Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

VP-16-213, a semisynthetic podophyliotoxin, was tested for antitumor and clinical toxicity in 126 children. The drug was administered iv daily x 5 days every 2 weeks at a starting dose of 75 mg/m2/day. The dose was increased by 25 mg/m2/day/course until clinical response or significant toxicity occurred. The only major toxicity was hematologic, with neutropenia as the most predominant feature. There was one local allergic reaction at the site of injection. No systemic allergic responses were reported. The drug demonstrated significant activity in acute myelomonocytic leukemia with four responses among 19 patients, less activity in acute myelocytic leukemia with two responses among 44 patients, and little activity in acute lymphocytic leukemia with only one partial response among 12 patients. Objective partial responses occurred in ten of 48 patients with solid tumors: two each with Wilms' tumor, lymphoma, and histiocytosis X, and one each with rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma, and undifferentiated carcinoma. The inclusion of VP-16-213 in combination chemotherapy for childhood acute myelomonocytic leukemia and acute myelocytic leukemia appears indicated in patients relapsing after initial therapy. For solid tumors this is an interim report, with further patient accrual required before specific comments can be made.
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PMID:Phase II study of VP-16-213 in childhood malignant disease: a Children's Cancer Study Group Report. 29 6

Cytologic smears obtained by needle aspiration biopsy of lytic bone lesions in 15 patients with histiocytosis X, Ewing's sarcoma and neuroblastoma were reviewed. After conventional staining, histiocytosis X could be diagnosed and differentiated from small cell tumours such as Ewing's sarcoma and neuroblastoma. The need for sampling material for cytochemical and ultrastructural analysis of these small cell tumours by needle aspiration is emphasized.
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PMID:Needle aspiration biopsy in the diagnosis of lytic bone lesions in histiocytosis X, Ewing's sarcoma and neuroblastoma. 30 67

Closed trephine needle biopsy of the bone marrow has become an established procedure in the evaluation of many malignant and benign diseases in adults; however, its role in pediatric pathology has not yet been defined. In the period from February 1974 to April 1978 we have performed 164 such biopsies in 111 children under 15 years of age. A representative specimen has been obtained in over 80% of cases. This series included, in order of frequency, non-Hodgkin lymphomas, Hodgkin lymphomas, aplastic anemias, rhabdomyosarcomas, neuroblastomas, miscellaneous solid tumors, and single cases of histiocytosis X, malignant histiocytosis, sarcoidosis, malignant histiocytoma, and Castleman lymphoma of the hyaline-vascular type. Histology has been found superior to cytology in the detection of neuroblastoma invasion; the evaluation of the true cellularity in aplastic anemia, and the detection of granulomatous tissue in the only case of sarcoidosis. In other diseases histology and cytology gave similar information, except for the few cases of acute leukemia in partial relapse, which has been better defined in the aspirate smears than in the core specimen. Further evaluation of this technique in other patient series appears advisable.
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PMID:Bone marrow biopsy in children: a study of 111 patients. 44 Feb 5

Rhabdomyosarcoma usually presents as a soft tissue mass, which may invade adjacent bone. However, the patient presents occasionally with bony metastases. Recognition of these is important for staging and management. Fifty-eight cases have been reviewed; 14 of these had local bone invasion by the soft tissue tumour. All bones involvel were flat bones; 12 showed permeated bone destruction and two showed geographic destruction. Bone expansion was seen in half the involved bones. Twelve of the 58 cases showed secondary bone deposits, which were the presenting feature in five. Although 10 cases had permeated bone destruction, two were very well defined with a wide range of radiological appearances. The radiological differential diagnosis includes neuroblastoma, leukaemic infiltration, lymphoma, histiocytosis X, solitary and multifocal osteosarcoma and other deposits.
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PMID:The radiology of bone changes in rhabdomyosarcoma. 62 1

32 children with different types of tumors have bben treated with peptichemio. The efficacy was excellent in rhabdomyosarcoma and embrional sarcoma; encouraging in neuroblastoma, Wilms tumor and histiocytosis X. Side and toxic effects were minimal. In conclusion we can say that peptichemio is effective in oncologic diseases, but a larger number of patients is required in order to have a better knowledge about the antitumor activity of this drug.
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PMID:Peptichemio in children with neoplastic disease. 79 81

Between 1987 and 1991, 248 long-term survivors of childhood cancer have been studied at the Oncology Unit of the Children's Hospital of Buenos Aires. The tumors were diagnosed between 1965 and 1986 as, retinoblastoma: 65, lymphoma: 57, nephroblastoma: 36, neuroblastoma: 25, germ-cell tumors: 18, sarcoma: 19, bone tumors: 7, lymphoepithelioma of cavum: 4, histiocytosis X: 9, others: 8. The treatment consisted of, surgery: 25, surgery+chemotherapy: 40, surgery+radiotherapy: 8, chemotherapy: 23, chemotherapy+radiotherapy: 42, and surgery+chemotherapy+radiotherapy: 110. There are alive without evidence of cancer disease 234 survivors between 5 and 25 years after diagnosis. Severe organic disabilities were observed in 181 survivors and moderate in 142. Thirteen patients died because of second malignant neoplasia and 1 patient with lung metastasis 9 years after diagnosis of nephroblastoma. In 180 survivors the data of instruction was available. Seventy participate in sports and 13 in artistic activities. Eight survivors are university graduates and 24 are employees. Five young women were mothers and one young man was a father. The meaning of concept of "cure" is discussed from the point of view of the physicians and the survivors. In order to detect deleterious late effects of cancer and their treatment the follow-up must be continuous.
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PMID:[The concept of cure in children with cancer]. 182 19

Obstructive jaundice secondary to external compression of the extrahepatic bile duct caused by tumor of non-liver origin was found in 5 of 199 consecutive children with cancer between 1986 and 1988 at the Department of Pediatrics, National Taiwan University Hospital. Of the 5 patients, 2 had non-Hodgkin's lymphoma and the other 3 had acute promyelocytic leukemia, histiocytosis X and neuroblastoma, respectively. Extrahepatic biliary obstruction occurred as part of the initial presentation of malignancy in 3 cases, and later in the course of disease in the other 2 cases. In each instance, abdominal ultrasonography and computed tomography revealed dilatation of intrahepatic biliary trees due to mass compressing effects. A huge multilobulated tumor and multiple enlarged lymph nodes near the porta hepatis were found in all 3 patients who underwent an exploratory laparotomy. Wedge biopsy of the liver showed no cancer cell invasion. One case died before chemotherapy had commenced. The other 4 patients received chemotherapy and 3 of them received additional radiotherapy. Although jaundice and tumor regressed dramatically with this mode of treatments, subsequent recurrence of tumor without jaundice rapidly ensued in 3 patients. They all died, except 1 case, within 18 months from the occurrence of jaundice. This suggests that these patients were in an advanced stage of disease and should be diagnosed early and treated vigorously. Accordingly, cancer of non-liver origin, although rare, should be considered in the differential diagnosis of obstructive jaundice if survival is to be improved in these cancer children.
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PMID:Extrahepatic biliary obstruction caused by cancer of non-liver origin in children: report of 5 cases. 259 45

All cases diagnosed in Finland as non-Hodgkin's lymphoma (NHL), Hodgkin's disease or histiocytosis X in children younger than 15 years in 1953 to 1973, according to the Finnish Cancer Registry, were reexamined histologically. Only 55% of the cases originally diagnosed as NHL were regarded as such at reexamination. The others were mainly malignant nonlymphatic tumors such as neuroblastoma and different kinds of sarcomas. Seventy-two NHLs were diagnosed in 50 boys and 22 girls. The corrected age-specific incidence rate was 0.32/10(5). The most common histologic types were Burkitt's lymphoma (BL) (30 cases), lymphoblastic lymphoma (LBL) (26), large cell lymphomas (LCL) (six), and non-Burkitt's lymphoma (n-BL) (three). There were marked differences between BL and LBL in the course of the disease: BL was extranodal in 83%, LBL only in 4% (mediastinum was regarded as nodal); BL showed initial abdominal or pelvic involvement in 60% whereas LBL showed none; BL had initial mediastinal involvement in 7%, and LBL had it in 62%; all patients with LBL died whereas 23% of those with BL survived. Other types of NHL resembled BL in their course of disease. Patients with initial tonsillary involvement appeared to have the best prognosis and patients with mediastinal involvement the poorest. The importance of accurate histologic classification is emphasized. It appears to be most important to differentiate LBL from other types of NHL.
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PMID:Non-Hodgkin's lymphomas in childhood. A clinicopathologic and epidemiologic study in Finland. 349 36

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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PMID:Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. 631 Feb 27


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