Infiltrative Astrocytomas With Granular Cell Features (Granular Cell Astrocytomas)

Abstract

Granular cell astrocytomas (GCAs) are rare, incompletely characterized infiltrative gliomas that contain a prominent component of granular cells. Such tumors can readily be mistaken for reactive conditions. We studied 22 cases to explore their morphologic spectrum, establish features useful in distinguishing GCA from nonneoplastic diseases, and to determine which parameters correlate with biologic behavior. Tumors occurred in 17 men and five women, ranging in age from 29 to 75 years, who presented mainly with seizures, headache, aphasia, or hemiparesis. Radiologically, high-grade GCAs were contrast-enhancing, cerebral hemispheric masses with prominent peritumoral edema. All contained sheets or interspersed large, round cells packed with eosinophilic, PAS-positive granules. Lymphocytic infiltrates, either perivascular or admixed with neoplastic cells, were present in 14 tumors. Transition to typical infiltrating astrocytoma was noted in 16 cases; of these, granular cells comprised 30-95% of cells. Six tumors consisted almost entirely of atypical granular cells. By WHO criteria, four GCA were grade 2, seven were grade 3, and 11 were grade 4. Glial fibrillary acidic protein staining was seen in all but one tumor, and the majority were immunoreactive for S-100 protein, KP-1, ubiquitin, and epithelial membrane antigen. Although MIB-1 proliferation indices increased with tumor grade, granular cells accounted for only a minority of immunoreactive cells. Among 18 cases with follow-up, 15 recurred after surgery and resulted in death (mean survival, 7.6 months). Two patients died postoperatively, and one was alive at 51 months. Granular cell astrocytoma is an uncommon morphologic variant that appears to be rapidly progressive and usually fatal.

Diagnosis and management challenge of a granular cell astrocytoma of the pineal region: case report

“…3,9,12 Although no systematic studies of this tumor have been undertaken to date, GCTs of the neurohypophysis and pineal region, unlike GCAs of the cerebral hemisphere, seem to be typically benign and indolent despite occasional invasion and recurrence. 2,3,10 Follow-up evaluation in our case, in the 4 previously reported pineal cases, and in cases in all other intracranial localizations has shown that subtotal resection is effective for symptomatic tumors. 6,[8][9][10]12,15 The role of radiation therapy for this benign, slow-growing tumor remains unclear.…”

Diagnostic implications of IDH1-R132H and OLIG2 expression patterns in rare and challenging glioblastoma variants

“…28,29 Generally, the use of GFAP and macrophage-specific markers (eg, CD163) substantiates the glial lineage. However, CD68 immunoreactivity can be misleading since the lysosome-rich glioma cells are often positive and GFAP may be difficult to interpret in cases with high background staining.…”

Established and emerging variants of glioblastoma multiforme: review of morphological and molecular features

“…One-year survival from GCA is reportedly 12% for high-grade GCA and 40% for low-grade GCA with extension to multiple cerebral lobes as seen in 35% of reported cases, may confer a poor prognosis despite a lack of aggressive histological features [150]. A study of 22 cases of GCAs (age range from 29 to 75 years) with a nearly 3 : 1 male : female ratio (17 men, 5 women) showed sheets of monomorphic round cells packed with eosinophilic, PAS-staining granules comprising 30-95% of cells [16]. Further more, this study demonstrated lymphocytic infiltration in 63% of cases, transition to infiltrating astrocytoma in 72% of cases, GFAP staining in 95% of tumours, common staining for S-100, KP-1, ubiquitin, and EMA, as well as MIB-1 index correlating with WHO grade.…”