Symptomatic glial cysts of the pineal gland

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✓ Small asymptomatic cysts of the pineal gland represent a common incidental finding in adults undergoing computerized tomography or magnetic resonance (MR) imaging or at postmortem examination. In contrast, large symptomatic pineal cysts are rare, being limited to individual case reports or small series. The authors have reviewed 24 cases of large pineal cysts. The mean patient age at presentation was 28.7 years (range 15 to 46 years); 18 were female and six male. Presenting features in 20 symptomatic cases included: headache in 19; nausea and/or vomiting in seven; papilledema in five; visual disturbances in five (diplopia in three, “blurred vision” in two, and unilateral partial oculomotor nerve palsy in one); Parinaud's syndrome in two; hemiparesis in one; hemisensory aberration in one; and seizures in one. Four lesions were discovered incidentally. Magnetic resonance imaging typically demonstrated a 0.8- to 3.0-cm diameter mass (mean 1.7 cm) with homogeneous decreased signal intensity on T1-weighted images, increased signal intensity on T2-weighted images, and a distinct margin. Hydrocephalus was present in eight cases. The cysts were surgically excised via an infratentorial/supracerebellar approach (23 cases) or stereotactically biopsied (one case). Histological examination revealed a cyst wall 0.5 to 2.0 mm thick comprised of three layers: an outer fibrous layer, a middle layer of pineal parenchymal cells with variable calcification, and an inner layer of hypocellular glial tissue often exhibiting Rosenthal fibers and/or granular bodies. Evidence of prior hemorrhage, mild astrocytic degenerative atypia, and disorganization of pineal parenchyma were often present. Postoperative follow-up review in all 24 cases (range 3 months to 10 years) revealed no complications in 21, mild ocular movement deficit in one, gradually resolving Parinaud's syndrome in one, and radiographic evidence of a postoperative venous infarct of the superior cerebellum with ataxia of 1 week's duration in one. Of the patients referred for study, the cysts were most often initially misdiagnosed as a pineocytoma in eight and a pilocytic astrocytoma in three. Only two patients were correctly diagnosed as having pineal cysts. This stresses the importance of recognizing the histopathological spectrum of pineal cysts, as well as correlation with radiographic findings, if a correct diagnosis is to be attained.

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Address reprint requests to: Bernd W. Scheithauer, M.D., Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905.

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    Examples of a pineal cyst. Left: Contrast-enhanced computerized tomography scan of the head showing a 3-cm cystic mass in the pineal region (arrow). Note obstructive dilatation of the lateral and third ventricles. Center and Right: Midcut T1-weighted (TR 6000 msec, TE 20 msec, center) and axial T2-weighted (TR 2000 msec, TE 90 msec, right) magnetic resonance images, sagittal view, of the head showing a 1.5-cm mass within the pineal gland (large arrow), with signals similar to cerebrospinal fluid on both sequences. The midbrain tectum and cerebral aqueduct (small arrow) are compressed, resulting in hydrocephalus.

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    Midcut T1-weighted (TR 800 msec, TE 25 msec) magnetic resonance images, sagittal view, of a pineal cyst prior to (left) and following (right) intravenous infusion of gadolinium-diethylenetriamine penta-acetic acid. Note enhancement of the cyst in a nodular pattern (arrow).

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    Photomicrograph showing the multilayer architecture of a pineal cyst. A scant, often discontinuous fibrous layer (arrow) surrounds the cyst, the wall of which consists in large part of lobulated pineal parenchyma and an inner layer of hypocellular glial tissue (upper right). H & E, × 105.

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    Photomicrographs of a pineal cyst. The pineal parenchyma usually retains its lobular architecture (A); in some instances lobules are separated by glial stroma (B), and in others the normal lobulation of pineal parenchyma is focally vague (arrows) or inapparent (C). H & E, × 100 (A); × 165 (B and C).

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    Photomicrographs of pineal cysts. Left: Bodian stains demonstrating the presence of pineal cell processes, many of which terminate in bulbous expansions around the capillaries (arrows). H & E, × 285. Right: Immunostains for glial fibrillary acidic protein demonstrating the presence of stromal astrocytes within the pineal tissue layer, a feature not seen in pincocytoma. H & E, × 180.

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    Photomicrographs of pineal cysts. A: The glial layer of this long-standing cyst contains numerous dark-staining Rosenthal fibers, a feature that on examination of tissue from a small biopsy may prompt an erroneous diagnosis of pilocytic astrocytoma. H & E, × 106. B and C: Astrocytes comprising the cysts showing nuclear hyperchromasia (B) or a variation in nuclear size (C). A portion of the compressed lumen of the cyst is visible in C. H & E, × 165.

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    Photomicrographs of pineal cysts. A: The three-layered nature of this cyst is demonstrated, although the pineal parenchymal layer is barely represented (arrow), whereas the glial layer is predominant and shows granular degenerative changes in the astrocytic cells. H & E, × 110. B and C: In long-standing cysts, degenerative changes may be apparent: B illustrates granular changes within the astrocytes as well as scattered perivascular lymphocytes; C shows an abundance of hemosiderin deposition due to prior hemorrhage. H & E, × 175.

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    Photomicrograph of an incidentally found pineal cyst. This horizontal, whole-mount autopsy specimen demonstrates considerable variation in wall thickness. The three-layer architecture is focally apparent (arrow), whereas portions of the cyst abutting brain show no appreciable fibrous layer (arrowhead) and others are lacking pineal tissue and consist of only a thin glial layer. H & E, × 6.5.

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