Anatomical Pathology Interesting Case Conference

Microscopic Description:

Histology reveals anastomotic vascular spaces lined by tumor cells showing pleomorphic nuclei and prominent nucleolus within the submucosa, mimicking prostate carcinoma.

IHC is positive for AE1/3, CK 7.

Discussion:

It is a rare vascular tumor originating from the vascular endothelial cells. It is aggressive in nature and can occur de novo (51.4%) or due to secondary causes (48.6%) like radiation(most commonly), smoking, CCB intake, vinyl chloride, arsenic, thorium dioxide, hemodialysis, organ transplant.

Hematuria is the most common symptom.

Grossly, hemorrhagic mass of trigone/dome.

Microscopically, it can be of epithelioid (91.5%), spindle-shaped (27.6%), and mixed morphology (19.1%). Tumor endothelial cells have pleomorphic nuclei with prominent nuclei. Irregular anastomising vascular channels with hemorrhage are seen in submucosa which can invade muscularis propria. Ulceration, necrosis are also seen.

Immunohistochemical analysis shows CD31+, AE1/31+, Vimentin+, VWF+ ,ERG+, Ki 67-high, CD34 + (73.9%), and Factor VIII-related antigen+(85%), CK 7+ (20.9%)/.

Treatment includes surgical resection, chemotherapy/radiation. Metastasis and recurrence are seen and associated with poor prognosis, with a median survival of <1 year.

No significant difference in survival between de novo and radiation-induced bladder angiosarcoma. Early detection and timely initiation of chemotherapy may improve patient survival.

References:

Silwal A, B R S, Rehman S, Daing A, Gopagoni R, Alyas Akram M, Saeed A, Sadiq KO, Farrukh AM, Harikrishna A. Bladder angiosarcoma: a systematic literature review and survival analysis. Proc (Bayl Univ Med Cent). 2025 Mar 14;38(3):305-312.

Elatreisy A, Hussein MRA, Shalkamy O, Aljubran AM, Aboelnasr M, Safar O, Shah S, Elhadad AYK, Alqahtani S, Ahmad N, Al-Ayafi M, Bosily MJ, Alhadi A. A Primary Epithelioid Angiosarcoma Arising in a Bilharzial Urinary Bladder: A Reappraisal and Case Report. Asian Pac J Cancer Prev. 2023 Jan 1;24(1):87-92.

Cito G, Santi R, Gemma L, Galli IC, Li Marzi V, Serni S, Nesi G. Angiosarcoma of the Urinary Bladder Following Radiotherapy: Report of a Case and Review of the Literature. Medicina (Kaunas). 2021 Apr 1;57(4):329.

 

Matoso A, Epstein JI. Epithelioid Angiosarcoma of the Bladder: A Series of 9 Cases. Am J Surg Pathol. 2015 Oct;39(10):1377-82.

 

Yan Q, Fernandez RA, Elmi M, Gelfond J, Davies MG. Outcomes of Interventions for Angiosarcoma. Front Surg. 2022 Apr 11;9:819099.

 

Pierce D, Connelly ZM, Boyd F, Heinsimer K.. De novo angiosarcoma of the bladder: a case report. Curr Probl Cancer Case Rep. 2023;11:100259. doi: 10.1016/j.cpccr.2023.100259.

Case Images