Tech Coloproctol DOI 10.1007/s10151-010-0613-0
Desmoid tumor of mesentery in familial adenomatous polyposis: a case report G. Basdanis • V. N. Papadopoulos • S. Panidis I. Tzeveleki • E. Karamanlis • A. Mekras • S. Apostolidis • A. Michalopoulos
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Ó Springer-Verlag 2010
Desmoid tumors are rare, with an estimated annual incidence of 2–5 per million population [1]. While FAPassociated desmoids account for only 2% or so of all cases, the incidence in patients with FAP is approximately 850 times that of the general population [2, 3].
3 years ago, presented with acute epigastric pain and obstructive ileum. His physical examination revealed a palpable mass in the left abdominal area extending to the hypogastrium. From family history, his brother and son were also diagnosed with FAP and underwent restorative colectomy and ileal pouch anal anastomosis. All the family members were found to have c.3260 3261delTC mutation in the APC gene. The abdominal CT scan revealed a mass in the left lateral abdominal cavity (Fig. 1). Mesenteric fibromatosis was suspected, and a laparotomy was performed due to the bowel obstruction. Adhesions of the small intestine were found and dissected. A large mass of the mesentery was revealed occupying the left lateral abdominal quadrant and hypogastrium (Fig. 2). Pathological examination revealed a desmoid tumor of the mesentery. After consultation from an oncologist, he received sulindac 150 mg twice a day and tamoxifen 20 mg 2 9 3. He had a normal postoperative period and was discharged after 5 days. His follow-up showed regression of the tumor, and he is free of symptoms until today.
Case presentation
Discussion
A 52-year-old male with FAP, who had been submitted to prophylactic colectomy and ileal pouch anal anastomosis,
Depending on localization, desmoid tumors can be either asymptomatic or cause small bowel obstruction, ureter compression, small intestinal ischemia, abscess formation, intestinal perforation or fistulas [4–6]. The etiology and pathogenesis of desmoid tumors, especially FAP associated, is not fully understood. Several factors have been proposed to contribute to tumor appearance. The female sex is reported, with a higher incidence, particularly those in childbearing age and a higher growth rate. The most well-documented phenomenon is the genotype–phenotype correlation, with a 30 germ line APC mutation of codon
Abstract Our case concerns a 52-year-old male with FAP, who was treated surgically by restorative colectomy and ileal pouch anal anastomosis. Three years later, he presented with acute epigastric pain and obstructive ileum. While a mass in the left lateral abdominal region was palpated. The patient underwent laparotomy, some adhesions were dissected and biopsies were taken from the mass. Pathological examination revealed a desmoid tumor of the mesentery. Keywords
FAP Desmoid tumor APC
Introduction
G. Basdanis V. N. Papadopoulos (&) S. Panidis I. Tzeveleki E. Karamanlis A. Mekras S. Apostolidis A. Michalopoulos 1st Propedeutic Surgical Department, A.H.E.P.A University Hospital, Medical School, Aristotle University of Thessaloniki, T.Ikonimidi 21, 551 31 Kalamaria, Thessaloniki, Greece e-mail:
[email protected]
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Tech Coloproctol
Fig. 1 Abdominal CT scan: a mass in the left lateral abdominal cavity
is commonly believed that resection triggers growth and therefore recurrences [6].
Conclusion There is poor understanding of the desmoids and its behavior, as there is also lack of properly randomized trials in the literature. Surgical treatment is indicated when complications appear, such as bowel or ureter obstruction and perforation, but should be avoided if possible, due to high recurrence and morbidity rates. Fig. 2 Large desmoid tumor of the mesentery
1444 manifesting a higher likelihood of developing desmoid tumor. Our patient was found to have c.3260 3261delTC mutation in the APC gene. CT imaging is the best in making diagnosis and establishing the relation to the surrounding structures, and T2weighted MRI imaging may reveal indications such as tumor’s behavior. There are no controlled trials on the treatment of desmoid tumors. For intra-abdominally located tumors, treatment with sulindac or celecoxib (non-steroidal anti-inflammatory drug) and/or a selective estrogen receptor modulator (tamoxifen/torimifene) is advised as first-line therapy. Cytotoxic chemotherapy has been used for desmoids that are not responsive to less aggressive therapies. Surgery is generally not recommended as first-line therapy for intra-abdominal desmoids because recurrence rate and morbidity are reported to be extremely high, including bleeding, short bowel and postoperative death. In addition, it
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Conflict of interest The authors declare that they have no conflict of interest related to the publication of this article.
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