Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Preliminary clinical trials using cryopreserved autologous bone marrow reinfusion have now been carried out at our institution in 5 children and 2 adults with advanced stages of neuroblastoma, rhabdomyosarcoma, non-Hodgkin's lymphoma and small cell carcinoma of the lung. Normal numbers of in vitro colony forming cells (CFU-C) were obtained from these patients despite prior courses of combination chemotherapy. The dose of marrow cells cryopreserved ranged from 1-6 X 10(8) cells/kg and recovery of CFU-C after thawing averaged 50%. Partial or complete hematologic reconstitution was achieved in all patients. The time for recovery ranged from 10-43 days for leukocytes (greater than 1000 cells/mm3) and 23-45 days for platelets (greater than 50,000/mm3). Two patients have died of interstitial pneumonitis due to cytomegalovirus. Three patients have died of recurrent tumor at 40, 48 and 156 days post-transplant. Two patients have had significant therapeutic benefit. One of these had a stable partial response permitting the use of further post-transplant therapy and is alive and well 16+ months post-transplant. The other patient had a complete response and remains tumor-free 25+ months following therapy. We conclude: 1) Autologous bone marrow reinfusion permits hematologic reconstitution following marrow-ablative therapy. 2) A quantity of marrow sufficient for this purpose remains viable following cryopreservation even when obtained from patients previously exposed to chemotherapy. 3) Autologous bone marrow reinfusion now allows the exploration of more intensive cytoreductive therapy in selected malignancies.
 ...
PMID:Autologous bone marrow transplantation in the treatment of selected human malignancies: The Johns Hopkins Oncology Center Program. 40 Jun 94

Twenty-one children were admitted to a single paediatric institution between 1964-1990 with histologically proven primary liver tumours. The diagnosis was hepatoblastoma (HBL) in 15 patients, hepatocellular carcinoma (HCA) in 2, rhabdomyosarcoma (RMS) in 2, non-Hodgkin's lymphoma (NHL) in 1, and haemangioendothelioma (HE) in 1. The common presenting clinical features were anaemia, abdominal mass, and abdominal pain. Serum alpha-foetoprotein was useful in establishing a diagnosis in HBL and in monitoring disease activity. Computed tomographic (CT) scan, ultrasound, and angiography were useful preoperative investigations for assessing site and resectability of tumour. There were no survivors in patients with malignant hepatic tumours (n = 10) who had surgery alone prior to 1981. Of 7 patients with HBL diagnosed after 1981 who had adequate surgical resection and chemotherapy, 5(72%) are currently alive and disease free between 15 months and 8 years from diagnosis. We conclude that adequate surgical resection and adjuvant chemotherapy can improve disease free survival for children with HBL. Optimal treatment has yet to be devised for other malignant hepatic tumours.
 ...
PMID:Primary hepatic tumours in children: a 26-year review. 131 8

The production and detailed immunostaining properties of a new rat monoclonal antibody (ICR.2) to epithelial membrane antigen are reported. The antibody was selected for its ability to compete with the polyclonal antiserum (M7), used in the original immunohistological studies, in order that it might serve as a direct replacement in diagnosing epithelial tumours. Most of the staining reactions on normal tissues were identical to those previously reported with M7 but there were some important differences. They included: positivity of renal and adrenal capsular fibroblasts, perineurium, some myoepithelial and smooth muscle cells, occasional osteoblasts and squamous and thyroid follicular epithelium in the normal state. The intercellular canaliculi of sweat glands and secretory canaliculi of gastric oxyntic cells were clearly demonstrated. These staining reactions could be obtained with M7 when a sensitive detection system was used although the results were usually weak and inconsistent. Nearly all adenosquamous and transitional carcinomas were positive. The remaining tumours fell into three major groups: (1) those which were consistently or nearly consistently negative--melanoma, seminoma, rhabdomyosarcoma, alveolar soft part sarcoma, adrenal cortical carcinoma, granulocytic sarcoma, paraganglioma, non-Hodgkin's lymphoma. Hodgkin's disease and embryonal carcinoma: (2) those which were either negative or positive with distinctive patterns of staining--basal cell carcinoma, embryonal tumours: and (3) non-epithelial tumours that were consistently positive--epithelioid sarcoma, synovial sarcoma, osteosarcoma, chordoma and myeloma--or positive in a significant minority of cases--leiomyosarcoma, malignant fibrous histiocytoma, clear cell sarcoma of tendon sheath, various neuroectodermal tumours.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Detailed investigation of the diagnostic value in tumour histopathology of ICR.2, a new monoclonal antibody to epithelial membrane antigen. 169 88

The levels of peripheral progenitor cells was measured serially after cancer chemotherapy in 4 patients with non-Hodgkin's lymphoma and one patient with rhabdomyosarcoma who received recombinant human granulocyte colony-stimulating factor (rG-CSF). This study was composed of two independent phases: in the first phase, patients received a course of cytotoxic chemotherapy only, and in the second phase, they received the same chemotherapy followed by subcutaneous injection of rG-CSF (2 micrograms/kg/day) for 10-14 days. In the control phase, the levels of granulocyte-macrophage colony-forming units (CFU-GM) and erythroid burst-forming units (BFU-E) per milliliter increased during the early recovery phase, but rG-CSF treatment increased the number of CFU-GM 3 to 18-folds, and the number of BFU-E increased 1.3 to 4.6-folds. An overshoot in the blood progenitor levels occurred at the day 8-10 of rG-CSF administration. And then, the peak of neutrophil count followed 3-5 days later. After the discontinuation of rG-CSF, the number of blood CFU-GM and BFU-E fell rapidly. This results suggest that in vivo expansion of circulating hemopoietic progenitors can be achieved by the administration of rG-CSF, and this approach might be clinically applicable to cancer patients who are a candidate of peripheral blood stem cell autotransplantation.
 ...
PMID:[Expansion of peripheral blood progenitors by recombinant human granulocyte colony-stimulating factor]. 170

Northern analysis of tumour RNA has been used to examine the expression of members of the myf family of muscle determining genes (myf3, myf4, myf5 and myf6) in a series of 20 rhabdomyosarcomas. A 2.0 kb myf3 transcript was observed in 85% of tumours, a 1.8 kb myf4 transcript was detected in 70% of tumours and a 1.7 kb myf5 transcript was observed in 55% of tumours. Transcription of myf6 occurred in 28% of tumours, but there were several transcript sizes (1.2, 1.5, 2.0 and 3.5 kb) and in some individual tumours two or more transcripts were observed. Only two rhabdomyosarcomas, one classified as embryonal and one as pleomorphic, failed to exhibit transcription of members of the myf gene family. We were unable to detect transcription of myf genes in neuroblastomas, Wilms' tumours, hepatoblastomas, paediatric non-Hodgkin's lymphoma and leiomyosarcomas. When considered together these observations suggest that expression of myf genes could provide an extremely useful marker in the diagnosis of rhabdomyosarcoma.
 ...
PMID:Expression of members of the myf gene family in human rhabdomyosarcomas. 176 65

Fine needle aspiration (FNA) was performed under ultrasound guidance on 17 abdominal masses in 16 pediatric patients at Baragwanath Hospital. The aspirated cellular material was assessed by conventional cytomorphology and by electron microscopy (EM). A diagnosis of malignancy was rendered for all 15 tumors that were adequately sampled (88.2%); the remaining 2 masses yielded insufficient material for either light microscopy or EM. Cytologic cell typing (including the use of EM) was successful in 12 of the 15 tumors (80%) as compared with the histologic diagnosis. EM was in agreement with the initial cytologic diagnosis in eight tumors, but corrected the initial impression in four tumors. The tumors with adequate aspirates included nine nephroblastomas and single examples of neuroblastoma, hepatoblastoma, non-Hodgkin's lymphoma, rhabdomyosarcoma, renal carcinoma and malignant rhabdoid tumor. The last three were not accurately typed by cytology plus EM. These preliminary results suggest that FNA cytology with adjunctive EM could become a useful technique in the preoperative assessment of pediatric abdominal tumors.
 ...
PMID:Fine needle aspiration of pediatric abdominal masses. Cytologic and electron microscopic diagnosis. 185 56

The sarcomas, particularly those of soft-tissue origin, pose substantial diagnostic challenges for the clinician and pathologist. Several small round cell sarcomas, including Ewing's sarcoma, peripheral primitive neuroectodermal tumor, and alveolar rhabdomyosarcoma, can be difficult to distinguish from one another. These same sarcomas can be difficult to distinguish from other small round cell tumors, including non-Hodgkin's lymphoma and neuroblastoma. Spindle cell sarcomas, including malignant peripheral nerve sheath tumor, synovial sarcoma, and leiomyosarcoma, present similar diagnostic challenges. This review discusses 1) recent advances in immunohistochemistry, electron microscopy, and cytogenetics that enable a specific diagnosis in virtually all sarcoma cases; 2) cell biology and oncogenetic implications of novel morphologic and genetic findings in sarcomas; and 3) clinical implications of the recent characterization of several family cancer syndrome genes.
 ...
PMID:Laboratory investigation, genetics, and experimental models in sarcomas. 193 25

One hundred forty-three patients who received radiation therapy for childhood tumors, and survived to the age of skeletal maturity, were studied by retrospective review of oncology records and roentgenograms. Diagnoses for the patients were the following: Hodgkin's lymphoma (44), Wilms's tumor (30), acute lymphocytic leukemia (26), non-Hodgkin's lymphoma (18), Ewing's sarcoma (nine), rhabdomyosarcoma (six), neuroblastoma (six), and others (four). Age at the follow-up examination averaged 18 years (range, 14-28 years). Average length of follow-up study was 9.9 years (range, two to 18 years). Asymmetry of the chest and ribs was seen in 51 (36%) of these children. Fifty (35%) had scoliosis; 14 had kyphosis. In two children, the scoliosis was treated with a brace, while one developed significant kyphosing scoliosis after laminectomy and had spinal fusion. Twenty-three (16%) patients complained of significant pain at the radiation sites. Twelve of the patients developed leg-length inequality; eight of those were symptomatic. Three patients developed second primary tumors. Currently, the incidence of significant skeletal sequelae is lower and the manifestations are less severe than reported in the years from 1940 to 1970. The reduction in skeletal complications may be attributed to shielding of growth centers, symmetric field selection, decreased total radiation doses, and sequence changes in chemotherapy.
 ...
PMID:Skeletal sequelae of radiation therapy for malignant childhood tumors. 213 23

Survival rates were analysed for a population-based series of over 15,000 childhood cancers registered in Great Britain during 1971-85. There were highly significant improvements (P less than 0.001 for trend) in survival for many major diagnostic groups. Between 1971-73 and 1983-85 the actuarial 5-year survival rates increased from 37% to 70% for acute lymphoblastic leukaemia, from 4% to 26% for acute non-lymphoblastic leukaemia, from 76% to 88% for Hodgkin's disease, from 22% to 70% for non-Hodgkin's lymphoma, from 61% to 72% for astrocytoma, from 24% to 42% for medulloblastoma, from 15% to 43% for neuroblastoma, from 58% to 79% for Wilms' tumour, from 17% to 54% for osteosarcoma, from 26% to 61% for rhabdomyosarcoma, from 59% to 94% for malignant testicular germ-cell tumours and from 43% to 77% for malignant ovarian germ-cell tumours. These increases in population-based survival rates reflect the substantial advances in treatment of a wide range of childhood cancers since 1970. The two principal diagnostic groups for which there was no evidence of any trend were retinoblastoma, which already had an excellent prognosis with a 5-year survival rate of over 85%, and Ewing's sarcoma, for which the survival rate remained below 45%.
 ...
PMID:Trends in survival for childhood cancer in Britain diagnosed 1971-85. 217 43

Since May 1979, 47 patients with pediatric malignancy aged 1 to 18 years (median: 7) were treated with cryopreserved autologous bone marrow transplantation (ABMT) in the department of pediatrics, National Cancer Center Hospital. The malignancies were acute non-lymphocytic leukemia (n = 8), acute lymphocytic leukemia (n = 5), osteosarcoma (n = 7), neuroblastoma (n = 6), brain tumor (n = 5), rhabdomyosarcoma (n = 4), retinoblastoma (n = 3), Ewing's sarcoma (n = 3), non-Hodgkin's lymphoma (n = 2), malignant histiocytosis (n = 1), hepatoblastoma (n = 1), malignant melanoma (n = 1) and malignant neuroepithelioma (n = 1). Conditioning regimens for solid tumors were multi-agent high-dose chemotherapy, mainly consisted of cyclophosphamide (CY) 120 mg/kg or melphalan 180mg/m2 and that for hematological malignancies were CY with fractionated total body irradiation (12 Gy). In vitro purging by 4-hydroperoxycyclophosphamide was performed in 12 leukemia patients and 5 solid tumor patients. Of the 13 patients with acute leukemia, 1 died from relapse 1 year after the unpurged marrow transplantation and 1 relapsed in the testis. Remaining 11 patients are alive in continuous complete remission with a median follow up of 30 months (range, 2 to 65 months) after transplantation. The disease-free survival rate of them was 78%. Of the 34 patients with solid tumor, 21 patients died, their cause of death were relapse in 18 and each one of infection, graft failure and brain hemorrhage. Thirteen patients are alive without disease with a median follow up of 28 months (range, 2 to 107 months) posttransplant. The longest survivor is a brain tumor girl, and there are 5 other long survivors; 2 of them are osteosarcoma and each one of rhabdomyosarcoma, Ewing's sarcoma and malignant histiocytosis. The disease-free survival rate of total 34 solid tumor patients is 29%, but that of 17 patients who received ABMT in responsive and minimum tumor residue (MTR) period was 69%. These results suggest that autologous bone marrow transplantation is an effective and tolerable treatment for poor prognostic pediatric malignancies, especially for acute leukemia and such solid tumor as that in MTR state.
 ...
PMID:[Autologous bone marrow transplantation in pediatric cancer]. 226 Aug 67


1 2 3 4 5 6 Next >>