Angiosarcoma of the caecum

Abstract

Introduction. Primary angiosarcomas arising from the digestive system are rare, representing less than 1% percent of the malignant sarcomas. Only a few cases have been reported in the literature. Colorectal involvement is exceedingly uncommon and is a harbinger of a poor prognosis with widespread metastasis. Diagnosis is often delayed due to non-specific symptoms and pathology which mimics other tumors. Case outline. A 52-year-old woman was presented to the emergency room in July 2021, complaining of protracted malaise, and rapid fatigue and occasional sweating. Multislice computed tomography (MSCT) of the abdomen and small pelvis revealed the existence of lobular, well vascularized, supravesical lesion, located along the anterior contour of the uterine corpus, in the convolutes of the small intestine. The patient was subjected to operative treatment with curative intent. Intraoperatively, a completely tumor-altered caecum was verified Standard histopathological examination demonstrated a high grade epithelioid angiosarcoma with severe pleomorphism and solid growth pattern. The control MSCT of abdomen and small pelvis which was done 12 months after the operation did not show the existence of any pathological lesions. Conclusion. Both clinical and pathological diagnoses of colorectal angiosarcoma are challenging. Patients are presenting non-specific symptoms, which can lead to mismanagement and late diagnosis. A pathological diagnosis relies on immunohistochemical staining for endothelial markers. In limited tissue biopsies, it can be easily misdiagnosed as poorly differentiated adenocarcinoma or gastrointestinal stromal tumor. For now, surgical treatment with R0 resection seems to be the only effective treatment modality.