Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ovarian tumors in children are uncommon. We reviewed all cases of ovarian tumors treated at the Department of Pediatric Surgery of Vicenza Regional Hospital from 1979 to 1994. They include 6 cases of cystic teratoma and 1 gonadoblastoma; the malignant lesions included 1 immature teratoma, 1 dysgerminoma, 1 yolk-sac tumors and 1 patient with ovarian infiltration from malignant leukemia. Only the patient with leukemia died of progression of the disease. These data show that most ovarian masses are benign. Germ-cell tumors are the most common malignancy and epithelial cell tumors are less likely. Epithelial cyst and teratomas are the most common benign lesions. Although ultrasound was 100% accurate in the diagnosis of ovarian pathology, the ultrasonogram could not distinguish between benign and malignant lesions.
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PMID:[Ovarian tumors in children]. 876 76

Down syndrome may be associated with many complications. Among the malignancies associated with Down syndrome, leukaemia is the most common. This is a case report of a patient with Down syndrome associated with both a retroperitoneal teratoma and a Morgagni hernia.
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PMID:Down syndrome associated with a retroperitoneal teratoma and Morgagni hernia. 900 6

F 11782 is a newly identified catalytic inhibitor of topoisomerases I and II, without any detectable interaction with DNA. This study aimed to establish whether its catalytic inhibition of topoisomerase II was mediated by mechanisms similar to those identified for the bisdioxopiperazines. In vitro combinations of F 11782 with etoposide resulted in greater than additive cytotoxicity in L1210 cells, contrasting with marked antagonism for combinations of etoposide with either ICRF-187 or ICRF-193. All three compounds caused a G2/M blockade of P388 cells after an 18-h incubation, but by 40 h polyploidization was evident only with the bisdioxopiperazines. Gel retardation data revealed that only F 11782, and not the bisdioxopiperazines, was capable of completely inhibiting the DNA-binding activity of topoisomerase II, confirming its novel mechanism of action. Furthermore, unlike ICRF-187 and ICRF-193, the cytotoxicity of F 11782 appeared mediated, at least partially, by DNA damage induction in cultured GCT27 human teratoma cells, as judged by a fluorescence-enhancement assay and monitoring p53 activation. Finally, the major in vivo antitumor activity of F 11782 against the murine P388 leukemia (i.v. implanted) and the B16 melanoma (s.c. grafted) contrasted with the bisdioxopiperazines' general lack of activity. Overall, F 11782 and the bisdioxopiperazines appear to function as quite distinctive catalytic topoisomerase II inhibitors.
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PMID:Characterization of the biological and biochemical activities of F 11782 and the bisdioxopiperazines, ICRF-187 and ICRF-193, two types of topoisomerase II catalytic inhibitors with distinctive mechanisms of action. 1114 91

The outcome of 31 patients with malignant ovarian germ cell tumors treated by surgery and a medium dose etoposide containing short chemotherapy regimen between 1988 and 1997 is reported. Of the 31 patients, 16 (51.6%) had malignant teratomas, 8 (25.8%) had dysgerminomas, 6 (19%) endodermal sinus tumors and one (3.2%) mixed germ cell tumor. Twenty-four (77.4%) patients were at FIGO stage I (of which 18 were stage IA), 2 (6.5%) at stage II, 4 (12.9%) at stage III and 1 (3.2%) at stage IV. Twenty-five (80.6%) patients underwent conservative surgery, 1 (3.2%) underwent bilateral salpingo-oophorectomy and 4 (12.9%) had total hysterectomy with bilateral salpingo-oophorectomy and omentectomy. One (3.2%) patient refused definitive treatment. Three patients with stage IA grade 1 immature teratomas were not treated with adjuvant chemotherapy and one patient with a stage IA dysgerminoma refused chemotherapy. Two patients with endodermal sinus tumor returned to their countries of origin after surgery. Twenty-five patients received bleomycin, etoposide, and cisplatin (BEP) regimen with etoposide dosage fixed at 120 mg/m2 on day 1 and day 2, bleomycin 15 mg intravenous bolus on days 1 and 2 and cisplatin 100 mg/m2 on day 1. Chemotherapy was administered at four weekly intervals for 4 cycles or until complete response was achieved. The median number of cycles of chemotherapy was four (range 3-6) for stage I, 6 (range 4-7) for stage II and 5 (range 5-6) for stage III tumors. Of the entire cohort of 29 patients analyzed, the median follow up period was 5 years. One patient died from stage IIIC endodermal sinus tumor and one patient had persistent teratoma in the lungs. The overall disease free survival control rate was 93.1%. There were three cases of the growing teratoma syndrome involving the liver, abdominal peritoneum, and the pelvis, respectively. No mortality resulted from the growing teratomas. No pulmonary complications, secondary primary tumor or leukemia was detected. Menstrual function returned in all patients with fertility-preserving surgery and one pregnancy occurred. This interesting data suggest that a medium dose 2-day BEP postsurgical adjuvant chemotherapy regimen is effective and superior to expectant treatment of malignant ovarian germ cell tumors. This report, however, should be viewed as a pilot study. The result indicates that a prospective randomised controlled trial to demonstrate equivalence of this regimen with the standard BEP regimen is warranted.
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PMID:Experience of a 2-day BEP regimen in postsurgical adjuvant chemotherapy of ovarian germ cell tumors. 1124 Jun 46

A 14-year-old male presenting with a short history of right subclavicular chest pain was found to have a mediastinal tumor. Hematologic investigations and bone marrow examination showed features of myelomonocytic leukemia. The mediastinal tumor was excised, but the surgery was complicated by massive hemorrhage. The patient's condition deteriorated postoperatively and he died a week later. The histology of the mediastinal tumor showed the typical features of an immature teratoma with a yolk sac tumor. A prominent infiltrate of leukemic blast cells was present within blood vessels and in close proximity to the yolk sac component. The karyotypic analysis of leukemic cells isolated and cultured from the bone marrow showed 50XXY, +8, +21, +iso G-group marker chromosome karyotype.
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PMID:Mediastinal immature teratoma with yolk sac tumor and myelomonocytic leukemia associated with Klinefelter's syndrome. 1207 11

Neonatal tumours occur every 12,500-27,500 live births and comprise 2% of childhood malignancies, but there is little clarity as to their real prevalence, sites of origin and pathological nature as reported series vary. As an entity, neonatal tumours provide a unique window of opportunity to study tumours in which minimal environmental interference has occurred. The majority of tumours present with a mass at birth (e.g., teratomas, neuroblastomas, mesoblastic nephroma, fibromatosis), which are not infrequently identified on antenatal ultrasound. Histologically, teratoma and neuroblastoma remain the two main tumour types encountered with soft tissue sarcoma, renal tumours, CNS tumours and leukaemia being the next most common tumour types identified. Malignant tumours are uncommon in the neonatal period per se and benign tumours may have malignant potential. A particular problem exists in clinical classification, as histological features of malignancy do not always correlate with clinical behaviour. Benign tumours may also be life threatening because of their size and location. Other tumours may demonstrate local invasiveness, but no metastatic potential, and tumours that are clearly malignant may demonstrate unpredictable or uncertain behaviour. Screening programmes have brought more tumours to light, but do not appear to affect the overall prognosis. They may provide clues to the stage at which tumours develop in foetu. The aetiology of cancer in children is multifactorial and includes both genetic and environmental factors. The association between congenital abnormalities and tumours is well established (15% of neonatal tumours). Genetic defects are highly likely in neonatal tumours and include those with a high risk of malignancy (e.g., retinoblastoma), but also genetically determined syndromes with an increased risk of malignancy and complex genetic rearrangements. Tumours are mostly genetically related at a cellular level and factors influencing cellular maturation or apoptosis within the developing foetus may continue to operate in the neonatal period. Cytogenetics of neonatal neoplasms appear to differ from neoplasms in older children, thus possibly explaining some of the observed differences in clinical behaviour. Certain constitutional chromosome anomalies, however, specifically favour tumours occurring in the foetal and neonatal period. In support of this hypothesis, certain cytogenetic anomalies appear to be specific to neonates, and a number of examples are explored. Other environmental associations include ionizing radiation, drugs taken during pregnancy, infections, tumours in the mother and environmental exposure.
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PMID:The epidemiology of neonatal tumours. Report of an international working group. 1452 68

Fetal tumors are a diverse group of neoplasms, which are unique in their histologic characteristics, anatomic distribution, and pathophysiology. The biologic behavior of tumors in the fetus may differ dramatically compared with that of the same tumor detected later in life. Teratomas are the dominant histologic type and constitute the majority of both extracranial and intracranial neoplasms. Although often histologically mature, they may prove lethal because of their location and metabolic demands on the fetus. Large solid tumors may lead to cardiovascular compromise and hydrops fetalis. Extracranial teratomas are most commonly located in the sacrococcygeal area, followed by the head and neck, chest, and retroperitoneum. Fetuses with intracranial tumors have a poor prognosis regardless of histologic type. There are, however, two notable exceptions: lipomas and choroid plexus papillomas, both of which have a more favorable outcome. Neuroblastoma is the most common fetal malignancy. It may be either solid or cystic and is more often located on the right side. It typically has favorable biologic markers and stage at presentation. The prognosis for prenatally diagnosed cases is excellent. Other fetal neoplasms include soft-tissue tumors (both benign and malignant), leukemia, mesenchymal hamartoma of the kidney, and liver tumors (hemangioendothelioma, mesenchymal hamartoma, and hepatoblastoma).
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PMID:From the archives of the AFIP: a comprehensive review of fetal tumors with pathologic correlation. 1565 97

We present an unusual association of mediastinal germ cell tumor containing seminoma and angiosarcoma components and splenic histiocytic sarcoma. A 15-year-old boy presented with chest pain. Histopathologically, an anterior mediastinal mass contained typical seminoma, immature teratoma, embryonal carcinoma, angiosarcoma, yolk sac tumor, and polyembryoma. An abdominal ultrasonogram revealed a huge splenomegaly with multiple ill-defined low echogenic nodules, 1 month after the second cycle of chemotherapy. Histopathologically, large, round-to-oval tumor cells with abundant eosinophilic cytoplasm often contained eccentrically placed nuclei with vesicular chromatin and an irregular nuclear membrane. The tumor cells were immunoreactive for CD68, CD31, and CD4. The cytogenetic results showed deletion of the long arm of chromosome 5 and trisomy 8. This lesion might have been on the pathway of multistep tumorigenesis toward a final leukemia.
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PMID:Mediastinal germ cell tumor associated with histiocytic sarcoma of spleen: case report of an unusual association. 1608 90

Klinefelter syndrome is a well documented abnormality of sex differentiation, with an incidence of 1 in 600 newborn males. It is characterized by a 47,XXY or a mosaic karyotype and clinical findings of hypergonadotrophic hypogonadism, small testes, infertility, reduced body hair, gynecomastia, and tall stature. Other conditions like venous disease, autoimmune disorders, mild neurobehavioral deficit, diabetes mellitus, sexual precocity, and osteoporosis may also affect these patients. Different malignancies such as breast cancer, testicular tumors, leukemia, and lymphomas occur in 1%-2% of the cases. Klinefelter syndrome has been associated with other malignancies such as extragonadal germ cell tumors; however, some authors consider this association an unusual finding. We report the molecular cytogenetic studies performed in 4 young males with mediastinal germ cell tumors. In 2 cases, a 47,XXY karyotype was recognized in different tissues by fluorescent in situ hybridization, whereas the other 2 had a normal XY karyotype. We propose that in young patients with mediastinal teratoma, a cytogenetic analysis must always be performed.
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PMID:Extragonadal germ cell tumors are often associated with Klinefelter syndrome. 1656 24

A 24-year-old Japanese man was admitted due to bloody phlegm in May 2002. A diagnosis of mediastinal germ cell tumor, mixed type involving seminoma, immature teratoma and embryonal carcinoma, was made by transthoracic needle biopsy. Three months later, his complete blood counts revealed pancytopenia with high fever. Examination of bone marrow revealed increased atypical large histiocytes (5.6%) with hemophagocytosis, and thus, hemophagocytic syndrome related to germ cell tumor was diagnosed. In addition, chromosomal analysis of the bone marrow cells revealed a 47, XY, +9 genotype. Chemotherapies for germ cell tumor and hemophagocytic syndrome were performed without any improvement, and he died of diffuse alveolar damage. Autopsy revealed diffuse infiltration of immature histiocytes with hemophagocytosis in the liver, spleen and bone marrow. The atypical histiocytes were positive for CD68 and lysozyme and negative for lymphoid markers, and the diagnosis of true malignant histiocytosis associated with mediastinal germ cell tumor was made. The rare chromosomal abnormality of trisomy 9, a marker for benzene-related leukemia, was seen in the present case without apparent benzene exposure.
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PMID:True malignant histiocytosis with trisomy 9 following primary mediastinal germ cell tumor. 1680 92


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