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)

Using a database comprising 13,266 cytogenetically abnormal neoplasms, the geographic heterogeneity of neoplasia-associated chromosomal abnormalities was investigated by comparing the frequencies of characteristic aberrations in consecutive series of patients with the same diagnosis. Significant frequency differences between geographic areas were found for the aberrations +8, i(17q), +19, and an additional Ph1 chromosome in chronic myeloid leukemia (CML); -5, 5q-, and +8 in acute nonlymphocytic leukemia (ANLL); t(8;21) in ANLL-M2; t(15;17) in ANLL-M3; 5q- and -7 in myelodysplastic syndromes (MDS); t(9;22) and +21 in acute lymphocytic leukemia (ALL); t(14;18) in follicular lymphoma; -8 and -22/22q- in meningioma; and structural abnormalities of 12q in pleomorphic adenoma of the salivary glands (PAS). No geographic incidence variation was detected for -7 and +21 in ANLL; +8 in MDS; 6q- and +8 in ALL; +12 in chronic lymphocytic leukemia; 6q- in non-Hodgkin's lymphoma (NHL); t(8;14) in Burkitt's lymphoma; t(11;22) in Ewing's sarcoma; i(12p) in germ cell tumors; 1p- in neuroblastoma; structural abnormalities of 3q, 8q, and 9p in PAS; or 3p- in renal cell carcinoma. Intraregional frequency similarities between cytogenetically identical abnormalities in related tumor types were also analyzed. No significant correlations were found regarding the incidence of 5q- in ANLL and MDS, 6q- in ALL and NHL, -7 in ANLL and MDS, +8 in ANLL and CML, +8 in ANLL and MDS, +8 in ALL and ANLL, or +21 in ALL and ANLL. The findings indicate that some geographic heterogeneity of tumor-associated aberrations exists both in hematologic neoplasms and in solid tumors.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Geographic heterogeneity of neoplasia-associated chromosome aberrations. 195 98

With increasing survival rates of children grafted for different malignancies concerns about the longterm side effects of this treatment are growing. Therefore, investigations on the function of endocrine systems were conducted in a total 28 patients grafted for various reasons: ALL (N = 18), AML (N = 1), SAA (N = 3), CML(N = 4), neuroblastoma (N = 2). The results can be summarized as follows: 1. The extent of hormonal derangements is primarily dependent on the extent of irradiation prior to BMT. Integrity of hormonal systems was found in cases without irradiation (SAA) or if TBI did not exceed 3 Gy. 2. Primary hypogonadism was present in 18 patients. 3. Primary hypothyroidism was present in 2 patients. 4. Growth impairment was observed in 8 patients. In four of these cases growth hormone deficiency was the cause. In four other cases with graft-versus-host-disease and hepatic involvement SmC/IGF I levels were severely diminished. The data suggest that in most cases BMT itself has relatively few negative effects on the endocrine regulatory system. However, more detailed investigations before and after BMT will be needed to further validate these observations.
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PMID:Influence of allogeneic bone marrow transplantation on the endocrine system in children. 248 Mar 5

Many cancers have been cured by chemotherapeutic agents. However, other cancers are intrinsically drug resistant, and some acquire resistance following chemotherapy. Cloning of the cDNA for the human MDR1 gene (also known as PGY1), which encodes the multidrug efflux protein P-glycoprotein, has made it possible to measure levels of MDR1 RNA in human cancers. We report the levels of MDR1 RNA in greater than 400 human cancers. MDR1 RNA levels were usually elevated in untreated, intrinsically drug-resistant tumors, including those derived from the colon, kidney, adrenal gland, liver, and pancreas, as well as in carcinoid tumors, chronic myelogenous leukemia in blast crisis, and cell lines of non-small cell carcinoma of the lung (NSCLC) with neuroendocrine properties. MDR1 RNA levels were occasionally elevated in other untreated cancers, including neuroblastoma, acute lymphocytic leukemia (ALL) in adults, acute nonlymphocytic leukemia (ANLL) in adults, and indolent non-Hodgkin's lymphoma. MDR1 RNA levels were also increased in some cancers at relapse after chemotherapy, including ALL, ANLL, breast cancer, neuroblastoma, pheochromocytoma, and nodular, poorly differentiated lymphoma. Many types of drug-sensitive and drug-resistant tumors, including NSCLC and melanoma, contained undetectable or low levels of MDR1 RNA. The consistent association of MDR1 expression with several intrinsically resistant cancers and the increased expression of the MDR1 gene in certain cancers with acquired drug resistance indicate that the MDR1 gene contributes to multidrug resistance in many human cancers. Thus, evaluation of MDR1 gene expression may prove to be a valuable tool in the identification of individuals whose cancers are resistant to specific agents. The information may be useful in designing or altering chemotherapeutic protocols in these patients.
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PMID:Expression of a multidrug resistance gene in human cancers. 256 56

Association with family history of cancer of each site was examined for each type of childhood malignancies using data of National Childhood Malignancy Registry in Japan as materials (n = 16,555). 2,926 cases were with cancer family history. Family history of same type of malignancy was found significantly in excess for leukemia, a malignant lymphoma, brain tumor, neuroblastoma and retinoblastoma. When observed by cell type, association with family history of leukemia was most striking in acute myeloid leukemia. Median age at first diagnosis of retinoblastoma was 11 months earlier when family history of retinoblastoma existed. Family history of leukemia and history of exposure to prenatal radiation exposure was found to enhance relative risk for childhood leukemia when combined, suggesting existence of genetic environment interaction. Mode of interaction was interpreted as multiplicative.
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PMID:[Interaction of cancer family history and environmental factors in the risk of cancer--with special reference to childhood malignancies]. 303 33

In summary, carcinoma is the most frequent cancer that metastasizes to the skin; lung cancer in men and breast cancer in women. Clinically distinctive patterns of cutaneous metastasis of epithelial origin include alopecia neoplastica, pulsatile nodules, Sister Mary Joseph's nodules, morpheaform, and cellulitis-like lesions. Biopsying these lesions reveals adenocarcinoma, squamous cell carcinoma, or anaplastic carcinoma. The type of histologic pattern seen can be a clue to the organ of origin giving rise to the cutaneous metastasis. Skin that is damaged allows for circulating malignant cells, often of epithelial or leukemic origin, to lodge and proliferate locally (inflammatory oncotaxis). The commonest form of leukemia to affect the skin of elderly males is chronic lymphocytic leukemia. However, when leukemia involves the mucous membranes, acute myeloid leukemia (acute monocytic and acute myelomonocytic leukemia) is the most likely diagnosis. When papules, nodules, or plaques develop on the head, neck, or torso in a middle-aged male accompanied by lymphadenopathy, there must be a high index of suspicion that these lesions are metastatic lymphomatous deposits. Definitive histologic diagnosis on a skin biopsy specimen is difficult. In this situation, it is best to rely on histologic patterns seen in lymphoid tissue along with cellular marker studies. An elderly patient having bone pain, anemia, elevated blood calcium level, and renal failure along with purplish or skin-colored nodules and plaques on the trunk has a good chance of having multiple myeloma. Biopsying these lesions is most certain to reveal atypical plasma cells, and blood immunoelectrophoresis will demonstrate characteristic monoclonal gammopathy. There are two malignancies seen in children under 3 years of age that often times affect the skin in a characteristic fashion. Letterer-Siwe disease, which is distinguished from other histocytic disorders by its cell of origin, the Langerhans cell, clinically shows maculopapular and erosive lesions distributed in a seborrheic pattern. Neuroblastoma derived from cells of the neural crest demonstrates clinically widespread bluish papulonodules. Kaposi's sarcoma, a multifocal vascular malignancy, has a wide spectrum of clinical expression. Those patients who are immunocompromised secondary to concomitant disease or immunosuppressive therapy are more susceptible to a disseminated fulminant course accompanied by opportunistic infection. In conclusion, although specific signs of internal malignancy are less common than nonspecific ones, they are just as important; if the clinician managing the cancer patient is familiar with these clues to internal disease, proper patient management will ensue.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Specific cutaneous manifestations of internal malignancy. 307 47

Within the last decade, significant advances have been made both in treating children with cancer and in providing proper nutrition support. Oncologic treatment and nutrition research and their application to the nutrition care of children with cancer are reviewed. Quality nutrition care is now possible because of an improved understanding of (a) the prevalence and significance of protein-energy malnutrition (PEM) in high-risk groups, (b) the staging and assessment of nutritional status, and (c) the efficacy and limitations of nutrition support options. Nutrition staging, assessment, and support should be integrated into treatment protocols for children with neoplastic diseases. Common risk factors for the development of PEM have been identified from serial monitoring of newly diagnosed children with a variety of tumors. Certain tumor types and their treatment can be classified within either low or high nutritional risk groups. A comprehensive nutrition program (intense nutrition counseling, favorite nutritious foods) is preferred for low nutritional risk groups but is ineffective in preventing or reversing PEM in high-risk groups. For high-risk patients, central parenteral nutrition (CPN) is the method of choice as a relatively short-term but important support measure that allows children to withstand long intervals of intense treatment during periods of growth and development. Current data suggest that bone marrow suppression may be attenuated and treatment tolerance improved with the use of CPN in selected children with advanced cancer (e.g., acute nonlymphocytic leukemia or advanced neuroblastoma).
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PMID:Advances in nutrition care of children with neoplastic diseases: a review of treatment, research, and application. 309 11

Children undergoing ABMT, a procedure which entails massive doses of chemotherapy along with total-body irradiation, are candidate to develop severe gastrointestinal toxicity and prolonged anorexia requiring administration of Parenteral Nutrition (PN) for variable periods. We report a series of 35 consecutive children affected by malignancies who underwent 37 courses of PN after ablative therapy followed by ABMT. Age ranged from 8 months to 17 years; 16 were females, 19 males. There were 23 cases of neuroblastoma, 5 of Wilms' tumor, 3 of acute myelogenous leukemia, 2 of Ewing's sarcoma, 1 case each of rhabdomyosarcoma and acute lymphoblastic leukemia. All patients developed severe neutropenia for 9-42 days (median 18 d). Fever occurred in all patients; sepsis was documented in 10. Duration of PN ranged from 10 to 64 days (23 +/- 9; mean +/- SD). PN solution, containing crystalline L-Aminoacids (8.5%) mixed with 33% glucose, minerals, trace elements and vitamins provided for children a caloric intake of 49.8 +/- 17.3 Kcal/Kg/day with a nitrogen intake of 0.26 +/- 0.27 g/Kg/day. Nutritional assessment, utilizing percent ideal body weight, serum protein electrophoresis, C3, pseudocholinesterase and fibrinogen, was performed at the beginning and at the completion of each course of PN. Mean percent ideal body weight was 95.8 before PN, 98.5 on last day of PN (p less than 0.0005). Other parameters did not change significantly. No metabolic complication nor severe electrolyte imbalance were observed except for 5 patients who developed hypokalemia in coincidence with administration of Amphotericin B.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Autologous bone marrow transplantation in children. Use of parenteral nutrition]. 311 38

The medical records of 973 previously untreated patients diagnosed between January 1960 and December 31, 1978 with childhood cancer were reviewed. Siblings in 13 families were diagnosed with cancer 9/12 to 15 years after the diagnosis of cancer in the index sibling. Previously unreported association of acute lymphoblastic leukemia with Hodgkin's disease, neuroblastoma with malignant hemangiopericytoma, non-Hodgkin's lymphoma with malignant melanoma, Wilms' tumor with non-Hodgkin's lymphoma, Hodgkin's disease with malignant teratoma of the testis and craniopharyngioma with acute myeloblastic leukemia were identified. Two families appeared to transmit a predisposition to childhood tumors. The data from these families extend previous observations regarding multiple cases of cancer in sibships.
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PMID:Childhood cancer in siblings. 315 35

The fine needle aspiration (FNA) cytologic findings in 18 cases of metastatic neoplasms of the breast are reported. The cases were encountered in a combined series of 2,529 FNA breast biopsies, of which 666 were malignant; the metastatic neoplasms of the breast thus constituted 2.7% of all the malignant breast tumors. The series consists of 15 women and 3 men, with a mean age of 48 years (range of 11 to 73 years). Sixteen biopsies confirmed metastatic malignancy in patients with known extramammary primaries; the prebiopsy clinical diagnoses in six of the patients were benign breast lesions. In eight patients, the clinical differential diagnosis was either a benign or malignant primary breast lesion versus a metastatic malignancy. In two additional patients, the FNA biopsy identified metastatic neoplasms from unsuspected extramammary primaries. The metastatic neoplasms included three small-cell carcinomas of the lung, one squamous-cell carcinoma of the lung, two malignant melanomas, three ovarian malignancies, including a dysgerminoma, and one each of carcinoma of the fallopian tube, endometrial carcinoma, transitional-cell carcinoma of the urinary bladder, prostatic carcinoma, acute granulocytic leukemia, lymphoma, mycosis fungoides, hepatoma and neuroblastoma of the retroperitoneum. Recognition of unusual cytologic patterns raised the suspicion of, or confirmed the diagnosis of, malignancy in all cases, with no false-negative diagnoses. None of the cases were cytologically interpreted as a primary breast malignancy. Ancillary studies performed on the FNA material, including immunocytochemistry, contributed to a definitive diagnosis in three cases. FNA diagnosis of metastatic malignancy of the breast is essential in order to avoid unnecessary mastectomy and to ensure appropriate chemotherapy and/or irradiation treatment.
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PMID:Fine needle aspiration cytology of neoplasms metastatic to the breast. 347 62

Autologous bone marrow transplantation (ABMT) is a new technique which is currently being evaluated in the treatment of leukemias, lymphomas, and a few solid tumors. In patients with acute leukemia (AL), high dose therapy + ABMT is of little benefit if done at time of relapse. On the other hand, when used for consolidation of remission, either with cleansed or non cleansed marrow, it may improve disease-free survival. In patients with acute myelocytic leukemia (AML) autografted during their 1st remission, the probability of remaining in remission at 2 years is 70 p. 100. It is slightly lower for patients with acute lymphocytic leukemia (ALL): 55 p. 100. The different techniques of cleansing the marrow, monoclonal antibodies, immunotoxins, drugs, are reviewed in this paper. A comparison of these techniques in term of tumor log cell kill is provided. ABMT is the best second line therapy for non-Hodgkin lymphomas (NHL), either after relapse after conventional chemotherapy or partial failure (partial remission). In these patients, the probability of remaining in remission at 3 years is about 50 p. 100. ABMT is currently under trial in the treatment of solid tumors and some success has been obtained in carcinoma of the ovary, non-seminomatous tumor of the testis, neuroblastoma, and some selected breast cancers.
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PMID:[Autograft of bone marrow for the treatment of acute leukemia: in vitro efficacy of anti-leukemic purification]. 352 33


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