Angiosarcomas account for 1–2% of soft tissue sarcomas and are preferentially localized to skin and superficial soft tissue. Rare cases are described in heart, liver, spleen and adrenal glands, being the digestive tract localization an exceptionally rare occurrence [1][2][3] .

Malignant tumors of the small intestine make up only 1– 1.6% of all gastrointestinal tract tumors.

The most widely recognized predisposing factors for angiosarcomas of skin and soft tissue are radiation and chronic lymphedema (Stewart-Treves syndrome). There is also a strong association with contact to some chemical agents such as thorium dioxide, arsenic, vinyl chloride or to foreign material introduced iatrogenically like vascular graft material or by trauma such as foreign bodies [1][4].

Clinically, like other tumors of the small bowel, angiosarcomas have nonspecific symptoms including recurrent gastrointestinal bleeding, abdominal pain and nausea [2][5][6][7]. The few cases described in the literature report rapid dissemination and very reserved prognosis with average survival from two to six months after diagnosis [3][6].

The authors present a clinical case of a primary angiosarcoma of the small bowel.

A 73-year-old male, with irrelevant personal history and no known exposure to chemical toxins, surgery, chemotherapy or radiation was admitted to the emergency department with abdominal pain in the right lower quadrant and vomiting for three days. Physical examination revealed a palpable mass in the mesogastric region of the abdomen. Laboratory testing detected a mild anemia (Hb 11.3 mg/dL) with no other analytic abnormalities. The computed tomography demonstrated an abdominal tumor measuring approximately 12.3x11.6x7 cm in the proximal ileum, with regional lymph nodes and a moderate amount of intra-abdominal free fluid (Figure 1).

Laparotomy showed a small bowel obstructing tumor and a minimal amount of bloody ascites. No other lesions were observed. A segmental enterectomy was performed (Figure 2). Pathological analysis revealed an angiosarcoma and immunohistochemistry shows positivity for factor VIII, CD 34, CD31 and negativity for CD 117, S100 and MITF (Figure 3). The tumor was 7 cm in larger axis and had metastasis in 4 of 16 nodes removed.

Due to the rarity of randomized trials and prospective studies, the management guidelines for other soft tissue sarcomas tend to be utilized when dealing with angiosarcoma. According to 7th edition of AJCC Soft-Tissue Sarcoma Staging System, this case was classified as pT2bN1M0. The presence of positive nodes (N1) in M0 tumors is considered stage III.

There were no complications during the postoperative period. Adjuvant chemotherapy was decided by multidisciplinary team discussion.

One month after surgery was readmitted with abdominal pain, vomiting, ascitic abdominal distension and worsening anemia (Hb 9.2 mg/dL). Paracentesis was performed and 1000 mL of serous-hematic fluid was drained. The ascitic fluid was positive for malignant cells. CT scan showed extensive peritoneal carcinomatosis (Figure 4). Patient experienced a progressive abdominal distention, worsening of pain and anemia despite transfusions; loss of weight with severe malnutrition and rapid deterioration with multiorgan failure; he died within tw0 months after the initial diagnosis.

The primary angiosarcoma of the small bowel like other neoplasms on this location present with nonspecific symptomatology that may include abdominal pain, nausea, vomiting, intestinal obstruction, gastrointestinal bleeding and anemia [2][4].

The patient, in our case report, presented with nonspecific complaints of pain and vomiting, and had no identifiable risk factors described in the medical literature [1]. This may have been the reason for delayed diagnosis and consequent poor prognosis.

Magnetic resonance imaging (MRI) scan, computed tomography (CT) scan, abdominal X-rays and ultrasound can be used for diagnosis, but all of them have limited diagnostic utility [5][6].

Immunohistochemistry is the only method to confirm the diagnosis [7] . Immunohistologically, intestinal angiosarcomas are positive for endothelial markers as CD31, CD34, Von Willebrand factor and vascular endothelial growth factor and negative for epithelial, neuronal and melanocytic markers as Keratins, S-100 and HMBE-45 [1][4][7].

Despite its aggressive behavior, angiosarcoma of the small bowel is an extremely rare entity [3]. In a literature review only 22 cases of truly primary angiosarcoma of small intestine cases were described (Table 1) [8].

There is no established standard treatment, and treatment itself becomes difficult owing to late detection due to inaccessible localization and the nonspecific symptoms.

The attempt to treat these patients requires a multidisciplinary team that may include radiologists, pathologists, surgical oncologists, medical oncologists and radiation oncologists. Although the cornerstone of the treatment is surgical complete resection when possible to achieve maximal locoregional control, treatment often includes palliative resection of the bleeding or obstructing lesions, chemotherapy, radiotherapy and best supportive care which may include massive blood transfusions [3] .

In 1999, Aitola et al. reported a case of a small bowel tumor resection followed by combination chemotherapy with doxorubicin which survived one year and nine months after diagnosis [9]. The patient described had 14 years previously undergone total hysterectomy and salpingo-oophorectomy for a stage I adenocarcinoma of the uterine corpus and received 55.6 Gy external radiation therapy to the lower pelvis. In May 1997, at the age of 50 years, she was again admitted to hospital due to repeated symptoms of intestinal obstruction. Complementary study demonstrated a constant 5-cm-long stricture at the terminal ileum. Laparotomy revealed a 20 cm long segment of thickened terminal ileum, an extended ileocecal resection was performed. The patient received 6 adjuvant doses of doxorubicin (110 mg). Relaparotomy was undertaken one year and nine months after diagnosis of the angiosarcoma from the operative specimen, and this showed wide intra-abdominal spread and retroperitoneal recurrence. This case is the one with the highest survival described in literature in patients with this disease. Most patients die within a few months after diagnosis with an average survival from two to six months after diagnosis secondary to refractory bleeding and disease progression [2][3][6][10][11][12][13] .

Angiosarcoma of the small bowel is an exceptionally aggressive and rare entity with very poor prognosis. Early diagnosis is a challenge due to the nonspecific symptoms. Imaging studies can be extremely important in timely finding these lesions, but high clinical suspicion based on clinical history is necessary for diagnosis. There are no defined guidelines or demonstrated efficacy of adjuvant treatment due to the low incidence of this pathology, thus, the multidisciplinary approach of these patients is of utmost importance.

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