Rhabdoid meningioma

A.Prof Frank Gaillard and Dr Azza Elgendy et al.

Rhabdoid meningioma is a rare and aggressive subtype of meningioma, it is classified as WHO grade III. Rhabdoid morphology is associated with a poor prognosis, regardless of tumour histogenesis. It resembles other types of rhabdoid tumors with great tendency for recurrence 1-2 . Extensive necrosis in rhabdoid meningioma might be indicative of an additional predictor of aggressive clinical behavior 3.

It occurs in middle age group approximately 10 years earlier than typical meningioma. Like benign meningioma there is a slight increased predominance in females and in African Americans 2

The presentation depends on the location of the lesion. Headache, seizures, visual manifestations, and focal neurological deficits have been described.

The term rhabdoid morphology is referring to the characteristic resemblance of the cells to a rhabdomyoblast without true skeletal muscle differentiation. The term malignant rhabdoid tumour (MRT) was first used to describe a distinctive paediatric renal tumor, the term was applied to similar extra renal paediatric tumours. Rhabdoid cells show abundant eosinophilic cytoplasm, cytoplasmic hyaline inclusions, and eccentric nuclei 4

Grading

Generally, classified as WHO III malignant meningioma.

Rhabdoid meningioma tends to have cystic and solid components with homogenous enhancement of the solid component. There is extensive peri-tumoral oedema 2. Bone destruction, indistinct margins and central necrosis are known to correlate with aggressiveness. 

MRI

Signal characteristics that correlate with aggressive behaviour of meningiomas include:

  • T1: indistinct tumor border (saw teeth appearance of the margins) and marked mass effect
  • T2/FLAIR: marked peritumoral edema
  • DWI/ADC: hyperintense signals on DWI that show restricted diffusion on ADC correlate with hypercellularity and more aggressive behaviour
  • T1 C+ (Gd): avid enhancement that may extend into the bone if associated with bone destruction
  • MR spectroscopy: elevated level of alanine at short TE

The tumours has a tendency for recurrence and has a poor prognosis.

 

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Article information

rID: 33829
Section: Pathology
Synonyms or Alternate Spellings:
  • Malignant meningioma with rhabdoid features
  • Rhabdoid neningiomas

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