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

Since 1978, six boys with prostatic rhabdomyosarcoma have been treated at our institution. Three had localized disease and were managed by initial biopsy, vincristine, actinomycin-D, and cyclophosphamide (VAC) chemotherapy, and bladder-sparing surgery with or without irradiation. Further combination chemotherapy ("pulse" VAC, Adriamycin, VP-16, cisplatin, and ifosfamide) was continued for 20 to 22 months following the induction course. Two boys had microscopic residual disease undetected by frozen section and unresponsive to radiotherapy. Subsequent total cystectomy 4 and 7 months later resulted in eradication of disease. In one patient, preservation of the bladder was achieved at the age of 3 months for 8 years. Artificial sphincter inserted to cure his urinary incontinence failed because of ischemia secondary to cuff compression and scar tissue. He is alive today with a modified Koch pouch urinary diversion. Of the 50% who had metastatic disease at presentation, two were dead within 12 months despite aggressive chemotherapy and irradiation. The third is currently on treatment. Although chemotherapy has markedly improved the prognosis, surgery is still necessary in most cases for cure. Bladder salvage is a desirable goal; however, residual microscopic disease, difficulty with frozen-section disease detection, and poor tissue vascularization for subsequent sphincter replacement remain significant obstacles.
 ...
PMID:Rhabdomyosarcoma of the prostate in childhood: current challenges. 280 70

The long-term follow-up is presented of three infants with vulvovaginal rhabdomyosarcoma treated at 10, 11, and 15 months of age by radical surgery, pelvic radiation therapy, and chemotherapy. Two patients developed colonic stricture requiring operative repair. One of these two patients also has urinary incontinence secondary to severe bladder contraction. Pelvic radiation did not significantly affect stature. Lateral transposition of the ovaries at the time of surgery preserved endocrine function in one patient with a normal puberty. The other two patients developed normal secondary sexual characteristics with hormonal replacement therapy. These patients perform well academically and socially. They have adapted 14, 14, and 16.5 years after their therapy.
 ...
PMID:Long-term follow-up and complications of infants with vulvovaginal embryonal rhabdomyosarcoma treated with surgery, radiation therapy, and chemotherapy. 334 30

A vaginal rhabdomyosarcoma which occurred in an 11-month-old infant was treated with conservative surgical excision, radiation therapy and chemotherapy. The long-term sequelae of this therapy included rectal stenosis, which required surgical correction, and urinary incontinence. The patient may have had fewer long-term complications if a more aggressive surgical procedure had been performed at the time of diagnosis because a gross and microscopically complete excision would have obviated the need of postoperative pelvic irradiation. Strong consideration of total surgical excision should be given to infants with pelvic rhabdomyosarcoma.
 ...
PMID:Incontinence of urine and stool following treatment in infancy for embryonal rhabdomyosarcoma. 682 95

Between January 1985 and June 1995 a total of 12 patients (9 female, 3 male) underwent total reconstruction of the lower urinary tract using gastric tissue. Their mean age was 10 years (range, 5-25 years). Total gastric bladder substitution was performed in seven patients, whereas five other patients had composite continent reservoirs (stomach plus bowel) created. The diagnoses were cloacal exstrophy, classic bladder exstrophy, bladder rhabdomyosarcoma, bilateral ectopic ureters, and VACTERL association. The mean follow-up period was 4.5 years (range, 16 months to 9 years). The average bladder capacity was 309 ml, and the compliance averaged 12.9 ml/cmH2O. Continence was achieved in all patients, but the continence mechanism often required revision. Renal deterioration was noted only in one patient due to an obstruction at the site of a transureteroureterostomy. The complications included hyponatremic, hypochloremic metabolic alkalosis (two patients); prolapse of the neovagina (one patient); a ureterovesical junction obstruction (three patients); and revision of the neourethra or Mitrofanoff (six patients). Revision of the gastric tube used as the catheterizable channel was performed in three instances. Lengthening of the tube, including nippling in one instance, corrected persistent incontinence. Distal stenosis of a gastric tube required several plastic procedures for correction. A ureteral Mitrofanoff developed a distal stricture necessitating revision. Persistent leaking of an appendiceal Mitrofanoff was corrected with reimplantation. A tapered ileal neourethra required a tunneling procedure followed by Teflon injection to provide urinary continence. Complications seen most often involved the continence mechanism of these gastric neobladders or composite urinary reservoirs. Persistence, patience, and perseverance led to total urinary continence and adequate reservoirs for urine storage in all patients.
 ...
PMID:Continent gastric pouch. 873 Nov 28

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.
 ...
PMID:[Rhabdomyosarcoma in the urogenital region of a child]. 1731 57

Traditionally, the standard treatment of bladder-prostate rhabdomyosarcoma (BP-RMS) is being implemented to be total cystoprostatectomy and urinary diversion. However, current multimodal treatment approaches emphasize the importance of bladder-sparing surgery. In this case series, it was aimed to indicate the results of the laparoscopic bladder-sparing approach of two pediatric patients with BP RMS. They have admitted to the emergency department due to acute urinary retention (AUR). The tumors located in the prostate causing AUR were detected by Magnetic resonance imaging (MRI) and the pathological diagnosis was confirmed by biopsy. The patients were managed in a prosperous manner by implementing laparoscopic surgery with the cause of detection of a significant decrease in the size as well as the enhancement pattern of the tumors following neoadjuvant chemotherapy. No urinary incontinence, tumor recurrence or metastasis was observed at 36 and 28 months follow-up in case 1 and case 2, respectively. Laparoscopic bladder-sparing approaches may have an advantage in patients with BP RMS to decrease morbidity and mortality related to radical surgery. To our knowledge, these are the first cases of laparoscopic bladder-sparing approach in the treatment of pediatric prostate-derived embryonal RMS (PDERMS).
 ...
PMID:Laparoscopic Bladder-Sparing Approach in Patients with Prostatic Rhabdomyosarcoma: A Case Series of Two Pediatric Patients. 3132 49