Embryonal rhabdomyosarcoma of urinary bladder in an adult.
Rhabdomyosarcoma is the commonest soft tissue sarcoma encountered in children.,  In a combined study of 231 cases of rhabdomyosarcoma representing all age groups, the incidence in the genitourinary system was 27 per cent., , , ,  However, rhabdomyosarcoma of the urinary bladder is exceedingly rare in adults.,  The first case was published in 1882 by Williams, in a 50 year old man. By 1964, less than 10 cases of rhabdomyosarcoma in adults were reported and two more were added by Jones and Oberman. Joshi et al reported one case in 1966 and reviewed 13 cases from the literature. In 1976, Krumerman and Katatikarn found only 14 cases reported in the American literature.
We have not come across a case report of rhabdomyosarcoma of urinary bladder in an adult in Indian literature. Such rarity of this tumour in adults has prompted us to report one case which we have come across recently.
A 32 year old female was admitted on 12th April 1981 with complaints of painless haematuria of 2 months' duration, and dysuria and frequency for 4-5 weeks. She had no history of similar illness in the past. She gave history of 2 full term normal deliveries. On examination, she was pale; the pulse and B.P. were within normal limits. She had a firm, nontender lump in the suprapubic region, measuring 7 cm x 6 cm x 4 cm which could not be moved in any direction.
Her haemoglobin was 6 g% and the urine showed frank blood. Blood urea nitrogen, serum creatinine and serum electrolytes were within normal limits. On cystoscopic examination, active bleeding was noted inside the bladder. A large mass was seen occupying most of the bladder cavity. Bimanual examination showed fixed mass infiltrating the bladder wall and the extravesical tissues. A biopsy of the mass was obtained.
Excretory urography showed good concentration and prompt excretion of the contrast by both the kidneys. No back pressure changes were seen. There was large filling defect in the urinary bladder. Biopsy report revealed rhabdomyosarcoma.
Radiographic examination of the chest and a total body radio-isotope scan using E.H.D.P. did not reveal any evidence of metastasis in the bones, lungs or the liver.
Total cystectomy with hysterectomy and ureterosigmoid anastomosis was performed on 27th April 1981. The tumour was adherent to the lateral pelvic wall along the lateral ligaments. The infiltrated perivesical tissue was excised along with the urinary bladder.
This patient is free from symptoms and has normal renal chemistry 8 months after the operation. Also there is no evidence of distant metastasis.
On opening, the bladder revealed a polypoid, fieshy tumour measuring 3" x 3". It was attached to the trigone by a broad base [ Fig. 1 ].
Microscopic Examination: [Fig. 2 ].
The tumour was composed of loosely arranged small spindle cells and occasional racket shaped cells with deeply acidophillic cytoplasm. The nuclei were hyperchromatic. The matrix was pale basophilic and acellular. Dense cellularity was present near the surface. P.T.A.H. stain revealed cross strations in a few cells. The, diagnosis was embryonal rhabdomyosarcoma.
Rhabdomyosarcoma of the urinary bladder occurs predominantly in males in ail agea.,  In Clatworthy's series, males outnumbered females by 3 to 1. Thirteen cases reviewed by Joshi et al in 1966 showed slight difference in sex incidence. In their series, there were 6 females and 8 males. The case reported in this communication was a female.
Painless gross haematuria with or without palpable mass is the common mode of presentation. Local spread is more common in this type of tumour than distant metastasis. One case of Ghavimi had metastasis in the lungs while one reported by Krumerman and Katatikarn had multiple metastases in the pelvis, the vertebrae and the lungs. Rhabdomyosarcoma of embryonal variety is a common tumour of childhood in patients under the age of 5 years. Those occurring in the adults are either pleomorphic or alveolar type.
The results of treatment of rhabdomyosarcoma are generally disappointing. Surgical excision usually results in local spread of the tumour and/or the appearance of distant metastasis. Radiotherapy has been given by some workers; however, there has been no evidence of any benefit because rhabdomyosarcoma is not a radiosensitive tumour. One case treated by Joshi et al had received radiotherapy following surgery but died 3 months later.
The prognosis of rhabdomyosarcoma of bladder appears to be grave in adults. Out of 14 cases reviewed from the literature, 11 died within 3 months of presentation and treatment. The longest survial was 7 years in one patient and the next two patients survived for 4 years and 6 months respectively.