Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The prognosis and the quality of life of patients with carcinoid tumors is related either to symptoms from the substances secreted or to progressive tumor growth. Medical treatment with cytotoxic agents is of marginal value for increasing life expectancy and reducing clinical symptoms. Recent studies with interferon have shown interesting results. In the present investigation, 22 patients with carcinoid tumors and syndrome were treated with recombinant interferon alpha-2a (r-IFN alpha-2a) at the dose of 6 x 10(6) IU intramuscularly daily for 8 weeks and three times weekly thereafter. The primary tumor was localized in the foregut (n = 11), midgut (n = 7), hindgut (n = 1), and unknown site (n = 3). Most cases had liver metastasis. Seventeen patients had elevated 5-hydroxyindoloacetic acid (5-HIAA) excretion and 5 had flushing and/or diarrhea as the only clinical manifestation. Six cases presented a complete syndrome (flushing, diarrhea and 5-HIAA excretion). Control of symptoms was obtained in 80% and a 5-HIAA level reduction in 58% of the patients. The interferon treatment was more effective for control of the carcinoid syndrome than for control of tumor growth. The treatment was well tolerated and fever, myalgia, anorexia and fatigue were the most frequent side-effects.
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PMID:Treatment of carcinoid syndrome with recombinant interferon alpha-2a. 768 66

A 16-year-old boy with generalized myalgia and petechial hemorrhage was found to have a diffuse infiltrative disease in his bone marrow (BM). The BM aspirate contained a dense population of vacuolated blast-like cells. The BM biopsy displayed compact sheets of small round cells with clear cytoplasm, reminiscent of Ewing sarcoma. Immunostains were not diagnostically conclusive while transmission electron microscopy on the BM cells demonstrated a clear skeletal muscle differentiation. The morphologic findings led to a tentative designation of metastatic embryonal rhabdomyosarcoma (RMS). It was not until cytogenetic analysis revealed the specific translocation t(2;13)(q35;q14) did the alveolar RMS finally get confirmed. Despite an exhaustive search by imaging studies, a primary tumor was not detected. This case illustrates that the massive BM involvement by atypical alveolar RMS of unknown origin may pose serious diagnostic challenges. Multidisciplinary studies are required to reach a definitive diagnosis.
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PMID:Massive bone marrow involvement by clear cell variant of rhabdomyosarcoma. 1912 93

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children younger than 15 years. According to the World Health Organization, there are embryonal, alveolar and pleomorphic types of RMS. Most RMS patients present with a tumor mass in the head and neck region, urogenital tract or lower extremities. Unusual clinical presentation of the disease with massive bone marrow infiltration at the disease onset and mimicking hematologic neoplasm is rarely seen. A case is presented of a 14-year-old, previously healthy girl hospitalized for outpatiently detected leukocyte elevation. For the last two weeks, she had complained of fatigue, myalgia and frequent bruising. On admission, clinical examination revealed numerous petechiae and hematomas, enlarged left inguinal lymph node and palpable spleen 2 cm below left costal arch. Laboratory findings showed leukocytosis, anemia and thrombocytopenia. Bone marrow fine needle aspiration (FNA) produced a hypercellular bone marrow sample with suppression of all three hemocytopoiesis lines and bone marrow infiltration with numerous undifferentiated tumor cells. Considering the morphological, cytochemical and phenotypic characteristics, the cytologic diagnosis was: bone marrow infiltration with RMS cells. Abdominal computerized tomography revealed a primary tumor occupying the entire retropeoritoneal space. Tumor biopsy confirmed alveolar subtype of RMS. In conclusion, in cases of bone marrow infiltration with primitive, immature cells, RMS should be considered as differential diagnostic possibility. Adjuvant technologies (cytochemistry, immunocytochemistry, cytogenetic analysis, flow cytometry, and molecular analysis) can be very helpful in diagnostic work-up, and may lead to definitive diagnosis in some cases.
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PMID:Rhabdomyosarcoma with bone marrow infiltration mimicking hematologic neoplasia. 2069 43