Primary intracranial epidermoid carcinoma with diffuse leptomeningeal carcinomatosis: Report of two cases.

Primary intracranial epidermoid carcinoma with diffuse leptomeningeal carcinomatosis: Report of two cases.

World Neurosurg. 2015 Dec 8;

Authors: Raheja A, Eli IM, Bowers CA, Palmer CA, Couldwell WT

Abstract
BACKGROUND: Malignant degeneration of epidermoid cyst (EC) with accompanying leptomeningeal carcinomatosis (LC) at presentation is extremely rare. We add two cases to the literature, including the first case of primary brainstem involvement with simultaneous diffuse LC, and discuss clinical and radiological cues to differentiate benign and malignant epidermoid tumors for early diagnosis.
CASE DESCRIPTION: A 54-year-old woman presented with recurrent aseptic meningitis and hydrocephalus. Imaging revealed a pre- and parapontine extra-axial EC with an intra-axial brainstem ring-enhancing cystic lesion, diffuse leptomeningeal enhancement, and intradural extramedullary nodular deposits throughout the spine. Surgical decompression of the cysts confirmed the diagnosis of invasive primary squamous cell carcinoma (SCC) of the brainstem and benign epidermoid tumor of the cerebellopontine cistern. The second patient was a 37-year-old woman with extensive left-sided cranial neuropathies. Imaging revealed pre- and parapontine enhancing and non-enhancing deposits along multiple cranial nerves and diffuse leptomeningeal nodular enhancement in the thoracolumbar spine. A biopsy confirmed the diagnosis of infiltrative poorly differentiated carcinoma adjacent to a benign EC. Both patients had systemic screening to rule out metastatic disease.
CONCLUSIONS: These cases illustrate that a high index of clinical suspicion is necessary for early diagnosis of disseminated disease in cases of recurrent episodes of aseptic meningitis. In cases of primary benign EC, aggressive resection should be attempted to reduce the risk of malignant degeneration. Separate biopsy from the enhancing portion of the tumor is used to rule out an underlying coexisting malignancy. Multimodal management carries the best prognosis for primary intracranial SCC with LC.

PMID: 26679260 [PubMed - as supplied by publisher]

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