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原发性腹膜平滑肌肉瘤

Primary leiomyosarcoma of peritoneal cavity.

作者信息

Bharti Jyotsna Naresh, Dey Biswajit, Desai Parth, Gupta Richa, Khurana Nita, Gandhi Gauri

机构信息

Department of Pathology, Maulana Azad Medical College , New Delhi, India.

Department of Obstetrics and Gynecology, Maulana Azad Medical College , New Delhi, India.

出版信息

Rare Tumors. 2014 Mar 26;6(1):5165. doi: 10.4081/rt.2014.5165. eCollection 2014 Jan 23.

Abstract

Leiomyosarcomas of soft tissue are the rare tumors and the retroperitoneum is the most common site involved. We report a case of primary leiomyosarcoma of the peritoneal cavity which clinically presented with suprapubic, freely mobile, nontender mass which measured 10×10 cm in size. Contrast enhanced computed tomography revealed well defined heterogenous hypodense solid cystic mass. The mass was surgically excised out in its entirety. The histopathological examination revealed spindle cells arranged in alternating fascicles having pleomorphic nuclei, indistinct margin and eosinophilic cytoplasm with foci of haemorrhage, necrosis and 5-6 mitosis/HPF. The spindle cells were immunoreactive for smooth muscle actin, desmin and negative for S-100, CD-34 and c-kit. Histopathology and immunohistochemistry were helpful in making the final confirmatory diagnosis. Leiomyosarcomas are aggressive tumors, with poor prognosis and often difficult to treat. The survival rates are lowest among all soft tissue sarcomas.

摘要

软组织平滑肌肉瘤是罕见肿瘤,腹膜后是最常受累部位。我们报告一例原发性腹腔平滑肌肉瘤,临床表现为耻骨上可自由活动的无压痛肿块,大小为10×10 cm。增强CT显示边界清晰的不均匀低密度实性囊性肿块。该肿块被完整手术切除。组织病理学检查显示梭形细胞呈交替束状排列,细胞核多形性,边界不清,胞质嗜酸性,有出血、坏死灶,每高倍视野有5 - 6个核分裂象。梭形细胞对平滑肌肌动蛋白、结蛋白免疫反应阳性,对S - 100、CD - 34和c - kit免疫反应阴性。组织病理学和免疫组化有助于做出最终确诊。平滑肌肉瘤是侵袭性肿瘤,预后差,治疗往往困难。其生存率在所有软组织肉瘤中最低。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2c6/3977169/5cefd8f05db4/rt-2014-1-5165-g001.jpg

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