Abstract
Between January 1975 and December 1983, 24 of 183 patients (13%) with familial adenomatous polyposis (FAP) seen at the Mayo Clinic had mesenteric fibromatosis (MF). MF was found most often in FAP patients with associated extracolonic “Gardner” signs (19 patients) and those who had had previous abdominal surgery (20 patients). In 4 patients, MF appeared spontaneously. The male-to-female ratio was 0.4, with a median age of 31 years in women and 37 years in men. Ten of 24 patients (42%) had been asymptomatic prior to diagnosis at time of surgery for FAP. Complications of the disease included intestinal or urinary tract obstruction. Minimal surgical manipulation seemed to be associated with fewer postoperative complications and a lesser risk of regrowth of the tumor. Nonsurgical treatment, including tamoxifen and sulindac in combination, may be beneficial. Surgery should be reserved for relief of obstruction, and bypass is preferred to resection.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Nichols RW (1923) Desmoid tumors. Arch Surg 7:227–236
Smith WG (1958) Multiple polyposis, Gardner's syndrome and desmoid tumors. Dis Colon Rectum 1:323–332
Miller RH, Sweet RH (1937) Multiple polyposis of colon; familial disease. Ann Surg 105:511–515
O'Brien JP, Wels P (1955) The synchronous occurrence of benign fibrous tissue neoplasia in hereditary adenosis of the colon and rectum. NY State J Med 55:1877–1880
Shepherd JA (1958) Familial polyposis of the colon with associated connective tissue tumours. J R Coll Surg Edinb 4:31–38
Weiner RS, Cooper P (1955) Multiple polyposis of the colon, osteomatosis and soft-tissue tumors: report of a familial syndrome. N Engl J Med 253:795–799
Gardner EJ, Richards RC (1953) Multiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis. Am J Hum Genet 5:139–147
Gumpel RC, Carballo JD (1956) A new concept of familial adenomatosis. Ann Intern Med 45:1045–1058
Sener SF, Miller HH, DeCosse JJ (1984) The spectrum of polyposis. Surg Gynecol Obstet 159:525–532
Simpson RD, Harrison EG Jr, Mayo CW (1964) Mesenteric fibromatosis in familial polyposis: a variant of Gardner's syndrome. Cancer 17:526–534
Naylor EW, Gardner EJ, Richards RC (1979) Desmoid tumors and mesenteric fibromatosis in Gardner's syndrome: report of kindred 109. Arch Surg 114:1181–1185
Richards RC, Rogers SW, Gardner EJ (1981) Spontaneous mesenteric fibromatosis in Gardner's syndrome. Cancer 47:597–601
Gardner EJ (1962) Follow-up study of a family group exhibiting dominant inheritance for a syndrome including intestinal polyps, osteomas, fibromas and epidermal cysts. Am J Hum Genet 14:376–390
Waddell WR (1975) Treatment of intra-abdominal and abdominal wall desmoid tumors with drugs that affect the metabolism of cyclic 3′,5′-adenosine monophosphate. Ann Surg 181:299–302
Bessler W, Egloff B, Sulser H (1984) Case report 253. Diagnosis: Gardner syndrome with aggressive fibromatosis. Skeletal Radiol 11:56–59
Godfrey PJ, Moore AW, Clarke AM (1977) Intraabdominal desmoid causing death in a young man with Gardner's syndrome. Aust NZ J Surg 47:787–790
Harvey JC, Quan SHQ, Fortner JG (1979) Gardner's syndrome complicated by mesenteric desmoid tumors. Surgery 85:475–477
McAdam WAF, Goligher JC (1970) The occurrence of desmoids in patients with familial polyposis coli. Br J Surg 57:618–631
Chaves FJZC, Cruz I, Lopes C, De Morais M (1976)Polyposis coli associated with fibrosis of mesentery, mesocolon and retroperitoneal tissues: a rare variant of Gardner's syndrome. Am J Gastroenterol 65:163–167
Brasfield RD, Das Gupta TK (1969) Desmoid tumors of the anterior abdominal wall. Surgery 65:241–246
Das Gupta TK, Brasfield RD, O'Hara J (1969) Extraabdominal desmoids: a clinicopathological study. Ann Surg 170:109–121
Sirbasku DA (1978) Estrogen induction of growth factors specific for hormone-responsive mammary, pituitary, and kidney tumor cells. Proc Natl Acad Sci USA 75:3786–3790
Strode JE (1954) Desmoid tumors particularly as related to their surgical removal. Ann Surg 139:335–340
Dahn I, Jonsson N, Lundh G (1963) Desmoid tumours: a series of 33 cases. Acta Chir Scand 126:305–314
Caldwell EH (1976) Desmoid tumor: musculoaponeurotic fibrosis of the abdominal wall. Surgery 79:104–106
Lipschütz A, Grismali J (1944) On the antifibromatogenic activity of synthetic progesterone in experiments with the 17-caprylic and dipropionic esters of α estradiol. Cancer Res 4:186–190
Reitamo J, Häyry P, Nykyri E, Saxén E (1982) The desmoid tumor. I. Incidence, sex- age- and anatomical distribution in the Finnish population. Am J Clin Pathol 77:665–673
Bruzzone S, Elgueta H, Iglesias R, Lipschütz A (1948) Oestrogen-induced fibroids of thoracic serosa. Br J Cancer 2:267–272
Nadel EM (1950) Histopathology of estrogen-induced tumors in guinea pigs. J Natl Cancer Inst 10:1043–1065
Arellano Perez H, Guzman PC, Aguilar PE (1976) Extraabdominal desmoid tumor: one case of successful treatment with antiestrogens. Rev Invest Clin 28:45–51
Kinzbrunner B, Ritter S, Domingo J, Rosenthal CJ (1983) Remission of rapidly growing desmoid tumors after tamoxifen therapy. Cancer 52:2201–2204
Waddell WR, Gerner RE, Reich MP (1983) Nonsteroid antiinflammatory drugs and tamoxifen for desmoid tumors and carcinoma of the stomach. J Surg Oncol 22:197–211
Järvinen HJ, Peltokallio P, Landtman M, Wolf J (1982) Gardner's stigmas in patients with familial adenomatosis coli. Br J Surg 69:718–721
Khorsand J, Karakousis CP (1985) Desmoid tumors and their management. Am J Surg 149:215–218
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Lotfi, A.M., Dozois, R.R., Gordon, H. et al. Mesenteric fibromatosis complicating familial adenomatous polyposis: predisposing factors and results of treatment. Int J Colorect Dis 4, 30–36 (1989). https://doi.org/10.1007/BF01648547
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01648547