| Abstract|| |
We report a case of botryoid rhabdomyosarcoma with cartilaginous differentiation in the urinary bladder of a 24-year-old male. The tumor possessed the diagnostic histologic and immuno-histochemical characteristics of botryoid rhabdomyosarcoma. Foci of immature cartilage were noted within the tumor. Cartilaginous differentiation in rhabdomyosarcoma has been reported in tumors of the uterine cervix only. To the best of our knowledge, this is the first case reported of botryoid rhabdomyosarcoma with cartilaginous differentiation in the urinary bladder of an adult.
Keywords: Botryoid rhabdomyosarcoma, Immature cartilage, Urinary bladder.
|How to cite this article:|
Al-Shedoukhy A, Qayyum A. Urinary Bladder Botryoid Rhabdomyosarcoma with Immature Cartilage in a 24-year-old Male Patient: A Case Report. Saudi J Kidney Dis Transpl 2003;14:522-5
|How to cite this URL:|
Al-Shedoukhy A, Qayyum A. Urinary Bladder Botryoid Rhabdomyosarcoma with Immature Cartilage in a 24-year-old Male Patient: A Case Report. Saudi J Kidney Dis Transpl [serial online] 2003 [cited 2020 Sep 21];14:522-5. Available from: http://www.sjkdt.org/text.asp?2003/14/4/522/32993
| Introduction|| |
Rhabdomyosarcomas vary widely in histological appearance depending on the degree of cellular differentiation, cellularity and growth pattern. Embryonal rhabdomyosarcoma closely resembles the various stages in the development of normal muscle tissue. Botryoid rhabdomyosarcoma is a variant of embryonal type which accounts for no more than 5 to 10% of all rhabdomyosarcomas.  This tumor is frequent in mucosa-lined hollow visceral organs like vagina or urinary bladder. Occasionally, the embryonal rhabdomyosarcoma displays, in addition to its rhabdomyoblastic component, foci of immature cartilage. Daya and Scully observed cartilaginous differentiation in 45% of rhabdomyosarcomas of the uterine cervix.  Perrone et al also reported a case of heterologous cartilage of uterine cervix in a young female.  Yasui et al reported a case of rhabdomyosarcoma of the urinary bladder in an adult.  A Medline search did not reveal even a single case of botryoid rhabdomyosarcoma of the urinary bladder with cartilaginous differentiation. To our knowledge, ours is the first reported case of urinary bladder botryoid rhabdomyosarcoma with immature cartilage in an adult.
| Case Report|| |
A 24-year-old male patient was admitted to a private hospital with a complaint of hematuria of eight months duration. Cystoscopy and bladder biopsy done previously were reportedly normal. The patient continued to have hematuria. A recent ultrasound examination, done in a private hospital, showed a huge urinary bladder tumor. The patient was then referred to the King Khalid University Hospital, Riyadh for further management.
Past medical history was unremarkable. However, the patient's father had died due to lymphoma and his uncle and one other relative were also treated for lymphoma. On examination, the patient was afebrile and the vital signs were stable. Cardiovascular, respiratory and abdominal examinations revealed no abnormality. Results of complete blood count and other routine investigations were within normal limits. Urine examination showed many red blood cells (RBC) while cytological examination showed RBC, leukocytes, few benign urothelial cells and groups of atypical urothelial cells.
A computerized tomographic scan showed a large heterogeneous mass invaginating within the urinary bladder which showed different tissue characters extending from cystic, solid and calcific component. The bladder wall thickness was noted to be non-uniform, particularly in the postero-inferior region. The perivesical fat appeared rather hazy and congested. The seminal vesicle also appeared slightly congested. No ascites or lymphadenopathy was seen. The liver, spleen, pancreas, both kidneys and adrenals were unremarkable.
At operation, there was a normal anterior urethra and prostate. There was a big tumor coming from the upper and lateral wall of the bladder measuring about 5 x 6 cm with some areas of calcification on the surface. The bladder was trabeculated and the posterior wall epithelium looked abnormal. The left lateral wall, right lateral wall and other areas of the bladder looked normal. Urethral calibration was performed and the patient was found to have meatal stenosis; meatotomy was performed. The tumor was dissected and sent for histopathology. The samples received in the laboratory consisted of multiple small fragments of soft gray-brown tissue, measuring in aggregate 1.5 x 1 x 1cm. Microscopy of the tumor showed both cellular and hypocellular areas with characteristic cambium layer [Figure - 1],[Figure - 2]. Within the hypocellular areas, foci of immature cartilage were noticed [Figure - 3],[Figure - 4]. Immunohistochemistry showed the tumor cells to be positive for Desmin, Vimentin, Actin and Myoglobin. The cartilaginous areas showed positivity with S100 protein.
| Discussion|| |
In the early nineteenth century, rhabdomyosarcomas were considered as medical curiosities. There used to be long debates regarding their histogenesis. The publications of Intergroup Rhadcomyosarcoma studies have greatly contributed in understanding these tumors. Generally speaking, rhabdomyosarcomas are common in children but they also occur in adults. However, it is rare in persons older than 45 years.  Most of these tumors are classified as embryonal (Botryoid), alveolar or Pleomorphic.  Embryonal rhabdomyosarcomas are usually, but not exclusively, seen in children below 15 years of age. Botryoid rhabdomyosarcoma is a variant of embryonal rhabdomyosarcoma characterized by grapelike growth pattern. Microscopically, myxoid stroma is seen containing undifferentiated round to spindle cells. Cambium layer of Nicholson is its characteristic feature. Its distinctive microscopic appearance is the result of unrestricted growth rather than an intrinsic feature of the tumor. 
The median age for occurrence of urinary bladder rhabdomyosarcoma is four years. It is rare in adults.  The tumor originates mainly in the submucosa of posterior bladder wall with particular preference for the regions of bladder neck and trigone. Our patient presented with hematuria which occurs when the overlying mucosa becomes necrotic or ulcerated. After the publication of Mostofi and Morse, who reported 10 cases in 1952,  many publications were seen regarding rhabdomyosarcoma of urinary bladder in children. Adult cases, although less frequent, have been reported in different publications (Joshtri et al). 
The presence of immature cartilage in Botryoid rhabdomyosarcoma of the cervix has been reported by Daya et al.  Its presence in cervical rhabdomyosarcoma is important as its presence is said to be associated with a better prognosis.  However, there are other publications which have opinion to the contrary. Perrone et al reported a case with a word of caution that their case, a 20-year-old female, who presented with Botryoid rhabdomyosarcoma of cervix had an aggressive course despite surgery and chemotherapy.  Future progress of our patient will determine the significance of the immature cartilage within the Botryoid variant of embryonal rhabdomyosarcoma of urinary bladder.
| References|| |
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|3.||Perrone T, Carson LF, Dehner LP. Rhabdomyosarcoma with heterologous cartilage of the uterine cervix: a clinicopathologic and immunohistochemical study of an aggressive neoplasm in a young female. Med pediatr Oncol 1990;18 (1):72-6. |
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Department of Pathology (32), King Khalid University Hospital, P.O. Box 2925, Riyadh 11461
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]