Primary meningeal sarcomas with leiomyoblastic differentiation: a proposal for a new subtype of primary meningeal sarcomas

Two cases of primary meningeal sarcoma with leiomyoblastic differentiation are presented. In case no. 1, the tumor showed anaplastic spindle cell tumor components intermingled with anaplastic meningothelial components. Meningothelial tumor cells gradually became transformed into spindle tumor cells. Spindle tumor cells reacted with antisera to muscle actin (HHF-35) and alpha-smooth muscle actin. However, unchanged meningothelial tumor cells did not react with the antisera to HHF-35 and alpha-smooth muscle actin. Electron microscopy showed condensations of cytoplasmic fibers and pinocytotic vesicles in spindle tumor cells similar to those seen in smooth muscle cells. In case no. 2, the tumor cells consisted predominantly of sheets of round or polygonal cells as seen in an epithelioid leiomyosarcoma. The neoplastic cells had frequent nuclear inclusions, such as those seen in meningiomas. Immunohistochemically, the tumor cells reacted with antisera to desmin and to HHF-35. Electron microscopy showed a basal lamina around the cytoplasm of tumor cells. Intranuclear inclusions with various cytoplasmic organelles were frequently observed in the tumor cells, as in meningiomas. Interdigitating cytoplasmic processes and intercellular junctional complexes, however, were not found in the tumor cells. Two possible hypotheses explain the occurrence of leiomyoblastic characteristics of these cases. In case no. 1, leiomyoblastic cells originated from meningothelial cells with the advancement of meningothelial anaplasia. In case no. 2, pluripotential mesenchymal cells in the meninges differentiated into meningothelial and smooth-muscle cell lines at the time of tumor growth. With consideration of previous publications on primary meningeal sarcoma, these cases are the first reported primary meningeal sarcoma with leiomyoblastic and meningothelial differentiation.