A rare case of intraventricular gangliocytoma

A 49-year-old man presented to the emergency department after cranioencephalic trauma with an intraventricular tumor detected in the computed tomography (CT) scan ([Figure 1]). An magnetic resonance image (MRI) showed a heterogeneous expansive lesion with enhancing solid components and peripheral cysts located in the left lateral ventricle ([Figure 1]). The patient underwent excision of the lesion. Histopathologic ([Figure 2]) and immunohistochemical ([Figure 3]) analysis revealed the diagnosis of gangliocytoma. Gangliocytomas are rare low-grade central nervous system tumors composed of dysplastic ganglion cells, usually presenting in children or young adults and located in the cerebral hemispheres[1],[2]. Until now, there are no case reports of intraventricular gangliocytoma.

Figure 1 Coronal (A) and axial (B) nonenhanced brain CT shows a solid and cystic lesion within the lateral left ventricle and infiltrating adjacent white matter. Coronal and axial T2 (C, D), axial FLAIR (E) shows a well-demarcated, isointense and heterogeneous lesion with predominantly peripheral cysts located within the left lateral ventricle with infiltration of its lateral wall and the septum pellucidum. Sagittal and axial post-contrast T1 (F, G) sequence shows intense enhancement of the solid portion and increased rCBV on DSC perfusion (H).FLAIR: fluid attenuated inversion recovery; rCBV: relative cerebral blood volume; DSC: differential scanning calorimetry.

Figure 2 Clusters of atypical pleomorphic ganglion cells embedded in a haphazard manner within a delicate neuropil matrix. No neoplastic glial cells are present.

Figure 3 Immunohistochemical (IHC) studies. Glial fibrillary acidic protein (GFAP) positive in normal astrocytes with positive ATRX and negative isocitrate dehydrogenase. Neurofilament (NF) and synaptophysin (SYN) were positive in the neuropil. Ki-67 (Ki67), which determines the proliferative index, was low (< 2%).

Conflict of Interest

There is no conflict of interest to declare.

Authors’ contributions:

JAPC, LTD: JNS, FCK, LFF: manuscript composition; LATT, MLD: manuscript review.

• REFERENCES

• 1 Koeller KK, Henry JM. From the archives of the AFIP: superficial gliomas: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics 2001; 2 (06) 1533-1556 https://doi.org/10.1148/radiographics.21.6.g01nv051533
• 2 Li X, Guo L, Sheng S, Xu Y, Ma L, Xiao X. et al. Diagnostic value of six MRI features for central neurocytoma. Eur Radiol 2018; 28 (10) 4306-4313 https://doi.org/10.1007/s00330-018-5442-y

Publication History

Received: 03 July 2021

Accepted: 08 August 2021

Article published online:
30 January 2023