Background: Angiosarcoma (AS) of the urinary bladder is a very rare and aggressive malignancy with a dismal outcome.
Case report: Here, we report a primary epithelioid angiosarcoma (EAS) of the urinary bladder in a forty-nine-year-old male patient who presented with severe hematuria. Cystoscopic examination revealed hemorrhagic ulcerated bladder mucosa but no definite mass lesions. Intractable hematuria raised the initial clinical impression of idiopathic hemorrhagic cystitis. Analysis of the cystoscopic biopsy revealed features of old bilharzial cystitis, markedly atypical epithelioid endothelial cells arranged as primitive anastomosing vascular structures and expressing vascular markers. The diagnosis of EAS was established. The patient developed intractable severe hematuria, and a radical cystoprostatectomy was performed. The patient was started on chemotherapy but suddenly developed widespread distant metastasis (liver, lung, suprarenal glands, and lymph nodes) and succumbed to death two months after the surgery.
Conclusion: To the best of these authors' knowledge, we presented the first report of primary EAS arising in a bilharzial bladder. The relevant studies were discussed.
Keywords: Epithelioid; angiosarcoma; bilharzial; urinary bladder.