1/154. Mixed malignant germ cell tumor of the fallopian tube.Immature teratomas of the fallopian tube are exceedingly rare with only three reported cases in the English literature. Reported here is a case of primary mixed malignant germ cell tumor of the fallopian tube composed of immature teratoma and yolk sac tumor.- - - - - - - - - - ranking = 1keywords = fallopian tube, tube (Clic here for more details about this article) |
2/154. Diagnostic and therapeutic approach to sialoblastoma: report of a case.The natural history of a rare parotid tumor, the sialoblastoma (embryoma) is reported. It is a blastematous neoplasm said to recapitulate the epithelial differentiation of a gland at various stages of development. The tumor grew in a period of 5 years (from shortly after birth until it was excised) to a firm asymptomatic mass measuring 5 cm in greatest diameter. With a diagnosis of adenoid cystic carcinoma (a common misdiagnosis), the patient was referred to the reporting institution, where the diagnosis was revised. Five months later, completion parotidectomy for presumed recurrence failed to demonstrate residual tumor. One year later, the patient appears to be free of disease. The authors propose that sialoblastomas should be regarded neither as benign nor malignant, but as one single disease with local infiltrative potential. Based on this concept, sialoblastomas can be treated with early conservative surgery alone, provided that free margins are obtained.- - - - - - - - - - ranking = 0.00087802165109263keywords = disease (Clic here for more details about this article) |
3/154. radiation-induced brain calcification: paradoxical high signal intensity in T1-weighted MR images.BACKGROUND: Irradiation to the central nervous system (CNS) in childhood is known to induce cerebral calcification after a latent period. Calcification has been generally found to show nil or a reduction in signal intensity in magnetic resonance (MR) images. However, we have studied three patients with radiation-induced brain calcification, who manifested increased signal intensity on T1-weighted MR images. METHOD: Three girls had each been diagnosed as having a suprasellar germ cell tumour and were treated with conventional fractionated radiotherapy in their childhood. In one case, chemotherapy was given prior to the CNS irradiation. FINDINGS: All three patients survived their disease, and a follow-up CT scan revealed calcification in the brain, which has shown an increased signal intensity in the T1-weighted images of MR. INTERPRETATION: Cerebral calcification may be presented as a high signal intensity in the T1-weighted MR images. This may be explained by a surface-relaxation effect by the calcium salt particle, precipitated in the brain due to radiation-induced mineralising microangiopathy.- - - - - - - - - - ranking = 0.00043901082554632keywords = disease (Clic here for more details about this article) |
4/154. Pancreatoblastoma treated by delayed operation after effective chemotherapy.A case of pancreatoblastoma in a 4-year-old girl is presented. She was admitted with an abdominal mass and vomiting in August 1994. Computed tomography scan showed a 10- x 8- x 7-cm mass occupying both the head and body of the pancreas. serum alpha-fetoprotein (AFP) level was 9,600 ng/mL (normal, <8.3 ng/mL). Results of open biopsy of the tumor showed pancreatoblastoma. Chemotherapy was administered using the new A-1 regimen consisting of cyclophosphamide, etoposide, pirarubicin, and cisplatin. After 3 cycles of chemotherapy, the size of the tumor was reduced to 5 x 4 x 3 cm, the portal vein became patent, and the AFP value decreased to 98.1 ng/mL. Total removal of the tumor was performed leaving the head and tail of the pancreas. Postoperative chemotherapy continued for 2 years. The patient has been disease free for 5 years, and her serum AFP remained within normal levels.- - - - - - - - - - ranking = 0.00043901082554632keywords = disease (Clic here for more details about this article) |
5/154. Hepatic undifferentiated (embryonal) sarcoma in an adult: a case report and review of the literature.Undifferentiated (embryonal) sarcoma of the liver (USL) is a rare malignant tumour with a poor prognosis. The absence of specific symptoms, the rapid tumour growth, the normality of the common tumour markers, and the consequential delay in the diagnosis often result in significant enlargement of the tumour. To our knowledge, there have been only 42 reported cases of USL in adults worldwide during the 40 years since the clinicopathological entity of USL was defined. We report here a 27-year-old male with USL who has been treated successfully with surgical resection and adjuvant chemotherapy using ifosfamide, adriamycin and cisplatin. Although the prognosis of the disease remains generally poor, long-term survival has been achieved recently in patients with a combination of surgery and multi-agent chemotherapy.- - - - - - - - - - ranking = 0.00043901082554632keywords = disease (Clic here for more details about this article) |
6/154. Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature.Undifferentiated (embryonal) sarcoma of the liver (USL) is a highly malignant tumor of early life. Treatment choices for USL, especially with intraperitoneal rupture, are uncertain. Outcomes have been almost uniformly poor until recently. We describe two 7-year-old girls treated for ruptured USL. In the more recent patient, operative biopsy was followed by three cycles of cisplatin (CDDP), adriamycin (ADR), and cyclophosphamide (CPM). A fluid-filled cavity in the tumor showed enlargement and was drained. Two cycles of CDDP, ADR, vincristine (VCR), and ifosfamide were accompanied by reduction in tumor size, and trisegmentectomy was performed. She has no evidence of disease 3.5 years after surgery. In the other patient, left lobectomy was followed by a less intensive regimen, including CPM, VCR, and fluorouracil. This patient died of dissemination within 5 months. In 170 reported pediatric patients with USL, the 2-year disease-free survival was 17%. For the 96 such patients reported since 1980, 2-year disease-free survival had improved to 27%. More aggressive chemotherapy has been associated with this change. Of 8 patients with tumor rupture whose details have been reported (including the 2 present patients) after resection of the tumor, 4 died, 1 was alive with disease, and 3 were free of disease at 8, 49, and 58 months, respectively, after diagnosis. Ruptured USL should be treated with combination chemotherapy including CDDP and ADR, as well as with curative resection.- - - - - - - - - - ranking = 0.0021950541277316keywords = disease (Clic here for more details about this article) |
7/154. Immature hepatic tumor of bimodal differentiation in a young adult patient: a novel lesion expressing beta-catenin and mimicking a distinct phase of hepatogenesis.PATIENT AND methods: A large right-sided liver tumor was incidentally discovered and totally resected in a 21-year-old female patient without chronic liver disease. Histopathologic examination revealed that this malignant tumor does not fit any of the known types of primary liver tumors reported thus far. RESULTS: The lesion chiefly consisted of numerous solid nests composed of immature epithelial cells disclosing a marker profile typical for hepatocytes and strongly expressing beta-catenin. These nests were tightly associated with abnormal duct-like profiles exhibiting features of bile duct cells. CONCLUSIONS: The finding of numerous neoplastic hepatobiliary units ('liverlets') suggests that this tumor may mimic a distinct developmental phase of hepatogenesis close to ductal plate formation.- - - - - - - - - - ranking = 0.00043901082554632keywords = disease (Clic here for more details about this article) |
8/154. Cystic variant of embryonal sarcoma of liver.Embryonal sarcoma of the liver is an unusual tumor. The cystic form is rare and can mimic hydatid disease. We present a case that was mistakenly treated as a hydatid cyst for 3 months. Surgery was successful in removing the mass.- - - - - - - - - - ranking = 0.00043901082554632keywords = disease (Clic here for more details about this article) |
9/154. An unusual phenotype of frasier syndrome due to IVS9 4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis.The Wilms' tumor gene (WT1) encodes a zinc-finger transcription factor involved in the development of the kidneys and gonads and their subsequent normal function. Mutations in the WT1 gene were identified in patients with WAGR (Wilms' tumor, aniridria, genitourinary abnormalities, and mental retardation), denys-drash syndrome, and frasier syndrome (FS). Constitutional heterozygous mutations of the WT1 gene, almost all located at intron 9, are found in patients with FS. This syndrome is characterized by female external genitalia in 46,XY patients, late renal failure, streak gonads, and high risk of gonadoblastoma development. We report a male with FS with an unusual phenotype characterized by normal penis size with perineal hypospadias, end-stage renal failure at the age of 19 yr, normal adult male serum T levels, extremely elevated gonadotropin levels, para-testicular leiomyoma, unilateral testicular germ cell tumor, bilateral gonadoblastoma, and absence of gonadal dysgenesis. Automatic sequencing identified the IVS9 4C>T mutation in the WT1 gene, which predicts a change in splice site utilization. WT1 transcript analysis showed reversal of the normal positive/negative KTS (lysine, threonine, and serine) isoform ratio, confirming the diagnosis of FS. This patient with FS presents an external genitalia of denys-drash syndrome, suggesting that these two syndromes are not distinct diseases but may represent two ends of a spectrum of disorders caused by alterations in WT1 gene. This case expands the spectrum of phenotypes associated with WT1 mutations, by including predominantly male ambiguous genitalia and absence of gonadal dysgenesis, extremely high gonadotropin levels, and delayed adrenarche, and presence of a para-testicular leiomyoma, bilateral gonadoblastoma, and germ cell neoplasia.- - - - - - - - - - ranking = 0.00043901082554632keywords = disease (Clic here for more details about this article) |
10/154. Lectin-reactive alpha-fetoprotein (AFP-L3%) curability and prediction of clinical course after treatment of non-seminomatous germ cell tumors.OBJECTIVE: Alpha-fetoprotein (AFP) is an important tumor marker for non-seminomatous germ cell tumors (NSGCTs) that greatly affects diagnosis and the evaluations of therapy and therapeutic policy. However, it is sometimes very difficult to make the distinction between tumors and falsely elevated AFP levels due to benign liver disease. We assessed the usefulness of lectin-reactive alpha-fetoprotein (AFP-L3%), which has been reported to be superior to total AFP in both sensitivity and specificity in hepatocellular carcinomas, for the evaluation of predictions of clinical courses after the treatment of NSGCTs. methods: Frozen sera of 25 tumor-bearing patients with testicular cancers whose AFP levels were 5.0 ng/ml or higher were used. The total AFP levels and the ratio of L3 fraction to total AFP (AFP-L3%) were measured by liquid-phase binding assay (LBA). RESULTS: The total AFP levels were 6.3-14 907 ng/ml (median: 105.9 ng/ml). The median AFP-L3% was 69.9% (range;: 1.1-88.1%). Except for one patient, 24 patients (96.0%) with evident disease showed high levels of AFP-L3% of >50%, regardless of their total AFP levels. In nine patients whose sera were sequentially measured, AFP-L3% was considered highly useful for the detection of residual tumors (n = 2) and recurrence (n = 1) and for the exclusion of false-positive cases (n = 1). CONCLUSIONS: When the total AFP level increases slightly (e.g. to 5-20 ng/ml), a measurement of AFP-L3% may provide additional useful information for monitoring NSGCT patients and in distinguishing falsely elevated AFP.- - - - - - - - - - ranking = 0.00087802165109263keywords = disease (Clic here for more details about this article) |
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