Choroid plexus papilloma and acoustic scwhannoma

Case contributed by RMH Neuropathology
Diagnosis certain

Presentation

Right hearing loss.

Patient Data

Age: 50 years
Gender: Female

There is a homogeneously enhancing mass, measuring 14 x 6 mm, arising from  within the right internal acoustic canal. The mass expands the internal  acoustic meatus, extends along the course of the vestibulocochlear nerve and  just projects into the cerebello-pontine angle where it does not contact the  brainstem or cerebellar peduncle.  The left internal acoustic meatus is normal. 

There is a second, homogeneously enhancing lesion centered, and contained  within the boundaries, of the fourth ventricle measuring 11 x 9 x 19 mm ( AP, transverse, cc ).  It does not result in obstruction to CSF flow. The temporal horns and  lateral ventricles are not dilated. The mass returns intermediate  homogeneous T2 signal and does not exhibit restricted diffusion. No  surrounding FLAIR signal abnormality or substantial mass effect.

Deep white matter T2 hyperintensities are consistent with the patient's age. There are no remote extra axial lesions. The optic nerves and optic chiasm are unremarkable.

Conclusion:

  1. right acoustic schwannoma
  2. well-circumscribed enhancing lesion contained to the fourth ventricle.

The most likely diagnosis is that of an ependymoma, especially given the enhancement, and co-existing acoustic schwannoma which raises the possibility of neurofibromatosis type II. If these two lesions are unrelated than a subependymoma (usually little if any enhancement), hemangioblastoma (usually prominent flow-voids) or choroid plexus lesions (papilloma or even metastasis) can be considered, but are even then thought to be less likely.

 

4th ventricle mass histology

pathology

MICROSCOPIC DESCRIPTION:

The sections show a moderately cellular tumor comprising well-defined  papillary structures with fibrovascular cores. No solid areas are  seen. The papillae are lined by cuboidal epithelium which shows  occasional pseudostratification. The tumor cells have round nuclei  with even chromatin, inconspicuous nucleoli and moderate amounts of  eosinophilic cytoplasm. No mitoses, microvascular proliferation or  necrosis is seen. There are scattered spots of microcalcification in  the background. The Ki-67 index is about 2%. The tumor cells are  transthyretin, thyroglobulin, TTF-1 and BER-EP4 negative. 

DIAGNOSIS: 4th ventricle lesion: Choroid plexus papilloma (WHO grade I).

Case Discussion

Although Occam's razor is usually thought of in situations such as this, and as such finding a unifying underlying abnormality (e.g. NF2) is tempting, sometimes unrelated dual pathology is encountered, especially when one of the lesions is common. 

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