Kishore, Manjari and Monappa, Vidya and Lakshmi, Rao and Kudva, Ranjini (2014) Mixed malignant germ cell tumour of third ventricle with hydrocephalus: A rare case with recurrence. Journal of Clinical and Diagnostic Research, 8 (11). FD03-FD05. ISSN 0973-709X

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Abstract

Malignant Germ Cell Tumours (GCTs) are rare, accounting for 3% of intracranial tumours and just like their extracranial counterparts represent a wide array of disease. Combination of Germinoma with Teratoma is very rare. Here in, we describe a case of Mixed Malignant Germ cell tumor of third ventricle with recurrence with emphasis on histopathological and radiological findings.channels surrounded by cytotrophoblasts and syncytiotrophoblasts were seen suggestive of choriocarcinoma [Table/Fig-4a,b].Based on the above findings a final diagnosis of Mixed Malignant Germ Cell Tumour (Immature Teratoma + Germinoma + Choriocarcinoma) was made.Three months later, the child presented with nocturnal urinary incontinence and difficulty in walking. CT scan [Table/Fig-1c&d] showed a lesion in suprasellar region with evidence of hydrocephalus. Histopathological examination of excised lesion showed tumour recurrence. Patient was advised Radiotherapy and further follow up.Manjari KiShore1, Vidya MonaPPa2, LaKShMi rao3, ranjini KudVa4Table/Fig-2ATable/Fig-2B[Table/Fig-1a,b]: Pre- operative CT Scan showing extra-axial enhancing lesion.[Table/Fig-1c,d]: CT Scan showing recurrent lesion (after 3 months of complete surgical clearance)[Table/Fig-2a]: Monomorphic large germ cells representing Germinoma (H&E: 200X)[Table/Fig-2b]: Atypical mitotic figure seen (H&E: 400X)DISCUSSIONPediatric Germ Cell Tumors involving CNS are uncommon. Primary involvement of the CNS occurs in around 18% cases, with metastatic involvement being more common [1]. Just like their extra-cranial counterparts, they present a wide array of diseases [2,3]. They may be seen from birth and throughout childhood with varying incidence depending upon patient’s age and gender. They arise primarily in two locations in the center of brain, the suprasellar in 40% cases and pineal region in 50% cases. 5-10% cases have both suprasellar and pineal gland tumour called as doublet lesions [2-4].These tumours are considered to arise from nests of embryonic cells (rests) located in the midline, both intra-cranially as well as extra-cranially[5]. Malignant GCTs are classified in similar way as their gonadal counterparts, i.e. germinoma, (being the most common), followed by teratoma and a number of Non-germinomatous tumours including embryonal Carcinoma, Choriocarcinoma, endodermal sinus tumours & combination of these tumours have been reported.

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