Extensive gastrointestinal metastasis of cutaneous angiosarcoma

We would like to present a rare and noteworthy case of a 78-year-old male diagnosed with extensive intestinal metastases originating from a primary scalp angiosarcoma. Angiosarcoma is an aggressive, malignant tumour arising from vascular or lymphatic endothelial cells and accounts for less than 2% of all soft tissue sarcomas.^{1, 2} The majority of angiosarcomas occur in the head and neck region, but gastrointestinal metastases are exceedingly rare, with only 13 reported cases worldwide.

Our patient initially presented with mild abdominal pain and was found to have multiple intestinal polypoid masses during exploratory laparotomy. The resected specimens revealed poorly differentiated carcinoma, later confirmed by immunohistochemical staining to be angiosarcoma (Fig. 1). Further review of the patient's medical history revealed a previous resection of a scalp angiosarcoma 2 years prior, leading to the conclusion of scalp angiosarcoma with widespread gastrointestinal metastasis. Despite palliative surgery, our patient did not receive radiotherapy or chemotherapy postoperatively. He was readmitted 3 months later due to secondary multiple intestinal perforations.

This case highlights the aggressive nature of angiosarcoma, which often presents with nonspecific symptoms, leading to diagnostic delays. Histopathological and immunohistochemical evaluation, particularly with markers such as CD31, CD34, and ERG, were crucial in confirming the diagnosis.³ Due to its aggressive behaviour, angiosarcoma carries a poor prognosis, especially in cases of metastasis, with a median survival of only 10 months.⁴ Given its rarity, there are no standard treatment guidelines for intestinal metastasis from angiosarcoma. The combination of surgery, radiotherapy, and chemotherapy remains the most commonly employed approach.

Given the rarity of intestinal metastasis from angiosarcoma, our report contributes to the limited body of literature on this condition. We believe that a heightened awareness of this potential metastatic pattern is essential for timely diagnosis and management.

Lei He: Conceptualization; investigation; writing – original draft. Ting Lu: Investigation; writing – original draft. Qin Shen: Investigation; resources; writing – review and editing. Ying Liu: Investigation; writing – review and editing. Yang Zhang: Conceptualization; investigation; resources.

This is a case report. When obtaining an informed consent for surgical procedure, a general consent was also obtained from the patient, for publication and presentation.