Choroid plexus carcinoma

Case contributed by Ryan Thibodeau
Diagnosis certain

Presentation

Progressively worsening gait instability and left-sided weakness over the past several months.

Patient Data

Age: 6 years
Gender: Male
mri

There is a large hyperintense T2/FLAIR, lobulated, and frond-like mass expanding the right atrium of the lateral ventricle. The mass demonstrates avid, homogeneous enhancement and has large central flow voids. There is vasogenic edema within the right frontal and parietal lobes.

There is enlargement of the right temporal horn, right atrium and third ventricle, as well as compression of the right frontal horn and foramen of Monroe.

There is leftward midline shift. The midbrain is displaced to the left and there is mild right uncal herniation.

Case Discussion

This is a case of choroid plexus carcinoma. The patient underwent a right-sided craniotomy for the excision of the mass within the right lateral ventricle.

Pathologic analysis showed that the tumor is composed of epithelioid cells with clear cell changes and demonstrated worrisome features including:

  • largely solid, compact, hypercellular, and highly mitotically mass, with only focal areas of papillary architecture

  • significant cytologic pleomorphism

  • high p53 labeling

There was evidence suggestive of brain invasion by the tumor. No evidence of tumor necrosis. The tumor was positive for Cam-5.2, MNF-116, and P53 (>90%), with diffuse but weak staining for Syn.9

When combining the abovementioned histomorphology and stain profiles, this atypical tumor was classified as a choroid plexus carcinoma, CNS WHO grade 3.

Co-author:
Travis Bevington, MD

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