A Review of Management and Outcome of Colorectal Angiosarcoma

Research Article

Austin J Surg. 2014;1(7): 1034.

A Review of Management and Outcome of Colorectal Angiosarcoma

Dhebri AR1*, Chadwick M1, Chowdhury J2 and Loganathan S1

1Department of Surgery, Royal Albert Edward Infirmary, UK

2Department of Pathology, Salford Royal NHS Foundation Trust, UK

*Corresponding author: AR Dhebri, Department of Surgery, Royal Albert Edward Infirmary, Wigan Lane, Wigan, WN1 2NN, UK

Received: August 30, 2014; Accepted: September 22, 2014; Published: October 07, 2014


Purpose: Colorectal angiosarcoma (AS) is a rare aggressive tumour, with no known natural history. We aim to determine the clinical course, management and prognosis of colorectal AS by an analysis of reported cases.

Methods: The cases were identified from PubMed, bibliographies of the retrieved papers and included one patient from our hospital.

Results: A total of 38 cases, including our one case, were analysed. The median (range) age at presentation was 59 (16-85) years and 55% were women. The most frequent site was sigmoid colon (39%). The size of the tumour ranged from 0.3-12 cms. (median: 5 cms.). The commonest symptom was rectal bleeding in 53%. Metastases were present in 48%, excluding the four cases which itself were metastases from AS at other sites. Surgical resection was carried out in 81%; chemotherapy given in 26% and radiotherapy in 28%. The median and 5-year survival rates were eight months and 2.9% respectively. On univariate analysis age, size of tumour, tumour ≥ 5 cm., recurrence and presence of metastases were significant but surgical resection including R0 resection, radiotherapy (XRT), chemotherapy and gender were not significant for survival. On multivariate analysis only the presence of metastases was significant.

Conclusion: AS has a poor prognosis compared to other colorectal tumours. The review suggests treatment of choice to be complete resection (R0), though because of very few cases published, there is no definite evidence for it. One must be aware of this rare entity, particularly in patient with colorectal malignancy presenting with unusual symptoms.

Keywords: Angiosarcoma; Sarcoma; Colon; Colorectal tumours


Angiosarcoma (AS) is a rare soft tissue tumour accounting for less than 1% of all soft tissue sarcomas [1,2]. It rarely occurs in the Gastrointestinal Tract (GIT) [3,4], accounting for less than 1% of all GIT malignancies [5] and usually occurs in the stomach [6] and small intestine [4,6]. Primary colorectal AS are exceedingly rare, accounting for less than 0.001% of all colorectal cancers [3,7-9]. The first case of AS of the colon was described by Steiner et al in 1949 [10].

They can arise anywhere in the body but most commonly occur in the skin and superficial soft tissues of the head and neck (50 to 60%) [11,12], particularly in the scalp [11] of elderly white men. Rarely, intra-abdominal AS arises in the liver [13], spleen [14], ovary [15] and adrenal glands [16] or as diffuse angiosarcomatosis [17]. Metastases to the colon from AS elsewhere are exceedingly rare; the first case to be described was by Fujii et al. [18]. AS principally spreads haematogenously, with the lungs the most common site for metastases. Other sites for metastases include heart, pericardium, liver, spleen, adrenal glands, kidneys, lymph nodes, bones, brain and breast [11,19-25].

Because of the paucity of published cases, there is no overview of the clinical course and management of colorectal AS. The rarity of cases, difficulty in tissue diagnosis and the confusion of nomenclature in the past, may have caused a variety of cases to have been erroneously diagnosed (Table 1).