Primary cutaneous meningioma associated with von Recklinghausen's disease

Cutaneous meningeal tumors are rare and can pose a diagnostic problem. We present a case of a 12-year-old girl with a family history of von Recklinghausen's disease. The patient was asymptomatic until the age of 11, when she developed two lesions on the head, both diagnosed as plexiform neurofibroma. Subsequently, she presented with a subcutaneous nodule on the left posterior occipital scalp which was excised. On histology, the tumor was composed of spindle-shaped cells with diffuse and nested patterns. A whorled configuration of the cells, with occasional giant cells and psammoma bodies, was present. There was no evidence of connection between the tumor and the underlying tissues. Immunohistochemical studies were positive for epithelial membrane antigen, vimentin, and weakly for neuron-specific enolase. Cytokeratin, S-100 protein, and muscle markers were negative. Based on these features, the diagnosis of cutaneous meningioma was made. An MRI examination failed to detect any communication between the tumor site and the meninges; however, asymptomatic bilateral acoustic neuromas were identified. This case, besides being of interest as a primary cutaneous meningioma, also documents a unique combination of findings, i.e., plexiform neurofibroma, meningioma, and cerebellopontine acoustic neuromas, which should alert the clinician to a forme fruste presentation of von Recklinghausen's disease.