Rhabdoid meningioma

Case contributed by Dr Bruno Di Muzio

Presentation

Recurrence of a previously resected brain tumour.

Patient Data

Age: 64-year-old

MRI Brain (first presentation) - 7 years ago

Modality: MRI

Overlying the left frontoparietal region there is a large extra-axial lesion that is slightly hypointense relative to grey matter on T1 and iso to hyperintense to grey matter on T2. There is mass effect with compression of the underlying sulci and a small contralateral midline shift. The lesion demonstrates moderate but slightly heterogeneous contrast enhancement. There is also dural enhancement and dural tail. Minimal subjacent white matter T2 hyperintensity but no significant vasogenic oedema. Medially the mass abuts the superior sagittal sinus which is not occluded or overtly invaded. Several prominent flow voids are noted within the mass. No invasion of the overlying bone and no significant hyperostosis. Incidental left middle cerebellar peduncle developmental venous anomaly. Nonspecific high T2 signal foci are noted in the white matter. The midline structures are unremarkable.

CT Stereotaxis

Modality: CT

Frameless post-contrast CT brain performed to assist surgical approach. The large left frontoparietal vivid enhancing mass consistent with meningioma again noted.

MRI Brain (current presentation)

Modality: MRI

Since the previous study (not shown), there has been unequivocal disease progression. Once again, there is a combination of gliosis and vasogenic oedema. There is a narrowing of the superior sagittal sinus but maintained flow voids are noted, unchanged compared to previously. This is well demonstrated on the axial T2 imaging. Susceptibility artefact is once again noted in the surgical bed. There is no further enhancing lesion noted.

Histology (latest surgical resection)

Modality: Pathology

MICROSCOPIC DESCRIPTION: 1&2. Paraffin sections show a dural-based densely hypercellular meningioma. Tumour cells have a diffuse sheeted arrangement and the majority have rhabdoid cytological features - strap-like and epithelioid cells with pleomorphic round and oval vesicular nuclei and a large amount of densely eosinophilic cytoplasm. Frequent mitotic figures are identified (14 in 20HPF). Several microscopic foci of necrosis are noted. There is no evidence of brain invasion. Immunohistochemistry shows patchy moderate membrane staining in tumour cells for epithelial membrane antigen (EMA). No nuclear staining for progesterone receptor (PgR) is seen. The topoisomerase labelling index is approximately 35%. The features are of rhabdoid meningioma (WHO Grade III).

DIAGNOSIS: 1&2. Brain tissue: Rhabdoid meningioma (WHO Grade III).

Case Discussion

This case illustrates an aggressive histological subtype of meningiomarhabdoid meningioma, which is classified as WHO grade III. Rhabdoid refers to the characteristic resemblance of the cells to a rhabdomyoblast without true skeletal muscle differentiation. It is not possible to differentiate this subtype on imaging. As it has been showed in this case, this subtype has a tendency for recurrence.

 

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Case Information

rID: 46145
Case created: 23rd Jun 2016
Last edited: 23rd Jun 2016
Inclusion in quiz mode: Included

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