Atypical Presentation of Transcranial Extension of Intracranial Meningiomas

Meningiomas are the most common primary central nervous system tumors. These tumors predominantly arise from the neural crest-derived meningothelial cells of the arachnoid dural layer. Intracranial meningiomas are stratified with the World Health Organization classification of tumors. Cutaneous meningiomas present rarely and have their own criteria classification (Lopez classification) of 3 types. The first type is congenital. The second consists of ectopic soft-tissue meningiomas. The third involves tumors that extended into the dermis or subcutis that include the neuroaxis. We present a case of a 56-year-old woman with 4 facial tumors that clinically seemed to be cutaneous cysts or lipomas. She reported a history of surgical resection of an intracranial meningioma on the left forehead scalp line 15 years ago. A recent surgical resection of a glabellar tumor revealed a glistening white mass. Pathologic examination revealed a poorly circumscribed mass in the deep dermis and subcutaneous area with sheets of epithelioid and plasmacytoid tumor cells with nuclear pleomorphism. Mitotic figures and necrosis were also evident. Immunohistochemistry revealed positivity for epithelial membrane antigen, p63, and ERG. The tissue had negative staining for p40, CK7, SOX10, CD68, SMA, desmin, and CD34. The patient's medical history was remarkable in that these tumors had only been growing for several months. Brain magnetic resonance imaging demonstrated widespread tumors in bilateral frontal lobes, skull, orbits, and sinuses. Considering the transcranial extensions and 15-year recurrence time, she was diagnosed with a recurrent atypical brain meningioma type II and cutaneous meningioma Lopez type III.