Primary germinoma of the posterior fossa with CSF and extracranial metastases.

We describe the case of a 1-year-old boy presenting clinically and radiologically with a haematoma occurring in the cerebellum. Histological examination revealed a germ cell tumour with a pure germinomatous component. The patient subsequently succumbed with extensive tumour metastases in the spinal cord and the subcutaneous tissues of the scalp. This represents the first comprehensive documented report of a germinoma arising in the posterior fossa.

Advanced ovarian dysgerminoma with cure of tumor persistent in meninges.

Isolated meningeal recurrence of ovarian cancer is uncommon. It is generally assumed that such cases are not accompanied by prolonged survival. We report the cure of a patient with advanced ovarian dysgerminoma who developed febrile carcinomatous meningitis 2 weeks after receiving her fifth course of combination chemotherapy (5 months after initiation of chemotherapy). No parenchymal brain disease was identified. The persistence of disease in the leptomeninges is related to the ability of the blood-brain barrier to exclude chemotherapeutic agents. The patient responded to craniospinal radiation and remains free of disease 2 years after completion of treatment.

Placental alkaline phosphatase as a tumor marker for primary intracranial germinoma.

A sensitive enzyme-linked immunosorbent assay (ELISA) was used in a retrospective study of placental alkaline phosphatase (PLAP) levels in serum, cerebrospinal fluid (CSF), and intratumoral cyst fluid in primary intracranial germinoma. The ELISA showed no cross-reactivity with intestinal alkaline phosphatase except in very high concentrations, after samples had been heat-treated. Three patients with germinoma were studied for serum PLAP levels and in all the levels were elevated (3.78, 0.52, and 2.11 IU/liter). Two of the germinoma patients were studied for PLAP levels in the CSF, and both had elevated levels (0.83 and 9.83 IU/liter). The intratumoral cyst fluid in one case of germinoma was tested for PLAP and the level was found to be very high (603 IU/liter). These PLAP levels decreased concomitantly with the reduction in tumor size during irradiation. Serum PLAP levels were measured in 40 control adult male individuals and in the CSF of 20 nonpregnant patients with subarachnoid hemorrhage. The upper normal limits were 0.20 and 0.11 IU/liter in the serum and the CSF, respectively. All PLAP levels measured in the serum of patients with various brain tumors were 0.18 IU/liter or less. This study strongly suggests that PLAP is a clinically useful tumor marker for primary intracranial germinoma.

Pineal region tumors in children.

The authors believe that the preferred treatment for pineal region tumors in children requires definitive surgery with a histological diagnosis and that a conservative approach consisting of shunting and radiation therapy no longer seems to be appropriate. The results are reported of a retrospective review of the presentation, treatment, and outcome of 36 children under the age of 18 years treated between 1974 and 1986. Eleven children had germinomas (two-cell type), seven had astrocytomas, and the remaining 18 had 15 histologically different tumor types. Surgery was performed on 30 patients; there were no deaths, but a 10% rate of persistent morbidity was found. The median follow-up period was 4 years. Nine (82%) of 11 patients with germinomas are alive without evidence of recurrence; one child died from recurrent tumor in the pineal region and another is presently being treated for recurrent tumor of the spinal cord. Six (86%) of the seven patients with astrocytoma are well after biopsy and radiation therapy. Of the remaining 18 children, five (28%) died from tumor progression. The cerebrospinal fluid (CSF) tumor markers alpha-fetoprotein and beta-human chorionic gonadotropin were helpful in determining the presence of malignant germ-cell tumors, particularly those with a poor prognosis. Magnetic resonance imaging was useful for diagnosis and for planning the operative approach. Magnetic resonance images showed the presence of pineal region tumors in four children with hydrocephalus who had no evidence of tumor on computerized tomography scans. Because the great variety of tumor types found in the pineal region must be treated in different ways and because improved microsurgical and stereotaxic surgical techniques have made mortality and morbidity rates acceptably low, a biopsy diagnosis should be obtained in all patients. Preoperative assessment of CSF tumor markers and cytology is useful for the identification of patients who have a poor prognosis.

Pineal germinoma: diagnosis, treatment and tumor response.

We report the case of pineal germinoma in a 25 year old man. The neurological signs and symptoms were insidious in onset; Parinaud syndrome allowed a topical diagnosis, confirmed by CT scan. Computerized Tomography and CSF cytological examination were of the utmost importance to diagnosis and treatment and it confirmed the success of radiotherapy.

Primary germinoma of the brain. Immunocytochemical demonstration of tumour cells in the cerebrospinal fluid.

A patient is presented, who developed a suprasellar tumour. Stereotactical biopsy of the tumour revealed the diagnosis of a dysgerminoma. Immunocytochemical examination of the CSF showed neoplastic cells staining for human chorionic gonadotropin and for alpha-fetoprotein. The authors stress the possibility to diagnose primary intracranial germ cell tumours without operation.

Presence of growth hormone-releasing factor-like immunoreactivity in human cerebrospinal fluid.

Immunoreactive human growth hormone-releasing factor (I-hGRF) in human cerebrospinal fluid (CSF) was measured by radioimmunoassay using antiserum specific to the C-terminal portion of hGRF(1-44)NH2. Dilution curves of I-hGRF in the CSF were completely parallel to that of synthetic hGRF(1-44)NH2 standard. On Sephadex G-50 column chromatography a single peak of I-hGRF in the CSF was eluted at the position of synthetic hGRF(1-44)NH2. I-hGRF was present in the CSF of all control patients without any endocrine disease (mean +/- SE, 29.3 +/- 2.0 pg/ml) whereas I-hGRF in the CSF was not detectable (less than 5.8 pg/ml) in any of the patients with hypothalamic germinoma. In all patients with idiopathic GH deficiency, I-hGRF in the CSF was measurable but its concentration (15.1 +/- 1.0 pg/ml) was significantly (p less than 0.05) lower than that in the control subjects. No difference in I-hGRF levels of the CSF was observed between patients with acromegaly and control subjects. These findings demonstrate for the first time that I-hGRF is present in human CSF. Measurement of I-hGRF in the CSF may be useful for understanding the pathophysiology of hypothalamo-pituitary diseases.

[Germ cell tumor in the basal ganglia with elevated serum and CSF alpha-fetoprotein levels].

Intracranial germ cell tumors usually locate either in the pineal region or in the suprasellar region or both. Primary occurrence of germ cell tumor in the basal ganglia is rare. To our knowledge, only 17 cases have been reported in the literatures, and all the patients were Japanese. None of these patients presented elevated serum and CSF AFP levels in the literatures. We are reporting a patient with a germ cell tumor which developed in the left basal ganglia and showed high serum and CSF alpha-fetoprotein (AFP) levels. The patient was a 12-year-old boy who had been completely well until five months prior to the first admission when he developed weakness of right extremities. On admission he showed a right hemiparesis of mild degree and right hypesthesia. CT-scans demonstrated a homogeneous high density area over the caudate head and the putamen on the left side. The anterior limb of the left internal capsule and a part of the posterior limb were also involved. The lesion was moderately enhanced by the contrast medium. Stereotaxic biopsy of the lesion was uncontributory and he was discharged for follow up. Five months later, CT-scans demonstrated the enlargement of the high density area with perifocal edema. Then we performed a craniotomy, and the tumor which was soft and dark red in colour was biopsied at the wall of the anterior horn of the left lateral ventricle. Histopathology of the specimen showed germinoma of two-cell pattern.(ABSTRACT TRUNCATED AT 250 WORDS)

Alphafetoprotein and human chorionic gonadotropin determination in cerebrospinal fluid. An aid to the diagnosis and management of intracranial germ-cell tumors.

The cerebrospinal fluid (CSF) and serum of six patients with histologically verified intracranial germ-cell tumors were assayed serially for the presence of alphafetoprotein (AFP) and the beta subunit of human chorionic gonadotropin (HCG). Two patients had embryonal carcinomas, two had choriocarcinomas, and two had dysgerminomas. The marker profile for a given tumor in either CSF or serum correlated with the histological diagnosis; that is, embryonal carcinoma produced AFP and HCG, choriocarcinoma produced HCG, and dysgerminoma produced no markers. The marker levels in serum and CSF declined with therapy and rose usually prior to the development of overt clinical symptoms if the patient's tumor recurred. A CSF-to-serum gradient of the marker levels was present in three of four patients, and the serum levels were often normal when the CSF values were elevated. Ventricular marker levels were lower than the lumbar levels in two of two patients. The assay of these biological markers is a sensitive indicator of the success of therapy, and the presence of a CSF-to-serum gradient suggests that the major portion of the neoplasm rests within the central nervous system. A histological diagnosis can be inferred without the necessity of surgery in appropriate clinical contexts.