Clin J Gastroenterol (2013) 6:46–49 DOI 10.1007/s12328-012-0358-6
Jejunal tubulovillous adenocarcinoma in adenoma presenting with entero-enteric intussusception Nobuhiro Takeuchi • Shuho Semba • Kazuyoshi Naba • Ryota Aoki • Yu Nishida • Yusuke Nomura • Tetsuo Maeda • Hidetoshi Tada
Received: 21 November 2012 / Accepted: 26 December 2012 / Published online: 12 January 2013 Ó Springer Japan 2013
Abstract A 73-year-old male was admitted to our institution with complaints of nausea, vomiting, and abdominal distension. Plain abdominal computed tomography (CT) suggested intussusception in the jejunum. Enhanced abdominal CT revealed the ‘target-like’ sign and ultrasonography revealed the ‘multiple concentric ring’ sign; therefore, a diagnosis of entero-enteric intussusception was made. The small intestinal obstruction and cause of the intussusception were not evident. The patient was treated conservatively with fasting and transfusion therapy to prevent intestinal obstruction. However, with no spontaneous resolution of intussusception, surgical treatment was decided. The operative findings revealed a jejunal tumor about 30 cm from the Treiz ligament, and the jejunum including the tumor with a 5 cm margin were partially resected. The resected tumor was a 35 9 50 mm soft mass spreading laterally with nodules. The pathological examinations revealed tubulovillous adenocarcinoma in the adenoma. Intussusception is rare in adults compared to children. About 45 % of cases of intussusception in adults are due to small intestinal tumors such as malignant lymphoma or lipoma, but a tubulovillous adenocarcinoma with N. Takeuchi (&) R. Aoki Y. Nishida Y. Nomura T. Maeda H. Tada Department of Gastroenterology, Kawasaki Hospital, 3-3-1 Higashiyama-cho, Hyogo-ku, Kobe, Hyogo 652-0042, Japan e-mail: [email protected]
S. Semba Division of Pathology, Department of Pathology, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Cyuo-ku, Kobe, Hyogo 650-0017, Japan K. Naba Department of Laboratory Medicine, Kawasaki Hospital, 3-3-1 Higashiyama-cho, Hyogo-ku, Kobe, Hyogo 652-0042, Japan
adenoma is a rare cause of intussusception. We present a rare case of jejunal tubulovillous adenocarcinoma in adenoma presenting with entero-enteric intussusception. Keywords Intussusception Jejunal tumor Tubulovillous adenocarcinoma in the adenoma
Introduction Intussusception is a condition in which a part of the intestine invaginates into another section of the intestine. Most intussusception occurs in childhood and is idiopathic. However, adult intussusception is rare and [90 % cases involve some organic disease . We present a rare case of jejunal tubulovillous adenocarcinoma in adenoma presenting with entero-enteric intussusception.
Case presentation A 73-year-old male with a history of atrial fibrillation, diabetes mellitus, hypertension, and cerebral infarction was admitted to our institution with complaints of nausea, vomiting, and abdominal distension. He had been taking an anticoagulant agent (dabigatran at 220 mg per a day) for the prevention of thrombotic events in addition to an antihypertensive drug and an antidiabetic drug. He denied both alcohol and tobacco use. Although he had experienced upper and lower endoscopy, no abnormal findings were revealed. He denied being diagnosed with Peutz–Jeghers syndrome, Crohn’s disease, familial adenomatous polyposis, or hereditary nonpolyposis colorectal cancer. His father and mother did not have cancer. He had occasionally felt dull abdominal pain; his blood pressure was 131/66 mmHg,
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heart rate was 91 beats/min, and body temperature was 35.6 °C. Blood chemistry analyses revealed inflammation (white blood cell count 10,100/ll), renal dysfunction (serum creatinine 1.12 mg/dl) and a coagulation dysfunction (prothrombin time 31 %, international normalized ratio 2.41, activated partial thromboplastin time 68.9 s). Tumor markers including serum carcinoembryonic antigen and serum carbohydrate antigen 19-9 levels were within normal limits. The abdomen was distended with normal peristalsis; moreover, mild tenderness was observed in the upper abdomen. No mass was palpable and no signs of peritoneal irritation were observed over the abdomen. Plain abdominal radiography revealed normal gas distribution. Plain abdominal computed tomography (CT) revealed a mass, suggesting intussusception in the jejunum (Fig. 1a). Enhanced abdominal CT revealed the ‘target-like’ sign (Fig. 1b) and ultrasonography revealed the ‘multiple concentric ring’ sign (Fig. 1c). Therefore, a diagnosis of enteroenteric intussusception was made. The small bowel obstruction and the cause of the intussusception were not evident. The patient was treated conservatively with fasting and transfusion therapy to prevent a small bowel obstruction. With no spontaneous resolution of the intussusception, a surgery was planned for day 6 after admission. The operative findings revealed a jejunal tumor about 30 cm from the Treiz ligament, and the jejunum including the tumor with a 5 cm margin were partially resected. The resected tumor was a 35 9 50 mm soft lobulated mass that spread laterally (Fig. 2). No invasion into the serosa was detected. The pathological findings revealed tubulovillous adenoma in almost all parts of the tumor and well-differentiated adenocarcinoma with atypical architecture invading the submucosa, but no vessel or lymphatic invasion (Fig. 3a, b). Immunostaining for p53 proteins revealed positive cells with densely stained nuclei in the adenocarcinoma component (Fig. 3c). Postoperative pathological findings confirmed TisN0M0 small intestinal cancer of stage 0. The postoperative course was uneventful, and the patient was discharged on foot.
Fig. 1 Computed tomography (CT) and ultrasonography. Plain abdominal CT (a) suggested invagination of the jejunum. Enhanced abdominal CT (b) revealed invagination of the jejunum but no obvious signs of intestinal obstruction. Ultrasonography (c) revealed a ‘target-like appearance’
Intussusception is a condition in which a part of the intestine invaginates into another section of the intestine. The part that prolapses into the other is called the intussusception and the part that receives it is called the intussuscipiens. Most intussusception occurs in childhood and is idiopathic. However, adult intussusception is rare and accounts for up to 16 % of all cases . Acute intestinal obstruction is uncommon, and most patients present with subacute, chronic, or intermittent symptoms . About 45 % of adult intussusception is due to small intestinal
tumors and one-third of these are malignant tumors. Surgical resection is considered to be one of the treatments, as malignancy is a common cause of adult intussusception . Intussusception can be divided into ileocolic, enteroenteric, and colocolic types. Ileocolic types are the most common because of local constriction of the mobile ileum end by abnormal intestinal peristalsis. A small intestinal intussusception is more often observed in the jejunum than in the ileum, and malignant lymphoma or lipoma is the most common tumors in the small intestine. As malignant
lymphoma or lipoma develops inside the intestinal lumen, it can cause intussusception; conversely, a smooth muscle
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Fig. 2 Resected specimen. Gross specimen from the resected jejunal segment revealed a 35 9 50 mm soft lobulated tumor
tumor that develops outside the intestinal lumen rarely causes intussusception. Small intestinal tumors are rare in adults with an incidence of 0.7–1.7/100,000 and account for 1.7–6.5 % [3–5] of all gastrointestinal tumors. Benign tumors occur at onethird the incidence of malignant tumors . Approximately 70 % of small intestinal tumors are jejunal tumors and 30 % are ileum tumors. Jejunal tumors usually develop within 60 cm from the Treiz ligament, and ileal tumors develop within 40 cm from the Bauhin’s valve. Despite the fact that the small intestine has about a ten-fold larger surface area than that of the colon, the rate of incidence of small intestinal tumors is low. Several factors have been suggested to explain the low occurrence of small intestinal tumors [6, 7]. The small intestinal mucosa has low carcinogenic potential and low production of carcinogens because of the low bacterial counts. The increase in benzyl peroxidase and immunoglobulin A may prevent tumor development. Widespread gut lymphoid tissue may play an important role in preventing tumor growth. Rapid intestinal
Fig. 3 Histological examination. Macroscopic examination revealed tubulovillous adenoma parts (red lines) and well-differentiated adenocarcinoma parts (black lines) (a). Microscopic examination revealed tubulovillous adenoma coexisting with tubulovillous adenocarcinoma, locally invading the lamina propria [hematoxylin and
eosin (H&E) stain, low-power field] (b). Tubulovillous adenoma and tubulovillous adenocarcinoma are observed (c) (H&E stain, highpower field). Immunostaining examinations for p53 proteins revealed positive cells with densely stained nuclei in adenocarcinoma component (d) (high-power field)
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flow through the small intestine limits contact as well as mucosal irritation. The most common malignant small intestinal tumors are adenocarcinomas, carcinoid tumors, malignant gastrointestinal stromal tumors, and lymphomas. Small intestinal adenoma accounts for 10–20 % of all small intestinal benign tumors . Most small intestinal adenomas develop in the duodenum and rarely develop in the lower small intestine. Small intestinal adenomas and colonic adenomas are divided into tubular adenomas, tubulovillous adenomas, and villous adenomas; among these, villous adenomas have the highest potential for malignant transformation, and 30 % of villous adenomas coexist with adenocarcinomas . Similar to adenomas in the colon, adenomas in the small intestine have the potential to proceed to adenocarcinomas. We searched for cases of intussusception caused by jejunal adenocarcinoma in adenoma; however, we could find only one report ; therefore, it is extremely rare that jejunal adenocarcinoma in adenoma is found after the occurrence of intussusception. A pathological review reported that 25 % of small intestinal adenoma is accompanied by adenocarcinoma . In contrast, 24 % of small intestinal adenocarcinoma is accompanied by adenoma . The improvement in small intestinal adenocarcinoma is suggested to be strongly associated with adenoma. In our case, tubulovillous adenocarcinoma coexisted with adenoma, suggesting that a small intestinal adenoma is a precursor of adenocarcinoma, as in the colon . Surgical treatment is the standard therapy for small intestinal cancer. If the lesion can be completely resected, the small intestine including the cancer should be resected with a margin of 5–10 cm with and/or regional lymph node resection. Only few cases of small intestinal cancer have been reported; therefore, there is no guideline for the treatment of small intestinal carcinoma and confirmed statements about the extent of resection of tumors and lymph nodes . Colonic adenoma is generated by adenomatous polyposis coli (APC) gene mutations and develops by ras gene mutations. Moreover, malignant transformation is associated with p53 gene mutations [13, 14]. p53 protein overexpression in the colon is supposed to be an index for malignant transformation. Some authors have reported that overexpression of p53 proteins is often found in small intestinal adenocarcinoma. Therefore, the p53 mutation plays an important role in malignant transformation in the small intestine .
adult intussusception is associated with some organic disease, surgery should be considered for the treatment and diagnosis, with or without the presence of a small intestinal obstruction. Conflict of interest of interest.
The authors declare that they have no conflict
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Conclusion We encountered a rare case of tubulovillous adenocarcinoma in adenoma presenting with intussusception. As most