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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2003  |  Volume : 14  |  Issue : 4  |  Page : 522-525
Urinary Bladder Botryoid Rhabdomyosarcoma with Immature Cartilage in a 24-year-old Male Patient: A Case Report


Department of Pathology (32), King Khalid University Hospital, Riyadh, Saudi Arabia

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   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 Top


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. [1] 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. [2] Perrone et al also reported a case of heterologous cartilage of uterine cervix in a young female. [3] Yasui et al reported a case of rhabdomyosarcoma of the urinary bladder in an adult. [4] 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 Top


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 Top


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. [1] Most of these tumors are classified as embryonal (Botryoid), alveolar or Pleomorphic. [5] 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. [1]

The median age for occurrence of urinary bladder rhabdomyosarcoma is four years. It is rare in adults. [6] 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, [7] 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). [8]

The presence of immature cartilage in Botryoid rhabdomyosarcoma of the cervix has been reported by Daya et al. [2] Its presence in cervical rhabdomyosarcoma is important as its presence is said to be associated with a better prognosis. [5] 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. [3] Future progress of our patient will determine the significance of the immature cartilage within the Botryoid variant of embryonal rhabdomyosarcoma of urinary bladder.

 
   References Top

1.Enzinger FM, Weiss SW. Soft tissue tumors. Fourth Ed, Washington DC: 539­43, Mosby 2001.  Back to cited text no. 1    
2.Daya DA, Scully RE. Sarcoma Botryoides of the uterine cervix in young woman: a clinicopathological study of 13 cases. Gynecol Oncol 1988;29: 290-304.  Back to cited text no. 2  [PUBMED]  
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.  Back to cited text no. 3    
4.Yasui T. Tanaka H, Sasaki S, Kohri K. Rhabdomyosarcoma of the bladder in an adult. Urol Int 1999;63 (2): 144-6.  Back to cited text no. 4    
5.Rosai Juan. Ackerman's Surgical Pathology, Vol (2), Eighth ed., Newyork; 1347-8, Mosby 1995.  Back to cited text no. 5    
6.Bostwick DG, Eble JN. Urologic Surgical Pathology, Minnesota: 275-6, Mosby 1997.  Back to cited text no. 6    
7.Mostofi FK, Morse WH. Polypoid rhabdomyosarcoma (sarcoma botryoides) of bladder in children. J Urol 1952;67:681.  Back to cited text no. 7  [PUBMED]  
8.Joshtri DP, Wessely Z, Seery WH, et al. Rhabdomyosarcoma of the bladder in an adult: case report and review of the literature. J Urol 1966;96:214.  Back to cited text no. 8    

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Correspondence Address:
Ahlam Al-Shedoukhy
Department of Pathology (32), King Khalid University Hospital, P.O. Box 2925, Riyadh 11461
Saudi Arabia
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PMID: 17657126

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  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures
 

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