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 is the most common tumor of the lower genitourinary tract in children in the first 2 decades of life. Most cases of genitourinary rhabdomyosarcoma are of the embryonal histologic subtype and include tumors of the bladder, prostate, testes and paratesticular sites, penis, perineum, vagina, and uterus. The natural history, pattern of metastatic spread, treatment, and prognosis of childhood rhabdomyosarcoma vary with the anatomic site of the lesion. In children with rhabdomyosarcoma of the bladder or prostate, presenting signs and symptoms include urinary or fecal retention, dysuria, urinary tract infection, and hematuria. Paratesticular rhabdomyosarcoma produces painless scrotal swelling, which may be ignored until the tumor has reached a large size. Vaginal tumors may manifest as a prolapsing mass in the introitus. Radiologic studies of children with genitourinary rhabdomyosarcoma reflect the nonspecific gross features of the tumor, which may be ill defined with infiltrative margins or well circumscribed by a pseudocapsule of compressed tissue. The botryoid variant of embryonal rhabdomyosarcoma results when submucosal tumor produces a polypoid mass resembling a cluster of grapes within a hollow structure. Botryoid morphology is characteristic, but not specific, for rhabdomyosarcoma within the vagina or urinary bladder, since yolk sac tumor and "tumoral" cystitis may have a similar appearance. Invasion of adjacent structures by the primary tumor may make the precise anatomic origin of genitourinary rhabdomyosarcoma difficult to determine on cross-sectional images. Recent refinements in multidisciplinary therapeutic regimens combining chemotherapy, radiation therapy, and surgery have dramatically improved outcome for children with genitourinary rhabdomyosarcoma. Diagnostic imaging plays an important role in monitoring response to therapy.
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PMID:From the archives of the AFIP. Genitourinary rhabdomyosarcoma in children: radiologic-pathologic correlation. 922 91

Ifosfamide is an active drug in the therapy of paediatric tumours such as rhabdomyosarcoma, Ewings' sarcoma, Wilms' tumour, neuroblastoma, germ cell tumours and lymphomas. Myelosuppression is the major toxicity along with haemorrhagic cystitis. The latter is largely prevented by the use of concomitant mesna.
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PMID:The role of ifosfamide in paediatric cancer. 967 60

Eosinophilic cystitis, an uncommon lesion, is rare in children; < 25 cases have been reported. The intense inflammatory changes in the bladder wall associated with this lesion may produce heaped-up excrescences, which resemble vesical rhabdomyosarcoma. Our experience with 3 patients shows that the initial diagnosis of eosinophilic cystitis may not be easily made, and that the lesions produced may mask other disease processes. We alsoreport the fifth case of eosinophilic infiltration of the bladder occurring in association with chronic granulomatous disease.
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PMID:Extensive inflammatory eosinophilic bladder tumors in children: experience with three cases. 1235 7

A 3-year-old boy presented with a single episode of gross hematuria and no history of previous urinary tract disorder. Imaging studies revealed a large complex polypoid filling defect in the bladder lumen. Several attempts at transurethral biopsy and cytological examination of the urine revealed clumps of benign epithelial cells, but suspicion of a malignant neoplasm such as rhabdomyosarcoma remained high and the lesion was resected. The specimen measured 15 cm, had a narrow zone of attachment to the bladder mucosa, and was grossly botryoid. Changes typical of cystitis cystica et glandularis were present at and near all surfaces. Myxoid stroma contained scattered benign fibroblasts, myofibroblasts, and smooth muscle cells. Inexplicably, one of two karyotyped stromal cells demonstrated a translocation usually associated with rhabdomyosarcoma. This child is well without evidence of bladder abnormality 1.5 years after surgery.
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PMID:Giant botryoid fibroepithelial polyp of bladder with myofibroblastic stroma and cystitis cystica et glandularis. 1257 20

From March 1991 through 31st December 2007, 2042 patients underwent stem cell transplantation at the Hematology-Oncology and Stem Cell Transplantation Research Center, affiliated to Tehran University of Medical Sciences. These transplantations included 1405 allogeneic stem cell transplantation, 624 autologous stem cell transplantation, and 13 syngeneic stem cell transplantation. Stem cell transplantation was performed for various diseases including acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, thalassemia major, sickle cell thalassemia, sickle cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combine immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. We had 105 cellular therapies for postmyocardial infarction, multiple sclerosis, cirrhosis, head of femur necrosis, and renal cell carcinoma. About 30 patients were retransplanted in this center. About 74.9% of the patients (1530 of 2042) remained alive between one to 168 months after stem cell transplantation. Nearly 25.1% (512 of 2042) of our patients died after stem cell transplantation. The causes of deaths were relapse, infections, hemorrhagic cystitis, graft versus host disease, and others.
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PMID:Stem cell transplantation; Iranian experience. 1911 Oct 33

Although not uncommon in adults, bladder tumors are rare in children. In addition, the histologic types of tumors seen in the pediatric population differ from those seen in adults. Although rhabdomyosarcoma is the most common pediatric bladder tumor, many other benign, malignant, and reactive lesions can be encountered. All may present clinically as a mass or polyp in the bladder. This study was designed to describe the pathology and patient demographics of pediatric bladder masses, because there are few studies describing these entities. Retrospectively reviewing our experience over a 21-year period, we identified 98 specimens from 65 patients with polyps or masses in the urinary bladder. As expected, the most frequent diagnosis was rhabdomyosarcoma. This was followed by fibroepithelial polyp and a variety of additional nonurothelial tumors. Only 7 urothelial tumors were identified, including 1 low-grade papillary urothelial carcinoma. Inflammatory lesions, such as cystitis cystica and nephrogenic adenoma, were invariably associated with an irritating factor when a history was provided. Our findings emphasize that diagnoses made in the pediatric urinary bladder are distinct from those in adults, although a wide variety of lesions may still be seen.
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PMID:Polyps and masses of the pediatric urinary bladder: a 21-year pathology review. 2179 66

A retrospective study of the clinical presentation, diagnosis, and treatment of eight patients whose common characteristic was an exophytic, solid urinary bladder lesion (botryoid rhabdomyosarcoma, transitional-cell carcinoma, nephrogenic adenoma, cystitis cystica, von Brunn's nests, hemangioma, and squamous-cell metaplasia) is presented. Exophytic bladder lesions, some of which are of great importance, are rare entities in childhood and present nonspecific clinical pictures. Therefore, whenever a space-occupying lesion is detected on a complementary study of the lower urinary tract, the differential diagnosis should be considered. Advantages of each of the currently available diagnostic methods are discussed and the essential role of cystoscopy is stressed.
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PMID:Exophytic urinary bladder lesions in childhood. 2405 39


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