Markus Holling1, Uwe Wildförster2, Raphaela Verheggen3, Klaus Müller4, Walter Stummer5, Astrid Jeibmann6. 1. Department of Neurosurgery, University Hospital Münster, Münster, Germany. Electronic address: hollingm@ukmuenster.de. 2. Department of Neurosurgery, Bergmannsheil Gelsenkirchen-Buer, Gelsenkirchen, Germany. 3. Department of Neurosurgery, Bathildiskrankenhaus, Bad Pyrmont, Germany. 4. Institute of Neurology (Edinger Institute), Johann Wolfgang Goethe-University, Frankfurt/Main, Germany. 5. Department of Neurosurgery, University Hospital Münster, Münster, Germany. 6. Institute of Neuropathology, University Hospital Münster, Münster, Germany.
Abstract
BACKGROUND: Myopericytomas are benign vascular tumors composed of perivascular myoid cells that usually arise in superficial soft tissues of the extremities. METHODS: We here report on 5 cases of myopericytoma, malignant myopericytoma, and glomangiopericytoma arising from subcutaneous, paraspinal, orbital, and sellar regions that secondarily involved the nervous system. Patients experienced orbital swelling, progressing neuropathic pain, dermatoma specific pain, and oculomotor paresis. RESULTS: Mean age at diagnosis was 56.8 years (range, 38-74 years). After a mean follow-up of 38 months, no recurrence was detected. All tumors were unencapsulated but relatively well-circumscribed lesions, showing typical histologic and immunohistochemical features of soft-tissue myopericytoma, including one malignant case. CONCLUSION: We conclude that myopericytoma may affect the nervous system and for prognostic and therapeutic reasons, needs to be differentiated from other vascular tumors.
BACKGROUND: Myopericytomas are benign vascular tumors composed of perivascular myoid cells that usually arise in superficial soft tissues of the extremities. METHODS: We here report on 5 cases of myopericytoma, malignant myopericytoma, and glomangiopericytoma arising from subcutaneous, paraspinal, orbital, and sellar regions that secondarily involved the nervous system. Patients experienced orbital swelling, progressing neuropathic pain, dermatoma specific pain, and oculomotor paresis. RESULTS: Mean age at diagnosis was 56.8 years (range, 38-74 years). After a mean follow-up of 38 months, no recurrence was detected. All tumors were unencapsulated but relatively well-circumscribed lesions, showing typical histologic and immunohistochemical features of soft-tissue myopericytoma, including one malignant case. CONCLUSION: We conclude that myopericytoma may affect the nervous system and for prognostic and therapeutic reasons, needs to be differentiated from other vascular tumors.