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Presented by Jonathan Epstein, M.D. and prepared by Julie M. Wu, M.D.
Case 6: A 5 year old male was noted to have a bladder mass following hematuria.
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1. Question
Week 349: Case 6
A 5 year old male was noted to have a bladder mass following hematuria.images/jmw051908/6.1.jpg
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images/jmw051908/6.5.jpgCorrect
Answer: Rhabdomyosarcoma
Histology: The lesion at low magnification has a polypoid growth with club-like structures. There appears to be a condensation of cellular tissue beneath the unremarkable urothelium. The cells within the stroma are spindled with eosinophilic cytoplasm. The nuclei appear fairly uniform without significant pleomorphism. In areas one can appreciate within the cytoplasm cross striations.
Discussion: This is an example of a rare subtype of embryonal rhabdomyosarcoma termed botryoides variant. These lesions have a grape-like gross appearance at cystoscopy due to the polypoid nature of the lesion which is often filled with edema. Embryonal rhabdomyosarcoma of the bladder is typically associated with a good prognosis with the botryoides variant of rhabdomyosarcoma associated with even a better prognosis. Typically following biopsy these patients are treated with chemotherapy and in many cases the response is excellent which can save the patient from undergoing radical surgery. In this case the diagnosis is straight forward without the use of immunohistochemistry as one can see well formed cross striations in rhabdomyoblasts. In other cases with more primitive undifferentiated cytology the use of immunohistochemical stains for desmin and myogenin can help identify the cells as showing rhabdomyoblastic differentiation.
Incorrect
Answer: Rhabdomyosarcoma
Histology: The lesion at low magnification has a polypoid growth with club-like structures. There appears to be a condensation of cellular tissue beneath the unremarkable urothelium. The cells within the stroma are spindled with eosinophilic cytoplasm. The nuclei appear fairly uniform without significant pleomorphism. In areas one can appreciate within the cytoplasm cross striations.
Discussion: This is an example of a rare subtype of embryonal rhabdomyosarcoma termed botryoides variant. These lesions have a grape-like gross appearance at cystoscopy due to the polypoid nature of the lesion which is often filled with edema. Embryonal rhabdomyosarcoma of the bladder is typically associated with a good prognosis with the botryoides variant of rhabdomyosarcoma associated with even a better prognosis. Typically following biopsy these patients are treated with chemotherapy and in many cases the response is excellent which can save the patient from undergoing radical surgery. In this case the diagnosis is straight forward without the use of immunohistochemistry as one can see well formed cross striations in rhabdomyoblasts. In other cases with more primitive undifferentiated cytology the use of immunohistochemical stains for desmin and myogenin can help identify the cells as showing rhabdomyoblastic differentiation.
