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

Rhabdomyosarcoma of the prostate in children is rare. This is a case report of a six-year-old boy with rhabdomyosarcoma of the prostate who was admitted to our hospital, complaining of pain on urination and dysuria. Further examination revealed metastasis to the lungs and lymph nodes. He was treated with vincristine, actinomycin-D, cyclophosphamide, but this therapy was not effective. He died of respiratory failure due to the diffuse pulmonary and pleural metastasis of tumor, 41 days after admission. This is only the 9th reported case in Japan of rhabdomyosarcoma of the prostate in a child.
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PMID:[A case of rhabdomyosarcoma of the prostate in children]. 389 70

We describe the case of a 3-year-old girl who developed veno-occlusive disease of the liver while receiving chemotherapy for parameningeal rhabdomyosarcoma. After suffering lethargy and oral mucosal bleeding for one day, the patient exhibited a sudden weight gain and refractoriness to platelet transfusions. Symptoms rapidly worsened with elevation of liver enzymes, bleeding diatheses, and respiratory failure. An ultrasound scan of the liver demonstrated reversed flow in the portal vein. Maximal supportive care, including tracheal intubation and mechanical ventilation, was required. The patient gradually recovered with no respiratory and minimal neurological sequelae. Veno-occlusive disease of the liver should be considered in children receiving chemotherapy who develop weight gain, a sudden drop in platelet count and derangement of liver enzymes. Aggressive supportive measures should be instituted if necessary, as patients surviving the acute phase can expect to make a full recovery.
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PMID:Veno-occlusive disease of the liver after chemotherapy for rhabdomyosarcoma: case report with a review of the literature. 756 14

A 56-year-old male suffered from primary rhabdomyosarcoma on the left internal thoracic wall, which was treated by chemotherapy, and local irradiation following biopsy. Four months after the diagnosis, he suddenly complained of headache and left paresthesia occurred followed by generalized convulsion and left hemiplegia. CT scan revealed a high density mass in the right parietal lobe. The patient was referred to our department and underwent emergency evacuation of the hematoma together with tumor removal. The pathological specimen showed spindle or oval-like cells with hypercellularity and some mitotic figures. Immunohistochemical study demonstrated that many cells were positive for desmin and myoglobin, which is specific to myogenic tumor. These characteristics were compatible with those of the primary thoracic lesion, and a diagnosis of metastatic rhabdomyosarcoma was made. Five weeks after the craniotomy, the metastatic brain tumor recurred in the same site and also in the bilateral occipital lobes. Although radiotherapy to the brain decreased the tumor size, the patient died of respiratory failure eleven and a half months after the initial diagnosis. Sarcomas metastasizing to the brain are rare and only 16 cases of rhabdomyosarcoma metastasizing to the brain have been reported so far. Recent advances in chemotherapy, however, have been able to show the increased incidence of sarcoma metastasis to the brain. Therefore, the necessity of follow-up CT scan for sarcoma patients of long survival is to be stressed, even if the patient shows no neurological symptoms.
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PMID:[Brain metastasis of rhabdomyosarcoma with intratumorous hemorrhage: a case report and literature review]. 825 24

A rare case of carcinosarcoma (sarcomatoid carcinoma) with rhabdomyoblastic and osteoblastic differentiation occurring in the gastric remnant is reported. A 69-year-old Japanese man who had undergone a partial gastrectomy for a duodenal ulcer 30 years earlier, presented with anemia, epigastralgia, and an abdominal mass. The diagnosis of gastric carcinosarcoma was made based on the findings of endoscopic biopsies. The patient was thus scheduled to undergo a surgical operation, but he died of respiratory failure. At autopsy, a huge polypoid tumor measuring 20 x 18 x 8 cm was located on the greater curvature of the gastric remnant. Microscopically, the tumor consisted of intimately mixed tubular adenocarcinoma and heterologous mesenchymal elements containing rhabdomyosarcoma and osteosarcoma. Between these components, a morphological transition from the adenocarcinoma element to the sarcomatous element was observed. Ultrastructually, rhabdomyoblastic differentiation was confirmed in the sarcomatous areas. Immunocytochemical expressions of epithelial markers including epithelial membrane antigen and cytokeratins (35bH11 and 34bE12) were recognized not only in the carcinomatous cells but also in the sarcomatous cells. These findings suggest that carcinomatous cells appear to transform into cells with sarcomatous features.
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PMID:Gastric carcinosarcoma (sarcomatoid carcinoma) with rhabdomyoblastic and osteoblastic differentiation. 929 37

Pediatric patients undergoing induction regimens of chemotherapy may require intubation and mechanical ventilation either for respiratory failure or airway compromise as a complication of their therapy. We describe a case of difficult airway management resulting from pseudomembrane formation in a 16-yr-old girl. The patient was undergoing induction chemotherapy for stage IV rhabdomyosarcoma and developed severe mucositis that led to progressive airway obstruction.
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PMID:Mucositis and airway obstruction in a pediatric patient. 1528 4

We present an 8-year-old male with metastatic alveolar rhabdomyosarcoma (ARMS) who developed precipitous cardiopulmonary collapse with severe tumor lysis syndrome (TLS) 48 hr after initiation of chemotherapy. Despite no detectable pulmonary metastases, acute hypoxemic respiratory failure developed, requiring extracorporeal membrane oxygenation (ECMO). Although TLS has been reported in disseminated ARMS, this singular case of life-threatening respiratory deterioration developing after initiation of chemotherapy presented unique therapeutic dilemmas. We review the clinical aspects of this case, including possible mechanisms of respiratory failure, and discuss the role of ECMO utilization in pediatric oncology.
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PMID:Severe Tumor Lysis Syndrome and Acute Pulmonary Edema Requiring Extracorporeal Membrane Oxygenation Following Initiation of Chemotherapy for Metastatic Alveolar Rhabdomyosarcoma. 2671 72

Somatic hotspot DICER1 mutations, which frequently coexist with germline inactivating mutation (i.e., DICER1 syndrome), have been identified in various types of benign and malignant conditions. Herein, we report an autopsy case of prostatic rhabdomyosarcoma (RMS) with a hotspot DICER1 c.5125G>A (p.D1709N) mutation. A 26 year-old man presented with a prostatic mass, hematuria, and urinary retention. He underwent total pelvic exenteration, colostomy, ileal conduit construction and partial urethrectomy. Five months postoperatively, he developed multiple metastases to the lungs, brain, iliopsoas muscles and bones. He died of respiratory failure, and autopsy was performed. Microscopically, the tumor was primarily composed of uniform primitive mesenchymal cells infiltrating to the prostate with cambium layer. Rhabdomyoblasts and anaplastic cells were focally observed. Immunohistochemically, tumor cells were positive for desmin, myogenin, PAX7, HMGA2. Multinodular goiter was detected at autopsy. Because the morphology is similar to pleuropulmonary blastoma and DICER1-mutant RMS of the female genital tract, we tested and identified a hotspot DICER1 mutation with Sanger sequencing. Recognizing DICER1-mutant tumor is important because of its frequent association with germline DICER1 inactivation and potential therapeutic implication. Further research is needed to clarify whether this case can be classified as embryonal RMS with anaplasia or 'DICER1-associated sarcoma'.
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PMID:An autopsy case of prostatic rhabdomyosarcoma with DICER1 hotspot mutation. 3311 96