Secondary Logo

Myofibroblastic Tumor of the Pancreatic Head: Recurrent Cholangitis

Zanchi, C.*; Giurici, N.*; Martelossi, S.*; Cheli, M.; Sonzogni, A.; Alberti, D.

Journal of Pediatric Gastroenterology and Nutrition: December 2015 - Volume 61 - Issue 6 - p e28–e29
doi: 10.1097/MPG.0000000000000350
Case Report

*Division of Pediatric Gastroenterology, Hepatology and Nutrition, Institute for Maternal and Child Health—IRCCS “Burlo Garofolo,” Trieste

Department of Pediatric Surgery and Pathology, Ospedali Riuniti di Bergamo, Bergamo

Department of Pediatric Surgery and Pathology, Ospedali Civili di Brescia, Brescia, Italy.

Address correspondence and reprint requests to Chiara Zanchi, MD, Department of Paediatric Gastroenterology, Hepatology and Nutrition, Institute for Maternal and Child Health—IRCCS “Burlo Garofolo,” Via dell’Istria 65, Trieste, Italy (e-mail:

Received 19 February, 2014

Accepted 19 February, 2014

The authors report no conflicts of interest.

A 13-year-old girl was referred to our pediatric surgery department with a 1-year history of recurrent episodes of acute cholangitis. She complained of recurrent upper acute abdominal pain, nausea, anorexia, vomiting, jaundice, and weight loss during the course of the previous 2 months. Her family history was negative for any malignant disease.

The patient's past medical history was unremarkable until October 2009 when she was admitted to a regional hospital because of abdominal pain, vomiting, and jaundice. Laboratory examination showed high levels of conjugated bilirubin (6.02 mg/dL), alkaline phosphatase (448 U/L), aspartate transaminase (193 U/L), alanine aminotransferase (430 U/L) and gamma-glutamyl transpeptidase (105 U/L). Abdominal ultrasound (USS) showed dilatation of the intrahepatic and extrahepatic biliary tree. Endoscopic retrograde cholangiopancreatography (ERCP) confirmed the biliary stenosis, and an endoscopic papillotomy was performed. The girl was discharged in good condition. Subsequently, the patient had 6 episodes of cholangitis, associated with mild pancreatitis, treated with urgent ERCP and insertion of stent, which provided only temporary benefit. Between each cholestatic episode, she was in good health and resumed normal activity.

In October 2010, she was referred to our hospital. On admission, abdominal USS showed the presence of a mass in the distal portion of the choledochal stent. A magnetic resonance cholangio-pancreatography showed the presence of a mass, 25 mm in diameter, with bilobate morphology around the choledochal stent which was imprinting the duodenal periampullary wall (Fig. 1). Cancer markers were negative: carbohydrate antigen 19-9 was 2.3 I.U./mL (n.v. <27 I.U./mL), cancer antigen 125 was 1.4 U/mL (n.v. <35 U/mL), carcinoembrionic antigen was 0.3 ng/mL (n.v.< 3.4 ng/mL), human chorionic gonadotropin was 0.2 mUI/mL (n.v. <5 mUI/mL in absence of pregnancy), and alpha fetoprotein was 0.8 ng/mL (n.v. 0.50–66 ng/mL).



USS-guided biopsy of the mass was performed. The histological examination revealed a mesenchymal neoplasm of pancreatic origin. Computerized tomography of the thorax and abdomen was negative for metastases. The patient underwent a pylorus-preserving pancreaticoduodenectomy. Histological examination showed an inflammatory myofibroblastic tumor (IMT) with compact fascicular proliferation of spindle and plump myofibroblasts and fibroblast. The biopsy contained myxoid, edematous, and collagenized regions as well as a distinctive inflammatory infiltrate of lymphocytes, plasma cells, and granulocytes (Fig. 2). Immunohistochemical analysis showed the anaplastic lymphoma kinase (ALK) expression, a marker considered highly suggestive for IMT (Fig. 3). No further treatment was required. She was discharged under oral pancreatic enzyme replacement therapy. The follow-up was uneventful: she reported feeling well, normal stool frequency, and normoglycemia. The patient has been monitored for 4 years without clinical or radiological evidence of recurrence.





Pancreatic tumors are rare in childhood, accounting for only 0.2% of childhood malignancies (1). IMTs are benign solid lesion of unclear etiology, commonly found in the lungs. Histological features include the presence of myofibroblastic proliferation and a varying degree of inflammatory infiltrates, mainly consisting of lymphocytes, histiocytes, and plasma cells.

Constitutional symptoms, such as fever, malaise, and weight loss, due to the inflammatory nature of these lesions are reported in around 20% of cases. These symptoms resolve with treatment and their recurrence often signifies recurrence of the disease (2).

Because complete surgical excision is the treatment of choice for localized IMTs, early diagnosis is important for pancreatic tumors to prevent recurrent acute cholangitis and pancreatitis and consequently avoid risky and ineffectual operative ERCP. Enucleation alone has an increased risk of local recurrence, in particular for extrapulmonary lesions (25% vs <2% for lung lesions), with the highest risk of recurrence within 6 months. Distant metastasis of IMTs is rare, occurring in <5% of cases (3). There are no specific chemotherapeutic regimens, although the various agents used include cyclosporin, cyclophosphamide, methotrexate, and 5 fluorouracil. Radiotherapy has been used for residual tumors; however, its use in children is limited. The ALK inhibitor Crizotinib could be used for IMTs which are shown to carry chromosomal rearrangements involving the ALK gene (approximately 50%–70% of total IMTs) (2,4)(2,4).

Back to Top | Article Outline


1. Dagash H, Koh C, Cohen M, et al. Inflammatory myofibroblastic tumor of the pancreas: a case report of 2 pediatric cases—steroids or surgery? J Pediatr Surg 2009; 44:1839–1841.
2. Gopal M, Tan Y-W, Barret AM, et al. Intra-abdominal inflammatory myofibroblastic tumours in children—a report of three cases in 3 years. Eur J Pediatr Surg 2012; 22:254–256.
3. Gleason BC, Hornick JL. Inflammatory myofibroblastic tumours: where are we now? J Clin Pathol 2008; 61:428–437.
4. Butrynski JE, D’Adamo DR, Hornick JL, et al. Crizotinib in ALK-rearranged inflammatory myofibroblastic tumor. N Engl J Med 2010; 363:1727–1733.
© 2015 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,