PRIMARY EPITHELIOID ANGIOSARCOMA OF THE JEJUNUM: A RARE CAUSE OF UPPER GASTROINTESTINAL BLEED

Background:

Gastrointestinal angiosarcoma is a rare and highly malignant soft tissue tumor constituting < 1% of all gastrointestinal malignancies and only 1% of all sarcomas. The objective of this case report, is to increase awareness of this rare tumour.

Case description:

A 73 year old man with type 2 diabetes, COPD, hypertension and obstructive sleep apnoea was admitted to hospital with melaena. He had a 5 year history of iron deficiency anemia. Initially, the anaemia was mild (Hb 11 – 12 g/dl) but over the past 5 months, became more severe and symptomatic (Hb 4.5 – 8 g/dl), requiring 24 units of blood over 3 months. Gastroscopy and colonoscopy were normal whilst CT scan showed lytic lesions in the spine (T6/T8). Targeted spinal biopsies were inconclusive. Capsule endoscopy showed a punched out bleeding ulcer in the jejunum. Double Balloon Enteroscopy (DBE) via the anal route failed to find any abnormality. DBE via the oral route did locate the jejunal ulcer, and 30 cm distal to the ulcer, there was a 2 cm polyp which was not seen on capsule endoscopy or CT. The ulcer was biopsied and the polyp was resected en bloc using Endoscopic Mucosal Resection. The histology of the jejunal polyp and ulcer showed epithelioid angiosarcoma; the tumour cells were strongly positive for CD31. Review of biopsies from the spinal lytic lesions revealed metastasis from this primary jejunal angiosarcoma. The patient was referred to a specialist Sarcoma Unit but died 3 months after diagnosis.

Conclusion:

The diagnosis of small bowel angiosarcoma is challenging due to the non-specific clinical, radiological and histopathological features. Enteroscopy may help detect the source of bleeding and biopsies/polypectomy with immunohistochemical studies may provide definitive diagnosis. A high degree of suspicion is required to ensure early diagnosis and treatment.

Reference:

Nai Q etal, J Clin Med Res. 2018.10 (4):294 – 301. Primary Small Intestinal Angiosarcoma: Epidemiology, Diagnosis and Treatment.