Papillary Glioneuronal Tumor: A New Variant of Mixed... : The American Journal of Surgical Pathology (original) (raw)

Original Articles

A New Variant of Mixed Neuronal-Glial Neoplasm

Komori, Takashi M.D., Ph.D.; Scheithauer, Bernd W. M.D.; Anthony, Douglas C. M.D., Ph.D.; Rosenblum, Marc K. M.D.; McLendon, Roger E. M.D.; Scott, R. Michael M.D.; Okazaki, Haruo M.D.; Kobayashi, Makio M.D., Ph.D.

Department of Pathology and Laboratory Medicine, Mayo Clinic (T.K., B.W.S., H.O.), Rochester, Minnesota, USA; the Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience (T.K.), Fuchu City, Tokyo, Japan; the Department of Pathology and Neurosurgery, Children's Hospital and Harvard Medical School (D.C.A., R.M.S), Boston, Massachusetts, USA; the Department of Pathology, Memorial Sloan-Kettering Cancer Center (M.K.R.), New York, New York, USA; the Department of Pathology, Duke University Medical Center (R.E.M.), Durham, North Carolina; the Department of Pathology, Tokyo Women's Medical College (M.K.), Tokyo, Japan.

Address correspondence and reprint requests to Dr. B. W. Scheithauer, Department of Pathology and Laboratory Medicine, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA. E-mail: [email protected]

Presented in part at the 14th Annual Meeting of the Japan Society of Brain Tumor Pathology, Tokyo, Japan on April 24, 1996, the 72nd Annual Meeting of the American Association of Neuropathologists, Vancouver, Canada on June 15, 1996, and the International Association of Neuropathology, Perth, Australia, on September 9, 1997.

Abstract

We describe the clinicopathologic features of nine cases of a unique papillary glioneuronal tumor (PGNT) exhibiting astrocytic as well as extensive and varied neuronal differentiation. The four male and five female patients studied ranged in age from 11 to 52 years (mean 27.7 years). They either presented with mild neurologic symptoms or were asymptomatic. Magnetic resonance imaging showed demarcated cystic, 1.5-cm to 7-cm contrast-enhancing masses; five involved the temporal lobe, two the parietal, and two the frontal. All but one were totally resected. No recurrence was noted despite a follow-up period of 3 years. Two microscopic components were evident: 1) compact pseudopapillae composed of hyalinized vessels covered by a single layer of glial fibrillary acid protein (GFAP)-positive astrocytes and 2) synaptophysin-positive neuronal cells of varying size, including neurocytes, ganglioid cells, and ganglion cells within neuropil. Immunostains for chromogranin-A were negative, as was in situ hybridization for chromogranin-A mRNA. Ultrastructurally, neuronal cells featured microtubule-containing processes and aberrant synaptic terminals, but dense core granules were rare. Overall, cellularity was moderate and atypia was minimal. No mitotic activity or necrosis was noted. The proportions of the two components varied, but essential morphologic findings were identical in all cases. In that the clinical, radiographic, and morphologic characteristics of PGNT are distinctive, it appears to represent a previously undescribed form of mixed neuronal-glial tumor of the central nervous system.