1/7. Congenital intracranial teratoma.Congenital intracranial teratoma is a rare disease. A fetus with a congenital intracranial teratoma presenting with a disproportionately enlarged head at 27 weeks gestation is presented. Prenatal ultrasonography and fetal magnetic resonance imaging demonstrate a huge, heterogenous intracranial mass in the left supratentorial region, with the left cerebral hemisphere being compressed and flattened. The infant died of respiratory failure within 24 hours of birth at 28 weeks gestation. On postmortem examination the histologic report revealed an immature teratoma. Fetal MRI is helpful in the prenatal diagnosis and evaluation of intracranial tumor.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Malignant struma ovarii: report of a case and review of the literature.Malignant struma ovarii is a very rare disease and therefore there is neither common agreement on treatment regimens nor sufficient follow-up experience. The case of a 38-year-old woman with malignant struma ovarii is described. The patient presented with a clinically silent ovarian neoplasm discovered incidentally during investigations for metrorrhagia. The ovarian mass was resected and the tumor was found to be a teratoma. Within the teratoma a papillary thyroid carcinoma of the follicular variant was found. Subsequently, in order to make follow-up of the patient possible by thyroglobulin measurement and radioiodine whole body scintigraphy, near-total thyroidectomy was performed and the thyroid was found to be normal on histology. Whole body radioiodine scintigraphy with (131)I and ablation of the thyroid remnant by the administration of 80 mCi (131)I was performed. The patient recovered uneventfully and is now well.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Paraneoplastic limbic encephalitis masquerading as chronic behavioural disturbance in an adolescent girl.AIM: To describe an unusual but treatable cause of behavioural disturbance in adolescence. methods: The case is reported of a 15-y-old girl presenting with acute confusion, memory problems and psychotic symptoms following an 18-mo history of change in personality, school failure and running away from home. A review of the literature is also presented. RESULTS: microbiology, toxicology, computed tomography and magnetic resonance imaging did not show any pathology of the central nervous system. Bilateral ovarian immature teratomas were eventually diagnosed and removed. The rapid improvement in the patient's mental and cognitive functions after corticosteroid treatment and the abnormality shown on the single photon emission computed tomography suggested a diagnosis of paraneoplastic limbic encephalitis. CONCLUSION: Paediatricians and neurologists should be aware of this rare disease entity among the more common conditions of behavioural problems and substance abuse in adolescents.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. A case of autoimmune hemolytic anemia associated with an ovarian teratoma.Autoimmune hemolytic anemia associated with an ovarian teratoma is a very rare disease. However, treating teratoma is the only method to cure the hemolytic anemia, so it is necessary to include ovarian teratoma in the differential diagnosis of autoimmune hemolytic anemia. We report herein on a case of a young adult patient who had severe autoimmune hemolytic anemia that was induced by an ovarian teratoma. A 25-yr-old woman complained of general weakness and dizziness for 1 week. The hemoglobin level was 4.2 g/dL, and the direct and indirect antiglobulin tests were all positive. The abdominal computed tomography scan revealed a huge left ovarian mass, and this indicated a teratoma. She was refractory to corticosteroid therapy; however, after surgical resection of the ovarian mass, the hemoglobin level and the reticulocyte count were gradually normalized. The mass was well encapsulated and contained hair and teeth. She was diagnosed as having autoimmune hemolytic anemia associated with an ovarian teratoma. To the best of our knowledge, this is the first such a case to be reported in korea.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. Extrarenal adult Wilms' tumor.Extrarenal Wilms' tumor is a very rare disease, occurring mostly in children. Sixteen cases have been reported in the world medical literature. A case of retroperitoneal extrarenal Wilms' tumor is reported in a 36-year old white male and the literature reviewed. Pathologic examination revealed both undifferentiated renal blastoma plus differentiated areas containing immature tubules and glomeruli. No evidence of extrarenal teratomatous tissue was seen. Theories for the histogenesis of this rare tumor are discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. Intrapericardial teratoma in infancy: a rare disease.Intrapericardial teratoma is a rare, and generally benign, tumor originating from the 3 germinal layers. It occurs in 60% of the cases in the first two years of life. Severe cardio-respiratory distress due to mediastinal compression and pericardial effusion are the main clinical symptoms. Echo-cardiography and cine-angiography confirm the diagnosis and early surgical removal brings immediate and durable improvement.- - - - - - - - - - ranking = 4keywords = rare disease (Clic here for more details about this article) |
7/7. Malignant thyroid teratoma of an adult: a long-term survival after chemotherapy.Adult primary malignant teratoma of the thyroid is a rare disease that has a poor outcome despite aggressive therapy. A 32-year-old woman underwent a simple lobectomy of the thyroid for a progressively enlarged nodule in August 1990. Pathologic assessment of the tumor revealed a malignant teratoma. The physical examinations and image studies failed to find tumor in other sites. She subsequently received 6 courses of chemotherapy with cisplatin, etoposide, and bleomycin. She experienced a complete response to chemotherapy and remained disease-free for 6.5 years. This tumor must be included in the differential diagnosis of a thyroid tumor and may be treated as a testicular germ-cell tumor for which chemotherapy may be recommended.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |