Abstract
Forty-two of 416 familial adenomatous polyposis (FAP) patients in the FAP registry at the Cleveland Clinic had desmoid tumors. The role of cytotoxic chemotherapy and radiation therapy in the management of these patients was investigated. Eight intra-abdominal desmoid tumors were treated by systemic cytotoxic chemotherapy. Two had complete remission, and one had partial remission. Five patients died as a result of the desmoid tumor or late complications of chemotherapy. Three intra-abdominal desmoids were treated by radiation therapy with no response. Neither cytotoxic chemotherapy nor radiation therapy is recommended as a first-choice treatment for intra-abdominal desmoid tumors in patients with FAP.
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References
Sener SF, Miller HH, DeCosse JJ. The spectrum of polyposis. Surg Gynecol Obstet 1984;159:525–32.
Järvinen HJ, Peltokallio P, Landtman M, Wolf J. Gardner's stigmas in patients with familial adenomatosis coli. Br J Surg 1982;69:718–21.
Arvanitis ML, Jagelman DG, Fazio VW, Lavery IC, McGannon E. Mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 1990;33:639–42.
Jones IT, Fazio VW, Weakley FL, Jagelman DG, Lavery IC, McGannon E. Desmoid tumors in familial polyposis coli. Ann Surg 1986;204:94–7.
Klemmer S, Pascoe L, DeCosse J. Occurrence of desmoids in patients with familial adenomatous polyposis of the colon. Am J Med Genet 1987;28:385–92.
Järvinen HJ. Desmoid disease as a part of familial adenomatous polyposis coli. Acta Chir Scand 1987;153:379–83.
Tsukada K, Fazio VW, Church JM,et al. Surgical treatment of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis (in preparation).
Tsukada K, Church JM, Jagelman DG,et al. Noncytotoxic drug therapy for intra-abdominal desmoid tumor in patients with familial adenomatous polyposis. Dis Colon Rectum (in press).
Douglass HD, Karakousis C. Alternating administration of Adriamycin and vincristine-actinomycin D in advanced sarcomas. Cancer Chemother Rep 1975;59:1045–7.
Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J, Flake G. Desmoid fibromatosis: a clinicopathologic study of 25 children. Semin Diagn Pathol 1986;3:138–50.
Karakousis CP, Berjian RA, Lopez R, Rao U. Mesenteric fibromatosis in Gardner's syndrome. Arch Surg 1978;113:998–1000.
Hutchinson RJ, Norris DG, Schnaufer L. Chemotherapy: a successful application in abdominal fibromatosis. Pediatrics 197;63:157–9.
Bataini JP, Belloir C, Mazabraud A,et al. Desmoid tumors in adults: the role of radiotherapy in their management. Am J Surg 1988;155:754–60.
Leibel SA, Wara WM, Hill DR,et al. Desmoid tumors: local control and patterns of relapse following radiation therapy. Int J Radiat Oncol Biol Phys 1983;9:1167–71.
Kiel KD, Suit HD. Radiation therapy in the treatment of aggressive fibromatosis (desmoid tumors). Cancer 1984;54:2051–5.
Assad WA, Nori D, Hilaris BS, Shiu MH, Hajdu SI. Role of brachytherapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys 1986;12:901–6.
Greenberg HM, Goebel R, Weichselbaum RR, Greenberger JS, Chaffey JT, Cassady JR. Radiation therapy in the treatment of aggressive fibromatosis. Int J Radiat Oncol Biol Phys 1981;7:305–10.
Pack GT, Ehrlich HE. Neoplasms of the anterior abdominal wall with special consideration of desmoid tumors: experience with 391 cases and a collective review of the literature. Int Abstr Surg 1944;79:177–98.
Gaches C, Burke J. Desmoid tumor (fibroma of the abdominal wall) occurring in siblings. Br J Surg 1971;58:495–8.
Kim D-H, Goldsmith HS, Quan SH, Huvos AG. Intra-abdominal desmoid tumor. Cancer 1971;27:1041–5.
Reitamo JJ. The desmoid tumor. IV. Choice of treatment, results, and complications. Arch Surg 1983;118:1318–22.
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Tsukada, K., Church, J.M., Jagelman, D.G. et al. Systemic cytotoxic chemotherapy and radiation therapy for desmoid in familial adenomatous polyposis. Dis Colon Rectum 34, 1090–1092 (1991). https://doi.org/10.1007/BF02050067
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DOI: https://doi.org/10.1007/BF02050067