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Excerpt
Intracranial germ cell tumors are a heterogeneous group of lesions that arise in patients of all ages. Germ cell tumors most frequently arise in the pineal and suprasellar region and, in general, pineal region germ cell tumors outnumber suprasellar tumors by a ratio of 2:1. Males are approximately two times more likely than females to develop germ cell tumors. The male predominance of germinomas is primarily limited to the pineal region, because suprasellar germinomas are more frequent in females. Intracranial germ cell tumors are usually diagnosed between 10 and 21 years of age. 1 Surgery is required in the majority of patients for diagnosis. 2 Radiotherapy has been the backbone of treatment for patients with intracranial germ cell tumors. Chemotherapy has only recently been integrated in the management of intracranial germ cell tumors. 3 Pineal germ cell tumors are often a cause of ventricular obstruction that necessitates a shunting procedure for relief of the increased intracranial pressure. We report the uncommon occurrence of ventriculoperitoneal shunt-associated extracranial metastasis in patient with pineal germ cell tumor.
A 23-year-old man sought treatment for headache and blurring of vision 5 years previously. Cranial magnetic resonance imaging (MRI) revealed mass in pineal gland. The patient underwent an occipital transtentorial exploration of this lesion. A subtotal decompression was performed and a ventriculoperitoneal shunt was inserted, which relieved his headache. Pathologic examination of the tumor revealed a diagnosis of pineal germ cell tumor. The patient received radiotherapy to the region of the pineal gland during the course of 5 weeks. Cranial MRI 1 month after radiation therapy showed no evidence of the tumor. Two years after radiotherapy, massive ascites developed. An abdominal computed tomography (CT) scan showed peritoneal mass, multiple liver metastases, and pelvic mass. There were nonlymphoid small round malignant cells on the cytologic examination of peritoneal fluid. Cranial MRI was normal at that time and the ventriculoperitoneal shunt was patent. Peritoneal fluid and serum beta human chorionic gonadotropin and α-fetoprotein were more than the normal levels. The patient was treated with four cycles of chemotherapy protocol consisting of Bleomycin 30 mg/d, days 1, 8, 15; etoposide 200 mg/d, days 1–3, cisplatin 35 mg/d, days 1–5, repeated every 21 days. After four cycles of therapy, abdominal CT scan and serum hormone levels were within normal limits. The patient has now been in remission for approximately 3 years.
Systemic dissemination of pineal germ cell tumor is relatively rare. With the advent of ventriculoperitoneal shunting for relief of increased intracranial pressure, tumor metastasis via that route has been described. Many case reports describing such a rare event were published in the literature. 4–10 These rare extracranial metastases may occur only in the peritoneal site or be disseminated, as in our case. Interestingly, these metastases respond well to systemic chemotherapy, as in our case. Abdominal ultrasonography should be considered in the routine follow-up of patients with intracranial germ cell tumors and ventriculoperitoneal shunts.
