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Year : 2015  |  Volume : 58  |  Issue : 3  |  Page : 409-410
The myofibroblastic sarcoma of retroperitoneum: A diagnostic dilemma

Department of Pathology, Sree Siddhartha Medical College, Tumkur, Karnataka, India

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Date of Web Publication14-Aug-2015

How to cite this article:
Geetha J P, Rashmi M V. The myofibroblastic sarcoma of retroperitoneum: A diagnostic dilemma. Indian J Pathol Microbiol 2015;58:409-10

How to cite this URL:
Geetha J P, Rashmi M V. The myofibroblastic sarcoma of retroperitoneum: A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2015 [cited 2022 Jan 22];58:409-10. Available from: https://www.ijpmonline.org/text.asp?2015/58/3/409/162938


Myofibroblastic sarcomas (MS), indicating malignant tumors with myofibroblastic differentiation, have been a controversial neoplasm for long time and have recently been better defined. [1],[2] The rarity of the disease and its low grade features make an accurate diagnosis difficult in most cases. [2],[3]

The differential diagnosis of this unusual tumor includes various benign entities as well as other sarcomas. [4] MS can occur at any age and in diverse sites such as head and neck especially oral cavity, bone, trunk, extremities, and other sites, [1],[2],[4] but rarely reported in retroperitoneum.

A 65 year old male presented with lower abdominal pain and fullness of one year duration, which was progressive in nature. On examination, there was a palpable mass in the lumbar region and revealed a retroperitoneal mass on ultrasonography. Fine needle aspiration cytology could not exclude the possibility of reactive or malignant neoplasm. Excision was done. Grossly it was a well circumscribed, nodular soft tissue mass measuring 11.5 6 7 cms. Cut sections revealed solid, lobulated, gray white mass along with areas of necrosis and hemorrhage [Figure 1]. Histopathologically, a partially circumscribed lesion composed of sheets of spindle cells in short fascicles, bundles, whorls and in storiform pattern were seen [Figure 2]. These were plump spindle cells with vesicular nucleus, inconspicuous nucleoli, and eosinophilic cytoplasm [Figure 3]a. A mixed inflammatory infiltrate composed of lymphocytes, plasma cells, neutrophils, eosinophils, and few multinucleated foreign body type giant cells were seen [Figure 3]b. Atypical mitosis [Figure 3]c and areas of necrosis were also noted [Figure 3]d. Immunohistochemically, it was positive for vimentin [Figure 4]a, smooth muscle actin [Figure 4]b, desmin [Figure 4]c, Ki-67 index of 20-30% [Figure 4]d. CK, ALK, CD117 (c-kit), CD34, and CD23 were negative. There was recurrence of the lesion by eight months and patient gradually deteriorated and succumbed to death within two months of recurrence.
Figure 1: Gross specimen showing solid, lobulated, gray white with areas of necrosis and hemorrhage

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Figure 2: Sheets of spindle cells in short fascicles, bundles, whorls and in storiform pattern (H and E, ×100)

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Figure 3: Plump spindle cells with a vesicular nucleus, inconspicuous nucleoli, and eosinophilic cytoplasm (a) A mixed inflammatory infiltrate (b) Atypical mitosis (c) And an area of necrosis seen (d)

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Figure 4: Immunohistochemically, it is positive for vimentin (a) Smooth muscle actin (b) Desmin (c) Ki-67 index of 20-30% (d)

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Myofibroblastic sarcoma was characterized as a distinct neoplasm in 1998 by Mentzel et al. [3] MS are rare lesions that have become better defined in the past few years, although clearly defined diagnostic criteria have not been established. [2] Histopathologically, MS are composed of slender spindle cells with variable nuclear pleomorphism and mitotic activity. Low grade MS lack necrosis and prominent nuclear pleomorphism. [2],[3] Immunohistochemically, myofibroblasts stain positively for at least one of the myogenic markers (desmin, smooth muscle actin, and smooth muscle myosin heavy chain) and vimentin. [1],[3] Other markers like CD34 and cytokeratin are occasionally expressed, S100 and EMA are absent. [2] After excluding inflammatory myofibroblastic tumor, leiomyosarcoma, malignant fibrous histiocytoma,(MFH) and dendritic cell sarcoma, the diagnosis of high-grade myofibroblastic sarcoma was considered.

The natural history indicates very poor survival. [2] Local recurrence is the major cause of mortality for retroperitoneal sarcomas in contrast with extremity sarcomas where the principal cause of death is distant metastases. This depends on the difference in anatomic location and tumor biology. [5] They should be managed by excision with a wide margin of normal tissue and adjuvant radiation therapy or systemic chemotherapy. [1] Due to the difference in prognosis, a precise pathologic diagnosis is essential, which requires a combination of thorough morphologic examination and immunohistochemistry.

   References Top

Meng GZ, Zhang HY, Bu H, Zhang XL, Pang ZG, Ke Q, et al. Myofibroblastic sarcomas: A clinicopathological study of 20 cases. Chin Med J (Engl) 2007;120:363-9.  Back to cited text no. 1
Koga S, Ikeda S, Urata J, Chijiwa R, Abe K, Hayashi T, et al. Primary high-grade myofibroblastic sarcoma arising from the pericardium. Circ J 2008;72:337-9.  Back to cited text no. 2
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 Rep 2010;4:121.  Back to cited text no. 3
Ni C, Xu YY, Zhou SH, Wang SQ. Differential diagnosis of inflammatory myofibroblastic tumour and low-grade myofibroblastic sarcoma: Two case reports with a literature review. J Int Med Res 2011;39:311-20.  Back to cited text no. 4
Bossi M, Paolino LA, Valenti A, Marciano L, 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;53:625-8.  Back to cited text no. 5

Correspondence Address:
Dr. J P Geetha
Department of Pathology, Sree Siddhartha Medical College, Tumkur, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.162938

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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