Jacobs Journal of Nephrology and Urology

Primary Angiosarcoma of the Urinary Bladder: A Review and Update of the Literature

Published on: 2019-03-15

Abstract

Primary angiosarcomas of the urinary bladder (PAUB) are very rare; in view of this their clinical and pathological features have not been extensively described. To the knowledge of the author, only 36 cases of primary angiosarcoma of the urinary bladder have been reported in the literature. Primary angiosarcoma of the urinary bladder has been reported in individuals whose ages had ranged between 32 years and 89 years. PAUB has been reported in men and women but PAUB has been reported more often in men. PAUB has presented most often with haematuria but other symptoms had included: Lower urinary tract symptoms, urinary frequency, loin pain, weight loss, weakness, lethargy, cachexia, swelling of the lower extremities. In some cases, a past history of radiotherapy or exposure to industrial chemicals had been documented. Diagnosis of PAUB has been based upon cytology, histopathology and immunohistochemistry features of a tumour and occasionally bladder washings which may show: Anastomosing vascular channels which are lined by plump hyperchromatic cells, as well as solid growth and epithelioid cytology. Typically the tumour invades the muscularis propria and the tumour may also involve the urinary bladder muscle; immunohistochemistry staining studies would tend to show positive staining for CD31, CD34, and Cytokeratin can be focally positive, especially in cases of epithelioid tumours. Treatment options that had been undertaken in various cases of PAUB included trans-urethral resection of a bladder tumour, partial cystectomy, radical cystectomy plus lymphadenectomy, cyst prostatectomy with lymphadenectomy, anterior pelvic exenteration, and these procedures were undertaken as sole procedures, or with adjuvant therapy in the form of radiotherapy alone, chemotherapy alone or chemotherapy and radiotherapy. With regard to outcome generally, the prognosis had tended to be poor and even though very few cases of short-term to medium-term survival, there was hardly any case of long-term disease-free survival. There was the development of early metastases even with cases of a tumour confined to the submucosa of the urinary bladder. There was no case report of disease-free survival of any patient who was initially diagnosed with metastases or any patient who subsequently developed metastasis pursuant to initial treatment of the disease and even though some patients were alive with the stable disease there was no report of complete destruction of tumour cells by chemotherapy or radiotherapy. Considering that some cases of PAUB developed pursuant to radiotherapy it would appear as if radiotherapy might not be the most appropriate adjuvant treatment for patients who have post-radiotherapy PAUB. Considering that the available chemotherapy agents have not been reported to have completely destroyed metastatic PAUB lesions, it would be most appropriate for pharmacotherapy researchers, oncologists, and urologist to identify a specific chemotherapeutic regimen that would effectively destroy metastatic PAUB tumour cells and prolong the long-term disease-free survival of patients who have PAUB. Finally, a global multi-center trial of treatment options for PAUB should be undertaken.

Keywords

Angiosarcoma; Primary, Urinary Bladder; Immunohistochemistry, Trans-Urethral Resection Of Bladder Tumour; Radiotherapy; Chemotherapy; Cystectomy; Cystoprostatectomy;CD31; CD34; Cytokeratin; Poor Prognosis