An Autopsy Case of Periaortic Malignant Fibrous Histiocytoma with Multiple Metastases to the Small Intestine

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1 Showa Univ J Med Sci 19(4), , December 2007 Original An Autopsy Case Periaortic Malignant Fibrous Histiocytoma with Multiple Metastases to Small Intestine Kyoko KASHIMA, Toshiaki KUNIMURA, Masashi SATO, Hiromi IMATAxA, Tomokazu HISAYUKI, Tomohiko YOSHIDA, Takahiro YAMAZAKI Toshio MOROHOSHI Abstract : A 67-year-old woman was admitted to our hospital with severe abdominal pain. Emergency laparotomy was performed, intestinal perforation was diagnosed. Wide resection distal ileum cecum with ileostomy was performed. Resected specimens revealed three small perforated ulcers two pedunculated tumors in small intestine. The ulcers tumors diagnosed histopathologically immunohistochemically as storiform-pleomorphic-type malignant fibrous histiocytoma (MFH). The patient died 1 month after surgery due to liver renal failure with aortic occlusion. At primary site, an encapsulated mass (4.0 ~1.0 ~2.0 cm) was situated around wall aorta at autopsy. The grayish white tumor tightly encircled half circumference at level celiac axis. It is very unusual for metastatic foci MFH to be found before discovery primary focus. In addition gastrointestinal (GI) involvement from MFH has rarely been reported. We found 11 cases metastasis MFH to small intestine in English language literature. Our case is first reported case MFH causing perforation small intestine. Key words : malignant fibrous histiocytoma, intestinal metastasis, perforation, aorta Introduction Malignant fibrous histiocytoma (MFH) usually metastasizes to lung, lymph nodes, liver, bone ; gastrointestinal (GI) involvement is rare. We found only 11 cases metastasis MFH to small intestine in English language literature. We reported a case periaortic MFH metastases to small intestine presenting as a perforation. We describe our case review previously reported cases this disease. Case Report A 67-year-old woman was admitted to our hospital in July 2005 due to severe, periodic lower abdominal pain that continued for 4 days. She had begun to notice occasional abdominal pain 6 months prior to admission. Physical examination yielded a temperature 37.4 C, blood pressure 194/94 mmhg, pulse rate 98 beats / min. The patient's abdomen was symmetrically rounded diffusely tender with signs peritoneal irritation. First Department Pathology, Showa University School Medicine, Hatanodai, Shinagawa-ku, Tokyo , Japan.

2 210 Kyoko Fig. 1. Abdominal KASHIMA, et al CT image shows free air around liver, distention small bowel, a low-density mass at anterior surface abdominal aorta at level celiac axis. Hematologic study revealed 16,600 / mm3. within Results normal Abdominal X-ray bowel, multiple a hemoglobin blood limits, showed but free air-fluid level chemistry air 10.4 g / dl tests, C-reactive beneath including protein a white liver level diaphragm, was blood renal elevated gaseous cell count function tests, to distention 25.1 mg / dl. small levels. Computed tomography (CT) revealed free air around liver, distention small bowel, ascites. Moreover, CT showed a low-density mass at anterior surface abdominal aorta, but at that time it was thought to be an intraluminal aortic thrombus (Fig. 1). Emergency laparotomy was performed, a clinical diagnosis intestinal perforation peritonitis was made. During surgery, we found purulent fluid in peritoneal cavity four perforated ulcers in small intestine, which confirmed to be about 80, 160, 260, 290 cm distal to ligament Treitz. Mucosal friability was noticed about cm distal to ligament Treitz. Two pedunculated tumors present in ileum. There was no evidence metastasis to lymph nodes, liver, or peritoneal cavity. Wide resection distal ileum cecum with ileostomy was performed. The small bowel looked mildly ischemic, extending about 50 cm from proximal terminal ileum. Histopathologic findings In resected found intestine, (Fig. 2). (Fig. 3a). cecal valve, solid tumor tissue invading Tumor three The nodules measured located muscularis direct invasion lesions cellular ulcers ulcerous located 30 ~4 mm mainly propria. tumor masses cells lesions in In (Fig. composed two in 8 ~9 pedunculated penetrated ileum mm. center 5 cm cut elevating Microscopically, entire The submucosa, 3b). tumors pleomorphic wall 34 cm surfaces nodules, small both spindle cells with with a proximal showed overlaying ulcers ulcerous forming central tumor crater infiltration to ileo- white, firm, mucosa formed by polypoid bundles

3 MFH with Metastases to Small Intestine 211 fascicles. Giant cells with bizarre nuclei predominant, mitotic activity was high (Fig. 3c). Specimens immunohistochemically positive for vimentin CD68 negative for cytokeratin, c-kit, CD-34, smooth muscle actin, S-100 protein, desmin. On basis se findings, tumors identified as malignant fibrous histiocytoma (MFH) showing a pleomorphic-storiform pattern. It was impossible to distinguish between primary site metastases ; re was no evidence that primary site was in extremities or peritoneal cavity. Post-surgical CT revealed that size aortic mass had not changed. CT angiography aorta confirmed obstruction by revealing a filling defect in entry celiac artery ; superior mesenteric artery left renal artery occluded. The superior mesenteric artery was enhanced from 3 cm distal to its origin, collateral veins not enhanced. The liver left kidney poorly enhanced due to arterial obstruction. The left kidney was atrophic much smaller than right kidney. The patient developed an anastomotic leak perforation due to small bowel infarction. Her condition deteriorated rapidly, liver kidney failure ensued, she died a month after surgery. Autopsy revealed dark discoloration entire ileum distal jejunum with two perforated lesions proximal anastomosis. The remaining portion small bowel was necrotic. There was a firm, longitudinally placed, grayish white tumor, 4.0 ~1.0 ~ 2.0 cm, which appeared to involve adventitia abdominal aorta. The tumor tightly encircled half circumference at level celiac axis thickening aortic wall to 1.0 cm, but overlying intima seemed largely intact. There was no direct spread to adjacent structures. The root celiac artery was occluded by a thrombus, superior mesenteric left renal arteries also occluded (Fig. 4). Microscopically histologic appearance periaortic tumor was similar to that seen in surgical material. The tumor extended mainly from adventitia through partly destroyed media to intima. Elastic van-gieson staining revealed that tumor had infiltrated intraluminal surface abdominal aorta, intima celiac artery, adventitia superior mesenteric artery, media left renal artery. Degenerative necrosis due to thrombosis was seen in liver spleen was considered cause death. No tumor embolus or metastasis was seen in any or organ. We realized this to be a case periaortic MFH with multiple metastases to small intestine., Discussion MFH is a st tissue sarcoma that occurs mainly in middle-aged individuals. According to an analysis 200 cases MFH, most frequent site is extremities (lower extremities, 49%; upper extremities, 19%) ; second most prevalent site is abdominal cavity or retroperitoneum (16%)1). MFH is a high-grade malignancy with an approximately 50% chance metastasis existing at time diagnosis 2) The most common sites metastasis are lungs (25%) lymph nodes (10%) 3). Tumor metastasis to gastrointestinal tract is extremely rare. Only 11 cases metastasis MFH to small intestine have been reported in English-language literature 3-11). These cases, including our case reviewed findings summarized (Table 1). Seven patients male five female. Mean age was 53.3 years (range, years). The common primary sites

4 Kyoko 212 Fig. KASHIMA, et al 2 Fig. Fig. 3 4

5 MFH with Metastases to Small Intestine 213 Table 1. Reported cases malignant fibrous histocytoma metastatic to small intestine SP ; Storiform-pleomorphic, I ; Inflammatory, P ; Plemorphic. ND ; not documented. M ; Multiple. A ; alive. D ; died. S ; Surgery. C ; Chemorapy. R ; Radiation. Fig. 2. Macroscopic findings resected intestine Three ulcers (A, B, C) two pedunculated tumors (D, E) found. The two polypoid polyps, 8 X 9 mm (D) 30 X 34 mm (E) located 34 cm (D) 5 cm (E) proximal to ileocecal valve also found. Fig. 3. Microscopic examination resected intestine a : An ulcerous lesion was associated with a tumor (A) penetrating entire wall. (hematoxylin eosin [H & E]) b : A pedunculated tumor (C) was located mainly in submucosa with infiltration to mucosal surface musculans propria (H & E ; ~400). C: The tumor is composed spindle cells in a storiform pattern, with nuclear atypia mitoses (H & E ; ~ 400). Fig. 4. Cross-section abdominal aorta Cut surface autopsied specimen shows a grayish white tumor thrombus in celiac artery, superior mesenteric artery, left renal artery.

6 214 Kyoko KASHIMA, et al extremities trunk (five cases). Metastatic tumors arose in jejunum in six patients, ileum in two patients, small intestine in four patients. The common clinical symptoms abdominal pain, two major manifestations GI involvement, GI bleeding (five cases) intussusception (two cases). The presence ulceration on surface tumors was documented in all cases with GI bleeding. Ours is first reported case MFH causing perforation small intestine. Metastatic tumors formed as polypoid or pedunculated tumors. The tumors seven patients with GI bleeding or intussusception varied in size from 0.8 cm to 13 cm (average size, 6.7 cm). Three cases solitary MFHs, but two cases multiple MFHs. With respect to or metastatic foci in 12 cases, six cases had lung metastases ors had liver, cerebrum, or adrenal metastases. In our case, it is remarkable that intestinal metastases existed without evidence MFH in lungs. We propose that our patient had primary retropentoneal MFH surrounding aorta that this metastasized to small intestine via abdominal aorta, celiac artery, superior mesenteric artery with an absence lung metastasis. Moreover, it is highly unusual that metastatic foci found before discovery primary MFH focus. Of 82 patients with metastatic MFH reported by Weiss Enzinger1) only one had metastases discovered before primary focus. Of 167 patients with st tissue MFH reported by Kearney et a112). 10 had pulmonary metastases at time diagnosis, but none had metastases predating discovery primary tumor 12). The overall prognosis MFH is poor, especially in cases in which metastatic disease is present. However, Atmatzidis et ally described a patient who was well 2 years after resection metastatic foci without discovery primary tumors. In our case, penaortic MFH produced ischemic trauma to small intestine, liver, spleen, kidney by causing aortic thrombosis ; thus, patient died multiple organ failure. If primary lesion had been found earlier treated, patient might have survived. In conclusion, when multiple ulcers tumors are found in alimentary tract, clinicians pathologists must include MFH in differential diagnosis. A careful search for or lesions should always be performed, especially near celiac superior mesenteric arteries. References 1) Weiss SW Enzinger FM: Malignant fibrous histiocytoma : an analysis 200 cases. Cancer 41: (1978) 2) Spanier SS Floyd JA : Clinicopathologic comparison malignant fibrous histiocytoma liposarcoma. Instr Course Lect 38 : (1989) 3) Blaauwwiekele EE, Koopal S Kreeftenberg HG : Metastasized malignant fibrous histiocytoma as a cause gastrointestinal tract hemorrhage. Sc J Gastroenterol 32: (1997) 4) Wasserman TH Stuard ID: Malignant fibrous histiocytoma with widespread metastases. Autopsy study. Cancer 33: (1974) 5) Hanada M, Maeda T Takeuchi N : Cystosarcoma phyllodes breast with features malignant fibrous histiocytoma. Acta Pathol Jpn 30: (1980) 6) Terashima K, Aoyama K, Nihei K, Nito T, Imai Y, Takahashi K Daidoji S : Malignant fibrous histiocytoma heart. Cancer 52: (1983) 7) Misonou J, Ka M, Shishido T, Abe M, Miyake T, Kawamura T, Maekawa I, Itou N, Atsuta T, Kubota H, Murakami T : An autopsy case malignant fibrous histiocytoma mediastinum, presenting multiple metastases to small intestine to brain-a rare case report with a review literature. Gastroenterol Jpn 25 : (1990)

7 MFH with Metastases to Small Intestine 215 8) Gorman RC, Jardines L, Brooks JJ Daly JM : Enteroenteric intussusception due to a metastatic malignant fibrous histiocytoma. J Surg Oncol 54: (1993) 9) Kanoh T, Shirai Y, Wakai T Hatakeyama K : Malignant fibrous histiocytoma metastases to small intestine colon presenting as an intussusception. Am J Gastroenterol 93 : (1998) 10) Tamura M, Oda M, Ohta Y, Omura K, Nonomura A Watanabe G : Small intestinal bleeding, secondary to metastatic malignant fibrous histiocytoma rib : report a case. Surg Today 32 : (2002) 11) Atmatzidis KS, Pavlidis TE, Galanis IN, Papaziogas BT Papaziogas TB : Malignant fibrous histiocytoma abdominal cavity : report a case. Surg Today 33 : (2003) 12) Kearney MM, Soule EH Ivins JC : Malignant fibrous histiocytoma, a retrospective study 167 cases. Cancer 45: (1980) [Received July 12, 2007: Accepted August 8, 2007]