Dados do Trabalho
BILATERAL BASAL GANGLIA GERMINOMA IN AN ADOLESCENT PRESENTING WITH CEREBRAL HEMIATROPHY
Apresentação do caso
A previously healthy 16 year old adolescent developed subacute behavior change characterized by depressed mood. Four months later she presented with progressive proportionate right hemiparesis associated with polydipsia and polyuria symptoms. In the
following months, she started with amenorrhea, dysphagia, dysarthria and slowly progressed to important cognitive impairment. One year after the onset of symptoms, patient was admitted to our service. Her first neurological examination showed poor language, pseudobulbar affect, dysarthria and proportionate right hemiparesis with bilateral pyramidal tract signs. Brain MRI showed: periventricular bilateral infiltrative lesion, hyperintense in T2/FLAIR with heterogeneous enhancement and perilesional edema associated with marked left frontal lobe and striated nucleus hemiatrophy. Germ cell tumor of the basal ganglia was suspected. Her alpha-fetoprotein and human chorionic gonadotropin in the serum were normal and biopsy confirmed primary germinoma. She was put on chemoradiotherapy and although motor sequelae persisted, a 5 year recurrent-free condition was achieved.
Germinoma is the most common intracranial germ cell tumor and usually arises from the midline. Basal ganglia germinomas are a rare entity, representing only 5-10% of all germinomas. These tumors are usually unilateral, and bilateral occurrence is extremely rare.
It has a slow clinical course characterized by hemiparesis, neuropsychiatry symptoms, endocrinological changes, oculomotor palsy, speech or visual disturbance. The diagnosis is suggested by peculiar neuroimage findings: one third of the patients present with cerebral and/or brain stem atrophy and usually image hyperintensity is commonly seen in T1 and T2-weighted images. Hemiatrophy can be subtle and occur prior to clinical manifestation. It has been hypothesized that the phenomenon of ipsilateral cerebral hemiatrophy is caused by wallerian degeneration of efferent and afferent fibers from the basal ganglia and thalamus due to destruction of ganglia cells and nerve fibers caused by the tumor and the consequent interruption of thalamocortical connections.
We report a rare case of bilateral basal ganglia germinoma. We recommend that this entity should be suspected in cases presenting with basal ganglia focal lesion, progressive hemiparesis, behavior symptoms and ipsilateral cerebral or brain stem hemiatrophy, aiming for an early diagnosis and treatment to reduce morbimortality.
Referências (se houver)
1. Li Y, Zhang S. Hemiatrophy as the Initial Sign of Germinoma in Corpus Callosum. Neurology. 2022 Sep 27;99(13):568-569. doi: 10.1212/WNL.0000000000201096. Epub 2022 Jul 19
2. Ozelame, R.V., Shroff, M., Wood, B. et al. Basal ganglia germinoma in children with associated ipsilateral cerebral and brain stem hemiatrophy. Pediatr Radiol 36, 325–330 (2006). https://doi.org/10.1007/s00247-005-0063-4
3.Okamoto, K., Ito, J., Ishikawa, K. et al. Atrophy of the basal ganglia as the initial diagnostic sign of germinoma in the basal ganglia. Neuroradiology 44, 389–394 (2002). https://doi.org/10.1007/s00234-001-0735-1
4.Oyama N, Terae S, Saitoh S, Sudoh A, Sawamura Y, Miyasaka K. Bilateral germinoma involving the basal ganglia and cerebral white matter. AJNR Am J Neuroradiol. 2005 May
Hemiatrophy; Germinoma; Basal Ganglia
Fonte de Fomento (se houver)
Declaração de conflito de interesses de TODOS os autores
The authors declare that they have no conflict of interest.
Escola Paulista de Medicina/UNIFESP - São Paulo - Brasil
Louise Scridelli Tavares, Thais dos Santos Rohde, Mateus Pradebon Tolentino , Felipe Arthur de Almeida Jorge , Vinicius Alves Lima, Bryan da Silva Marques Cajado, Alulin Tácio Quadros Santos Monteiro Fonseca, Marcelo de Melo Aragão, Ricardo da Silva Pinho