A 16-year-old girl undergoes total colectomy for familial adenomatous polyposis. Two years later there is a 4 cm asymptomatic abdominal wall mass in the inci¬sion. The appropriate management is (AIPG 2009)
|A||A course of dacarbazine and doxorubicin|
|B||A course of vincristine, actinomycin D, and cyclophosphamide|
|D||Wide resection of the mass|
a. Desmoid disease
b. Desmoids are histologically benign tumors that arise from fibroaponeurotic tissue and occur in 12% to 17% of FAP patients.
c. Unlike those in the general population, desmoids in FAP patients tend to be intra-abdominal (up to 80% of cases) and mainly occur after abdominal surgical procedures..
d. Desmoids often involve the small bowel mesentery making complete resection difficult or impossible, and they may also involve the ureters.
e. Patients present with small bowel obstruction.
f. The recurrence rate after attempted resection is also high, and the recurrent disease is often more aggressive than the initial desmoids.
g. Medical therapy. When desmoid tumors are clinically inert, they may be treated with sulindac.
h. Tamoxifen or other antiestrogens may be added for slow-growing or mildly symptomatic tumors.
i. More aggressive desmoid tumors may be treated with chemotherapy.
j. Vinblastin and methotrexate achieve some degree of response in 40% to 50% of patients.
k. For more rapidly growing desmoids, antisarcoma agents, such as doxorubicin and dacarbazine, may be administered.
l. Radiation therapy may also be effective, but it can result in substantial small bowel morbidity.
m. Surgical therapy. Surgical treatment of intra-abdominal desmoid tumors should be reserved for small, well-defined lesions with clear margins.
n. When intra-abdominal desmoids involve the small bowel mesentery, they should be treated according to their initial presentation and rate of growth.