MS. E, 32, went to her physician with a complaint of swelling in the right groin. She'd been diagnosed with a type of familial adenomatous polyposis (FAP) characterized by the presence of multiple colorectal adenomatous polyps 16 years before, when she underwent a total colectomy.1 Ms. E is a physically active, otherwise healthy woman who participates in strenuous physical activities every day.
Computed tomography (CT) revealed a large pelvic mass that extended from the anterior pelvis around the right hip into the right buttock. Her physician suspected a sarcoma or desmoid tumor. The results of a needle biopsy confirmed it was a desmoid tumor.
Before surgery, numerous consults were obtained to determine the tumor's size and to determine impingement on adjacent organs and structures. Surgery was planned to excise most, if not all, of the tumor with two incisions, anterior and posterior, for optimal access to the tumor. The team of surgeons was led by the surgical oncologist and included specialists from neurosurgery, urology, gynecology, and plastic surgery.
During the surgery, the tumor was found to have encircled the right peroneal nerve adjacent to the sciatic nerve. (See A closer look at the sciatic nerve.) Multiple approaches were attempted to dissect the tumor from the peroneal nerve. Because each attempt resulted in Ms. E having foot drop, the team ended its efforts to remove the entire tumor. Instead, only one-third of the tumor was removed. Ms. E experienced no postoperative functional limitations.
This article describes desmoid tumors, including who's at risk, and management as well as nursing considerations.
What are desmoid tumors?
Desmoid tumors have been defined as benign, slowly growing, invasive fibroblastic tumors that don't metastasize but that, depending on location, can cause significant morbidity and in rare cases, death.2,3
Two types of desmoid tumor have been described:
- Sporadic desmoid tumors occur mainly in the extremities, shoulder girdle, and abdomen.
- Desmoid tumors associated with FAP generally occur in the abdomen and frequently recur after surgical treatment.4,5
Desmoid tumors are rare, with only two to four new cases per million people per year.6 Patients with FAP have a 1,000 times higher risk of developing desmoid tumors than people in the general population.7,8 These nonmalignant tumors affect women more often than men, and these women tend to be of reproductive age.9
Mutations in the APC (adenomatous polyposis coli) gene cause desmoid tumors in people with FAP. Most desmoid tumors occur sporadically, are associated with mutations in the beta-catenin gene (CTNNB1), and occur in those ages 15 to 60.10,11 Up to 16% of desmoid tumors are caused by APC gene mutations, which are associated with FAP.12 These tumors are most common in women of childbearing age, usually ages 25 to 35.3,13,14
Three-quarters of all desmoid tumors occur after abdominal surgery.11 In people who have FAP, the tumors commonly develop in the area of prophylactic surgery performed to remove a portion or all of their colon.15 Although some authors speculate about the correlation between the role of trauma and the development of desmoid tumors in both those with FAP and the general population, Freitas and Barbosa have discussed an exaggerated inflammatory response at the surgical site that may lead to the development of this type of tumor.16
Pregnancy-related desmoid tumors are rare, but when they occur, most appear in the right rectus abdominal muscle.9 The combination of increased estrogen and decreased immunity in pregnancy may contribute to the growth of desmoid tumors, which may regress once the pregnancy is over.17
Desmoid tumors may be asymptomatic until their growth affects adjacent structures or organs.18 Many desmoid tumors progress to become a significant cause of morbidity. Possible complications include small bowel or ureteral obstruction, intestinal fistula, abscess, or hemorrhage.3,7,19
Disease severity is described in stages based on the presenting signs and symptoms. (See Staging desmoid tumors.) Staging may be assigned after surgery when the size and degree of obstructive complications could be determined.
If a desmoid tumor is suspected due to the patient's clinical presentation, diagnostic studies may include ultrasonography, magnetic resonance imaging (MRI), and CT.20 Diagnosis is confirmed by a biopsy of the mass to identify histologic components consistent with a desmoid tumor.15
Because of desmoid tumors' inconsistent growth patterns, treatment options vary depending on tumor size, location, and the presence of actual or potential complications. Multiple interventions may be used simultaneously. Multidisciplinary treatment plans should be individualized depending on the tumor site, staging, and patient preference.10,16,21,22
Historically, surgical intervention has been the treatment of choice. It's most successful when clear (negative) surgical margins are achieved. However, if surgery is performed and clear margins can't be attained, a high degree of tumor regrowth can be expected.23
Radiation may be used in patients who have a recurrence but aren't candidates for resection due to tumor location and/or its degree of invasiveness. Other treatment modalities such as chemotherapy, radiation therapy, or targeted molecular therapy may be used.23
The goal of pharmacologic therapy is to reduce tumor size or bulk. If the desmoid tumor shows aggressive growth with increased morbidity, surgical or other interventions may be needed.16
Freitas and Barbosa have suggested a “step-up strategy,” for intra-abdominal desmoid tumors, which is an approach based on disease progression that typically starts with nonsteroidal anti-inflammatory drugs (NSAIDs).16 If NSAIDs aren't successful, hormone therapy is added. If the tumor continues to grow, systemic chemotherapy is recommended. (See Treatment algorithm for desmoid tumors.)
Other treatment options
Interventions less frequently used include ablation therapy and isolated limb perfusion. Extra-abdominal desmoid tumors that are deemed inoperable may be treated with cryoablation (using freezing methods) or radiofrequency ablation (using extreme heat).22,24-26
A clinical trial is currently underway in France to look at outcomes using cryoablation techniques in patients who've been treated medically without success. The study was slated to conclude in May 2018. For more information about this study at Strasbourg's University Hospitals, see https://clinicaltrials.gov/ct2/show/NCT02476305.
To save the limb, surgical excision isn't used for desmoid tumors in the extremities. Treating these tumors with isolated limb perfusion using tumor necrosis factor and melphalan has had some success.27 These highly concentrated drugs are administered directly to the tumor site, preventing systemic toxicity by avoiding the liver and kidneys.28
“Wait and see” approach
Most recently, a “wait and see” or “active observation” approach with observation and medical management has been favored.29 Patients who have less aggressive tumors can be monitored carefully to avoid potentially life-altering surgery or debilitating adverse reactions from other treatment modalities.6,29,30
Some patients' tumors may regress on their own without intervention, but these patients require close follow-up to monitor for any change in the tumors. This may include MRI or CT scans monthly to trimonthly in the first year, then semiannually for 5 years. For patients being followed with the “wait and see” or “active observation” approach, who have tumors close to essential anatomy, frequent follow-up is paramount so that opportunities to surgically intervene won't be lost.29,31
Possibly significant adverse reactions are an important consideration when deciding on treatment options. Risks and benefits of every intervention need to be explained to the patient for an informed decision-making process. Preserving function is a priority.32
Because the clinical presentation of patients with desmoid tumors varies, nursing care must be individualized based on the site and extent of the tumor and specific type of treatment. Many patients have significant morbidity or disability and are concerned about the potential progression of their disease. Psychological support should be integrated throughout the plan of care.10,33
If the patient requires surgery, routine postoperative nursing care should be initiated. Teach measures to maintain optimum respiratory status such as coughing and deep-breathing exercises and incentive spiromety use.34 Perform a comprehensive pain assessment and intervene as indicated, including administering analgesics as prescribed. Suggest alternative modalities for pain relief such as guided imagery, meditation, relaxation techniques, acupuncture, and music.35,36 For patients who've undergone abdominal surgery, the use of splinting techniques can be helpful in managing pain.34
Additional nursing interventions include providing wound care, teaching self-care strategies, and reinforcing any prescribed dietary and activity modifications.18 The importance of early ambulation should be emphasized to prevent postoperative complications such as pneumonia and venous thromboembolism.34
When patients require limb amputation, rehabilitation includes physical therapy, occupational therapy, and prosthesis evaluation to maintain or improve quality of life.21
Before surgery, team members can help prepare the patient for the amputation. A discussion should include patient expectations about prosthesis options and rehabilitation.37
For patients who have extremity tumors, advocate for interdisciplinary support, such as physical and occupational therapy.37 Demonstrating assistive devices such as canes, crutches, or walkers with the patient's return demonstration is essential in the nursing plan of care. Teach patients to report difficulty walking, any new limitation in range of motion, or difficulty performing activities of daily living, which may be signs of tumor growth.38
Chemotherapy and radiation
Specific nursing interventions to address chemotherapy-related adverse reactions will be needed. Adverse reactions to the commonly used drugs include alopecia, mucous membrane irritation, nausea, and vomiting. The patient care plan includes anticipatory guidance and measures to reduce these adverse reactions as much as possible. Before chemotherapy begins, patients who desire to use a wig or head covering should be encouraged to investigate these options in case they lose their hair.39 Encourage meticulous mouth care with nonalcohol-based mouth care products. For example, a mixture of baking soda and salt solution may be very soothing and maintains oral hygiene.40 Antiemetics such as ondansetron may be prescribed.39
Refer patients receiving chemotherapy and/or radiation to a nutritionist to plan dietary interventions that may help prevent malnutrition. Review the dietary plan with the patient; usually, a diet high in calories and protein, divided into frequent small meals throughout the day as tolerated, is recommended. Patient food preferences should always be considered. Using pharmaceutical appetite stimulants such as corticosteroids or cannabinoids may also be beneficial. Nutritional supplements may be added as needed. Some patients may require parenteral nutrition or peripheral parenteral nutrition.41
Fatigue is a common manifestation in patients who are receiving chemotherapy and/or radiation. Anticipate this symptom in planning patient care and organizing activities of daily living. Encourage frequent rest periods.39
Specific nursing interventions to address radiation therapy–related adverse reactions will be needed. Nursing care will depend upon the anatomic area of treatment and treatment factors such as the cumulative dose, proximity of sensitive tissues and organs, and effect of other interventions, such as surgery and chemotherapy.42 For example, skin in the irradiated area should be regularly assessed throughout the course of treatment and for several weeks thereafter. Using nonalcohol-based, perfume-free lotions for skin hydration is recommended. Advise patients to inform the healthcare provider if their skin integrity becomes compromised.43
Nurses should emphasize the importance of adherence to the required imaging schedule. Make sure that patients have the appropriate contact information for their healthcare provider or clinic so they can report signs and symptoms that may indicate disease progression to areas adjacent to their existing tumor.
Resources for patients
Many patients who have desmoid tumors have significant morbidity and/or disability, and concern about the potential progression of their disease. Psychological support should be integrated into the plan of care.10
The Desmoid Tumor Research Foundation (www.dtrf.org) is a resource for patients and their families and caregivers. This international organization holds an annual patient meeting that includes educational sessions with information on the latest research and treatment. Lectures by experts in the field, breakout sessions, and social activities for participants are some of the highlights of these meetings. Due to the rarity of the disease, connecting with others can be daunting and this organization offers immeasurable support and networking for those affected around the world.
Case study follow-up
Four years after her desmoid tumor diagnosis and surgery, Ms. E continues to have follow-up imaging studies every 6 months. Results show no progression of the disease and no additional treatment has been recommended.
Desmoid tumors are rare neoplasms often causing significant morbidity and disability. Prompt recognition with a multidisciplinary approach to patient care and diligent follow-up can provide the best patient outcomes.
1. Jung WB, Kim CW, Kim JC. Clinical characteristics and adequate treatment of familial adenomatous polyposis combined with desmoid tumors. Cancer Res Treat
2. Park JS, Nakache YP, Katz J, et al Conservative management of desmoid tumors is safe and effective. J Surg Res
3. Kallam AR, Ramakrishna BV, Roy GK, Karthik KR. Desmoid tumours: our experience of six cases and review of literature. J Clin Diagn Res
4. Constantinidou A, Scurr M, Judson I, Litchman C. Chapter 2. Clinical presentation of desmoid tumors. In: Litchman C, ed. Desmoid Tumors
. Springer Science and Business Media; 2012:5–15.
5. Pinheiro LV, Fagundes JJ, Coy CS, et al Multiple desmoid tumors in a patient with Gardner's syndrome—report of a case. Int J Surg Case Rep
6. Eastley NC, Hennig IM, Esler CP, Ashford RU. Nationwide trends in the current management of desmoid (aggressive) fibromatosis. Clin Oncol (R Coll Radiol)
7. Quintini C, Ward G, Shatnawei A, et al Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients. Ann Surg
8. Kumar SS, Ramachandran P, Veena G, Madhusudhan N, Kumbhar U. Intra-abdominal desmoid tumour (DT) with pelvic extension—a case report. J Clin Diagn Res
9. Choi SH, Lee JH, Seo BF, Kim SW, Rhie JW, Ahn ST. Desmoid tumor of the rectus abdominis muscle in a postpartum patient. Arch Plast Surg
10. Kasper B. Systemic treatment approaches for sporadic desmoid-type fibromatosis: scarce evidence and recommendations. Oncol Res Treat
11. Ravi V, Patel SR, Raut CP, DeLaney TF. Desmoid tumors: epidemiology, risk factors, molecular pathogenesis, clinical presentation, diagnosis, and local therapy. UpToDate. 2017. http://www.uptodate.com
12. Koskenvuo L, Peltomäki P, Renkonen-Sinisalo L, et al Desmoid tumor patients carry an elevated risk of familial adenomatous polyposis. J Surg Oncol
13. Valesano JC, Schmitz JJ, Jensen NM, Schultz GR, Callstrom MR. Desmoid tumors: a review of their natural history, imaging, and treatment. J Radiol Nurs
15. Mirza RA, Ghesani MV, Enker WE. Radiological case: intra-abdominal desmoid tumor. Appl Radiol
16. Freitas ACR, Barbosa LER. Genetic profile, risk factors and therapeutic approach of desmoid tumors in familial adenomatous polyposis. J Coloproctol (Rio De Janiero)
17. Robinson WA, McMillan C, Kendall A, Pearlman N. Desmoid tumors in pregnant and postpartum women. Cancers (Basel)
18. Yao X, Corbett T, Gupta AA, et al A systematic review of active treatment options in patients with desmoid tumours. Curr Oncol
19. Kalady MF, Church JM. Monitoring and management of desmoids and other extracolonic manifestations in familial adenomatous polyposis. Semin Colon Rectal Surg
20. Braschi-Amirfarzan M, Keraliya AR, Krajewski KM, et al Role of imaging in management of desmoid-type fibromatosis: a primer for radiologists. Radiographics
21. Ghert M, Yao X, Corbett T, et al Treatment and follow-up strategies in desmoid tumours: a practice guideline. Curr Oncol
22. Molloy AP, Hutchinson B, O'Toole GC. Extra-abdominal desmoid tumours: a review of the literature. Sarcoma
23. Wang YC, Wong JU. Complete remission of pancreatic head desmoid tumor treated by COX-2 inhibitor—a case report. World J Surg Oncol
24. Bonvalot S, Desai A, Coppola S, et al The treatment of desmoid tumors: a stepwise clinical approach. Ann Oncol
. 2012;23(suppl 10):x158–x166.
25. Havez M, Lippa N, Al-Ammari S, et al Percutaneous image-guided cryoablation in inoperable extra-abdominal desmoid tumors: a study of tolerability and efficacy. Cardiovasc Intervent Radiol
27. van Broekhoven DL, Deroose JP, Bonvalot S, et al Isolated limb perfusion using tumour necrosis factor α and melphalan in patients with advanced aggressive fibromatosis. Br J Surg
29. Howard JH, Pollock RE. Intra-abdominal and abdominal wall desmoid fibromatosis. Oncol Ther
30. He XD, Zhang YB, Wang L, et al Prognostic factors for the recurrence of sporadic desmoid-type fibromatosis after macroscopically complete resection: analysis of 114 patients at a single institution. Eur J Surg Oncol
31. Grignol VP, Pollock R, Howard JH. Management of desmoids. Surg Clin North Am
32. Desurmont T, Lefèvre JH, Shields C, Colas C, Tiret E, Parc Y. Desmoid tumour in familial adenomatous polyposis patients: responses to treatment. Fam Cancer
33. Penel N, Chibon F, Salas S. Adult desmoid tumors: biology, management and ongoing trials. Curr Opin Oncol
34. Pusey-Reid E. Preventing postoperative pneumonia. Nurs Crit Care
35. Hole J, Hirsch M, Ball E, Meads C. Music as an aid for postoperative recovery in adults: a systematic review and meta-analysis. Lancet
36. Chou R, Gordon DB, de Leon-Casasola OA, et al Management of postoperative pain: a clinical practice guideline from the American Pain Society, the American Society of Regional Anesthesia and Pain Medicine, and the American Society of Anesthesiologists' Committee on Regional Anesthesia, Executive Committee, and Administrative Council. J Pain. 2016
37. Pasquina PF, Miller M, Carvalho AJ, et al Special considerations for multiple limb amputation. Curr Phys Med Rehabil Rep
38. Gronchi A, Colombo C, Le Péchoux C, et al Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm—a position paper from the Italian and the French Sarcoma Group. Ann Oncol
39. Roe H, Lennan E. Role of nurses in the assessment and management of chemotherapy-related side effects in cancer patients. Nurs Res Rev
40. Tipian C. Brushing up on mouth care for cancer patients with mucositis. Am Nurse Today
43. Bauer C. Understanding radiation dermatitis. Am Nurse Today