Management of hydrocephalus in a pediatric bifocal germinoma

Germinomas represent the most frequent tumour type in the pineal region. However, the synchronous involvement of the suprasellar region, frequently termed bifocal intracranial germinoma (BFG) is quite rare. A literature search identified a further of 48 pediatric cases with a median age of 12.9 year...

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Autores principales: Ricardo Santos de Oliveira, Rodrigo Inácio Pongeluppi, Elvis Terci Valera
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Lenguaje:EN
Publicado: Brazilian Society for Pediatric Neurosurgery 2020
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spelling oai:doaj.org-article:af0b536340d340bcb2f4f2d0338b2b582021-11-23T21:46:37ZManagement of hydrocephalus in a pediatric bifocal germinoma2675-362610.29327/apn.v2i1(January-April).22https://doaj.org/article/af0b536340d340bcb2f4f2d0338b2b582020-04-01T00:00:00Zhttps://www.archpedneurosurg.com.br/pkp/index.php/sbnped2019/article/view/22https://doaj.org/toc/2675-3626Germinomas represent the most frequent tumour type in the pineal region. However, the synchronous involvement of the suprasellar region, frequently termed bifocal intracranial germinoma (BFG) is quite rare. A literature search identified a further of 48 pediatric cases with a median age of 12.9 years. BFG is known to be highly radiosensitive. We presented a clinical case of pediatric bifocal germinoma. A 14-year-old boy presented with clinical features of progressive headaches since three months associated with vomits. MR showed two distinct lesions in the pineal and suprasellar regions. Neuroaxis spine MR was normal. Tumor markers for germ cell tumors from blood and cerebrospinal fluid were within the normal range. An endoscopic procedure was performed to treat the hydrocephalus (endoscopic third ventriculostomy) and, in the same procedure the suprasellar lesion was biopsied and the histology revealed a germinoma. She received chemotherapy and a focal radiotherapy covering the sites of primary disease and the ventricular system. Three months after the completion of treatment, the patient achieved a complete clinical and radiological response, which was maintained during the 5-years follow-up period. The optimum management of primary intracranial pineal and suprasellar (bifocal) germinomas still remains controversial. The definition as either localized or disseminated disease has major implications on required treatment and its associated late morbidity. Prospective studies with large cooperative groups worldwide and consensus meetings may reduce uncertainties regarding available management options.Ricardo Santos de OliveiraRodrigo Inácio PongeluppiElvis Terci ValeraBrazilian Society for Pediatric NeurosurgeryarticlehydrocephalustumorgerminomaPediatricsRJ1-570SurgeryRD1-811ENArchives of Pediatric Neurosurgery, Vol 2, Iss 1(January-April), Pp 55-55 (2020)
institution DOAJ
collection DOAJ
language EN
topic hydrocephalus
tumor
germinoma
Pediatrics
RJ1-570
Surgery
RD1-811
spellingShingle hydrocephalus
tumor
germinoma
Pediatrics
RJ1-570
Surgery
RD1-811
Ricardo Santos de Oliveira
Rodrigo Inácio Pongeluppi
Elvis Terci Valera
Management of hydrocephalus in a pediatric bifocal germinoma
description Germinomas represent the most frequent tumour type in the pineal region. However, the synchronous involvement of the suprasellar region, frequently termed bifocal intracranial germinoma (BFG) is quite rare. A literature search identified a further of 48 pediatric cases with a median age of 12.9 years. BFG is known to be highly radiosensitive. We presented a clinical case of pediatric bifocal germinoma. A 14-year-old boy presented with clinical features of progressive headaches since three months associated with vomits. MR showed two distinct lesions in the pineal and suprasellar regions. Neuroaxis spine MR was normal. Tumor markers for germ cell tumors from blood and cerebrospinal fluid were within the normal range. An endoscopic procedure was performed to treat the hydrocephalus (endoscopic third ventriculostomy) and, in the same procedure the suprasellar lesion was biopsied and the histology revealed a germinoma. She received chemotherapy and a focal radiotherapy covering the sites of primary disease and the ventricular system. Three months after the completion of treatment, the patient achieved a complete clinical and radiological response, which was maintained during the 5-years follow-up period. The optimum management of primary intracranial pineal and suprasellar (bifocal) germinomas still remains controversial. The definition as either localized or disseminated disease has major implications on required treatment and its associated late morbidity. Prospective studies with large cooperative groups worldwide and consensus meetings may reduce uncertainties regarding available management options.
format article
author Ricardo Santos de Oliveira
Rodrigo Inácio Pongeluppi
Elvis Terci Valera
author_facet Ricardo Santos de Oliveira
Rodrigo Inácio Pongeluppi
Elvis Terci Valera
author_sort Ricardo Santos de Oliveira
title Management of hydrocephalus in a pediatric bifocal germinoma
title_short Management of hydrocephalus in a pediatric bifocal germinoma
title_full Management of hydrocephalus in a pediatric bifocal germinoma
title_fullStr Management of hydrocephalus in a pediatric bifocal germinoma
title_full_unstemmed Management of hydrocephalus in a pediatric bifocal germinoma
title_sort management of hydrocephalus in a pediatric bifocal germinoma
publisher Brazilian Society for Pediatric Neurosurgery
publishDate 2020
url https://doaj.org/article/af0b536340d340bcb2f4f2d0338b2b58
work_keys_str_mv AT ricardosantosdeoliveira managementofhydrocephalusinapediatricbifocalgerminoma
AT rodrigoinaciopongeluppi managementofhydrocephalusinapediatricbifocalgerminoma
AT elvistercivalera managementofhydrocephalusinapediatricbifocalgerminoma
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