gms | German Medical Science

Objective: Myopericytoma is a rare, usually benign perivascular soft tissue tumor. We report a series of 5 myopericytomas affecting the peripheral or central nervous system. Usually myopericytomas arise in the subcutaneous and superficial soft tissues in the extremities, while localization elsewhere was found to be extremely rare even in the nervous system.

Method: Five cases of myopericytoma, atypical myopericytoma and glomangiopericytoma have been retrieved from the archives of the Institute of Neuropathology. Data on tumor location, extent of surgical resection and postoperative course were compiled by reviewing patient records. Moreover, general practitioners and neurosurgeons were contacted to provide follow-up information on recurrence and survival. Histological and immunohistochemical stainings of specimens were accomplished.

Results: The tumors occurred in two females and three males. Patients had a mean age of 56.8 years. All tumors examined were unencapsulated but relatively well circumscribed lesions, composed of oval or spindle shaped myoid appearing cells. In all but one case mitoses and necroses were absent. Infiltration of a peripheral nerve could be observed. All tumors investigated showed a typical staining pattern, except two cases, one showing focal positivity for desmin, the other displaying a Ki67 proliferation index up to 20%. During a mean follow-up of 33 months no patient revealed signs of recurrence.

Conclusions: In conclusion we describe five cases of myopericytoma impacting on the peripheral and central nervous system. Myopericytoma have varied locations including brain, spine and peripheral nerves. In rare cases these tumors may also have atypical features or even show a malignant phenotype. Surgery results in long-term stabilization.