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原发性肝骨肉瘤:病例报告及文献复习。

Primary Osteosarcoma of the Liver: Case Report and Literature Review.

机构信息

Department of Pathology, Xi Jing Hospital, 4th Military Medical University, No. 169 Chang Le Xi Road, Xi'an, 710032, Shaanxi, China.

出版信息

Pathol Oncol Res. 2020 Jan;26(1):115-120. doi: 10.1007/s12253-018-0483-8. Epub 2018 Oct 25.

Abstract

Extraskeletal osteosarcoma is a rare, highly malignant, osteoid formation mesenchymal neoplasm in the absence of bone involvement, associated with exceptionally poor prognosis. It frequently arises in the soft tissues of the extremities or in the retroperitoneum, but rarely in visceral organ. We describe a primary osteosarcoma of the liver in a 70-year-old man who presented with an episode of fever, accompanied by abdominal discomfort, after an accident abdominal strike. Ultrasonography and computed tomography revealed a large heterogeneous mass with areas of dense calcification involving most of the right lobe of liver. Radiography did not show evidence of primary tumor or primary bone lesion at any other site. Histologically, the tumor showed an essentially similar appearance as osteosarcoma originating in the skeleton, comprised of polygonal or spindle shaped cells, along with abundant eosinophilic lace-like osteoids, or irregularly arranged bone trabeculae. Immunohistochemistry showed that the tumor cells were positive for vimentin, CD10, and focally for SMA and CD56, but negative for other lineage-specific markers. Thus, the findings favored a primary hepatic osteosarcoma. This patient received palliative chemotherapy to ease the signs of his sickness due to the large size of the tumor and he died 4 moths later.

摘要

骨外骨肉瘤是一种罕见的、高度恶性的、形成骨样的间叶性肿瘤,在没有骨受累的情况下发生,与异常不良的预后相关。它常发生在四肢的软组织或腹膜后,但很少发生在内脏器官。我们描述了一名 70 岁男性的肝脏原发性骨肉瘤,他在腹部受撞击后出现发热和腹部不适。超声和计算机断层扫描显示一个大的混杂性肿块,伴有密集的钙化区域,累及右肝大部分。放射线检查在其他任何部位均未显示原发性肿瘤或原发性骨病变的证据。组织学上,肿瘤表现出与起源于骨骼的骨肉瘤基本相似的外观,由多边形或梭形细胞组成,伴有丰富的嗜酸性蕾丝状骨样组织,或不规则排列的骨小梁。免疫组织化学显示肿瘤细胞阳性表达波形蛋白、CD10,局灶性表达 SMA 和 CD56,但阴性表达其他谱系特异性标志物。因此,这些发现支持肝脏原发性骨肉瘤。由于肿瘤体积大,该患者接受了姑息性化疗以缓解他的疾病症状,4 个月后死亡。

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