Multiple chordoid meningiomas: post-radiotherapy

Case contributed by Melbourne Uni Radiology Masters

Presentation

Headaches. Previous history of prophylactic cranial irradiation (DXT) for acute lymphoblastic leukemia (ALL) in childhood.

Patient Data

Age: 39-year-old
Gender: Female

Multiple high-density lesions in an extra-axial location are identified on both sides of the brain, the biggest ones on the left. They have a vivid contrast enhancement and, some of them, necrotic hypoattenuating central areas. The largest arises from the left sphenoid wing in the left middle cranial fossa and promotes mass effect with compression over the left lateral ventricle and mild midline shift to the right, regional edema, as well as left transtentorial herniation downward. 

Conclusion: The lesions are in keeping with multiple meningiomas which are probably secondary to  radiotherapy treatment in childhood. 

The multiple extra-axial lesions are again demonstrated, showing iso-signal on T1 and high signal on T2 compared to the adjacent brain, keeping the vivid contrast enhancement and, some of them, with a necrotic hypoattenuating central areas. The largest one arises from the left sphenoid wing in the left middle cranial fossa and promotes mass effect with compression over the left lateral ventricle and mild midline shift to the right, regional edema, as well as left transtentorial herniation downward. 

The second largest mass, arising form the parietal bone on the left demonstrates increased T2 signal and facilitated diffusion on ADC.

Conclusion: The lesions are in keeping with multiple meningiomas which are probably secondary to prior radiotherapy treatment .

Case Discussion

This patient had total body radiation for acute lymphocytic leukemia when a child.

These are radiation-induced meningiomas, which are more frequently multiple and have a very long latency.

The patient went on to have resection of the two largest left sided tumors. 

Histology

The paraffin sections show a variably hypercellular dural based  tumor.  Tumor cells are a mixture of epithelioid and spindle  morphology and are arranged in diffuse sheets and loose fasciculi. In  many areas, a chordoid appearance is also noted with solid aggregates and cords of epithelioid tumor cells dispersed within a myxomatous stroma.

Tumor cells show moderate nuclear pleomorphism and an  occasional mitotic figure is identified (1/20 HPF). No areas of necrosis are seen. There is no brain invasion.

Immunohistochemistry shows strong nuclear staining in tumor cells for  progesterone receptor (PgR) and strong membrane staining for  epithelial membrane antigen (EMA). There is also moderate staining for  bcl-2 and patchy moderate staining for smooth muscle actin (SMA). No staining for CD34, ALK-1, desmin or pancytokeratin AE1/AE3 is seen in  tumor cells.  

DIAGNOSIS: Chordoid meningioma (WHO grade II).

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Case information

rID: 38122
Published: 22nd Nov 2015
Last edited: 14th Aug 2019
Tag: rmh
Inclusion in quiz mode: Included

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