Low-grade myofibroblastic sarcoma of retroperitoneum: a rare case

Suresh K. Choudhary, Shalu Gupta, Somendra Bansal, Narender Kumar


Low-grade myofibroblastic sarcoma (LGMFS) is an uncommon tumor which develops mainly in the bone or soft tissues of the head and neck region, trunk, or extremities and extremely rarely found in the abdominal cavity. The rarity of the disease and its low-grade features make an accurate diagnosis difficult in most cases. We recently encountered a giant LGMFS which had developed in retroperitoneum and surgically resected with gratifying results. An 18 years old female presented with complaint of left sided abdominal pain since 8 months and left sided abdominal lump since 5 months. Imaging examinations revealed retroperitoneal soft tissue mass, and surgical treatment was scheduled. During operation, a tumor about 20x18x15cm in diameter with its anterior aspect covered with the pancreas, mesocolon and splenic flexure of colon with densely adhered to splenic vessels and left renal vessels was found. The tumor had firm adhesions to the surrounding tissues, and it was excised with concomitant distal pancreato-splenectomy, left nephrectomy and resection of splenic flexure of colon with colo-colic anastomosis. Histopathologically, fusiform cells were arranged in a complicated or storiform pattern, and immunohistochemical staining revealed that the tumor was positive for vimentin and focally positive for α-smooth muscle actin (SMA), negative for desmin, CD34, CD117, EMA, DOG-1 and S-100. Diagnosis of LGMFS was made. During 6 months follow up patient is asymptomatic and ultrasound abdomen is normal. In case of LGMFS, favorable prognosis can be attained by complete resection of the primary lesion and regular follow up of patient by physical examination and imaging modality.


Myofibroblastic sarcoma, Retroperitoneum, Sarcoma

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Mentzel T, Dry S, Katenkamp D, Fletcher CD. Low-grade myofibroblastic sarcoma: analysis of 18 cases in the spectrum of myofibroblastic tumors. Am J Surg Pathol. 1998 Oct 1;22(10):1228-38.

Montgomery E, Goldblum JR, Fisher C. Myofibrosarcoma: a clinicopathologic study. Am J Surg Pathol. 2001 Feb 1;25(2):219-28.

Agaimy A, Wünsch PH, Schroeder J, Gaumann A, Dietmaier W, Hartmann A, et al. Low-grade abdominopelvic sarcoma with myofibroblastic features (low-grade myofibroblastic sarcoma): clinicopathological, immunohistochemical, molecular genetic and ultrastructural study of two cases with literature review. J Clin Pathol. 2008 Mar 1;61(3):301-6.

Koga S, Ikeda S, Urata J, Chijiwa R, Abe K, Hayashi T, et al. Primary high-grade myofibroblastic sarcoma arising from the pericardium. Circulation J. 2008;72(2):337-9.

Arora R, Gupta R, Sharma A, Dinda AK. A rare case of low-grade myofibroblastic sarcoma of the femur in a 38-year-old woman: a case report. J Med Case Reports. 2010 Dec;4(1):121.

Bossi M, Paolino LA, Valenti A, Marciano LE, Polliand C, Ziol M, et al. High-grade poorly differentiated retroperitoneal sarcoma. Report of a case and review of the literature. Rom J Morphol Embryol. 2012 Jan 1;53(3):625-8.

Meng DGZ, Zhang HY, Bu H, Zhang XL, Pang ZG, Ke Q, et al. Myofibroblastic sarcomas: a clinicopathological study of 20 cases. Chin Med J. 2007;120:363-9.

Niedzielska I, Janic T, Mrowiec B. Low-grade myofibroblastic sarcoma of the mandible: a case report. J Med Case Reports. 2009 Dec;3(1):8458.

Taccagni G, Rovere E, Masullo M, Christensen L, Eyden B. Myofibrosarcoma of the breast: review of the literature on myofibroblastic tumors and criteria for defining myofibroblastic differentiation. Am J Surg Pathol. 1997 Apr 1;21(4):489-96.

Bisceglia M, Magro G. Low-grade myofibroblastic sarcoma of the salivary gland. Am J Surg Pathol. 1998;22:1228-38.

Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK, Lee TJ, Ro JY, Silverman JS. A Case of Cutaneous Low‐grade Myofibroblastic Sarcoma. J Dermatol. 2001 Jul;28(7):383-7.

Roth TM, Fratkin J, Woodring TC, McGehee RP. Low-grade myofibroblastic sarcoma of the vulva. Gynecol Oncol. 2004 Jan 1;92(1):361-4.

Watanabe K, Ogra G, Tajino T, Tajino T, Hoshi N Suzuki T. Myofibrosarcoma of the bone: a clinicopathologic study. Am J Surg Pathol. 2001 Dec; 25(12):1501-7.

Zeng Y, Tang R. Low-grade myofibroblastic sarcoma of the right ilium. Arch Histopathol Differ Diagn. 2007;14:43-7.