Abstract
Desmoid tumors are locally invasive, nonmetastasizing fibrous tumors most frequently seen in patients with familial polyposis coli (FPC). Of 325 patients with FPC treated at the Cleveland Clinic, 29 (8.9%) were found to have a total of 36 desmoid tumors. These tumors occur in young patients (mean age: 29.8 years), particularly women (ratio 3:1), and most appeared after previous colectomy (86%). The majority (72% of all desmoids, 90% of patients) were located within the abdomen, specifically within the mesentery of the small intestine. In most cases, attempts at surgical resection were followed by recurrence, and other previous treatments were similarly ineffective. Six of the 29 patients (21%) died from the desmoid and three died from other causes. The recent use of sulindac (Clinoril) has produced some early encouraging results in four patients with these tumors that have proven so difficult to treat in the past.
Full text
PDF
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Belliveau P., Graham A. M. Mesenteric desmoid tumor in Gardner's syndrome treated by sulindac. Dis Colon Rectum. 1984 Jan;27(1):53–54. doi: 10.1007/BF02554079. [DOI] [PubMed] [Google Scholar]
- GARDNER E. J., RICHARDS R. C. Multiple cutaneous and subcutaneous lesions occurring simultaneously with hereditary polyposis and osteomatosis. Am J Hum Genet. 1953 Jun;5(2):139–147. [PMC free article] [PubMed] [Google Scholar]
- Harvey J. C., Quan S. H., Fortner J. G. Gardner's syndrome complicated by mesenteric desmoid tumors. Surgery. 1979 Apr;85(4):475–477. [PubMed] [Google Scholar]
- Hutchinson R. J., Norris D. G., Schnaufer L. Chemotherapy: a successful application in abdominal fibromatosis. Pediatrics. 1979 Jan;63(1):157–159. [PubMed] [Google Scholar]
- Khorsand J., Karakousis C. P. Desmoid tumors and their management. Am J Surg. 1985 Feb;149(2):215–218. doi: 10.1016/s0002-9610(85)80067-2. [DOI] [PubMed] [Google Scholar]
- Kiel K. D., Suit H. D. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors). Cancer. 1984 Nov 15;54(10):2051–2055. doi: 10.1002/1097-0142(19841115)54:10<2051::aid-cncr2820541002>3.0.co;2-2. [DOI] [PubMed] [Google Scholar]
- Kinzbrunner B., Ritter S., Domingo J., Rosenthal C. J. Remission of rapidly growing desmoid tumors after tamoxifen therapy. Cancer. 1983 Dec 15;52(12):2201–2204. doi: 10.1002/1097-0142(19831215)52:12<2201::aid-cncr2820521204>3.0.co;2-#. [DOI] [PubMed] [Google Scholar]
- Leibel S. A., Wara W. M., Hill D. R., Bovill E. G., Jr, de Lorimier A. A., Beckstead J. H., Phillips T. L. Desmoid tumors: local control and patterns of relapse following radiation therapy. Int J Radiat Oncol Biol Phys. 1983 Aug;9(8):1167–1171. doi: 10.1016/0360-3016(83)90175-x. [DOI] [PubMed] [Google Scholar]
- McAdam W. A., Goligher J. C. The occurrence of desmoids in patients with familial polyposis coli. Br J Surg. 1970 Aug;57(8):618–631. doi: 10.1002/bjs.1800570816. [DOI] [PubMed] [Google Scholar]
- Naylor E. W., Gardner E. J., Richards R. C. Desmoid tumors and mesenteric fibromatosis in Gardner's syndrome: report of kindred 109. Arch Surg. 1979 Oct;114(10):1181–1185. doi: 10.1001/archsurg.1979.01370340087015. [DOI] [PubMed] [Google Scholar]
- Schuchardt W. A., Jr, Ponsky J. L. Familial polyposis and Gardner's syndrome. Surg Gynecol Obstet. 1979 Jan;148(1):97–103. [PubMed] [Google Scholar]
- Waddell W. R., Gerner R. E. Indomethacin and ascorbate inhibit desmoid tumors. J Surg Oncol. 1980;15(1):85–90. doi: 10.1002/jso.2930150113. [DOI] [PubMed] [Google Scholar]
- Waddell W. R., Gerner R. E., Reich M. P. Nonsteroid antiinflammatory drugs and tamoxifen for desmoid tumors and carcinoma of the stomach. J Surg Oncol. 1983 Mar;22(3):197–211. doi: 10.1002/jso.2930220314. [DOI] [PubMed] [Google Scholar]