Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

The differential diagnosis is difficult in cases of metastatic neuroblastoma, Ewing's sarcoma, lymphoma, and rhabdomyosarcoma, the common so-called small round cell tumors of childhood. The distinction between Ewing's sarcoma and neuroblastoma in bone with no soft tissue mass in the adolescent is especially difficult. Ewing's tumor is usually characterized by its content of glycogen, neuroblastoma by its absence. A case of glycogen-containing neuroblastoma initially misdiagnosed as Ewing's tumor is presented. Diagnostic implications, including the role of electron microscopy in diagnosis, are discussed. Glycogen alone is unreliable as a diagnostic aid due to 1) its presence in several tumors other than Ewing's including neuroblastoma, and 2) its absence in some cases of Ewing's sarcoma.
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PMID:Glycogen-containing neuroblastoma with clinical and histopathologic features of Ewing's sarcoma. 63 2

The utility and limitations of 67Ga scintigraphy in children with solid tumors were evaluated. Thirty-five patients with malignancies (13 lymphoreticular neoplasms, 11 soft-tissue sarcomas, 8 neuroblastomas, and 3 primary bone tumors) had a 67Ga-citrate scan as part of their clinical evaluation. The sensitivity and specificity of the test were analyzed for the different tumor types. The overall sensitivity of the 67Ga-citrate scan for the lymphoma group was 87%. Higher values were obtained for the mediastinal and abdominal regions. Ninety-three per cent of the involved sites were correctly identified by 67Ga scintigraphy in the soft-tissue sarcoma group. Small lung metastases, however, were missed on scan. Thus, 67Ga scans should be complemented with chest radiographs and whole chest tomograms for both initial evaluation and follow-up in those patients. 67Ga had low sensitivity for neuroblastoma.
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PMID:Scintigraphic evaluation of childhood malignancies by 67Ga-citrate. 66 63

The main features of Dubowiz syndrome are bird headed dwarfism and a typical facial configuration. Including two own patients 13 cases among 8 siblings are known in the literature. Two own cases of Dubowitz syndrome in two sisters are described, one of them with hypogammaglobulinemia and neuroblastoma, the other one with complete Ig A deficiency and malignant lymphoma. A propable relationship between immundeficiency and malignancies is discussed. Until 1973 151 malignant tumors with primary immundeficiency had been registrated. This paper is the first description of two cases of Dubowitz syndrome with immunodeficiency and malignant neoplasms.
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PMID:[Dubowitz syndrome with immunodeficiency and solid malignant tumor in two siblings (author's transl)]. 91 26

Intradermal skin test antigen (Varidase, Mumps, Candidine) and contact sensitization with 2,4-dinitrochlorobenzene (DNCB) were employed in 35 previously untreated children with neuroblastoma and malignant lymphoma, to evaluate delayed cutaneous hypersensitivity. Cutaneous responses were correlated to stage of disease and survival. The limited number of patients prevents any definitive conclusion. It is apparent, however, that in childhood non-Hodgkin's lymphoma anergy to DNCB is correlated to advanced stages with rapid progressive disease. In neuroblastoma and Hodgkin's disease patients skin test sensitization seems to be of questionable clinical value.
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PMID:Skin tests in childhood lymphoma and neuroblastoma. 102 49

During the past 3 years, eight hospitals and one cooperative study group have reported their initial clinical results with cis-dichlorodiammineplatinum (II). The most popular clinical schedule was 15-25 mg/m2/day for 5 days repeated every 3-4 weeks. Almost all patients had extremely advanced disease. Of 323 patients in whom response could be evaluated, there were 12 complete responses, 25 partial responses (greater than 50% decrease in tumor size), and 23 improvements (greater than 50% decrease in tumor size) for a 19% overall response rate. The tumor most sensitive to cis-dichlorodiammineplatinum (II) was testicular carcinoma in which seven complete responses, three partial responses, and three improvements were observed in 16 patients treated at Roswell Park Memorial Institute. Other sensitive tumors were lymphoma (63% response and improvements), squamous cell carcinoma of the head and neck (41% response and imporvements), and ovarian carcinoma (40% response and improvements). Complete responses were also seen in one patient with thyroid carcinoma and two with bladder carcinoma, while partial remissions were recorded in two patients with breast carcinoma and one patient each with acute myelogenous leukemia, endometrial carcinoma, renal carcinoma, malignant thymoma, neuroblastoma, adenocarcinoma of the lung, and an undifferentiated tumor of unknown origin. Five major types of toxicity were encountered: gastrointestinal, hematopoietic, immunosuppressive, otologic, and renal, with the last two generally the most serious. Serial audiometry testing can generally warn of the otologic toxicity and thus prevent permanent acoustic damage. Renal toxicity, which is similar to that seen with heavy-metal poisoning, appears to be dose related, cumulative, and only partly reversible, thus, severely limiting the repeated administration of cis-dichlorodiammineplatinum (II). Recent laboratory studies suggest that combination chemotherapy with this drug may be rewarding. Studies of this nature should be pursued along with attempts to find more effective less toxic platinum compounds.
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PMID:Review of the current clinical status of platinum coordination complexes in cancer chemotherapy. 110 40

Ninety-eight children with solid tumors resistant to conventional chemotherapy received adriamycin 90 mg/m2, either as a single intravenous injection or in 6 divided doses administered every 6 hours. Of the 88 evaluable children, 6 (7%) achieved a complete response and 26 (29%) achieved a partial response. Tumors which demonstrated significant response rates were: neuroblastoma (9/18), Wilms' tumor (7/13), rhabdomyosarcoma (4/11), and lymphoma (4/8). The toxicities observed with this regimen included: alopecia, leukopenia, thrombocytopenia, nausea, vomiting, stomatitis, febrile episodes, and ST-segment changes.
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PMID:Adriamycin in the treatment of childhood solid tumors. A Southwest Oncology Group study. 119 48

131I is the radionuclide most commonly used in biologically targeted radiotherapy at the present time. Microdosimetric analysis has shown that microtumors whose diameters are less than the beta-particle maximum range absorb radiation energy inefficiently from targeted radionuclides. Micrometastases of diameters < 1 mm are likely to be spared if targeted 131I is used as a single modality. Because of this, combined modality therapy incorporating targeted 131I, external beam total-body irradiation (TBI), and bone marrow rescue has been proposed. In this study, the minimum necessary TBI component is shown to depend on the radiosensitivity of the tumor cells. The analysis shows that the TBI component, to achieve radiocurability, increases directly with tumor radioresistance. For the most radiosensitive tumors, a whole-body TBI treatment dose 2 x 2 Gy is calculated to be obligatory, whereas practical arguments exist in favor of higher doses. For more radioresistant tumors, the analysis implies that a TBI treatment delivery of 5 x 2 Gy is obligatory. In all situations, external beam TBI appears to be an essential factor in providing reasonable probability of cure of disseminated malignant disease. Reasonable prospects of tumor cure by combination strategies incorporating 131I exist for the more radiosensitive tumor types (e.g., neuroblastoma, lymphoma, leukemia, myeloma, seminoma), but more resistant tumors are unlikely to be curable at present. Superior targeting agents, and the possible use of panels of different radionuclides, may be necessary to achieve high cure probabilities for less radiosensitive tumor types.
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PMID:Optimum combination of targeted 131I therapy and total-body irradiation for treatment of disseminated tumors of differing radiosensitivity. 128 26

Paranuclear blue inclusions (PBIs) are frequently identified within metastatic undifferentiated small cell carcinoma (SCC) cells on air-dried bone marrow aspirates stained with Wright's stain. To determine the sensitivity and specificity of this finding, 116 bone marrow aspirates containing metastatic neoplasms were evaluated for the presence and frequency of PBIs. Bone marrow specimens included 47 cases of metastatic SCC of the lung, 13 cases of large cell lymphoma, 19 cases of neuroblastoma, five cases of small, noncleaved cell lymphoma, seven cases of rhabdomyosarcoma, three cases of Ewing's sarcoma, three cases of other sarcomas, and 19 cases of non-small cell carcinoma (adenocarcinoma). PBIs were identified in 40 of 47 (85%) cases of SCC and their frequency varied from 0 to 24% of tumor cells among different cases. In approximately half the cases of SCC, PBIs were identified in 1 to 4% tumor cells; and in eight cases, PBIs were present in 5% or more of tumor cells. PBIs were also identified in two of seven (29%) cases of rhabdomyosarcoma and one case of malignant peripheral nerve sheath tumor, but they were not seen in Ewing's sarcoma, small non-cleaved cell lymphoma, large cell lymphoma, neuroblastoma, or non-small cell carcinoma. In addition, PBIs were not seen in alcohol-fixed, Papanicolaou-stained cytology specimens containing SCC. Ultrastructurally, PBIs may represent phagocytized nuclear/cellular material. PBIs are a feature of small cell carcinoma on air-dried, cytologic material stained with Romanowsky type stains. Their presence may provide diagnostic information with regard to the differential diagnosis of metastatic SCC in the bone marrow. Future studies evaluating non-bone marrow Wright's stained fine-needle aspiration specimens are needed to determine if PBIs are useful in distinguishing SCC from other poorly differentiated tumors in the cytology laboratory.
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PMID:Paranuclear blue inclusions in metastatic undifferentiated small cell carcinoma in the bone marrow. 128 55

Primitive neuroectodermal tumor (PNET) is a small round cell malignancy arising in soft tissue and bone, predominantly in older children and adolescents. We report the cytomorphologic features and findings of ancillary studies of eight fine needle aspiration (FNA) biopsies from three patients (7-year-old male, 12-year-old female, 9-year-old female). Two of the biopsies suggested the initial diagnosis of PNET of the chest wall, while the remaining six documented recurrent or metastatic disease. In one of these cases the primary diagnosis made by FNA biopsy enabled the pediatric oncologists to give specific therapy for the unresectable tumor and achieve remission. Local recurrences included the chest wall (two cases), pleura (one case) and pericardium (one case), while metastatic disease involved the supraclavicular lymph node and breast. All the cases consisted of small malignant cells with a high nuclear/cytoplasmic ratio and hyperchromatic nuclei without prominent nucleoli. Homer Wright rosettes were seen in only two of the aspirates, and neuropil and ganglion cells were not present. Ancillary studies, including electron microscopy (two cases), immunocytochemistry (four aspirates from two cases) and cytogenetics (11/22 translocation, one case) performed on the aspirated material were aids in making a specific diagnosis and excluded other small round cell tumors of childhood, such as malignant lymphoma, rhabdomyosarcoma and Ewing's sarcoma. The differential diagnosis between PNET and neuroblastoma can be difficult on the basis of an FNA biopsy alone, although light microscopic morphologic differences exist. Clinical features (e.g., age, primary site, metastatic patterns), catecholamine levels, electron microscopy and cytogenetics are necessary in establishing the correct diagnosis.
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PMID:Fine needle aspiration cytology of primitive neuroectodermal tumors. A report of these cases. 132 99


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