Radiation-associated peritoneal angiosarcoma

Abstract

Angiosarcomas account for only 1-2% of all soft tissue sarcomas, with the most common site of origin being in the head and neck region. Peritoneal angiosarcoma is an extremely rare tumour and few cases have been reported previously. Presentation of peritoneal angiosarcoma can be very variable, hence making diagnosis difficult. Herein, we review the current literature and describe a rare case of a patient who presented with haemorrhagic ascites, 17 years after radiotherapy for endometrial carcinoma and was subsequently diagnosed with peritoneal angiosarcoma. Due to extensive disease, surgery was not a viable option. She was started on palliative chemotherapy, but despite treatment, her condition deteriorated further and she eventually passed away. We highlight the diagnostic challenges and considerations in these patients as well as current treatment and management options available.

Keywords: Cancer intervention; Surgical oncology.

Figure 1

(A, B) Axial and sagittal views showing the cystic mass situated atop the vaginal stump with thick enhancing walls, which was aspirated via endoscopy.

Figure 2

Interval decrease in size of collection on follow-up imaging from 8 cm to 4 cm.

Figure 3

Interval resolution of the collection above the vaginal stump.

Figure 4

Interval increase in ascites but no significant masses or collections noted.

Figure 5

Fibroadipose tissue with scattered inflammatory cells reactive fibrosis. No malignancy present.

Figure 6

Exploratory laparotomy showing fibrinous tissue covering peritoneum.

Figure 7

Histology slide showing dyscohesive clusters of large epithelioid cells embedded in a haemorrhagic and fibrin-rich stroma. These cells show large pleomorphic nuclei, prominent nucleoli and pale vacuolated cytoplasm. The tumour cells immunostain positively for the endothelial marker CD31. Immunostains for keratins, mesothelial and macrophage markers are negative, confirming the diagnosis of angiosarcoma.