Meningiomas (Tumors of the Meninges)


Meningiomas: Overview and General Characteristics

Definition

  • Neoplasms arising from meningothelial (arachnoidal) cells; are usually attached to the inner surface of the dura mater (dural-based tumors)

Clinical Features

Epidemiology

  • Comprise about 25% to 30% of primary intracranial tumors

  • Incidental finding 1.4% at autopsy

  • All age groups affected; most common in middle-aged to elderly patients; peak in fifth to seventh decades

  • More common in females (F : M ratio ranges from 2 : 1 to 3 : 1); F : M ratio is 10 : 1 for spinal cord meningiomas

  • Usually sporadic; multiple in about 10% of cases

  • May arise secondary to radiation exposure; both low dose and high dose

  • Multiple meningiomas may arise with neurofibromatosis type 2 (NF2)

  • Chromosomal abnormalities include deletion of chromosome 22

Presentation

  • Slow-growing tumors; symptoms result from location and compression of adjacent structures; headache and seizures are the most common presenting symptoms

  • Most commonly found in intracranial, intraspinal, or orbital locations

  • Intracranial locations: cerebral convexities (50% along sagittal sinus), olfactory grooves, parasellar/suprasellar, petrous ridges, sphenoid ridges, tentorium, posterior fossa

  • Rarely located in intraventricular or epidural spaces

  • Extra-axial, intradural location most common in spinal cord

Prognosis and Treatment

  • Most meningiomas are WHO Grade I and are generally treated by gross total resection; may recur if not complete excised

  • Radiation therapy or radiosurgery may be used for recurrent tumors or for small tumors in surgically inaccessible areas (i.e., cavernous sinus)

Imaging Characteristics

  • MRI typically shows a circumscribed, isodense, contrast-enhancing, dural-based mass with classic “dural tail” sign (enhancement of dura adjacent to tumor)

  • May have peritumoral edema resulting from vascular compromise

  • CT scan may demonstrate tumor calcification or bone formation

Pathology

Gross

  • Well-demarcated, firm, rubbery, yellow-tan, round to lobulated mass attached to dura

  • Usually compresses adjacent brain or spinal cord

  • May invade skull and cause hyperostosis (thickening of the cortical bone)

Histology

  • General features of the most common meningothelial variant:

    • Lobules of mostly uniform cells arranged in a syncytium

    • Meningothelial whorls and psammoma bodies are classic

    • Bland, oval nuclei, and fine chromatin that may demonstrate central clearing

    • May have nuclear pseudoinclusions

    • Many variants exhibit a wide range of morphologic appearances (see the next three topics)

Immunopathology/Special Stains

  • Immunoreactive for vimentin and epithelial membrane antigen (EMA)

  • S-100 variably reactive but usually not prominent

  • Progesterone receptor expressed in about 60% of cases (most often in women)

Main Differential Diagnoses

  • Metastatic carcinoma

  • Schwannoma

  • Solitary fibrous tumor

  • Sarcoma

Fig 1, Meningioma. This postcontrast T1 MRI shows extensive growth of a bifrontal meningioma.

Fig 2, Meningioma. A typical lateral convexity meningioma is shown on the right with classic enhancement of the adjacent dura mater (“dural tail sign”).

Fig 3, Meningioma. This gross image of the brain shows a right lateral convexity meningioma that compresses the adjacent brain.

Fig 4, Meningioma. Meningiomas are typically firm, solid, well-circumscribed neoplasms that are attached to the dura ( upper right ).

Fig 5, Meningioma. Tumor cells are arranged in distinctive whorls in the most common histologic pattern (meningothelial).

Fig 6, Meningioma. Meningothelial whorls and psammomatous calcifications ( left ) are classic features of the meningothelial variant. Bland oval nuclei, some of which contain prominent clearing or intranuclear cytoplasmic inclusions, are classic features of meningiomas.

Fig 7, Meningioma. Meningiomas are immunoreactive for epithelial membrane antigen (EMA).

Fig 8, Meningioma. Immunoreactivity for progesterone receptors, as shown in this image, is observed in up to two thirds of meningiomas.

Fig 9, Meningioma. Bone invasion with hyperostotic reaction is a feature of some meningiomas.

Meningioma: Who Grade I Variants

Definition

  • World Health Organization (WHO) Grade I variants include meningothelial, fibrous (fibroblastic), transitional (mixed), psammomatous, angiomatous, microcystic, secretory, lymphoplasmacyte-rich, metaplastic

Clinical Features

  • See previous topic, Meningiomas: Overview and General Characteristics, for details

Epidemiology

  • Similar to meningothelial variant

Presentation

  • General symptoms result from location and compression of adjacent structures

  • Headache and seizures are the most common presenting symptoms

  • Psammomatous meningiomas are typically found in thoracic spinal region of middle-aged women

Prognosis and Treatment

  • Surgical excision in most cases

Pathology

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