Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Several clinical trials have demonstrated that cisplatin-based chemotherapy for primary intracranial germ-cell tumors is effective as a neoadjuvant chemotherapy. In this report, we describe a 6-year-old boy, Down syndrome and Hirschsprung's disease with intracranial pure yolk sac tumor treated by combined chemotherapy with cisplatin, vinblastine, bleomycin and cyclophosphamide (modified VAB-6 regimen). He had been admitted to our hospital because of intractable vomiting, and left facial nerve palsy since 1 month before. An MRI revealed an enlarged mass, 4cm in diameter, in the left cerebello-pontine angle with uniformal enhancement by Gd-DTPA, and bilateral ventricular dilatation. He was found to have increased serum alpha-fetoprotein level (AFP 11, 786ng/ml), but not human chorionic gonadotropin beta-subunit. After a partial resection of the tumor, diagnosed as pure yolk sac tumor, and ventriculo-peritoneal shunt, three courses of combined chemotherapy with cisplatin, bleomycin, vinblastine and cyclophosphamide (modified VAB-6 therapy) were carried out. The serum AFP level returned to normal, and the tumor mass entirely disappeared (a complete response) on MRI after the second course of chemotherapy. However, cisplatin-induced vomiting and mild neutropenia and renal tubular injury developed after the third course of chemotherapy. Irrespective of administration of recombinant human G-CSF and broad spectrum antibiotics, he suffered from pneumonia and died of septic shock and multiple organ failure. Autopsy showed microscopic residual tumors. The combination chemotherapy with cisplatin, bleomycin, vinblastine and cyclophosphamide is effective for initial treatment of childhood intracranial yolk sac tumor. It is necessary, however, to reevaluate the cisplatin dosage and treatment schedule in order to reduce such side effects as bone marrow suppression and renal damage.
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PMID:[A case report of a 6-year-old boy with intracranial yolk sac tumor treated by VAB-6 regimen]. 753 Dec 96

Cases of embryonal carcinoma arising in the basal ganglia are rarely reported. According to the literature available, only 5 cases of embryonal carcinoma, arising from the basal ganglia, have been reported to date. This paper reports one such case we recently encountered. The patient was a 15-year-old boy. He was first admitted to another hospital because of occasional headache and vomiting. During the hospital stay, CT scans revealed abnormalities. For this reason, the patient was referred to our critical care center. Upon admission to our center, a physical examination revealed no abnormalities, but neurological examination disclosed left hemiparesis. CT scans revealed a large mass lesion of a low to high density in the right basal ganglia, accompanied by midline shift and ventricular dilatation. Elevation of human chorionic gonadotrophin (HCG) and alpha-fetoprotein (AFP) in both serum and cerebrospinal fluid (CSF) was observed. The tumor with multiple cysts was removed totally by craniotomy. The removed tissue was rated histopathologically as mixed-type germ cell tumor composed of germinoma and embryonal carcinoma. The removed tumor cells were found immunohistologically to contain HCG and AFP. Postoperative CT scans showed complete disappearance of the tumor. Taking into account a recent report that a combined cisplatin and etoposide therapy (PE chemotherapy) was effective in treating intracranial germ cell tumors, we used this chemotherapy for postoperative management of this patient. The patient underwent 3 cycles of PE chemotherapy during the 3 months after surgery. The elevated HCG and AFP levels in serum and CSF returned to their normal range within 2 months after surgery. CT and tumor markers revealed no signs of recurrence.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cisplatin-etoposide chemotherapy of an embryonal carcinoma arising in the basal ganglia of the cerebrum: a case report]. 807 94

Since its introduction in the neurosurgical field, neuroendoscopy has played an important role in the treatment of pineal germ cell tumors. The authors report a case of pineal germinoma revealing spontaneous regression before neuroendoscopic surgery. A 15-year-old boy presented with chronic headache, deterioration of his visual acuity, and diplopia. Head magnetic resonance imaging (MRI) revealed a large tumor in the pineal region and triventricular hydrocephalus due to occlusion of the aqueduct. As levels of tumor markers in serum and cerebrospinal fluid, including those of alpha-fetoprotein, carcinoembryonic antigen, human chorionic gonadotropin (hCG), and the beta-subunit of hCG, were all within the normal ranges, endoscopic surgery for biopsy and third ventriculocisternostomy was performed. Under endoscopic view, the tumor was revealed to be smaller than expected from preoperative images. MRI obtained 14 days after admission disclosed marked shrinkage of the tumor. The histopathological diagnosis was pure germinoma, and the patient underwent chemotherapy combined with fractionated radiotherapy. The tumor disappeared, and the patient did not show any signs of relapse. Spontaneous regression is a very rare phenomenon in malignant brain tumors, and in the literature it has been uncommonly described in pineal germ cell tumors. Our case disclosed a pitfall of modern strategy for this pathology with neuroendoscopy. Dilated ventricles and a certain amount of lesion are factors for safe endoscopic biopsy of pineal tumors. Radiographic images shortly before surgery are warranted, especially in patients whose symptoms of intracranial hypertension have already improved before surgery.
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PMID:Spontaneous regression of germinoma in the pineal region before endoscopic surgery: a pitfall of modern strategy for pineal germ cell tumors. 2094 6