Abstract
Mesenteric fibromatosis or intrabdominal desmoid tumour is a rare proliferative disease that frequently involves the gastrointestinal tract. These tumours account for less than 3% of all tumours among soft tissue neoplasms. Most such lesions are large (10cm or more in diameter) at the time of the excision. Although grossly well-circumscribed, they lesions typically infiltrate the surrounding soft tissues including the bowel wall. The case described here concerns a 62-year-old woman with a partly cystic mesenteric fibromatosis incorporating the large bowel wall, which degenerated into an abscess. A right colectomy was undertaken, and the sample was sent for histological examination. Based on the morphological and immunohistochemical findings, a diagnosis of abdominal fibromatosis was established. The surgical margins were free of disease, and the examined lymph nodes showed cortical hyperplasia and histiocytosis of the lymph sinuses. The patient recovered from surgery with no complications; her fever subsided, and she was discharged from hospital 12 days later. Surgery is the primary therapy for extra-abdominal and intra-abdominal desmoid tumours. Considering the unique biology of desmoid tumours, conservative treatment strategies merit further investigation in both unresectable primary disease and resectable disease. The optimal criteria for choosing surgical or non-surgical treatment still need further evaluation with randomized controlled studies.
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References
Li M, Cordon-Cardo C, Gerald WL, Rosai J. Desmoid fibromatosis is a clonal process. Hum Pathol 1996; 27:939–4.
Haggitt RC, Reid BJ. Hereditary gastrointestinal polyposis syndromes. Am J Surg Pathol 1986; 10:871–8.
Yantiss RK, Spiro IJ, Compton CC, Rosenberg AE. Gastrointestinal stromal tumor versus intra-abdominal fibromatosis of the bowel wall: A clinically important differential diagnosis. Am J Surg Pathol 2000; 24:947–5.
Bhattacharya B, Dilworth HP, Iacobuzio-Donahue C, et al. Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions. Am J Surg Pathol 2005; 29:653–9
Zhou YL, Boardman LA, Miller RC. Genetic testing for young-onset colorectal cancer: case report and evidencebased clinical guidelines. Radiol Oncol 2010; 44:57–6.
Alman BA, Li C, Pajerski ME, Diaz-Cano S, Wolfe HJ. Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). Am J Pathol 1997; 151:329–3.
Munker R, Thomas JO, Li BD, Burton GV. Review Update on desmoid tumors. Escobar Ann Oncol 2012; 23:562–9
Bonvalot S, Eldweny H, Haddad V, Rimareix F, Missenard G, Oberlin O, et al. Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol 2008; 34:462–8
Fiore M, Rimareix F, Mariani L, Domont J, Collini P, Le Péchoux C, et al. Desmoid-type fibromatosis: a front-line conservative approach to select patients for surgical treatment. Ann Surg Oncol 2009; 16:2587–93. doi: 10.1245/s10434-009-0586-2
Bertani E, Chiappa A, Testori A, Mazzarol G, Biffi R, Martella S, et al. Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature. Ann Surg Oncol 2009; 16:1642–9. doi: 10.1245/s10434-009-0439-z
Smith AJ, Lewis JJ, Merchant NB, et al. Surgical management of intra-abdominal desmoid tumours. Br J Surg 2000; 87:608–1.
Salas S, Dufresne A, Bui B, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic des- moids tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol 2011; 29:3553–8
Azzarelli A, Gronchi A, Bertulli R, et al. Low-dose chemotherapy with methotrexate and vinblastine for patients with advanced aggressive fi-bromatosis. Cancer 2001; 92:1259–6.
Hansmann A, Adolph C, Vogel T, et al. High-dose tamoxifen and sul-indac as first-line treatment for desmoids tumors. Cancer 2004; 100:612–2.
Patel SR, Evans HL, Benjamin RS. Combination therapy in adult desmoid tumors. Cancer 1993; 72:3244–7
Ballo MT, Zagars GK, Pollack A. Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol 1999; 17:158–6.
Patel SR, Evans HL, Benjamin RS. Combination therapy in adult desmoid tumors. Cancer 1993; 72:3244–7
Nuyttens JJ, Rust PF, Thomas CR, et al. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer 2000; 88:1517–2.
Fiore M, Rimareix F, Mariani L, et al. Desmoid-type fibromatosis: a frontline conservative approach to select patients for surgical treatment. Ann Surg Oncol 2009; 16:2587–9.
Bonvalot S, Eldweny H, Haddad V, et al. Extra-abdominal primary fibromatosis: aggressive management could be avoided in a subgroup of patients. Eur J Surg Oncol 2008; 34:462–8
Salas S, Dufresne A, Bui B, et al. Prognostic factors influencing progression-free survival determined from a series of sporadic des-moids tumors: a wait-and-see policy according to tumor presentation. J Clin Oncol 2011; 29:3553–8
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Demosthenous, M., Stavrakis, I., Nazari, E. et al. A rare case of solitary colonic fibromatosis mimicking a cystic tumour. Hellenic J Surg 86, 221–225 (2014). https://doi.org/10.1007/s13126-014-0134-9
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DOI: https://doi.org/10.1007/s13126-014-0134-9