Primary cerebellar germinomas of the posterior fossa

Abstract

Primary cerebellar germinomas, in the absence of germ-cell tumours outside the nervous system or elsewhere in the cranial cavity and CSF pathways, are exceptional; only two previous cases have been reported in the literature. Two personal observations are described from our 20-year records of intra-axial posterior fossa tumours. The patients were a 32-year-old man and a 17-year-old woman with a clinical history of posterior fossa tumour, studied by computed tomography. The first patient with slight cerebellar signs had a small right hemispheric cerebellar tumour, and the other had a left cerebellar mass with hydrocephalus and progressive intracranial hypertension. Both were treated by tumour removal and irradiation to the whole posterior fossa. The survival times were 58 and 49 months, respectively. The diagnosis of primary cerebellar germinoma cannot be suspected before pathological confirmation. The clinical, neuroradiological and surgical findings are non-specific and quite similar to those of other malignant cerebellar tumours, such as anaplastic gliomas or metastases. Surgery and radiotherapy ensure adequate tumour control in the early stages; cases of recurrence or disseminated disease may be treated by irradiation and chemotherapy.

Primary pontomedullary germinoma in a 12 year old boy

“…7,8 Germinomas located in the cerebellopontine angle commonly present with facial nerve palsies. 9,10 Imaging with CT and MRI are important for diagnosis but are often unable to differentiate germinomas from other neoplastic pathologies in the brainstem. Biochemical markers, including serum and CSF a-fetoprotein and b-HCG, are useful.…”

Late recurrence of primary cerebellar germinoma at unusual site after complete response to radiotherapy

“…Only six cases were reported in previous studies, in which only three patients had isolated cerebellar germinoma, as was the case of our patient. [9] Maiuri suggested some criteria for the diagnosis of primary cerebellar germinoma, Of note, the diagnosis of primary cerebellar germinoma may not be suspected preoperatively. [9] erefore, surgery is usually indicated in primary cerebellar germinomas because of its favorable site for total resection and histopathological examination.…”

“…Besides, immunohistochemical staining may be helpful to rule out differential diagnoses in patients with unusual localizations and untypical histological features. [9] For example, pineal parenchymal neoplasms, oligodendrogliomas, central neurocytomas, malignant lymphomas, melanomas, and metastatic carcinomas do Despite high radiosensitivity, the proportion of recurrent germinoma after complete response to radiotherapy is estimated to be 6-17%. [10,11] e median time to recurrence was 30.3 months.…”

“…Only six cases were reported in previous studies, in which only three patients had isolated cerebellar germinoma, as was the case of our patient. [ 9 ] Maiuri suggested some criteria for the diagnosis of primary cerebellar germinoma, which include: (1) absence of germ cell tumors outside the nervous system; (2) absence of pineal, suprasellar, and other intracranial or spinal localization; (3) presence of single or multiple cerebellar lesions without CSF spread at the diagnosis. Of note, the diagnosis of primary cerebellar germinoma may not be suspected preoperatively.…”

“…Of note, the diagnosis of primary cerebellar germinoma may not be suspected preoperatively. [ 9 ] Therefore, surgery is usually indicated in primary cerebellar germinomas because of its favorable site for total resection and histopathological examination.…”