1/4. Intracranial germ cell tumors in children with and without down syndrome.PURPOSE: Two Chinese children with down syndrome affected by intracranial germ cell tumors are described. Because they represent two of eight affected patients in the current series from 1990 to 1996, it is postulated that such occurrence may be more than a coincidental event. patients AND methods: Two children with down syndrome developed germ cell tumors in atypical intracranial sites that affected basal ganglion and cerebellum. The pathology showed germinoma and yolk sac tumor, respectively. These were treated by radical surgical resection and chemotherapy with cisplatin, etoposide, and bleomycin, but without radiotherapy. RESULTS: One patient survived 3 years without radiologic evidence of tumor. The other died from infective complications caused by severe myelosuppression after chemotherapy. CONCLUSIONS: Subtle neurologic manifestations in developmentally handicapped patients with intracranial space-occupying lesions could result in delayed diagnosis. Children with down syndrome suffering from brain tumors may have a higher chance for germ cell tumors. Assay for alpha-fetoprotein and beta-human chorionic gonadotrophin could hasten diagnosis in some cases. This observation and review of literature suggest an increased risk of developing intracranial germ cell tumors in subjects with down syndrome.- - - - - - - - - - ranking = 1keywords = basal ganglion, ganglion (Clic here for more details about this article) |
2/4. Bilateral metachronous germinoma of the basal ganglia occurring long after total removal of a mature pineal teratoma: case report.OBJECTIVE AND IMPORTANCE: We report the extremely rare occurrence of a second germ cell tumor at a different site and with different histological types long after total removal of a mature pineal teratoma. CLINICAL PRESENTATION: A 10-year-old boy who presented with headache and vomiting was admitted to our hospital. Neuroradiological studies revealed a tumor in the pineal region. The tumor was totally removed. Histologically, the tumor proved to be a mature teratoma. The patient's postoperative course was uneventful. The patient received no adjuvant therapy and was followed in the outpatient clinic. Three years later, he was readmitted with transient left upper limb weakness and vomiting. Neuroradiological studies showed a tumor in the bilateral basal ganglia. INTERVENTION: The second tumor, which was located in the right basal ganglion, was partially removed for biopsy. Histologically, the tumor proved to be a germinoma. The patient received three cycles of combination chemotherapy consisting of carboplatin and etoposide with radiotherapy. After the second course of chemotherapy, magnetic resonance imaging studies revealed no evidence of the tumor. CONCLUSION: The second tumor was considered to be a de novo metachronous neoplasm rather than a recurrence of the original mature teratoma. We think that if primordial germ cell groups exist along the midline of the brain, more than two primordial germ cell groups could give rise to metachronous neoplasms at different sites and with different histological types.- - - - - - - - - - ranking = 1keywords = basal ganglion, ganglion (Clic here for more details about this article) |
3/4. germinoma of the basal ganglia and thalamus--CT and MRI findings.A case of germinoma originating in the basal ganglia and thalamus is presented. This tumour most commonly originates during childhood and adolescence, at pineal and suprasellar regions. In the early stages, the diagnosis of germinoma in the basal ganglion and thalamus is difficult because of its rarity and non-specific findings. The computed tomography (CT) and magnetic resonance imaging (MRI) findings though non-diagnostic, are discussed here. A few differential diagnoses had been discussed with radiological abnormality. Open biopsy done in this case proved to be two-cell pattern germinoma. Early detection of the tumour is desirable, since this tumour is highly sensitive to radio and chemotherapy and is potentially curable. Our patient was treated with combined chemotherapy and the response was well and no residual tumour or recurrence was seen on the repeated imaging modality, however his neurological deficits remained unchanged.- - - - - - - - - - ranking = 1keywords = basal ganglion, ganglion (Clic here for more details about this article) |
4/4. Mediastinal mature teratoma with coexistence of angiosarcoma, granulocytic sarcoma and a hematopoietic region in the tumor: a rare case of association between hematological malignancy and mediastinal germ cell tumor.An association between mediastinal germ cell tumors (MGCT) and hematological malignancies (e.g. acute leukemia, malignant histiocytosis) has been recognized since 1984. A rare case of mediastinal mature teratoma with angiosarcoma, a hematopoietic region and granulocytic sarcoma is reported in a 29-year-old male. The resected tumor was 9.0 x 6.5 cm, weighed 65 g and showed extensive necrosis, forming a cyst. The histological features of the tumor showed a mature teratoma, which contained a large gland lined by ciliated epithelium, hyalinous cartilage, a paraganglion-like structure, well-differentiated angiosarcoma with atypical hematopoiesis composed of CD34-positive cells, and malignant round cells. The malignant round cells did not stain for CD34 but were positive for leukocyte common antigen (LCA) and c-kit product. From these findings, the round cells were diagnosed as granulocytic sarcoma. The patient died of metastasis of the granulocytic sarcoma in the tonsils and cervical lymph nodes 8 months after surgery. A leukemic condition was not present throughout the clinical course. The association between MGCT and hematological malignancy is a distinctive syndrome. However, its pathogenesis is still obscure and the origin of the hematopoietic malignancy has not been fully elucidated. In this particular case, it is suggested that the granulocytic sarcoma might have arisen from the abnormal hematopoietic area in the mediastinal teratoma.- - - - - - - - - - ranking = 0.25246854210735keywords = ganglion (Clic here for more details about this article) |