Proceedings of the Japan Pancreas Society, 2020, Suizo, the Journal of the Japan Pancreas Society, Volume 35, 2020, Naohiro Sata, MD, PhD, Editor-in-Chief : Pancreas

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Proceedings of the Japan Pancreas Society, 2020, Suizo, the Journal of the Japan Pancreas Society, Volume 35, 2020, Naohiro Sata, MD, PhD, Editor-in-Chief

Pancreas 50(6):p 895-903, July 2021. | DOI: 10.1097/MPA.0000000000001836
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Observation of Mucinous Cystic Neoplasms (MCN): A Study of 46 Patients With MCN and MCN-like Lesions

Takanobu Taniguchi,* Takashi Sasaki,* Tsuyoshi Takeda,* Takaaki Furukawa,* Takafumi Mie,* Masashi Sawada,* Ryo Kanata,* Akiyoshi Kasuga,* Masato Matsuyama,* Masato Ozaka,* Yosuke Inoue,† Yu Takahashi,† Manabu Takamatsu,† Yutaka Takazawa,‡ Naoki Sasahira,*

Departments of * Hepato-Biliary-Pancreatic Medicine, † Hepato-Biliary-Pancreatic Surgery, and ‡ Pathology, Cancer Institute Hospital of Japanese Foundation for Cancer Research, Tokyo, Japan. Suizo. Vol 35, No 2, pp 201-212.

Suspected pancreatic mucinous cystic neoplasms (MCN) are often resected in Japan,1 but MCN without malignant findings have been followed-up without resection in the United States2 and Europe.3 Therefore, we examined the propriety and methods of observation of patients with suspected MCN. Forty-six patients with pathologically confirmed or suspected of MCN in our hospital were included. The positive predictive value and the sensitivity of preoperative diagnosis of MCN were 71% and 89%, respectively. Epidermoid cyst of an intrapancreatic accessory spleen was the most frequent diagnosis among false positive cases. After final diagnosis, patients were classified into confirmed MCN (malignant 7/benign 20), MCN-like diseases (malignant 3/benign 7), and suspected MCN (not resected 9). There were no deaths among patients with adenomas and noninvasive carcinomas presenting as MCN. All MCN with mural nodules <5 mm and cyst diameter <40 mm were adenomas. We could also distinguish malignant from benign by this criterion in patients with MCN-like diseases (Fig. 1). It was suggested that an increase in size of >10 mm/year and calcification might be a positive predictive factor for malignancy. The diagnostic sensitivity of neoplastic nodules was 96% for computed tomography (CT) scan and 100% for magnetic resonance imaging (MRI) and endoscopic ultrasound (EUS). All MCN showed a gradual increase annually except one which appeared suddenly and increased rapidly. In conclusion, based on the criteria of “a mural nodule <5 mm and cyst diameter <40 mm,” it is reasonable to follow-up suspected MCN by CT scan or MRI or EUS every 6-12 months after re-evaluation at 3 months after the initial imaging study.

Height of mural nodule and cyst diameter in 10 patients with non MCN(MCN-like lesion) and 29 patients with MCN. MCC, mucinous cystic carcinoma; MCA, mucinous cystic adenoma).


1. Tanaka M, Fernández-del Castillo C, Adsay V, et al. International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology. 2012;12:183-197.

2. Vege SS, Ziring B, Jain R, et al; Clinical Guidelines Committee; American Gastroenterology Association. American Gastroenterological Association Institute guideline on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology. 2015;148:819-822; quiz e12-e13.

3. European Study Group on Cystic Tumours of the Pancreas. European evidence-based guidelines on pancreatic cystic neoplasms. Gut. 2018;67:789-804.


The Preoperative Interval and Surgical Outcomes of Longitudinal Pancreaticojejunostomy for Patients With Chronic Pancreatitis

Hideaki Sato,* Masaharu Ishida,* Fuyuhiko Motoi,* Hideo Ohtsuka,* Masamichi Mizuma,* Takanori Morikawa,* Hiroki Hayashi,* Kei Nakagawa,* Takashi Kamei,* Takeshi Naitoh,* Atsushi Masamune,† Shinichi Egawa,‡ Michiaki Unno,*

* Department of Surgery, Tohoku University Graduate School of Medicine, Miyagi; † Department of Gastroenterology, Tohoku University, Miyagi; and ‡ Division of International Cooperation for Disaster Medicine, International Research Institute of Disaster Science, Tohoku University, Miyagi, Japan. Suizo. Vol 35, No 6, pp 551-558.

The purpose of this study was to investigate the optimal timing of surgery for patients with chronic pancreatitis (CP). Surgery for patients with CP is recommended when medical and endoscopic treatments fail in the Japanese practice guidelines. However, the optimal timing of surgery is not mentioned. The medical records of 50 patients who underwent longitudinal pancreaticojejunostomy, from January 2005 to April 2016 at our institution were retrospectively reviewed. The patients were divided into two groups, early surgery (preoperative interval less than 5 years, N = 30) and late surgery (5 years or more, N = 20), and the postoperative outcomes were compared between the two groups. The comparison of both groups is shown in Table 1. There were no significant differences in patients’ background or perioperative outcomes. Thirty-eight patients were analyzed one year after surgery (early group: N = 20, late group: N = 18). Nutritional status improved in both groups, and there were no significant differences between the two groups. Pain was also significantly improved in both groups. There was no new onset of diabetes mellitus or pancreatic cancer during the follow-up period. The occurrence of postoperative hepatic steatosis was observed in only one patient in the late surgery group. Regardless of the preoperative interval, surgical intervention was effective for patients with CP. Because previous reports have shown early surgical intervention is more useful for patient with CP than late surgery,1-3 early surgical intervention may improve quality of life by reducing disease duration.

TABLE 1 - Nutritional and Pain Assessment Before and After Longitudinal Pancreaticojejunostomy With Comparison of the Early and Late Groups
Early Surgery (n = 20) Late Surgery (n = 18)
Preoperative Postoperative Preoperative Postoperative P
Pain relief, % 85 61 0.27
Body weight, kg 53.0 (48.9–60.6) 53.5 (50.1–56.8) 53.5 (50.0–56.9) 54.7 (48.3–58.8) 0.46
Albumin, g/dL 3.7 (3.3–4.2) 4.0 (3.8–4.4) 3.9 (3.4–4.2) 4.3 (4.0–4.4) 0.61
Diabetes mellitus 7 7 8 1
Hepatic steatosis 0 0 0 1


1. Yang CJ, Bliss LA, Schapira EF, et al. Systematic review of early surgery for chronic pancreatitis: impact on pain, pancreatic function, and re-intervention. J Gastrointest Surg. 2014;18:1863-1869.

2. Ke N, Jia D, Huang W, et al. Earlier surgery improves outcomes from painful chronic pancreatitis. Medicine (Baltimore). 2018;97:e0651.

3. Yang CJ, Bliss LA, Freedman SD, et al. Surgery for chronic pancreatitis: the role of early surgery in pain management. Pancreas. 2015;44:819-823.


Survival of a Patient With Multiple Liver Metastases From a Neuroendocrine Tumor of the Pancreas Treated With Peptide Receptor Radionuclide Therapy

Noritoshi Kobayashi,* Takeshi Shimamura,† Yuma Takeda,* Naoki Okubo,* Motohiko Tokuhisa,* Yukihiko Hiroshima,* Ayumu Goto,* Shoko Takano,‡ Itaru Endo,§ Yasushi Ichikawa,*

* Division of Oncology, Yokohama City University Hospital, Kanagawa; † Shimamura Clinic, Kanagawa; and ‡ Department of Radiology and § Division of Gastroenterological Surgery, Yokohama City University Hospital, Kanagawa, Japan. Suizo. Vol 35, No 1, pp 97-103.

We report the long-term survival of a patient with multiple liver metastases from a pancreatic neuroendocrine tumor who underwent peptide receptor radionuclide therapy (PRRT) in Switzerland. A 60-year-old Japanese woman was diagnosed with a pancreatic neuroendocrine carcinoma and underwent a Whipple resection in 2002. She was diagnosed with liver metastases in 2003. Despite proton beam treatment, multiple liver metastases developed, and she was treated with systemic chemotherapy including platinum-based chemotherapy between 2004 and 2010. However, this treatment was ineffective and the liver tumors gradually progressed and was admitted (Fig. 1A). We re-examined the pathological findings and diagnosed a well-differentiated neuroendocrine tumor (WHO 2010: NET grade 2 Ki-67 labeling index 6%). Immunohistochemical findings showed that somatostatin receptor type 2 was strongly positive on the tumor membranes (Fig. 1B). We informed the patient of these findings and recommended PRRT. She underwent PRRT with 90Y-DOTATOC (4.4 GBq) and 177Lu-DOTATOC (7.4 GBq) three times at three intervals in Switzerland in 2011 (Fig. 1C). Computed tomography scan findings revealed a partial response that continued for two years (Fig. 1D). However, the liver lesions progressed in 2013, and she underwent PRRT twice in 2014. The liver tumors showed a slight response after these treatments, but stable disease persisted. Despite this, the tumors progressed in 2016. She refused other treatment, and underwent PRRT twice. However, the multiple metastases could not be controlled, and multiple metastatic bone lesions appeared. She died of liver failure in 2017. She survived for six years with PRRT treatment. Somatostatin-based PRRT was first introduced in Europe in the 1990s.1 PRRT was evaluated in many retrospective and prospective clinical trials, but only recently, a randomized phase III study was performed with 177Lu-DOTATATE in patients with mid-gut NETs.2,3 177Lu-DOTATATE was approved by the FDA for the treatment of somatostatin receptor-positive NETs in 2018. PRRT is an innovative and powerful treatment: which achieves a high tumor response and a long progression-free survival. Additionally, PRRT provides better quality of life than other treatments in patients with NETs.4 Based on these promising results, we hope that PRRT will become available in Japan.

A, D, Abdominal contrast enhanced computed tomography scan. Multiple hypervascular lesions are seen in the liver before peptide receptor radionuclide therapy (A). B, Immunohistochemical findings by somatostatin receptor type 2 (SSTR2a).Diffuse hole tumormembraneswere stainedwith SSTR2a. C, SPECT-CT imaging of initial peptide receptor radionuclide therapy. Moderate to intense uptake revealed multiple liver metastases.Multiple hypovascular lesions showed a partial response after peptide receptor radionuclide therapy (D).


1. Otte A, Mueller-Brand J, Dellas S, et al. Yttrium-90-labelled somatostatin-analogue for cancer treatment. Lancet. 1998;351:417-418.

2. Kwekkeboom DJ, de Herder WW, Kam BL, et al. Treatment with the radiolabeled somatostatin analog [177 Lu-DOTA 0,Tyr3]octreotate: toxicity, efficacy, and survival. J Clin Oncol. 2008;26:2124-2130.

3. Strosberg J, El-Haddad G, Wolin E, et al; NETTER-1 Trial Investigators. Phase 3 trial of 177Lu-Dotatate for midgut neuroendocrine tumors. N Engl J Med. 2017;376:125-135.

4. Strosberg J, Wolin E, Chasen B, et al; NETTER-1 Study Group. Health-related quality of life in patients with progressive midgut neuroendocrine tumors treated with 177Lu-Dotatate in the phase III NETTER-1 Trial. J Clin Oncol. 2018;36:2578-2584.


Factors Associated With Fatigue 3 Months After Pancreatectomy

Rio Minami,* Naoko Sato,† Fuyuhiko Motoi,‡ Fumiko Sato,† Michiaki Unno,‡

* Department of Nursing, Tohoku University Hospital, Miyagi; and Departments of † Oncology Nursing and ‡ Hepato-Biliary Pancreatic Surgery, Tohoku University Graduate School of Medicine, Miyagi, Japan. Suizo. Vol 35, No 1, pp 83-90.

Pancreatic tumor resection is associated with impaired quality of life (QOL) due to poor prognosis and extensive surgery, and 70%–100% of patients with pancreatic cancer experience fatigue, which has been shown to substantially impact QOL.1 This study investigated correlations between fatigue and health-related QOL (FACT-HEP2), body composition, nutritional status (Controlling Nutritional Status score: CONUT), and depression (Self-rating Depression Scale: SDS) in patients 3 months after pancreatectomy, and examined factors associated with and interventions to reduce fatigue.2 Univariate analysis of data from 77 patients who underwent pancreatectomy revealed that numerous subjective symptoms (P ≤ 0.001–0.049) and depression (P = 0.041) worsened after surgery (Table 1). Body composition items (P ≤ 0.001–0.006) and nutritional status (P ≤ 0.001) also worsened after surgery, suggesting that patients need symptomatic relief as well as both mental and physical care (Table 1). Correlation analysis, but not multivariate analysis, revealed important factors associated with fatigue. Fatigue in patients 3 months postoperatively was significantly correlated with weight loss, dyspepsia, appearance changes, fever, taste changes, dry mouth, abdominal discomfort, weakness, and nausea (r = −0.296–0.469; P ≤ 0.001–0.039) (Table 1). Management of these symptoms may be important to reduce fatigue. Fatigue was also significantly negatively correlated with body weight, muscle mass, and impaired nutritional status (r = −0.361–0.205; P = 0.014–0.048). Nutritional status is reported to affect postoperative muscle mass.3 Along with postoperative nutritional management, early postoperative rehabilitation to prevent weight and muscle loss may also be effective. Fatigue also significantly correlated with depression (r = 0.485, P < 0.001) and can be affected by emotions; therefore, emotions may be an important target for intervention to reduce fatigue. A moderate correlation was found between fatigue and unhappiness about changes in one’s appearance (r = 0.464; P ≤ 0.001). A study of patients with advanced cancer found that those with low body image satisfaction were more depressed.4 Therefore, appearance care may reduce depression and improve fatigue.

TABLE 1 - Comparison of Fatigue Factors Between Pre- and Post-3 Months Operative Period, and Correlation With Fatigue Factors in Pancreatic Cancer Patients (N = 77)
Preoperative Score Mean (SD) Score 3 mo After Surgery Mean (SD) P Correlation Coefficient for Fatigue 3 mo After Surgery P
Subject symptoms
1. I have swelling or cramps in my stomach area 0.25 (0.65) 0.64 (0.99) 0.847 0.133 0.250
2. I am losing weight 1.18 (1.36) 1.45 (1.46) 0.001* 0.236 0.039
3. I have control of my bowels 2.23 (1.48) 2.32 (1.35) 0.32 -0.052 0.652
4. I can digest my food well 2.44 (1.24) 1.90 (1.13) 0.044 -0.296 0.009*
5. I have diarrhea 0.25 (0.63) 1.22 (1.51) 0.489 0.215 0.060
6. I have a good appetite 2.81 (1.29) 2.06 (1.33) 0.007* -0.180 0.118
7. 1 am unhappy about a change in my appearance 0.91 (1.14) 1.49 (1.40) <0.001 0.464 <0.001
8. I have pain in my back 0.92 (1.30) 0.99 (1.10) <0.001 0.086 0.456
9. I am bothered by constipation 0.84 (1.19) 0.61 (1.10) <0.001 0.087 0.451
10. I feel fatigued 1.05 (1.20) 1.38 (1.11) 0.003*
11. I am able to do my usual activities 2.75 (1.27) 2.18 (1.14) 0.106 -0.137 -0.236
12. I am bothered by jaundice or yellow color to my skin 0.23 (0.78) 0.09 (0.37) 0.589 -0.030 0.796
13. I have had fevers (episodes of elevated body temperature) 0.38 (0.84) 0.30 (0.71) 0.799 0.259 0.023
14. I have had itching 0.52 (0.90) 0.66 (0.98) 0.271 0.020 0.860
15. I have had a change in the way food tastes 0.48 (1.00) 1.34 (1.37) 0.007* 0.298 0.008*
16. I have had chills 0.48 (0.90) 0.94 (1.07) 0.049 0.185 0.108
17. My mouth is dry 1.06 (1.25) 1.38 (1.38) <0.001 0.387 0.001*
18. I have discomfort or pain in my stomach area 0.73 (1.17) 1.31 (1.06) 0.002* 0.254 0.026
19. I have lack of energy 1.16 (1.25) 1.97 (1.17) <0.001 0.469 <0.001
20. I feel ill 0.23 (0.65) 0.57 (0.90) 0.127 0.243 0.033
21. I have pain 0.73 (0.98) 1.10 (0.98) 0.396 0.150 0.191
Body composition items, CONUT, and SDS
 Weight, kg 56.529 (9.48) 51.606 (8.66) <0.001 -0.236 0.110
 BCM, kg 27.15 (4.98) 25.20 (4.58) <0.001 -0.330 0.025
 LBW, kg 42.31 (7.34) 40.26 (6.85) <0.001 -0.304 0.040
 Body mass index, kg/m2 21.968 (2.98) 19.91 (2.86) <0.001 -0.306 0.039
 BFP, % 24.346 (8.55) 22.498 (9.65) 0.006* 0.141 0.343
 %TSF 163.28 (48.62) 134.54 (44.39) <0.001 -0.188 0.211
 %AMC 94.13 (7.73) 90.94 (8.74) <0.001 -0.361 0.014
 CONUT 12.274 (1.55) 11.430 (1.59) <0.001 0.205 0.077
 SDS 42.19 (8.66) 44.44 (10.03) 0.041 0.485 <0.001
Nos. 3, 4, 6, and 11 are reverse items; the higher the number, the better the condition.
%TSF and %AMC represent "patient status/standards," with 80-90% being mild, 60-80% being moderate, and below 60% being severe.
*P <0.01
P <0.05
P <0.001
Spearman product-moment correlation coefficient: P < 0.001; *P< 0.01; P < 0.05 (two-sided?test)
AMC indicates arm muscle circumference; BCM, body cell mass; BFP, body fat percentage; CONUT, Controlling Nutritional Status score; LBW, lean body mass; SDS, Self-rating Depression Scale, SD, standard deviation; TSF, triceps skinfold.


1. Sato N, Katayose Y, Motoi F, et al. [Strategy of symptom-targeted intervention based on patient quality of life at three months after pancreatectomy]. [Article in Japanese]. Suizo. 2015;30:654-662.

2. Heffernan N, Cella D, Webster K, et al. Measuring health-related quality of life in patients with hepatobiliary cancers: the functional assessment of cancer therapy-hepatobiliary questionnaire. J Clin Oncol. 2002;20:2229-2239.

3. Sugita Y, Hara T, Kubo A. [Relationship between changes in physical activity and physical function of perioperative gastrointestinal cancer patients]. [Article in Japanese]. Rigakuryoho Kagaku. 2020;35:843-848.

4. Rhondali W, Chisholm GB, Filbet M, et al. Screening for body image dissatisfaction in patients with advanced cancer: a pilot study. J Palliat Med. 2015;18:151-156.


Early Detection and Diagnosis in Two Patients With Thrombotic Microangiopathy Induced By Gemcitabine for the Treatment of Pancreatic Cancer

Yuzo Shimokawa,* Tsukasa Miyagahara,* Katsuhito Teramatsu,* Yuta Suehiro,* Satoshi Hisano,† Keijiro Ueda,‡ Ichiro Yamamoto,§ Yuichi Yamada,∥ Yoshinao Oda,∥ Akira Aso,¶ Nao Fujimori,¶ Takamasa Oono,¶

* Department of Gastroenterology, Nakatsu Municipal Hospital, Ohita; † Department of Pathology, Fukuoka University School of Medicine, Fukuoka; ‡ Department of Gastroenterology, Kitakyushu Municipal Medical Center, Fukuoka; § Department of Pathology, Nakatsu Municipal Hospital, Ohita; and Departments of ∥ Anatomic Pathology, Pathological Sciences, and ¶ Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. Suizo. Vol 35, No 5, pp 403-411.

Thrombotic microangiopathy (TMA) is a rare complication of gemcitabine (GEM) therapy which can be life-threatening and adversely affect renal function.1,2 We report two patients with TMA who were diagnosed early and improved without specific treatment. Two patients with locally advanced pancreatic cancer (a 40-year-old with heavy iron radiotherapy and a 70-year-old) started GEM monotherapy. Due to myelosuppression, dose reduction was required with relative dose intensity of 50% and 55%, respectively. Both patients developed slowly progressive mild renal dysfunction with edema and hypertension and were diagnosed with TMA by pathologic findings from a renal biopsy (Fig. 1). They required no specific treatment other than withdrawal from GEM monotherapy. Delay in the diagnosis of GEM-induced TMA is common because of its low incidence, varied presentation, and low awareness of this condition. To improve the clinical management of TMA, a multidisciplinary approach is proposed.3 Early diagnosis of TMA in these two patients was facilitated by the slow progression of renal dysfunction associated with low relative dose intensity of GEM and early consultation with a nephrologist. Careful evaluation of nonspecific findings such as hypertension, edema, weight gain, and urinary occult blood that emerged during GEM therapy is the key to the early detection of GEM-induced TMA.2

Pathologic findings from a renal biopsy. Renal biopsy revealed a platelet-rich thrombus in a small artery (A, periodic acid-Schiff staining), glomerular basement membrane thickening and duplication (B, periodic acid-Schiff staining), with expansion of the subendothelial space with mesangial interposition (C, periodic acid methenamine silver staining).


1. Humphreys BD, Sharman JP, Henderson JM, et al. Gemcitabine-associated thrombotic microangiopathy. Cancer. 2004;100:2664-2670.

2. Izzedine H, Isnard-Bagnis C, Launay-Vacher V, et al. Gemcitabine-induced thrombotic microangiopathy: a systematic review. Nephrol Dial Transplant. 2006;21:3038-3045.

3. Gordon CE, Chitalia VC, Sloan JM, et al. Thrombotic microangiopathy: a multidisciplinary team approach. Am J Kidney Dis. 2017;70:715-721.


Analysis of 5 Patients With Isolated Lung Metastases After Resection of Pancreatic Cancer

Hidehiro Tajima, Isamu Makino, Ryosuke Gabata, Hiroyuki Shimbashi, Mitsuyoshi Okazaki, Yoshinao Ohbatake, Shinichi Nakanuma, Tomoharu Miyashita, Tetsuo Ohta.

Department of Gastroenterological Surgery, Kanazawa University, Ishikawa, Japan. Suizo. Vol 35, No 4, pp 331-335.

Although the prognosis of patients with pancreatic ductal adenocarcinoma (PDAC) who develop postoperative recurrence is poor, lung metastases may have a relatively better prognosis than other sites of recurrence.1 Among 112 patients who underwent surgery for PDAC in our department from 2006 to 2017, five had isolated lung metastases as shown in Table 1. Three of five patients underwent resection of the lung metastases, and two patients were unresectable due to multiple lung metastases and one with respiratory dysfunction. Resection for primary PDAC was performed after neoadjuvant chemotherapy in all patients, and postoperative adjuvant chemotherapy was given to four. Pathologically, four patients had lymph node metastases, and an R0 resection was performed for the primary lesion in four patients. All five patients have an overall survival greater than four years, and only two patients died of lung metastases. The mechanism of direct lung metastases from PDAC has been suggested to be a hematogenous pathway via lymph node metastases and/or the collateral circulation around the splenic vein occluded by the tumor.2 There are many reports that resection of lung metastases in patients with PDAC improves the patient’s prognosis.3 However, it is necessary to accumulate and examine more patients to show the effectiveness of metastasectomy.

TABLE 1 - Five Cases With Isolated Lung Metastasis After Resection for Pancreatic Cancer
No. Sex Age, y Primary Lesion NAC TS, mm N (No.) Stage R AC DFS, y No. LM Chemotherapy for LM Operation for LM Prognosis Cause of Death Survival After LM, y OS, y
1 Male 67 H GEM 35 1b (4) IIB 0 GEM 2.0 3 S-1 → GEM + Dead Peritonitis carcinomatosa 5.3 7.8
2 Male 71 BT GEM 95 1b (7) IIB 1 S-1 0.8 2 GEM → mFOLFIRINOX Dead LM 3.1 5.0
3 Male 75 BT GnP 25 1a (1) IIB 0 None 2.0 3 GnP + Alive 3.6 5.7
4 Male 66 BT GS 5 0 (0) IIA 0 S-1 3.2 Multiple None Dead LM 1 4.2
5 Male 67 H GS 35 1b (10) IIB 0 S-1 1.2 1 GnP + Dead LM 4.7 5.9
H, head, BT, body and tail; NAC, neoadjuvant chemotherapy; GEM gemcitabine; GnP, GEM + nab-paclitaxel; GS, GEM + S-1; TS, tumor size; N, lymph node metastasis; R, residual tumor; AC, adjuvant chemotherapy; DFS, disease free survival; LM, lung metastasis; OS, overall survival, mFOLFIRINOX, modified combination of leucovorin, fluorouracil, irinotecan, oxaliplatin


1. Wangjam T, Zhang Z, Zhou XC, et al. Resected pancreatic ductal adenocarcinomas with recurrence limited in lung have a significantly better prognosis than those with other recurrence patterns. Oncotarget. 2015;6:36903-36910.

2. Zheng B, Ohuchida K, Yan Z, et al. Primary recurrence in the lung is related to favorable prognosis in patients with pancreatic cancer and postoperative recurrence. World J Surg. 2017;41:2858-2866.

3. Arnaoutakis GJ, Rangachari D, Laheru DA, et al. Pulmonary resection for isolated pancreatic adenocarcinoma metastasis: an analysis of outcomes and survival. J Gastrointest Surg. 2011;15:1611-1617.

Epidermoid Cyst of the Spleen Preoperatively Diagnosed as a Mucinous Cystic Neoplasm in the Tail of the Pancreas

Yusuke Nakayama,* Eiji Toyoda,* Jun Matsubayashi,* Yoshinobu Ikeno,* Kazuhiko Kitaguchi,* Tatsuo Ito,* Hideaki Oe,* Tomoko Okuno,† Tetsuro Hirose,* Ryuichiro Doi,*

Departments of * Surgery and † Pathology, Otsu Red Cross Hospital, Shiga, Japan. Suizo. Vol 35, No 1, pp 91-96.

A 47-year-old woman presented with a cystic lesion in the tail of the pancreas, and was referred for further investigation. Contrast-enhanced computed tomography scan revealed a multilocular cyst with septa, measuring 46 mm in diameter. On magnetic resonance imaging, the cyst had heterogeneous hyperintensity on T2-weighted images. Contrast-enhanced endoscopic ultrasound revealed a cyst-in-cyst-like structure with cystic portions giving a honeycomb appearance. The lesion was diagnosed as a mucinous cystic neoplasm in the tail of the pancreas and treated with laparoscopic distal pancreatectomy. Histopathologically, the cyst originated in the spleen and was invaginated into the tail of the pancreas. The cystic wall was lined by stratified squamous epithelium and the final diagnosis was an epidermoid cyst of the spleen. An epidermoid cyst of the spleen invaginating into the tail of the pancreas should be taken into consideration when a cystic lesion is located at the very distal end of the pancreas.

Three Patients With B Cell Lymphoma Diagnosed by Endoscopic Ultrasound-guided Fine Needle Aspiration (EUS-FNA) of Pancreatic Lesions

Ai Kasai,*† Shinichi Hashimoto,* Kengo Tsuneyoshi,† Masayuki Kabayama,† Hiromichi Iwaya,† Shiho Arima,* Hiroki Taguchi,*† Shuji Kanmura,* Michiyo Higashi,‡ Akio Ido,*

* Department of Digestive and Lifestyle Diseases, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima; † Department of Gastroenterology, Saiseikai Sendai Hospital, Kagoshima; ‡ Department of Pathology, Field of Oncology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. Suizo. Vol 35, No 2, pp 213-221.

Three patients with pancreatic lymphoma were seen. Primary pancreatic lymphoma accounts for less than 2% of extranodal malignant lymphomas and less than 0.5% of malignant tumors of the pancreas. In patients with pancreatic lymphoma, an accurate diagnosis is crucial because the tumor is difficult to distinguish from other pancreatic tumors—especially pancreatic cancer—based on imaging findings alone, and because the treatment depends on the specific type of lymphoma. Two of these patients improved after chemoradiation therapy. EUS-FNA was useful for determining the type of lymphoma and determining the treatment strategy.

Acinar Cell Carcinoma of the Pancreas With Repeated Shrinkage and Expansion of the Tumor Until Operation: A Case Report

Taketoshi Fujimoto,* Yo Kato,† Takahiro Okai,‡ Atsushi Nakanishi,§ Masahiro Irimoto,∥ Yoshikazu Yasuda,¶

* Department of Surgery, Hiratsuka Gastroenterological Hospital, Tokyo (Current institution: IMS Fujimi General Hospital, Saitama); † Department of Pathology, Nikko Medical Center, Dokkyo Medical University, Tochigi; ‡ Department of Internal Medicine, Kawakita General Hospital, Tokyo; § Department of Radiology, Juntendo University, Tokyo;Department of Radiology, Irimoto Medical, Tokyo; ¶ Department of Gastroenterological Surgery, Jichi Medical University, Tochigi, Japan. Suizo. Vol 35, No 2, pp 222-233.

Acinar cell carcinoma (ACC) of the pancreas developed in a 71-year-old man. The tumor behaved in an unusual manner with two episodes of alternate shrinkage and expansion without any treatment until operation. Although ACC is alleged to have a poor prognosis, this patient survived for more than five years after surgery without adjuvant chemotherapy. He developed dull back pain and presented in April 200X. Imaging studies had shown an unrecognized solid ellipsoid tumor, 40 mm in diameter, between the liver and the pancreatic head in June 200X-6. After two episodes of alternate shrinkage and expansion, the lesion became a hypervascular inhomogeneous ellipsoid mass, 65 mm in diameter, in April 200X and demonstrated expansive growth with portal vein depression. Laboratory data showed an elevated CEA (62.0 ng/ml), and transabdominal fine needle aspiration biopsy revealed adenocarcinoma. Tumor extirpation was performed in July 200X. Pathology demonstrated ACC of the pancreas. The patient died of primary pulmonary cancer without recurrence of the pancreatic ACC 6 years after resection.

Difficulty in Preoperative Diagnosis of a Patient With a Neuroendocrine Neoplasm of the Pancreas

Tatsuya Kakegawa,* Mitsuyoshi Honjo,* Atsushi Sofuni,* Takayoshi Tsuchiya,* Kentaro Ishii,* Reina Tanaka,* Ryosuke Tonozuka,* Shuntaro Mukai,* Kenjiro Yamamoto,* Hiroshi Yamaguchi,† Takao Itoi,*

Departments of * Gastroenterology and Hepatology and † Anatomic Pathology, Tokyo Medical University, Tokyo, Japan. Suizo. Vol 35, No 2, pp 234-240.

A 47-year-old woman was incidentally found to have a mass in the tail of the pancreas on a contrast-enhanced CT scan. EUS-FNA was performed but the specimen was non-diagnostic and she was referred to our hospital. Contrast-enhanced CT scan showed a hypovascular mass with delayed enhancement in the periphery. Repeat EUS showed a heterogenous hypoechoic mass in B mode and contrast-enhanced EUS using Sonazoid® showed poor enhancement in the central part of the mass but early contrast enhancement in the margin. Several imaging findings highly suggested the mass to be a pancreatic duct adenocarcinoma. Prompt surgery without re-biopsy was preferred and curative distal pancreatectomy performed. Pathological examination showed the mass to be a NET G2. Pathological findings of hyaline necrosis and fibrosis in the central part of the tumor also reflected the imaging findings. While typical pancreatic NET tumors are hypervascular and relatively easy to diagnose, atypical lesions with disparate imaging findings are often encountered. A NET with a reduced contrast effect is considered to be relatively high grade and the poor enhancement is generally seen in larger masses. This tumor was small but highly atypical.

Surveillance for the Early Diagnosis of Familial Pancreatic Cancer (Expert Consensus)

Masayuki Kitano,* Chigusa Morizane,† Susumu Hijioka,† Hiroyuki Matsubayashi,‡ Reiko Ashida,§ Tsukasa Ikeura,∥ Tetsuhide Ito,¶ Terumi Kamisawa,# Takahisa Kawaguchi,** Ken Kawabe,†† Shinji Kosugi,‡‡ Yuzo Kodama,§§ Kyoko Shimizu,∥∥ Hideaki Takahashi,¶¶ Shinichi Yachida,## Takeshi Terashima,*** Masako Torishima,††† Keiji Hanada,‡‡‡ Toru Furukawa,§§§ Masayuki Furukawa,∥∥∥ Junji Furuse,¶¶¶ Hiroyuki Maguchi,### Yoshiyuki Majima,**** Nobumasa Mizuno,†††† Masamichi Mizuma,‡‡‡‡ Masaki Mizumoto,§§§§ Takeichi Yoshida,* Keita Wada,∥∥∥∥ Kyoichi Takaori,¶¶¶¶

* Second Department of Internal Medicine, Wakayama Medical University, Wakayama; † Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo; ‡ Division of Endoscopy & Genetic Medicine Promotion, Shizuoka Cancer Center, Shizuka; § Departments of Cancer Survey and Gastrointestinal Oncology, Osaka International Cancer Institute, Osaka; ∥ The Third Department of Internal Medicine, Kansai Medical University, Osaka; ¶ Neuroendocrine Tumor Centre, Fukuoka Sanno Hospital, Fukuoka; # Internal Medicine, Tokyo Metropolitan Komagome Hospital, Tokyo; ** Center for Genomic Medicine, Kyoto University Graduate School of Medicine, Kyoto University, Kyoto; †† Department of Gastroenterology, National Hospital Organization Kyushu Medical Center, Fukuoka; ‡‡ Medical Ethics/Medical Genetics, Kyoto University Graduate School of Medicine, Kyoto; §§ Department of Gastroenterology, Kobe University Graduate School of Medicine, Hyogo; ∥∥ Department of Gastroenterology, Tokyo Women's Medical University, Tokyo; ¶¶ Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital East, Chiba; ## Department of Cancer Genome Informatics, Graduate School of Medicine Osaka University, Osaka; *** Department of Gastroenterology, Kanazawa University Hospital, Ishikawa; ††† Clinical Genetics Unit, Kyoto University Hospital, Kyoto; ‡‡‡ Department of Gastroenterology, JA Onomichi General Hospital, Hiroshima; §§§ Department of Investigative Pathology, Tohoku University Graduate School of Medicine, Miyagi; ∥∥∥Department of Hepato-biliary-pancreatology, National Hospital Organization Kyushu Cancer Center, Fukuoka; ¶¶¶ Department of Medical Oncology, Kyorin University Faculty of Medicine, Tokyo; ### Education and Research Center, Teine-Keijinkai Hospital, Hokkaido; **** NPO PanCAN Japan, Tokyo; †††† Department of Gastroenterology, Aichi Cancer Center Hospital, Aichi; ‡‡‡‡ Department of Surgery, Tohoku University, Miyagi; §§§§ Department of Surgery, Takeda General Hospital, Kyoto; ∥∥∥∥Department of Surgery, Teikyo University School of Medicine, Tokyo; ¶¶¶¶ Division of Hepato-Biliary Pancreatic Surgery and Transplantation, Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan. Suizo. Vol 35, No 2, pp 234-240.

The incidence of pancreatic cancer in families with at least two affected first-degree relatives who have pancreatic cancer is significantly higher than in the general population. In 2014, the Japan Pancreas Society started the nationwide Japan Familial Pancreatic Cancer Registry to investigate familial pancreatic cancer in Japan. The aim of this paper is to describe an expert consensus for surveillance in people with an increased risk using the Japan Familial Pancreatic Cancer Registry. A working group constituted by experts in imaging and genetic diagnosis of pancreatic cancer first proposed 22 statements regarding 1) the definition of those at high risk, 2) initial work-up, and 3) follow-up of the high-risk group. Agreement of the councilors of Japan Pancreas Society was obtained for the 21 of the 22 statements by a vote. We expect that this expert consensus will contribute to the early diagnosis and an improved prognosis in people with familial pancreatic cancer.

A Patient With Trousseau’s Syndrome and Meningeal Carcinomatosis Due to Pancreatic Cancer During Chemotherapy

Yusuke Sano, Makoto Ueno, Kuniyuki Kawano, Taito Fukushima, Hiroyuki Asama, Satoshi Kobayashi, Manabu Morimoto.

Department of Hepatobiliary and Pancreatic Oncology, Kanagawa Cancer Center, Kanagawa, Japan. Suizo. Vol 35, No 4, pp 336-343.

A 66-year-old female with lesions in the body of the pancreas and liver on computed tomography (CT) scan was diagnosed with adenocarcinoma by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). She was treated with gemcitabine plus nab-paclitaxel combination therapy (GnP). She presented to our hospital with dysarthria and right hemiparesis eight days later. Magnetic resonance imaging (MRI) scan revealed multiple cerebral infarctions and she was diagnosed with Trousseau’s syndrome. Since her neurological symptoms improved spontaneously, we continued systemic chemotherapy and no new neurological symptoms developed. Hypercoagulability was ameliorated by combination chemotherapy with GnP. The pancreatic lesions and hepatic metastases responded partially to systemic chemotherapy. However, metastases to the spine were found 4 months later after 5 courses of GnP. Therefore, we changed the regimen to modified FOLFIRINOX (mFOLFIRINOX). Nausea, appetite loss, and vesicorectal disturbances developed 3 months later following 4 courses of mFOLFIRINOX. Meningeal carcinomatosis was diagnosed by cerebrospinal fluid cytology. Her general condition deteriorated and she died three weeks later.

Resection of Residual Pancreatic Recurrence and Abdominal Wall Metastasis Associated With Long-Term Survival in a Patient With Intraductal Papillary Mucinous Carcinoma: A Case Report

Nana Mamuro,* Yoshihito Masuoka,* Taro Mashiko,* Akira Nakano,* Kenichi Hirabayashi,† Toshio Nakagohri,*

Departments of * Surgery and † Pathology, Tokai University School of Medicine, Kanagawa, Japan. Suizo. Vol 35, No 4, pp 344-353.

A 59-year-old woman presented with upper abdominal pain. Imaging studies showed a 30 mm polycystic tumor in the head of the pancreas. ERCP showed dilation of the main pancreatic duct, and pancreatic fluid cytology and brushing cytology were both class V. The diagnosis was intraductal papillary mucinous carcinoma (IPMC). The patient underwent pancreatoduodenectomy. The final pathological diagnosis was IPMC. Twelve months after resection, the patient had elevated serum CA 19-9 levels. CT scan showed recurrence at the pancreatointestinal anastomosis, and the patient underwent further resection. Histopathological examination showed the same pattern as that observed in the previously resected specimen. IPMC with residual pancreatic recurrence was diagnosed. The serum CA 19-9 levels were again elevated 51 months after re-excision. CT scan showed a 20 mm mass in the abdominal wall. Abdominal metastasis from IPMC was suspected, and resection was performed. The resected mass was white and well-defined. Based on histopathological findings, abdominal wall metastasis from IPMC was diagnosed. The patient remains alive 88 months after the first resection. We report a patient for whom long-term survival was achieved by resection of the residual pancreatic recurrence and abdominal wall metastasis after resection of IPMC in the head of the pancreas.

Carcinoma of the Head of the Pancreas in an Annular Pancreas Complicated by Branch Duct Type Intraductal Papillary Mucinous Neoplasm: A Case Report

Keisuke Kinoshita,*† Satoshi Fukuchi,† Kazunari Murakami,*

* Department of Gastroenterology, Oita University Faculty of Medicine Graduate School of Medicine, Oita; † Department of Gastroenterology, Almeida Memorial Hospital, Oita, Japan. Suizo. Vol 35, No 4, pp 354-360.

Annular pancreas is a congenital anomaly in which pancreatic parenchyma surrounds the second part of the duodenum, with a frequency of 0.015%. Pancreatic cancer is extremely rare in patients with annular pancreas. We report a patient with invasive ductal carcinoma of the head of the pancreas in an annular pancreas. A 76-year-old woman was diagnosed with branch duct intraductal papillary mucinous neoplasm and annular pancreas and was admitted with fatigue and anorexia. Laboratory data showed elevation of serum bilirubin and hepatobiliary enzymes. Contrast-enhanced CT scan revealed dilatation of the intrahepatic and common bile ducts, and showed a 2 cm hypovascular tumor in the head of the pancreas. Pancreatography showed disruption of the main pancreatic duct in the head, and dilatation of the caudal main pancreatic duct. Cholangiography showed stenosis of the distal bile duct and the diagnosis was obstructive jaundice due to carcinoma of the head of the pancreas. A subtotal stomach preserving pancreaticoduodenectomy was performed. Pathology revealed invasive ductal carcinoma of the pancreas. Ph, TS2 (33 mm), pT3, pN1, M0, pStage IIB.

A Patient With a Pancreatic Metastasis From Synovial Sarcoma With Long-Term Survival Following Resection

Yuko Kobashi,* Junichi Matsui,† Yutaka Takigawa,† Takayuki Suzuki,* Koichi Masuda,* Shinji Yamazoe,* Takuji Mogami,* Aya Sasaki,‡

Departments of * Radiology, † Surgery, and ‡ Pathology, Tokyo Dental College Ichikawa General Hospital, Tokyo, Japan. Suizo. Vol 35, No 4, pp 361-369.

A 43-year-old female presented with a tumor in the head of the pancreas. She had a history of a synovial sarcoma in the left axilla resected 4 years previously. The pancreatic tumor was 4.5 cm in diameter and showed cystic and solid components and enhancement on contrast CT scan. The pancreas tumor showed intermediate signal intensity on T2-weighted images and low signal intensity on T1 weighted images on MRI. There was no evidence of main pancreatic duct dilatation suggestive of pancreatic cancer on either CT or MRI. The ERP also showed a normal main pancreatic duct. Laboratory data and tumor makers were all normal. Based on examinations, the pancreatic head tumor was diagnosed as a metastasis from the synovial sarcoma. We performed a pylorus-preserving pancreaticoduodenectomy. The final pathologic diagnosis was a metastasis from the synovial sarcoma. She has been well for 83 months without recurrence. Pancreatic metastasis from synovial sarcoma is quite rare. The CT/MRI findings of synovial sarcoma are varied because both synovial sarcomas and their metastases usually include calcification, cystic component and hemorrhage. If patients have only a pancreatic metastasis, pancreatic tumor resection may be associated with a good prognosis.

Gastric Tube-Preserving Pancreaticoduodenectomy for Mixed-Type IPMN After Esophageal Cancer Surgery

Ryosuke Arata,* Yasuhiro Matsugu,* Akihiko Oshita,* Tamito Sasaki,† Hideki Nakahara,* Toshiyuki Itamoto,*

Departments of * Gastroenterological Surgery and † Gastroenterology, Hiroshima Prefectural Hospital, Hiroshima, Japan. Suizo. Vol 35, No 5, pp 378-386.

A 70-year-old man with esophageal cancer underwent subtotal esophagectomy with combined right thoracotomy and laparotomy including lymph node dissection and retrosternal gastric tube reconstruction. There was no recurrence of cancer postoperatively but he had been diagnosed with branch-type IPMN in the pancreatic head 2 years before. During follow-up, dilatation of the main pancreatic duct progressed and pointed outward with thickened enhanced cyst walls. Mural nodules were present in the branch and main pancreatic ducts. The patient was diagnosed with mixed-type IPMN (high-risk stigmata). PPPD was performed while preserving the gastroduodenal artery, right gastroepiploic artery, and right gastric artery and vein. Histopathological diagnosis revealed a mixed-type IPMN with intermediate dysplasia. The patient was discharged on postoperative day 12 without any complications. Pancreatoduodenectomy is rarely performed after radical surgery for esophageal cancer, and only 28 cases have been reported as of 2018. It is necessary to consider blood flow preservation after gastric tube reconstruction, and 3D-CTA is useful to confirm the vascular morphology and arterial anatomy. In patients after gastric tube reconstruction, right gastroepiploic artery/venous preservation is typical, but preservation and revascularization of the right gastric artery/vein should be considered when resecting.

Difficult Preoperative Diagnosis of Pancreatic Hamartoma

Atsuhito Tsuchihashi,* Shinjiro Kobayashi,* Junichi Tsuchiya,* Masafumi Katayama,* Satoshi Koizumi,* Yasushi Ariizumi,† Takehito Otsubo,*

Departments of * Gastrointestinal and General Surgery and † Pathology, St. Marianna University School of Medicine, Kanagawa, Japan. Suizo. Vol 35, No 5, pp 387-393.

We describe a patient with a pancreatic hamartoma. The patient is a 65-year-old woman followed for a hepatic cyst. She was asymptomatic, but a pancreatic tumor was incidentally found and she was referred for further evaluation. Serum tumor markers and hormone levels were within normal limits. Dynamic computed tomography scan showed a well-demarcated tumor in the head of the pancreas measuring 1.5 cm in maximum diameter. An arterial phase image shows a relatively well-circumscribed nodule with enhancement in the portal venous phase. Endoscopic ultrasonography-fine needle aspiration was non-diagnostic. The patient underwent pylorus-preserving pancreatoduodenectomy with a preoperative diagnosis of a pancreatic neuroendocrine tumor. A well-demarcated solid nodule was found in the head of the pancreas. Microscopically, the lesion consisted of disarranged ductal and acinar cells embedded in fibroinflammatory stroma but lacked concentric elastic fibers in the duct walls, peripheral nerves, and islets of Langerhans. The histological diagnosis was pancreatic hamartoma. Pancreatic hamartoma is a rare tumor and it is very difficult to obtain a preoperative histological diagnosis. We report a patient with a pancreatic hamartoma resembling a non-functional pancreatic neuroendocrine tumor.

Gastric Metastasis From Needle Tract Seeding After Endoscopic Ultrasound-Guided Fine Needle Aspiration of a Cancer of the Pancreatic Body and Tail

Masahiro Fuse,* Yasuhiro Yabushita,* Nobuhiro Tsuchiya,* Yu Sawada,* Yuki Homma,* Takafumi Kumamoto,* Ryusei Matsuyama,* Shoji Yamanaka,† Kunihiro Hosono,‡ Itaru Endo,*

Departments of * Gastroenterological Surgery, † Pathology, and ‡ Gastroenterology and Hepatology, Yokohama City University, Yokohama, Japan. Suizo. Vol 35, No 5, pp 394-402.

A 78-year-old female underwent distal pancreatectomy for cancer of the pancreatic body and tail diagnosed by trans-gastric endoscopic-guided fine needle aspiration (EUS-FNA). Pathological findings showed invasive ductal carcinoma T2N0M0 (JPS7th). The patient received S-1 therapy as postoperative chemotherapy for 6 months. Four and a half years after resection, a positron emission tomography computed tomography scan showed strong uptake in the proximal posterior gastric body wall (SUV max = 6.9), and esophagogastroduodenoscopy (EGD) revealed a submucosal tumor (SMT) near the puncture site from the previous EUS-FNA. Biopsy of the SMT showed tubular adenocarcinoma resembling the resected pancreatic cancer. A partial gastrectomy was performed for suspected needle tract seeing in the gastric wall. Comparing immunostaining of the primary pancreatic cancer and the gastric wall SMT, we diagnosed needle tract seeding caused by EUS-FNA. Needle tract seeding caused by EUS-FNA must be considered, and we suggest EGD as regular follow-up for the early diagnosis of needle tract seeding.

Retroperitoneal Schwannoma in the Differential Diagnosis of a Pancreatic Tumor: Report of a Laparoscopically Resected Tumor

Kohei Yoshimura,* Yoshinobu Ikeno,* Eiji Toyoda,* Tomoharu Kawanami,† Tomoko Okuno,‡ Ryuichiro Doi,*

Departments of * Surgery, † Gastroenterology, and ‡ Pathology, Otsu Red Cross Hospital, Shiga, Japan. Suizo. Vol 35, No 5, pp 412-417.

A 64-year-old man was referred because of a mass detected by ultrasonography in the body of the pancreas. Although preoperative histopathological diagnosis was not obtained, neuroendocrine neoplasm was suspected based on imaging studies. Due to a family history of pancreatic cancer, we recommended surgical resection. Laparoscopic distal pancreatectomy was successfully performed, and histopathological evaluation revealed the tumor to be a retroperitoneal schwannoma. Generally, it is difficult to diagnose this disease preoperatively. However, it is important to take this entity into consideration when contrast-enhanced computed tomography scan reveals a tumor with delayed enhancement near the pancreas.

Two Patients With Postoperative Recurrence of Pancreatic Acinar Cell Carcinoma Successfully Treated by FOLFIRINOX

Takeyuki Sawano,* Yutaka Umehara,* Koji Shimaya,† Masaki Munakata,† Akihiko Murata,* Kenichi Takahashi,* Hidekachi Kurotaki,‡ Kenichi Hakamada,§

Departments of * Surgery, † Gastroenterology, and ‡ Diagnostic Pathology, Aomori Prefectural Central Hospital, Aomori; § Department of Surgery, Division of Digestive Surgery, Hirosaki University Graduate School of Medicine, Aomori, Japan. Suizo. Vol 35, No 5, pp 418-428.

Patient 1: A 29-year-old man was suspected to have pancreatic cancer and underwent SSPPD-IIA-1, right hemicolectomy, and partial resection of the SMV. He was diagnosed with pancreatic acinar cell carcinoma (PACC) (T3N1aM0 Stage IIB) and received adjuvant chemotherapy with S-1. A local recurrence appeared and was treated with nab-PTX + GEM therapy, but the lesion enlarged. He was then treated with modified FOLFIRINOX therapy. The recurrent lesion disappeared after 12 courses, and he was alive 24 months after surgery.

Patient 2: A 69-year-old man was suspected to have a pancreatic neuroendocrine tumor or PACC and underwent distal pancreatectomy, The lesion was PACC (T3N0M0 Stage IIA). He received adjuvant chemotherapy with S-1, but multiple liver metastases and an elevated AFP developed. He was then treated with nab-PTX + GEM therapy, but the liver metastases enlarged and the AFP level increased. He underwent modified FOLFIRINOX as the next treatment, the liver metastases resolved and AFP decreased to within normal limits after 12 courses. He was alive at 17 months after surgery.

Conclusions: FOLFIRINOX therapy may enable long-term survival for patients with postoperative recurrence of PACC.

Medical Treatment of Unresectable Malignant Insulinoma in an Elderly Patient

Kazuhide Matsumoto,* Nao Fujimori,* Yuta Suehiro,* Masatoshi Murakami,* Katsuhito Teramatsu,* Yu Takamatsu,* Takehiro Takaoka,* Takamasa Oono,* Hisato Igarashi,*† Tetsuhide Ito,‡§

* Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka; † Igarashi Medical Clinic, Yamaguchi; ‡ Neuroendocrine Tumor Center, Fukuoka Sanno Hospital, Fukuoka; § Department of Gastroenterology, International University of Health and Welfare, Fukuoka, Japan. Suizo. Vol 35, No 5, pp 429-438.

An 86-year-old woman presented with loss of consciousness and had Whipple’s triad. Enhanced abdominal computed tomography scan showed a pancreatic tumor and multiple liver tumors with hypervascularity. The patient was diagnosed with malignant insulinoma, with secretion from both lobes of the liver based on results of a 48-hour fasting test, and selective arterial secretagogue injection test. Somatostatin receptor scintigraphy was positive in the pancreatic tumor and multiple liver metastases. Surgical treatment was considered to be ineffective, and the patient was treated with somatostatin analog and diazoxide. Symptoms of hypoglycemia were diminished with this treatment. This approach can improve the quality of life for elderly patients with unresectable malignant insulinoma.

Multimodal Therapy of Metachronous Anaplastic Pancreatic Carcinomas Including Two Pancreatic Resections

Takashi Ito,* Yoshinori Kito,† Takumi Kozu,* Yusuke Abe,* Akihiro Kaneda,* Naoto Nakamura,* Jun Matsubayashi,* Yusuke Nakayama,* Kazuhiko Kitaguchi,* Katsuaki Ura,* Eiji Toyoda,* Hideaki Oe,* Tetsuro Hirose,* Ryuichiro Doi,*

* Department of Surgery, Otsu Red Cross Hospital, Shiga; † Department of Surgery, Yamatotakada Municipal Hospital, Nara, Japan. Suizo. Vol 35, No 5, pp 439-446.

A 70-year-old man was diagnosed with carcinoma of the pancreatic head because of worsening diabetes, and pancreatoduodenectomy was performed. The tumor was 9 mm in diameter (TS1 in JPS classification), and histopathological examination revealed an osteoclast-like giant cell-type anaplastic pancreatic carcinoma. Twenty-nine months after surgery, abdominal CT scan revealed a 22-mm mass in the residual pancreatic body with a dilated pancreatic duct, indicating another tumor. Residual pancreatectomy (total pancreatectomy) was then performed. Histopathologically, a spindle cell-type anaplastic carcinoma was found that was different from the subtype of the initial anaplastic carcinoma. We regarded this as a new lesion, not a recurrence of the initial one. This is the first report in Japan of metachronous anaplastic pancreatic carcinomas with different subtypes. Anaplastic pancreatic carcinoma is very rare and is associated with a poor prognosis. The current patient achieved long-term survival with multimodal therapy including two pancreatic resections. Aggressive surgical treatment can contribute to the prognosis of patients with this disease.

Type 2 Autoimmune Pancreatitis Diagnosed After Resection and Difficult to Differentiate From Cancer of the Pancreatic Body

Hiroki Yano,* Shinichi Hashimoto,* Hiroki Taguchi,* Shiroh Tanoue,* Hiromichi Iwaya,* Shiho Arima,* Fumisato Sasaki,* Shuji Kanmura,* Michiyo Higashi,† Akio Ido,*

* Digestive and Lifestyle Diseases and † Pathology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan. Suizo. Vol 35, No 5, pp 447-454.

The patient is a 46-year-old woman admitted with epigastric pain and nausea. She was found to have elevated pancreatic enzymes and treated for acute pancreatitis. Imaging findings showed uneven contrast enhancement of the pancreatic body to tail and enlargement of the caudal pancreatic duct. A pancreatic tumor was suspected. Although no definitive histologic diagnosis was obtained using endoscopic ultrasonography-guided fine needle aspiration (EUS-FNA), the possibility of pancreatic body cancer could not be ruled out, and resection was performed. The resected specimen showed characteristic histologic findings, leading to a diagnosis of type 2 autoimmune pancreatitis.

Anaplastic Carcinoma (Osteoclast-Like Giant Cell Type) in the Remnant Pancreas Six Years After Resection of Pancreatic Carcinoma

Takayoshi Nakajima,* Shinichi Ikuta,* Meidai Kasai,* Noriko Ichise,* Ayako Tomono,* Ikumi Hamano,* Ryo Okamoto,* Yoshihiko Nakamoto,* Tsukasa Aihara,* Hidenori Yanagi,* Takanori Yoshimoto,† Yoshiki Nando,† Tomotaro Kawazoe,† Noriko Kajimoto,‡ Ayako Kakuno,‡ Naoki Yamanaka,*

Departments of * Surgery, † Internal Medicine, and ‡ Pathology, Meiwa Hospital, Hyogo, Japan. Suizo. Vol 35, No 5, pp 455-462.

A 64-year-old man underwent distal pancreatectomy for moderately differentiated tubular adenocarcinoma. Adjuvant chemotherapy with S-1 was administered for 1 year. He was followed and recurrence-free for over 6 years. Seventy-five months after resection, CA 19-9 became elevated and enhanced abdominal computed tomography scan revealed a 30 mm tumor in the remnant pancreas. Anaplastic carcinoma was diagnosed by biopsy of the tumor using endoscopic ultrasound-guided fine needle aspiration, and total remnant pancreatectomy performed. The histological diagnosis was anaplastic carcinoma (osteoclast-like giant cell type). There are few reports of carcinoma in the remnant pancreas associated with anaplastic carcinoma, and to the best of our knowledge, this is the first report of metachronous anaplastic carcinoma in the remnant pancreas.

An 83 Year-Old Male With a Solid Pseudopapillary Neoplasm of the Pancreas

Takuro Fuke,* Hironobu Suto,* Bunpei Nishiura,* Hidemi Ibuki,† Hideki Kamata,‡ Hisashi Usuki,* Keiichi Okano,* Yasuyuki Suzuki,*

Departments of * Gastroenterological Surgery, † Diagnostic Pathology, and ‡ Gastroenterology and Neurology, Faculty of Medicine, Kagawa University, Kagawa, Japan. Suizo. Vol 35, No 6, pp 559-567.

An 83-year-old male was noted to have an elevated serum amylase level. Contrast enhanced CT scan revealed a heterogeneous mass in the head of the pancreas. The lesion was diagnosed as a solid pseudopapillary neoplasm (SPN) by EUS-FNA. We performed a subtotal stomach-preserving pancreaticoduodenectomy and lymph node dissection. Macroscopically, the tumor was 40 × 30 × 25 mm, composed of blood, cysts and necrotic tissue. The histological findings included solid and partial pseudopapillary structures. Immunohistochemical staining was positive for beta-catenin and vimentin and partially positive for CD10. There was no staining for chromogranin or progesterone receptor. The final diagnosis was SPN. The patient is doing well and has no evidence of recurrence 2-years postoperatively. The number of male patients with SPN has been increasing recently, but there are no patients reported to date with SPN older than age 80. We believe that this patient is the oldest male yet reported with SPN.

High-Grade Pancreatic Intraepithelial Neoplasm With a Mass and a High Level of Serum Elastase 1: A Case Report

Arihito Yoshizumi,* Takashi Hatori,* Masaru Miyazaki,* Osamu Itano,* Atsushi Kato,* Shunichi Imai,* Hajime Higuchi,* Shinsuke Aida,†

* Digestive Disease Center and † Department of Pathology, International University of Health and Welfare, MITA Hospital, Tokyo, Japan. Suizo. Vol 35, No 6, pp 568-574.

The patient is a man in his 70s. Elevated serum Elastase1 and dilatation of the main pancreatic duct were detected on routine medical evaluation and he was referred for farther examination. A dynamic CT-scan and MRI showed an inhomogeneous 20 mm mass in the pancreatic uncus with mild contrast enhancement. Pancreatic ductal adenocarcinoma was suspected. Pancreatic acinar cell neoplasm or neuroendocrine neoplasm were also considered and pylorus preserving pancreaticoduodenectomy was performed. The histopathological findings showed a papillary proliferation associated with nuclear atypia in the stenotic area of the main pancreatic duct. No cell invasion was seen beyond the pancreatic duct. The pathological diagnosis was a high-grade pancreatic intraepithelial neoplasm. The mass seen on the CT-scan was composed of marked fibrosis with invasion of fat tissue. The postoperative course was uneventful. Local fatty changes and atrophy of the pancreatic parenchyma were recently described as characteristic of early-stage pancreatic ductal adenocarcinoma, as seen in this patient. We report this patient with a literature review of the molecular mechanisms of fibrotic change.

Cystic Pancreatic Tumor With Adjacent SCN and IPMN: A Case Report

Naoto Nakamura,* Takumi Kohdu,* Jun Matsubayashi,* Yusuke Nakayama,* Kazuhiko Kitaguchi,* Katsuaki Ura,* Eiji Toyoda,* Hideaki Oe,* Tetsuro Hirose,* Tomoyuki Shirase,† Ryuichiro Doi,*

Departments of * Surgery and † Pathology, Otsu Red Cross Hospital, Shiga, Japan. Suizo. Vol 35, No 6, pp 575-582.

A 59-year-old asymptomatic woman was found to have a cystic mass in the tail of the pancreas during a periodic health examination and was referred for further evaluation. A 5 × 3 cm multilocular lesion was seen on contrast-enhanced computed tomography scan. The central part of the lesion was solid and deeply enhanced in the early phase and surrounded by larger multi-lobed cysts. The lesion was also recognized to have multi-lobed cysts with high intensity on T2-weighted images of MRI and MRCP. Intra-cystic nodules and a honeycomb-like area were seen on endoscopic ultrasound imaging. One of the pancreatic cysts appeared to communicate with the main pancreatic duct using endoscopic retrograde pancreatography. Cytology of the pancreatic fluid did not show malignant cells. Distal pancreatectomy and splenectomy was performed. Histopathological evaluation showed a serous cystic neoplasm (SCN, microcystic type) and intraductal papillary neoplasm (IPMN, gastric type) located next to each other. Synchronous pancreatic SCN and IPMN are extremely rare. This paper reports our experience together with a review of the literature and comment.

A Small Solid-Pseudopapillary Neoplasm With Atypical Ultrasound Images

Keisuke Yonamine, Shinsuke Koshita, Yoshihide Kanno, Takahisa Ogawa, Kaori Masu, Hiroaki Kusunose, Toshitaka Sakai, Kazuaki Miyamoto, Toji Murabayashi, Fumisato Kozakai, Kazuki Endo, Yutaka Noda, Kei Ito

Department of Gastroenterology, Sendai City Medical Center, Miyagi, Japan. Suizo. Vol 35, No 6, pp 583-591.

A 47-year-old man presented with a mass in the pancreatic tail diagnosed by transabdominal ultrasonography. A contrast-enhanced CT scan showed a 15 mm mass with central calcifications which gradually enhanced from early to late phases. Based on endoscopic ultrasonography imaging, the mass was hyperechoic mainly in the central area despite the margin being hypoechoic. Based on imaging studies, a solid-pseudopapillary neoplasm (SPN) and pancreatic neuroendocrine tumor were considered in the differential diagnosis. Endoscopic ultrasonography-guided fine needle aspiration (EUS-FNA) was performed, followed by hematoxylin-eosin staining and immunostaining, to definitively establish the diagnosis of SPN. We performed a distal pancreatectomy, and the mass was histologically confirmed to be a SPN from the resected specimen. In this patient, diagnosis using imaging studies alone was inconclusive since the mass had an atypical internal echo by ultrasonography. Therefore, EUS-FNA played a relatively important role in the preoperative diagnosis of SPN for this mass lesion with atypical ultrasonographic findings.

Neoplastic Aneurysms in Areas With Metastatic Pancreatic Choriocarcinoma During Chemotherapy

Hajime Sunagozaka,* Manami Utsunomiya,* Shotaro Kawase,* Kiyokuni Tanabe,* Yoshiaki Shimizu,* Azusa Kawasaki,* Yoshihide Naito,* Kenkei Hasatani,* Yasuharu Kaizaki,† Hiroyuki Aoyagi,*

Departments of * Gastroenterology and Hepatology and † Pathology, Fukui Prefectural Hospital, Fukui, Japan. Suizo. Vol 35, No 6, pp 592-600.

We describe a patient with neoplastic aneurysms which developed in areas of pancreatic choriocarcinoma during chemotherapy. A 43-year-old female presented with irregular metrorrhagia. CT scan demonstrated a 2.5 cm mass in the head of the pancreas and multiple lesion in the lung, liver, adrenal gland, and lumbar vertebra. The pancreatic tumor enhanced peripherally in the arterial phase and enhanced similar to the surrounding parenchyma of the pancreas in the delayed phase. We performed a lumbar vertebra biopsy and diagnosed multiple organ metastases from choriocarcinoma, histologically. EMA/CO chemotherapy was used to treat this patient for advanced choriocarcinoma. Neoplastic aneurysms developed in the pancreatic lesion of the choriocarcinoma while receiving chemotherapy. Preventive coil embolization was performed on the pancreatic aneurysms. After preventive coil embolization, EMA/CO chemotherapy was continued. Consequently, the EMA/CO chemotherapy was effective and complete remission was obtained. Pancreatic lesions of choriocarcinoma are extremely rare, however, choriocarcinoma results in neoplastic aneurysm formation in the primary and metastatic lesions. It is necessary to observe the clinical course of pancreatic choriocarcinoma carefully whether strongly stained areas in the tumor progress to neoplastic aneurysms. Chemotherapy should be given to patients with choriocarcinoma while considering the treatment for the aneurysm.

Two Patients With Dislocated Pancreatic Duct Stents After Pancreaticoduodenectomy

Masatsugu Ishii,* Kosuke Okuwaki,† Satoko Yamagishi,* Tatsuhiko Hoshikawa,* Takeshi Nakamura,* Masashi Tsugita,* Makoto Nakamaru,*

* Department of Surgery, Fussa Hospital, Tokyo; † Department of Gastroenterology Kitasato University, Tokyo, Japan. Suizo. Vol 35, No 6, pp 601-606.

A 55-year-old man underwent pancreaticoduodenectomy for extrabiliary cancer and 19 months after surgery presented with a fever. Serum hepatic and biliary enzyme levels gradually increased. DIC-CT scan showed intrahepatic stones in the left hepatic ducts. He was successfully treated with fasting and antimicrobial agents and then underwent endoscopic retrograde cholangiopancreatography. Biliary lithotripsy was performed and the pancreatic stent dislocated into the intrahepatic bile duct. A 53-year-old woman underwent pancreaticoduodenectomy for intraductal papillary mucinous adenoma. Six months after surgery, she presented with abdominal pain. CT scan showed an enlarged pancreas. She was treated successfully with fasting and ulinastatin and endoscopic retrograde pancreatography was performed. The pancreatic stent became dislocated. Based these findings, we conclude that dislocation of the pancreatic duct stent may be a long-term complication. Based on an individualized approach, a pancreatic duct stent may not be necessary when performing pancreaticoduodenectomy.

Xanthogranulomatous Inflammatory Tumor (Xanthogranulomatous Pancreatitis) in the Pancreatic Head Mimicking Pancreatic Cancer

Naomi Kuroki, Yosuke Inoue, Ryosuke Nomura, Yu Takahashi

Department of Gastrointestinal Surgery, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan. Suizo. Vol 35, No 6, pp 607-614.

An 81-year-old woman presented with epigastric pain. A tumor in the pancreatic head was found and she was referred for further evaluation and treatment. Contrast enhanced computed tomography scan showed a hypovascular 1.5 cm mass in the pancreatic head. We diagnosed resectable pancreatic cancer, cT3 cN0 cM0 cStage IIA (General rules for the study of pancreatic cancer, Japan Pancreas Society 7th Edition). We did not perform PET-CT or fine needle aspiration preoperatively and performed a subtotal stomach-preserving pancreatoduodenectomy. Histopathological examination failed to reveal malignancy, but revealed a xanthogranulomatous inflammatory tumor (xanthogranulomatous pancreatitis) of the pancreatic head. Xanthogranulomatous inflammatory tumor is extremely rare and difficult to differentiate from pancreatic cancer. Herein, we report a patient with xanthogranulomatous inflammatory tumor of the pancreatic head mimicking a pancreatic cancer, and summarize 27 previously reported cases.

Disseminated Varicella-Zoster Virus Infection During FOLFIRINOX Therapy in a Patient With Pancreatic Cancer With Local Recurrence and Distant Metastases

Masaya Yamanaka,* Hiroyuki Sugimoto,†

* Department of Surgery, Nakatugawa City Hospital, Gifu; † Department of Surgery, Komaki City Hospital, Aichi, Japan. Suizo. Vol 35, No 6, pp 615-621.

A 54-year-old man with cancer of the pancreatic body underwent distal pancreatectomy. Chemotherapy was given as second-line treatment for local recurrence and distant metastases. He underwent three courses of FOLFIRIOX (oxaliplatin, irinotecan, fluorouracil, and levofolinate calcium regimen) without difficulty. During the fourth course, he developed severe systemic blisters, abdominal pain, and back pain. Varicella was diagnosed based on the varicelliform rash. Acyclovir was given following hospitalization. Fever, hepatic dysfunction, and disturbance of consciousness subsequently developed. Varicella zoster virus (VZV) was detected in the cerebrospinal fluid and disseminated VZV infection diagnosed. His condition improved with supportive care including antiviral therapy, steroids, as well as transfusion. The VZV infection resolved. Varicella is known to be aggravated in patients who are in an immunosuppressed state with leukemia or after transplantation. However, reports of varicella infections during chemotherapy for solid tumors are rare. It is necessary to be aware of the potential for such patients to develop a viral infection when they are undergoing chemotherapy for solid tumors.

The Natural History of Recurrent Metachronous Intraductal Papillary Mucinous Neoplasm Imaged for 3 Years After Diagnosis

Gen Sugawara, Yasuhiro Kurumiya, Keisuke Mizuno, Ei Sekoguchi, Masaya Inoue, Takehiro Katoh, Naohiro Akita, Takayuki Minami

Department of Surgery, Toyota Kosei Hospital, Aichi, Japan. Suizo. Vol 35, No 6, pp 622-629.

The patient was a 77-year-old man who underwent distal pancreatectomy and splenectomy for intraductal papillary mucinous neoplasm (IPMN) at another institution 8 years previously. CT scan 7 and 6 years previously showed no abnormalities. CT scan 3 years before revealed dilatation of the main pancreatic duct with multiple cystic lesions. Recurrent metachronous IPMN was suspected, and surgery was recommended but follow-up alone was conducted in accordance with the patient’s wishes. CT 2 and 1 year previously showed enlargement of the cystic lesions. CT examination at presentation showed that multiple cystic lesions had become larger. Mural nodules were identified in the pancreatic body by endoscopic ultrasonography examination and remnant pancreatectomy was performed. The patient’s postoperative course was uneventful, and he was discharged 28 days postoperatively. The pathology results showed IPMN with high grade dysplasia, and metachronous recurrence diagnosed based on the absence of continuity with the previous lesion and multicentric pattern. This is a report of the natural history of metachronous recurrence after IPMN resection with imaging studies for 3 years

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