A 61 year-old male was admitted for constipation and a palpable mass in the periumbilical area. His medical history revealed the surgery for acute perforative appendicitis 20 years ago and occasional intestinal obstruction afterwards. He denied any history of radiation or occupational exposure to chemicals. The small bowel series showed an external compressive lesion in the distal ileum and the computerized tomographic (CT) scan revealed a 6 cm, well-marginated mass in the anteromedial aspect of the ascending colon (Figure ). Under the suspicion of foreign body granuloma due to gauze (gossypibioma), an exploratory laparotomy was performed. The whole intestine was found to be covered in severe adhesion; however, ascites or blood in the abdominal cavity was absent. In the anteromedial aspect of ascending colon, there was an encapsulated mass 5 cm in size, and it was adhered to ascending colon and distal ileum (Figure ). The mass was filled with abscess and granulation tissue, and the frozen biopsy reported the diagnosis of abscess. Only resection of the mass was performed, and the patient was discharged without any complications.
Enhanced abdominal CT scan of upper abdomen showing a 6 cm sized mass in the right lower quadrant of the abdomen at the first operation. The mass contained air bubbles, tiny calcifications and hematoma.
Macroscopic view of the mass in the right paracolic gutter showing a 5 × 3 cm sized, encapsulated mass filled with abscess and granulation tissue.
Forty days after the discharge, he was admitted again with anemia, abdominal distension, and melana. The colonoscopy revealed the hematoma and the stenosis of the lumen directly above the cecum. The abdominal CT scan showed large volume of blood in the abdomen and multiple peritoneal nodules. The angiography and 99 mTc labeled RBC scan showed active bleeding around the ileocecal valve. Emergent laparotomy was performed and multiple nodules were found on the wall of ileum, liver, mesentery and peritoneum. In the distal ileum, two nodular lesions with 3 cm and 2 cm in size were bleeding actively, and they were adhered to each other. The nodules were diagnosed as sarcoma through the frozen biopsy, and the distal ileum including the pathologic lesion was resected. Seven days later, reoperation was performed due to substantial hemorrhage from the peritoneum, the mesentery, and the small intestine wall. Two days after the last surgery, the patient expired of uncontrollable bleeding due to disseminated intravascular coagulopathy.
On the macroscopic examination of resected ileum, there were two ill-defined tan solid tumors with mucosal ulceration, each measured 3 × 1.5 cm and 2 cm in diameter, involving the entire intestinal wall and extending to the subserosa of adhered loop. (Figure ).
Macroscopic view of the segment of the small intestine showing an ill-defined tan solid mass that involved the entire intestinal layer.
Under microscope, spindle-shaped or epithelioid cells were arranged as a plate and the rudimentary vessel lumen were detected occasionally (Figure ). Separated from these two lesions, several small angiosarcomas containing foreign body granulomas were found in the subserosal layer of the intestine. Also, the nodules of liver and mesentery were diagnosed as metastatic angiosarcomas.
Figure 4 Microscopic findings of the angiosarcoma in the small intestine. 4a) Atypical spindle or epithelioid tumor cells were arranged in sheets, and rudimentary lumen formation was rarely noted. (H&E, × 200) 4b) The tumor cells are strongly positive (more ...)
Immunohistochemical staining was performed and tumor cells were positive for CD31, CD34, and vimentin (Figure ), whereas negative for factor antigen, CD117, and S-100. The tumor cells were also negative for cytokeratin (AE1/3) and EMA. The foreign body granulomas were surrounded by CD31 positive cells, partially or entirely.
The previously resected mass, which was diagnosed as abscess, was reviewed. In a low magnification field, abscess in the center and fibrosis with vascular proliferation in the periphery were noted (Figure ). However, when the periphery was examined under the high magnification, spindle-shaped or epithelioid cells were arranged as plate patterns, in some area, well-differentiated vessels were formed as similar to the lesions from the ileum. The additional serial sections revealed more foreign materials surrounded by epithelioid tumor cells and the invasion of tumor cells to the blood vessel. The tumor cells were positive for CD31 and CD34, and negative for cytokeratin. This supported the final diagnosis of a foreign body granuloma associated-angiosarcoma.
Figure 5 Microscopic finding of the angiosarcoma in the anteromedial aspect of ascending colon. Proliferation of malignant blood vessels was seen in the periphery of the abscess(left upper corner) (H&E, × 40). Inlet: Malignant epitheliod tumor (more ...)