Imaging findings of familial adenomatous polyposis-associated aggressive mesenteric fibromatosis: A case report

Srdjan Stošić; Slavica Sotirović-Seničar

doi:10.2298/VSP200722098S

Srdjan Stošić University Clinical Center of Vojvodina, Center for Radiology, Novi Sad, Serbia
Slavica Sotirović-Seničar University Clinical Center of Vojvodina, Center for Radiology, Novi Sad, Serbia

DOI: https://doi.org/10.2298/VSP200722098S

Keywords: biopsy, needle;, colonoscopy;, diagnosis;, fibromatosis, abdominal;, fibromatosis, aggressive;, immunohistochemistry;, magnetic resonance imaging;, mesentery;, tomography, x-ray computed

Abstract

Introduction. Aggressive fibromatosis, also known as desmoid type fibromatosis (DF) is a locally aggressive fibroblastic neoplasm that can arise anywhere in the body with no potential for metastasis and a high recurrence rate after surgical resection. Mesenteric fibromatosis are locally aggressive DF of the mesentery with a high propensity for bowel involvement. The real etiology of these tumors remains unknown, occurring sporadically or in association with familial adenomatous polyposis (FAP), as Gardnerʼs syndrome. Case report. A 34-year-old female patient presented with a palpable solid tumefactive mass in the left hemiabdomen. Contrast enhanced computed tomography (CT) and magnetic resonance imaging (MRI) revealed multiple massive solid tumefactions in the mesentery and in between the small bowel loops. Colonoscopy confirmed the presence of multiple sessile polyps characteristic of FAP. Tissue samples of the mesenteric mass were acquired via ultrasound guided biopsy with histopathologic confirmation of desmoid fibromatosis with imunohistochemical analysis. The risk of surgery was deemed too high at the time due to the size of the mass and proximity to mesenteric vascular structures, therefore the patient was planned for chemotherapy with a potential for further surgical reevaluation. Conclusion. Mesenteric fibromatosis is a rare neoplasm that presents with a wide range of histologic and imaging features. CT and MRI play a crucial role in evaluation and planning an optimal treatment model for patients with mesenteric fibromatosis.

Author Biography

Srdjan Stošić, University Clinical Center of Vojvodina, Center for Radiology, Novi Sad, Serbia

dr med.

Centar za radiologiju

Klinički centar Vojvodine

References

Mukut D, Ghalige HS, Santhosh R, Sharma MB, Singh TS. Mes-enteric fibromatosis (Desmoid tumour) – a rare case report. J Clin Diagn Res 2014; 8(11): ND01‒2.

Braschi-Amirfarzan M, Keraliya AR, Krajewski KM, Tirumani SH, Shinagari AB, Hornick JL, et al. Role of imaging in man-agement of desmoid-type fibromatosis: A primer for radiolo-gists. Radiographics 2016; 36(3): 767‒82.

Gari MK, Guraya SY, Hussein AM, Hego MM. Giant mesenter-ic fibromatosis: Report of a case and review of the literature. World J Gastrointest Surg 2012; 4(3): 79‒82.

Sinha A, Hansmann A, Bhandari S, Gupta A, Burling D, Rana S, et al. Imaging assessment of desmoid tumours in familial ade-nomatous polyposis: Is state-of-the-art 1.5 T MRI better than 64 MDCT? Br J Radiol 2012; 85(1015): e254‒61.

Tseng KC, Lin CW, Tzeng JE, Feng WF, Hsieh YH, Chou AL, et al. Gardner’s syndrome – emphasis on desmoid tumours. Tzu Chi Med J 2006; 18: 57‒60.

Wronski M, Ziarkiewicz-Wroblewska B, Slodkowski M, Cebulski W, Gornicka B, Krasnodebski IW. Mesenteric fibromatosis with intestinal involvement mimicking a gastrointestinal stromal tumor. Radiol Oncol 2011; 45(1): 59‒63.

Vasen HF, Moslein G, Alonso A, Aretz S, Bernstein I, Bertario L, et al. Guidelines for the clinical management of familial ade-nomatous polyposis (FAP). Gut 2008; 57(5): 704–13.

Bulow S. Results of national registration of familial adenoma-touspolyposis. Gut 2003; 52: 742–6.

Heiskanen I, Luostarinen T, Jarvinen HJ. Impact of screening ex-aminations on survival in familial adenomatous polyposis. Scand J Gastroenterol 2000; 35: 1284–7.

Vandevenne JE, De Schepper AM, De Beuckeleer L, Van Marck E, Aparasi F, Bloem JL, et al. New concepts in understanding evolution of desmoid tumors: MR imaging of 30 lesions. Eur Radiol 1997; 7(7): 1013–9.

Shinagare AB, Ramaiya NH, Jagannathan JP, Krajewski KM, Giardino AA, Butrynski J, et al. A to Z of desmoid tumors. AJR Am J Roentgenol 2011; 197(6): W1008–14.

Murphey MD, Ruble CM, Tyszko SM, Zbojniewicz AM, Potter BK, Miettinen M. From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correla¬tion. Radi-oGraphics 2009; 29(7): 2143–73.

McCarville MB, Hoffer FA, Adelman CS, Khoury JD, Li C, Skapek SX. MRI and biologic behavior of desmoid tumors in children. AJR Am J Roentgenol 2007; 189(3): 633–40.

Kasper B, Baumgarten C, Bonvalot S, Haas R, Haller F, Hohen-berger P, et al. Manage¬ment of sporadic desmoid-type fibroma-tosis: a European consensus approach based on patients’ and professionals’ expertise—a sarcoma patients EuroNet and Eu-ropean Organisation for Research and Treatment of Can-cer/Soft Tissue and Bone Sarcoma Group initiative. Eur J Cancer 2015; 51(2): 127–36.

Guglielmi G, Cifaratti A, Scalzo G, Magarelli N. Imaging of su-perficial and deep fibromatosis. Radiol Med 2009; 114(8): 1292–307.