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Neoplastic Meningitis as the Presentation of Occult Primitive Neuroectodermal Tumors

Vanderbilt University, Nashville, TN

Neal Slatkin, MD

City of Hope Hospital, Arcadia, CA

Marc D'Angelo, MD

Archbold Memorial Hospital, Thomasville, GA

Leena Ketonen, MD, PhD

University of Rochester, Rochester, NY

Mahlon D. Johnson, MD, PhD

Vanderbilt University, Nashville, TN

Marc Rosenblum, MD

Memorial Sloan-Kettering Cancer Center, New York, NY

Jeffrey Creasy, MD

Vanderbilt University, Nashville, TN

Noel Tulipan, MD

Vanderbilt University, Nashville, TN

Russell Walker, MD

Memorial Sloan-Kettering Cancer Center, New York, NY

Seven children and young adults initially presented with subacute meningitis and/or increased intracranial pressure. The diagnosis of neoplastic meningitis secondary to a primitive neuroectodermal neoplasm was delayed by the absence of an obvious primary tumor. The neuroradiologic appearance was that of a basimeningeal infiltrative process, complicated by communicating hydrocephalus or "pseudotumor cerebri." Myelography was important in the diagnosis of disseminated meningeal malignancy in four cases. Cerebrospinal fluid cytologic diagnosis was insensitive but ultimately confirmed in five cases. All seven patients experienced progressive disease despite neuraxis radiotherapy and intensive chemotherapy; six have died. Systemic dissemination to bone and/or peritoneum occurred in three patients while on therapy. In two, a primary parenchymal brain or spinal cord tumor could not be identified at postmortem examination. The presentation of a primitive neuroectodermal tumor as subacute meningitis without an evident primary tumor heralds an aggressive and refractory neoplasm. (J Child Neurol 1993;8:306-312).

Journal of Child Neurology, Vol. 8, No. 4, 306-312 (1993)
DOI: 10.1177/088307389300800403


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