Abstract
Fibromatosis covers a broad spectrum of benign fibrous tissue proliferation and is characterized by slow growing, locally infiltrative growth pattern with a high propensity for local recurrence. We report on a case of multicentric fibromatosis originating from the retroperitoneal space and submandibular triangle, in an 18-year-old lady. Computed tomography revealed a retroperitoneal abdominopelvic tumor extending from the left sub-diaphragmatic space to the pelvic inlet which had enveloped the solid viscera in the left upper quadrant with a displaced celiac axis. She also had a recurrent resectable fibromatosis in left submandibular gland. Histopathological evaluation revealed fibromatosis. Preoperatively, Vinblastin-, Methotrexate-, Tamoxifen-based systemic chemotherapy was offered for 12 weeks in an attempt to downsize the mass. An en bloc complete resection of tumor with multi-visceral resection was performed to achieve negative margins and since then, the patient has remained asymptomatic without any signs of tumor recurrence during the 12th month follow-up visit. Complete resection with negative margins is the treatment of choice and majority of the lesions are amenable for surgical resections. Adjuvant therapy using non-steroidal anti-inflammatory agents, tamoxifen, interferon, anti-neoplastic agents, and radiotherapy, either alone or in combination finds application for un-resectable or recurrent cases.
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References
Weiss SW, Goldblum JR (2001) Fibromatosis. In: Weiss SW, Goldblum JR (eds) Soft tissue tumors. Mosby, St. Louis, pp 309–346
Reitamo JJ, Scheinin TM, Häyry P (1986) The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor. Am J Surg 151:230–237
Campanacci M, Enneking WF (1999) Aggressive fibromatosis. In: Campanacci M, Enneking WF (eds) Bone and soft tissue tumors. Springer Verlag, Padova, pp 925–936
Enzinger FM, Weiss SW (2001) Soft tissue tumors, 4th edn. CV Mosby, St. Louis, pp 1472–1475
Stout AP (1961) The fibromatosis. Clin Orthop 19:11–18
Guglielmi G, Cifaratti A, Scalzo G, Magarelli N (2009) Imaging of superficial and deep fibromatosis. Radiol Med 114:1292–1307
Lath C, Khanna PC, Gadewar SB, Agrawal D (2006) Inoperable aggressive mesenteric fibromatosis with ureteric fistula. Case report and literature review. Eur J Radiol 59:117–112
Kabra V, Chaturvedi P, Pathak KA, deSouza LJ (2001) Mesenteric fibromatosis: a report of three cases and literature review. Indian J Cancer 38:133–136
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Sugoor, P., Gupta, A., Patkar, S. et al. Sporadic Retroperitoneal Fibrosis: a Gentle Giant. Indian J Surg Oncol 9, 71–73 (2018). https://doi.org/10.1007/s13193-017-0710-x
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DOI: https://doi.org/10.1007/s13193-017-0710-x