Small bowel perforation due to indistinguishable metastasis of angiosarcoma: case report and brief literature review
© Uchihara et al. 2016
Received: 8 September 2015
Accepted: 27 April 2016
Published: 8 May 2016
Intestinal metastasis of angiosarcoma is extremely rare. We herein report a case of intestinal perforation due to intestinal metastasis of angiosarcoma. The patient was a 72-year-old Japanese man with multiple recurrent angiosarcomas of the scalp. He developed acute abdominal pain with guarding, and we performed an emergency exploratory laparotomy. An intestinal perforation was found 80 cm from the ligament of Treitz, and partial jejunectomy was successfully performed. Macroscopic inspection revealed no obvious injury, ulcer, or tumor at or around the perforation site. Pathological examination revealed angiosarcoma cells penetrating through all layers of the jejunum at the site of intestinal perforation. This is the first reported case of intestinal perforation caused by indistinguishable intestinal metastasis of angiosarcoma. This case emphasizes intestinal metastasis of angiosarcoma as a possible cause of small bowel perforation in patients with advanced angiosarcoma, even when no visible tumor is present during surgery.
Angiosarcoma is a very rare tumor that originates from endothelial cells and constitutes approximately 2 % of soft tissue sarcomas . Angiosarcoma most commonly occurs in the cutaneous tissues of the head, neck, and face, particularly the scalp . The prognosis of this tumor is poor . The most common sites of recurrence are the regional lymph nodes and lungs, followed by the liver and spleen .
The incidence of malignant tumors has been increasing worldwide, and the management of abdominal oncologic emergencies has thus become more clinically important. Common causes of abdominal oncologic emergencies are obstruction, hemorrhage, and perforation. Among these, intestinal perforation often requires urgent surgical intervention. Lung and gynecologic cancers occasionally cause intestinal perforation by metastatic tumors [4, 5].
We herein report an extremely rare case of intestinal perforation due to intestinal metastasis of angiosarcoma, which was indistinguishable during the operation but pathologically confirmed after surgery. We also provide a brief review of the literature regarding intestinal metastasis of angiosarcoma.
Review of reported seven cases (including this case) with intestinal metastasis of angiosarcoma
Authors, year of publication (reference number)
Primary tumor site
Site of intestinal metastasis
Symptom at presentation
Visible or tactile tumor at the site of perforation
Outcome and time after surgery
Schmid E et al., 1984 
Present (visible hemorrhage)
Dead, approximately 14 months
Kunkel D et al., 1993 
Massive small intestine
Bandorski D et al., 2002 
Massive small intestine
Multiple jejunal and ileal resection
Hsu JT et al., 2005 
Present (visible hemorrhage)
Partial intestinal resection
Dead, 7 months
Ruffolo C et al., 2004 
Perforation (due to tumor ulceration)
Present (tactile ulcer)
Intestinal wedge resection
Dead, 16 days (due to respiratory distress)
Santonja C et al., 2001 
Perforation (due to tumor cell embolization in intestinal artery)
Dead, 18 days (due to acute renal failure, and liver infarction)
Uchihara et al., 2015
Perforation (due to invisible metastatic-tumor-cell penetration)
Partial jejunal resection
Dead, 23 days (due to hemorrhagic shock due to hemothorax)
According to our literature review, hemorrhage is a common initial symptom in patients with intestinal metastasis of angiosarcoma. Hemorrhage occurred in four cases (67 %), but peritonitis occurred in only two cases (33 %) (Table 1). Ruffolo et al.  reported a case of intestinal perforation due to invasion of an ulcerated tumor through all layers of the small intestine. In the present case, no visible or palpable ulcerative changes were present on the mucosal or serosal surface of the intestine at or around the perforation site. Only the pathological examination revealed intestinal metastasis of angiosarcoma. Santonja et al.  also reported a rare case of intestinal perforation caused by intestinal ischemia due to tumor cell embolization, but not due to direct intestinal metastasis. In their case, the primary tumor was located in the abdominal aorta, and the tumor cells spread into small mesenteric arteries, resulting in intestinal infarction. In the present case, there was no evidence of tumor cell embolization on pathological examination.
The growth pattern of angiosarcoma is usually infiltrative, without the formation of a capsule or clear border distinguishing the tumor from normal tissue. At the primary site (scalp) in the present case, a red-brown color was the only clue of demarcation between the tumor and normal tissue (Fig. 1a). Such a subtle color change may be more difficult to detect in a perforated intestine with inflammation than in the skin. In fact, although many tumor cells were present within the serosal layer at or around the perforation site, we found no metastatic changes in the intestine (Fig. 2a, b). Pathological examination would be necessary to confirm the presence or absence of metastatic angiosarcoma of the intestine.
Intestinal perforation during chemotherapy can be explained by necrotizing enteritis in the presence of neutropenia, metastatic tumor infiltration, and direct intestinal damage by chemotherapeutic agents characterized by mitotic arrest [11–14]. In the present case, there was no evidence of neutropenia, enteritis, or any histological findings of damaged cells with mitotic arrest. Although we cannot exclude the possibility of tumor necrosis by chemotherapy, the intestinal perforation in this case may be due to a metastatic tumor invading the whole wall of the intestine.
The prognosis of angiosarcoma is very poor. Lahat et al. reported a median disease-free survival duration of 43 months (range, 1–188 months), a 5-year disease-specific survival rate of 35 to 40 %, and a median survival duration of 10 months in patients with metastatic angiosarcoma [1, 15–18]. The prognostic factors of angiosarcoma are reportedly a large tumor (>5 cm) [19, 20], old age, distant metastasis, and poor performance status [19, 21]. In our review of seven cases (six previously reported cases plus ours) of intestinal metastasis from angiosarcoma, the prognosis of patients with peritonitis was remarkably poorer than that of patients with hemorrhage alone. Additional evidence is necessary to establish the surgical indications for intestinal perforation due to metastatic angiosarcoma.
In summary, intestinal perforation due to intestinal metastasis of angiosarcoma should be taken into account, in abdominal emergency cases with advanced angiosarcoma.
When obtaining an informed consent for surgical procedure, a general consent was also obtained from the patient, for publication and presentation, as usual.
We would like to express the deepest appreciation to Ms. Chiemi Nozaki, for the data collection.
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