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On-line version ISSN 1678-4227
Arq. Neuro-Psiquiatr. vol.67 no.1 São Paulo Mar. 2009
Multiple midline intracranial germinoma managed by neuroendoscopy
Germinoma intracraniano múltiplo tratado por neuroendoscopia
Joacil Carlos da SilvaI; Frederico de Melo Tavares de LimaI; Igor Vilela FaquiniI; Leonardo Ferraz CostaI; Marcelo Moraes ValençaII, Roberto José Vieira de MelloIII
IDepartment of Neurological Surgery, Restauração Hospital, Recife PE, Brazil
IIDepartment of Neurological Surgery, Federal University of Pernambuco, Recife PE, Brazil
IIIDepartment of Pathology, Federal University of Pernambuco, Recife PE, Brazil
Germinomas belong to the class of germ cell tumors that also comprise embryonal cell carcinoma, yolk sac tumor, teratoma (mature and immature) and choriocarcinoma. Extragonadal germ cell tumors typically arise in midline locations. The most common sites of origin in adults are the anterior mediastinum, retroperitoneum and the pineal and suprasellar regions of the brain1. Multiple primary intracranial germ cell tumors are uncommon. Only 510% of all germinomas are found as synchronous lesion in pineal and suprasellar region1. The cerebellar location is extremely rare with nine cases previously reported2.
The simultaneous midline triple location (pineal, suprasellar and cerebellum) was never documented1-6.
A 17-years-old male presented with anorexia, ataxia and paresis of upward gaze during a period of two months. Symptoms worsened one week before admission with intracranial hypertension signs and altered awareness. Neurological examination revealed papilledema and there was restriction of upward gaze and convergent nystagmus on attempted upgaze (Parinaud's syndrome). The patient had normal mental development and secondary sexual characters.
Magnetic resonance imaging (MRI) demonstrated obstructive hydrocephalus and three midline contrast-enhancing lesions located at pineal, suprasellar region and posterior fossa (Figure) There were no spine lesions. Cerebrospinal fluid examination was normal without atypical cells. The tumor markers human chorionic gonadotropin and α-fetoprotein in serum and cerebrospinal fluid had normal values.
Endoscopic third-ventriculostomy was performed and during the same procedure the suprasellar tumour was partially resected. Histological examination of the tissue was compatible with germinoma. The patient received radiotherapy with an excellent outcome result.
There is always controversy about the therapeutic modalities of intracranial germ cell tumors. Some neurosurgeons insist that histological verification is crucial before any treatment. On the other hand, the so-called diagnostic or blind radiotherapy is also advocated as a safer option1.
This more conservative approach was especially favored by the Japanese, because the high preponderance of radiosensitive germinomas in that population provided support for this rationale7,8. Unfortunately, this strategy can lead to unnecessary and potentially harmful radiation for many patients with benign and radiation-resistant tumors9.
Tissue diagnosis can be obtained by a stereotactic biopsy, craniotomy or neuroendoscopy. The approach is influenced by clinical and radiographic features and the surgeon's degree of experience with the procedures7. The obvious advantage of open resection is the ability to obtain larger amounts of tissue to histological analysis. The clinical advantages of tumor debulking are less apparent with malignant tumors like the germinoma1,7.
The relative technical ease of performing stereotactic biopsy procedures should be viewed cautiously with regard to pineal region lesions. There is an increased risk or hemorrhage from several mechanisms, including bleeding in highly vascular tumors or damage to the deep venous system with catastrophic intraventricular hemorrhage and acute hydrocephalus.10
Endoscopic biopsy through the ventricles has been reported as an alternative method of securing a tissue diagnosis and typically this procedure is combined with a third-ventriculostomy. Diagnostic sensitivity for endoscopic biopsy is reported as 75%11.
Neuroendoscopy is a safe and minimally invasive approach to these lesions. The possibility of direct hemostasis with bipolar or monopolar coagulation gives a huge superiority to stereotactic biopsy. Neuroendoscopic procedures can permit a precise histological diagnosis of intracranial germinomas and are also cost-effective in the management of hydrocephalus avoiding a more expensive shunt procedure.
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Received 31 July 2008, received in final form 3 October 2008. Accepted 13 November 2008.
Dr. Joacil Carlos da Silva Rua Agenor Lopes 424 / 701 - 51021-110 Recife PE - Brasil. E-mail: firstname.lastname@example.org