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)

Three children with rhabdomyosarcoma of the prostate treated with combination chemotherapy and radiation therapy underwent radical extirpation with continent diversion to the urethra. This urological procedure is technically feasible even in the small child (2 years old) and offers chance for cure. Radical cystectomy with continent diversion need not be considered as mutilating surgery.
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PMID:The use of continent diversion in the management of rhabdomyosarcoma of the prostate in childhood. 159 96

After high inguinal semicastration in group-1 paratesticular rhabdomyosarcoma (RMS), the patient having undergone chemotherapy can be followed closely by CT scanning without retroperitoneal lymphadenectomy. In contrast, retroperitoneal RMS should be operated on as radically as possible after downstaging the tumor mass. In RMS of the female genitalia locally limited organ-preserving surgery is the method of choice. The prognosis is excellent with adjuvant chemotherapy. Only 20% of all bladder RMS arise from the bladder dome or the movable part of the bladder, where primary partial resection including a safety margin of 3 cm of healthy tissue is possible. The majority, however, arising from the submucous tissue of the bladder base, trigonal area and bladder neck, infiltrates the prostatic urethra and the surrounding pelvic fascia. Therefore differentiation between primary bladder or prostate RMS is often doubtful. The current strategy of downstaging by chemotherapy or chemo-radiotherapy followed either by limited organ-preserving surgery or by a wait-and-see policy includes the high risk of residual tumor or local tumor recurrence, which has proved to have the worst prognosis of all. As organ-preserving therapy was recommended as the method of choice, mainly to avoid anterior pelvic exenteration and urinary diversion, the question of advantages in comparison to the risk of tumor recurrence is still open. The routine use of chemoradiotherapy and increasing follow-up has called attention to a variety of chronic sequelae, such as contracted bladders with reduced capacity and upper urinary tract deterioration, which subsequently require secondary urinary diversion. Progress in continent urinary diversion may be an alternative procedure for better life quality.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Extent of surgery in rhabdomyosarcoma of urogenital structures. 271 28

The authors report a case of rhabdomyosarcoma of posterior urethra they observed in a 10 year-old boy who was referred for an acute urinary retention. The story of the patient included other urinary tract troubles: macroscopic haematuria and stranguria had been observed when the boy was four year old and at that time cystography and cystoscopy grave normal results. In the following years the patient presented repeatedly episodes of haematuria, stranguria, dysuria and urinary tract infections. The authors stress the slow evolution of the tumor and the difficulties for diagnosing the disease in its early phase.
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PMID:[Unusual course of a case of urethral rhabdomyosarcoma in a 10-year-old boy]. 360 2

Only about 2% of the urinary tract are not of urothelial origin. Our knowledge of their morphology and biology is mainly based on single case reports, and therefore apart from a few exceptions very poor. Generally, the most often affected site is the urinary bladder (79.2%), followed by the urethra (12.7%), pelvis (4.9%) and ureter (3.2%). The urinary bladder also is the only organ in which all different histological types of these tumors were described. According to their histogenesis non-urothelial tumors (NUT) can be classified by the following main groups: soft tissue tumors, mixed epithelial and non epithelial tumors (carcinosarcomas), neuroendocrine carcinomas, carcinoids, malignant lymphomas, malignant melanomas and extragonadal germ cell tumors. Moreover some very interesting tumor-like lesions, like malakoplakia and inflammatory pseudosarcoma, mainly occur in this region. About 75% of all NUT of the urinary tract belong to the soft tissue tumors. Rhabdomyosarcomas in children and leiomyomas and -myosarcomas in adults are the more frequent histological types. Leiomyosarcomas can easily be confused with other tumor types or even with inflammatory pseudotumors. The use of immunohistochemistry to achieve a correct diagnosis is mandatory but not always successful. A relatively frequent tumor occurring in the bladder of young adults is the paraganglioma (pheochromocytoma), which usually produces typical symptoms of catecholamine excess. Carcinosarcomas of the urinary bladder contain both epithelial and mesenchymal components. They have to be distinguished from collision tumors (coexistent but separate carcinoma and sarcoma), spindle cell transitional carcinomas as well as from carcinomas with osseous or cartilaginous metaplasia. Carcinoids and neuroendocrine carcinomas developed from the neuroendocrine cells scattered all over the transitional epithelium of the bladder. Neuroendocrine carcinomas of the bladder are also called "oat cell carcinomas" since they show the same histological features and immunoreactivity as the oat cell carcinomas of the lung. They share also the same poor prognosis. The affection of the urinary tract in generalized malignant lymphomas and leukemias occur in more than 30% of cases. Lymphomas, primarily localised in the urinary bladder are, however, extremely rare. The most frequent ones are low grade non Hodgkin lymphomas, although 3 cases of Hodgkin disease and a few cases of primary extramedullary plasmacytoma of the bladder have been reported, too.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Non-urothelial tumors of the urinary tract]. 751 Dec 78

A patient with carcinosarcoma of the urethra occurring 15 years after a radical cystoprostatectomy for transitional cell carcinoma is presented. The tumor arose from the penile urethra and invaded the glans penis and penile corpus cavernosum. The tumor was composed of mixed carcinoma (squamous cell carcinoma, adenocarcinoma and transitional cell carcinoma) and rhabdomyosarcoma. Biopsy of the right inguinal lymph node showed simultaneous metastasis of sarcomatous and transitional cell carcinoma elements.
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PMID:Urethral carcinosarcoma following total cystectomy for bladder carcinoma. 846 Apr 43

Rhabdomyosarcoma is the most common soft tissue sarcoma, representing 10-15% of all malignant solid tumours in children and can occur in any site. The prognosis for rhabdomyosarcoma of the prostate has improved significantly in the last few decades. The management involves a combination of chemotherapy, radiotherapy and surgery. Several approaches to resect the prostate in adults have been described. These include radical retropubic, transperineal, posterior sagittal as well as the transpubic approach. In the child, the transpubic approach allows direct access to the prostate and proximal urethra whilst obviating problems noted in adults. We describe this approach to the prostate in a 3-year-old boy.
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PMID:Transpubic radical prostatectomy: a novel approach for rhabdomyosarcoma of the prostate in children. 1659 9

We describe a relatively rare case of botryoid rhabdomyosarcoma (RMS) in a 15-month-old female presenting with fever, abdominal pain, urinary retention, haematuria and a tumour protruding through the urethra. The diagnosis was verified by cystoscopy with biopsy and MRI. She was treated with combined chemotherapy and surgery. Early diagnosis and treatment are crucial to the prognosis. RMS should be considered as a differential diagnosis in cases whose symptoms include urinary retention, haematuria, urinary incontinence and atypical abdominal pain, and a primary ultrasound scan should be done. Cystoscopy and MRI should be considered, especially in cases involving atypical urological symptoms.
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PMID:[Rhabdomyosarcoma in the urogenital region of a child]. 1731 57