CENTRAL AND PERIPHERAL

NERVOUS TUMORS

Gliomas

General definition: Tumor arising from

astrocytes, oligodendrocytes, ependymal cells, and plexus epithelial cells.

These tumors often exhibit a radial grouping of cell nuclei. The term rosette is used to refer to such a grouping around a nonvascular lumen. A radial arrangement of nuclei around a vascular structure or a virtual center is referred to as a pseudorosette.

Gliomas

Gliomas present with the following general clinical features:

The tumor is ill-defined due to its infiltrative growth into the tissue of the brain. Systemic metastases are extremely rare. The tumor often distorts the topography of the brain. Complications include death from increased intracranial pressure.

Gliomas

Gliomas are diagnosed by immunohistochemical findings of expressed GFAP (an acidic glial fiber protein).

astrocytoma

Well-differentiated astrocytoma. A, The right frontal tumor has expanded gyri, which led to flattening (arrows). B, Expanded white matter of the left cerebral hemisphere and thickened corpus callosum and fornices.

Low-Grade-Astrocytoma

Pilocytic astrocytoma in the cerebellum with a nodule of tumor in a cyst.

Pilocytic astrocytoma (HE) x 100

Grade-III-Astrocytoma

Gemistocytic astrocytoma (HE) x 100

Examination of crush preparation: Cerebral masses whose malignancy status is unknown are located and aspirated under stereotactic guidance. The needle biopsy obtained is flattened between two slides. Once the proper stain is applied, the cells contained in the specimen can be evaluated within a few minutes (C, D).

Oligodendroglioma
Occurrence: cerebrum. Age of manifestation: adults. Definition: Slowly growing tumor arising from oligodendrocytes. Morphology: Ill-defined tumor of small, densely packed tumor cells (exhibiting a dark nucleus in bright cytoplasm) that creates a honeycomb pattern. Signs of regression include bleeding, cysts, and calcification.

Oligodendroglioma

This rare tumor arises from cells that make up the fatty substance called myelin that covers the nerves like electrical insulation. These tumors usually occur in the cerebrum. They grow slowly and usually do not spread into surrounding brain tissue like astrocytomas do. They are most common in middle-aged adults.

Oligodendroglioma

Clinical presentation : Patients exhibit symptoms of epilepsy. The tumor tends to evolve into less differentiated forms.

Oligodendroglioma

ependymoma

ependymoma

Ependymoma. A, Tumor growing into the fourth ventricle, distorting, compressing, and infiltrating surrounding structures. B, Microscopic appearance of ependymoma.

Ependymoma, rosset

medulloblastoma

Medulloblastoma. A, CT scan showing a contrastenhancing midline lesion in the posterior fossa.

Medulloblastoma. Sagittal section of brain showing medulloblastoma destroying the superior midline cerebellum.

Medulloblastoma. C, Microscopic appearance of medulloblastoma

Retinoblastoma

Retinoblastoma ( eye )

RETINOBLASTOMA

Retinoblastoma ( eye ) (HE) x 50

RETINOBLASTOMA

meningioma

meningioma

Definition: Benign arachnoid cell tumor Morphology: Spherical or lobulated tumor consisting of spindle-shaped tumor cells (meningothelial cells of the arachnoid) that tend to assume an arrangement resembling the layers of an onion. The tumor is located between the soft meninges, successively leading to formation of a capsule and reactive hyperostosis of the skull.

Parasagittal multilobular meningioma attached to the dura with compression of underlying brain.

beningn meningioma

meningioma

meningioma

Meningioma with a whorled pattern of cell growth and psammoma bodies.

Psammomatous meningioma

Psammomatous meningioma exhibits dense epithelial clusters of tumors cells forming numerous corpuscles resembling the layers of an onion, leading to psammomatous calcification

Fibrous meningioma

Fibrous meningioma

Fibroblastic meningioma exhibits chains and swirls of tumor cells rich in collagen fibers with few onion-like corpuscles.

transisional meningioma

case

A 50-year-old woman presented with progressive headaches, unstable gait, short-term memory deficit, and mood swings. Bladder and bowel function were unaffected. No other focal deficits were noted. The patient had no history of trauma. Past medical history was significant for rheumatoid arthritis treated with piroxicam and hydroxychloroquine sulfate.

Serum electrolytes and white blood cell count were within normal limits. She was mildly anemic. Hemoglobin and hematocrit values were 9.5 g/dL and 28%, respectively.

A computed tomographic scan of the brain demonstrated a large, enhancing left lateral sphenoid wing tumor, measuring 5 to 6 cm in greatest dimension with surrounding edema. A follow-up magnetic resonance imaging/magnetic resonance angiography study confirmed a large, left frontotemporal, extra-axial tumor with generalized enhancement and evidence of hemorrhage within the tumor. No large vessels were noted to feed into the tumor; however, the left middle cerebral artery was markedly displaced. The patient was treated with phenytoin and a craniotomy was performed.

Progressive Headaches in a 50-Year-Old Woman

Surgery yielded a 3.0 2.5 1.0-cm aggregate of tan-pink, mucoid, focally hemorrhagic soft tissue fragments. Histopathology revealed abnormal trabeculae composed of vacuolated eosinophilic cells in a myxoid background (Figure 1 ). Also identified were small areas composed of whorled epithelial cells (Figure 2 ).

What is your diagnosis?

Pathologic Diagnosis: Chordoid Meningioma

Chordoid meningiomas feature a mixture of epithelioid and spindled cells within a myxoid matrix.15 The histologic appearance closely resembles a chordoma.15 The tumor exhibits cytoplasmic vacuolation and clustering or cords of tumor cells.2,5 Meningothelial foci are also usually present. In addition, these tumors are often surrounded by a heavy lymphocytic infiltrate, often showing follicles and germinal centers; however, this feature is not diagnostic.1,4 Most lymphocytic infiltrates in all meningioma types are composed of T cells; however, chordoid meningiomas of childhood are strongly associated with B lymphocytes and plasma cells.4

neurofibroma

Type I neurofibromatosis (skin)

S-100: neurofibroma (IH) x 75

Schwannoma

Schwannoma

These benign tumors arise from the neural crestderived Schwann cell and are associated with neurofibromatosis type 2. Symptoms are referable to local compression of the involved nerve or to compression of adjacent structures (such as brain stem or spinal cord). Sporadic schwannomas are associated with mutations in the NF2 gene on chromosome 22; there is usually absence of the NF2 gene product by Western blotting or immunostaining, even if there is no evidence of a mutation in the gene.

Schwannoma.
A, Bilateral eighth
nerve schwannomas.
(Courtesy of Dr. K.M. Earle.)

B, Tumor showing cellular areas (Antoni A), including Verocay bodies (far right), as well as looser, myxoid regions (Antoni B).

Schwannoma (HE) x 100

METASTATIC TUMOR