Alberghini M, Pasquinelli G, Zanella L, Pignatti G, Benini S, Bacchini P, Bertoni F
Department of Surgical Pathology, Rizzoli Institute, IRCCS, Bologna, Italy.
APMIS. 2007 Apr;115(4):376-80. doi: 10.1111/j.1600-0463.2007.apm_569.x.
A 55-year-old male presented with a 1-month history of localized pain caused by an osteolytic and destructive lesion in the right distal femur. Histologically, the tumour consisted of spindle cells intermingled with epithelioid eosinophilic cells arranged in small cords embedded in a hyalinized-to-chondromyxoid stroma. Electron microscopy and immunohistochemistry showed features of myoepithelial differentiation. RT-PCR failed to demonstrate chimeric transcripts of extraskeletal myxoid chondrosarcoma. The final diagnosis was primary malignant myoepithelioma of bone. The patient is alive with lung metastases 13 months after surgery. Primary malignant myoepithelioma of bone is an exceptionally rare neoplasm that should be considered in the differential diagnosis with the more aggressive myxoid spindle cell sarcomas.
一名55岁男性因右股骨远端溶骨性破坏病变出现1个月的局部疼痛。组织学上，肿瘤由梭形细胞与上皮样嗜酸性细胞混合组成，呈小条索状排列，包埋于玻璃样变至软骨黏液样基质中。电子显微镜和免疫组织化学显示有肌上皮分化特征。逆转录聚合酶链反应未能证实骨外黏液样软骨肉瘤的嵌合转录本。最终诊断为原发性骨恶性肌上皮瘤。该患者术后13个月存活，有肺转移。原发性骨恶性肌上皮瘤是一种极其罕见的肿瘤，在与侵袭性更强的黏液样梭形细胞肉瘤进行鉴别诊断时应予以考虑。
