Central neurocytoma

Case contributed by Antonio Rodrigues de Aguiar Neto
Diagnosis certain

Presentation

This patient came to the emergency department complaining of a headache that started three months ago and evolved with progressive worsening, associated with ocular pain and reduced visual acuity.

Patient Data

Age: 20 years.
Gender: Female

Subtraction

mri

MR images of the brain, including post-contrast sequences, demonstrate a large lobulated intraventricular mass filling and expanding the right body of the lateral ventricle, with a broad contact to the right lateral margin of the septum pellucidum, which is bowed to the left. The mass has a low T1 signal, heterogenous hyperintense T2 signal with small cysts, and focal areas with low signal on the SWI sequence, which may represent calcifications or blood products. There is a slight restriction on DWI / ADC and faint irregular contrast enhancement. There is dilatation of the lateral ventricles, notably the right, without transependymal cerebrospinal fluid permeation.

Impression: The findings are consistent with an intraventricular tumor, favoring the diagnosis of a central neurocytoma. 

The patient underwent a craniotomy and excision of the intraventricular mass.

pathology

Histology report:

Microscopic pathologic examination reveals that the tumor consists of small round cells and uniform nuclei with a finely granular chromatin pattern. Many cells have a perinuclear halo and a prominent clear and lightly eosinophilic granular cytoplasm. Neither pleomorfism nor mitosis was present. There is no vascular proliferation or necrosis. 

Immunohistochemistry shows that tumor cells are positive for neuron-specific enolase and synaptophysin. 

Conclusion: The features are those of a central neurocytoma (WHO Grade II).

mri

Post-surgical follow-up MRI study performed without contrast administration revealed evidence of right craniotomy and extensive resection of the intraventricular tumor.

Case Discussion

Central neurocytomas are rare well-differentiated tumors that typically develop in the lateral ventricles and usually occur in young adults 1-6. This case demonstrates typical appearances of a large central neurocytoma with histopathologic confirmation.

Case courtesy

  • Patrícia Menandro, MD - PGY-3, radiology resident, Department of Radiology

  • Luciana de Oliveira Borges, MD – pathologist, Department of Pathology

  • Fernanda Lima Didjurgeit, MD – pathologist, Department of Pathology

  • Antonio Rodrigues de Aguiar Neto, MD - radiologist, Department of Radiology

Hospital da Restauração – Recife, PE – Brazil

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