Abstract
Background
Desmoid-type fibromatosis is a locally aggressive tumor known to have high potential for local recurrence after resection, while it exhibits self-limiting behavior and shows growth arrest or spontaneous regression in many patients. Thus, its natural course is not well known, and the proper treatment has not yet been established.
Methods
We retrospectively reviewed clinical outcome and changes in tumor size in 11 consecutive patients with extremity and trunk desmoid-type fibromatoses, who were basically observed without any treatment after diagnosis.
Results
For two patients in whom the tumors were initially incorrectly diagnosed as other tumors, surgical resection was performed. For another two patients, surgical resections were performed in the follow-up periods due to tumor enlargement or joint contracture. In all four patients who underwent surgery, tumors recurred shortly after resection and re-resection was not performed. During the follow-up periods with a median length of 56 months, ten tumors eventually stopped growing, and three of them regressed spontaneously. At the time of final follow-up, ten patients were alive with residual disease without severe morbidity. In one patient, the tumor enlarged to over 30 cm in diameter with a substantial functional deficit.
Conclusions
Simple observation is a noninvasive and function-preserving treatment for desmoid-type fibromatosis.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Weiss SW, Goldblum JR. Extraabdominal fibromatosis. In: Enzinger and Weiss’s soft tissue tumors. 4th ed. St Louis: Mosby; 2001. p. 320–329.
Rock MG, Pritchard DJ, Reiman HM, Soule EH, Brewster RC. Extra-abdominal desmoid tumors. J Bone Joint Surg Am 1984; 66:1369–1374.
Pritchard DJ, Nascimento AG, Petersen IA. Local control of extra-abdominal desmoid tumors. J Bone Joint Surg Am 1996;78:848–854.
Nuyttens JJ, Rust PF, Thomas CR, Turrisi AT III. Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: a comparative review of 22 articles. Cancer 2000;88:1517–15123.
Gronchi A, Casali PG, Mariani L, Lo Vullo S, Colecchia M, Lozza L, et al. Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol 2003;21:1390–1397.
Leithner A, Gapp M, Leithner K, Radl R, Krippl P, Beham A, et al. Margins in extra-abdominal desmoid tumors: a comparative analysis. J Surg Oncol 2004;86:152–156.
Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF. Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer 1999;86:2045–2052.
Miralbell R, Suit HD, Mankin HJ, Zuckerberg LR, Stracher MA, Rosenberg AE. Fibromatoses: from postsurgical surveillance to combined surgery and radiation therapy. Int J Radiation Oncology Biol Phys 1990;18:535–540.
Dahn I, Jonsson N, Lundh G. Desmoid tumours: a series of 33 cases. Acta Chir Scand 1963;126:305–314.
Jenkins NH, Freedman LS, McKibbin B. Spontaneous regression of a desmoid tumour. J Bone Joint Surg Br 1986;68:780–781.
Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan MF. The enigma of desmoid tumors. Ann Surg 1999;229:866–873.
Dalén BP, Bergh PM, Gunterberg BU. Desmoid tumors. a clinical review of 30 patients with more than 20 years’ follow-up. Acta Orthop Scand 2003;74;455–459.
Mendenhall WM, Zlotecki RA, Morris CG, Hochwald SN, Scarborough MT. Aggressive fibromatosis. Am J Clin Oncol 2005;28:211–215.
Hosalkar HS, Fox EJ, Delaney T, Torbert JT, Ogilvie CM, Lackman RD. Desmoid tumors and current status of management. Orthop Clin North Am 2006;37:53–63.
Enzinger FM, Shiraki M. Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid). Analysis of thirty cases followed up for ten or more years. Cancer 1967:20;1131–1140.
Vandevenne JE, De Schepper AM, De Beuckeleer L, Van Marck E, Aparisi F, Bloem JL, et al. New concepts in understanding evolution of desmoid tumors: MR imaging of 30 lesions. Eur Radiol 1997;7:1013–1019.
Reitamo JJ, Scheinin TM, Häyry P. The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 1986;151:230–237.
Alman BA, Naber SP, Terek RM, Jiranek WA, Goldberg MJ, Wolfe HJ. Platelet-derived growth factor in fibrous musculoskeletal disorders: a study of pathologic tissue sections and in vitro primary cell cultures. J Orthop Res 1995;13:67–77.
Liegl B, Leithner A, Bauernhofer T, Windhager R, Guelly C, Regauer S, et al. Immunohistochemical and mutational analysis of PDGF and PDGFR in desmoid tumours: is there a role for tyrosine kinase inhibitors in c-kit-negative desmoid tumours? Histopathology 2006;49:576–581.
Heinrich MC, McArthur GA, Demetri GD, Joensuu H, Bono P, Herrmann R, et al. Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol 2006;24:1195–1203.
Author information
Authors and Affiliations
About this article
Cite this article
Nakayama, T., Tsuboyama, T., Toguchida, J. et al. Natural course of desmoid-type fibromatosis. J Orthop Sci 13, 51–55 (2008). https://doi.org/10.1007/s00776-007-1187-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00776-007-1187-1