A Case of Undiferentiated Smal Intestinal Carcinoma with Pan 圃

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1 peritonitis 42 Report J l To 86(3) 11 日 2(2016) A Case of Uiferentiated Smal Intestinal Carcinoma with Pan 圃 Yoshizumi DEGUCHI 2,. 1 Takayuki SAT0 2,. 1 Hiroyasu SUGA Shinobu SUZUKP, Takao NAKAGA W A 1 Eiji ISOTANP 1. 2, 1 Department of Emergency Critical Care Medicine, Isesaki Sawa Medical Asociation Hospital 2Emergency Critical Care Center, Tokyo Women's Medical University Medical Center East 3 Department of Pathology, Isesaki Sawa Medical Asociation Hospital (Acepted March 18, 2016) Uiferentiated cancer of the smal intestine caries a por prognosis has rarely ben reported. A 63- year-old man presented with intermitent pain of the left lower abdomen that had ben left untreated. Blod tests showed inflamation high levels of tumor markers. Contrast-enhanced CT of the abdomen showed a smal intestinal tumor with a difuse thickened wal, fat tisue, peritoneal inflamation, peritoneal fluid were observed arou the tumor. Acute pan-peritonitis due to perforation caused by a smal intestinal tumor diagnosed. Perioperative fiings in the jejunum 10 cm from the ligament of Treitz showed a 3 司 cm,mas at 20 cm an 1 a side of this mas, there a 6 cm tumor invading into the sigmoid colon with perforation. Swolen lymph nodes of mesenterium omentum, a gastric tumor were also obserbed. Disection radical surgery were performed. Histopathologicaly, primary uiferentiated smal intestinal carcinoma diagnosed. The postoperative course god postoperative chemotherapy with an oral anticancer agent, S-l started after the patient resumed eating solid fod. He discharged on postoperative day 23, but with peritonitis carcinomatosa on postoperative day 75 subsequently died on postoperative day 18. readmited Key W ords: smal intestinal cancer, uiferentiated cancer, S-l Introduction Primary smal in testinal carcinoma s i very rare, uiferentiated smal intestinal carcinoma s i particularly rare, with very few reported cases of l 1 3 long-term postoperative survivai - ). We report a case of uiferentiated smal intestinal carcinoma where the subjective symptom of left lower abdominal pain went untreated peritonitis developed. Past medical history family history: Nothing Patien Case Report : t A 63-year-old man. of note. Life history ocupation: Corporate management; Smoking history: 20/ day for about 40 years; Drinking history: Social drinker. History of the present ilnes: Left lower abdominal pain apeared on December 25, 2012, intermitent pain persisted. On J anuary 8, 2013, he visited a nearby urology department with the complaint of oliguria. Urology failed to fi a problem, but black stol noted abdominal ultrasou iicated a pseudo-kidney sign, thus he refered to our department for a suspected tumor of the gastroin testinal trac. t Present ilnes at admision: At admision, the patient lucid, with a blod presue of 94/74 mmhg, pulse of 50/min with a regular heart bea, t 図 : Yoshizumi DEGUCHI Emergency Critical Care Center, Tokyo Women's Medical University Medical Center Eas, t Nishi-Ogu, Arakawa-ku, Tokyo, JAPAN yoshizumi08@gmai. l com

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3 4 open abdominal surgery on hospital day. 8 Intraoperative fiings: A moderate amount of turbid ascites observed in the abdominal cavity. A tumor measuring 3 cm observed in the jejunum, about 10 cm from the ligament of Treitz, a tumor measuring 6 cm had directly infiltrated into the sigmoid colon at 20 cm further to the anal side. An enlarged lymph node measuring 4 cm observed in the mesentery. An enlarged lymph node measuring 3 cm also fou in the omentum. Blunt disection of the smal intestine where the sigmoid colon infiltrated showed a pinholelike perforation in the center of the tumor. The smal in testinal tumor excised en bloc along with the lymph nodes, folowed by e-to-e anastomosis. The site of tumor infiltration in the sigmoid colon resected the omental lymph node disected; then, 2 cm of tumor in the gastric body partialy resected, a drain placed in Douglas' pouch in the inferior surface of the liver, to conclude the surgery. Histopathological fiings: The 6-cm tumor that had directly infiltrated into the sigmoid colon had an ulcer acompanied by partial perforation, exhibited a comparatively clearly-defined nodule with a periphery elevated in the maner of an embankmen. t The tumor nodule solid, filed with an aray of atypical cels having a large - r i regular nucleus, clearly-defined nucleolus, a wide amphophilic cytoplasm. The tumor cels had no clear, specific tre in their arangemen. t Some mitotic figures were also sen, some were fou to have sucumbed to necrosis. Tumor growth spaned the full thicknes of the wal, there exposure below to the delaminated surface serosal surface. Lymphatic invasion also observed. The tumor nodule measuring 3 cm that observed in the jejunum, about 10 cm from the ligament of Treitz orad of there included an ulcer, there growth of a tumor exhibiting similar histological signs. Tumor growth here spaned from the mucosa to the subserosal laye. r The 2-cm tumor nodule of the stomach also included an ulcer, exhibited growth of a tumor showing similar histological signs from the mucosa to the muscle layer, with clearly-defined nodule formation with a periphery elevated in the maner of an embankmen. t The greatest tumor diameter in the smal intestine, where the depth of invasion spaned the full thicknes, with growth infiltrating into a broad range, lymphatic invasion also observed; however, the tumor nodule of the stomach confined to betwen the mucosa muscle layer. Thus, we concluded that the 6 圃 cm tumor of the smal intestine the primary lesion. Immunostaining showed that the tumor cels were slightly positive for cytokeratin (AE1/ AE3). They were negative for UCHL-, l CD20, CD30, epithelial membrane antigen (EMA), Thus, these histological signs imunostaining results iicate smal intestine primary uiferentiated carcinoma (Fig.. ) 3 Acording to 8th edition of the J apanese Clasification of Colorectal Carcinoma, the carcinoma clasified as SE, N 3, H 0, P3, M, l Stage IV. He folowed a favorable course. other than developing postoperative wou infection, started on fluids on postoperative day ; 5 on postoperative day 7, he gained the ability to eat sanbugayu J ( apanese poridge), on postoperative day 23, he started on an oral anticancer agen, t S-l discharged. On postoperative day 75, he 1 e-hospitalized with a fever abdominal pain. Abdominal contrast-enhanced CT fiings: A 63-m tumor observed in the nearby mesentery after the smal intestinal carcinoma surgery, this also fou to be suroued by lymph node metastasis nodules believed to be peritoneal disemination. A 65 x 48. mm tumor also fou in the sigmoid colon. The left adrenal tumor had increased in sized as compared to the previous CT (Fig.. ) 4 Lower gastrointestinal eoscopy fiings: A Borman Type I tumor ocupied the majority of the lumen in the sigmoid colon about 37 cm from the pectinate line. This corespoing to the site of direct invasion of the smal intestinal carcinoma at the time of the previous surgery, biopsy results showed porly diferentiated carcinoma, which desmin.

4 54.1/~ 3ylacipocsorcaM ipyta yllac lac enil.ylralugerri evit rof nitarekotyc 4 Enhaced 57 showed sisatsatem noitanimes a e pyt 2 tumor si devresbo ni eht munje sllec htiw egral ralugerri suelcun era ylesuffid detartlifni rehtruf lacimehcotsihonumi seiduts show eht noisel (AE l/ AE3) :B( HE x 05 :C HE x 02 :Dnitarekotyc lanimodba CT noevitarepotsop romut ly mph dna laenotirep elpitlum tnerucer no eht muiretnsm. ) swora( yad edon -sid del he dei cinoma ot be recunce ( gained lanitsetni bypas the ytiliba 48 he uerwent surgery ot eat but retal colstomy sit inotirep a -icrac adilos -isop.x)002 abdominal dnuof ni eht law noitartlifni eht tsrif.yregus Discuion erit lanitsetniortsag -er -ipocsorcim dna mrof ot ebylthgils lea( /AE3) 81 retfa on day The day ot tfel -rac.)5 On evitarpos d na 5 Type II tumor ecnerucer noloc by.ypocsonoloc diomgis nomatosa devilb )A( % ni terms nat tumors acounts tcart rof ni terms fo latot mucosal ecafrus ylera ocur ni eht 57 % fo the fo htgnel are but -ne 09 -gilam

5 46 the frequency of primary smal intestinal malignant tumors malignant s i about 1 to 3 % of l a gastrointestinal tract 4 tumors. ) Acording to Yao et I a 5 ), the frequency of smal intestine malignant tumors by tissue type s i 32.6 % cancer, % malignant lymphoma, % gastrointestinal stromal tumor (GIST). Thus, primary smal intestinal cancer s i the most comon smal intestine cancer s i adenocarcinoma, at 87.5 %, 6.3 % s i uiferentiated carcinoma; thus uiferentiated smal intestinal carcinoma can be described as an extremely rare disease. In terms of site of ocurence, 56.7 % s i jejunal cancer 43.3 % s i ileal cancer; frequent sites of ocurence are thought to be within 60 cm from the ligament of Treitz within 40 cm from Bauhin's valve. In the present case, a 3 αn tumor fou in the jejunum about 10 cm from the ligament of Treitz, a 6 圃 cm tumor also had infiltrated directly into the sigmoid colon from 20 cm toward the anal side. The J apanese Clasification of Colorectal Carcinoma defines ui 旺 erentiated carcinoma as when cancer does not show a teency toward diferentiating into any of the types of cancer-adenocarcinoma, mucinous carcinoma, signet ring cel carcinoma, squamous cell carcinoma, ぽ0 r adenosquamous carcinoma 一 does not exhibit glular cavity formation or have clearly-evident mucus produ 山 1 児 C 嗣 tion が 1 9 )10 玖 0 i 加 ng 郡 s for u 必 f i 妊 ferentia 抗 ted smal in testinal carcinoma would make t i dificult to reach a diagnosis simply from an HE-stained tisue specimen, because or smal polynuclear cels or spile cels grow without asuming a certain structure. In many reports, a definitive diagnosis of uiferentiated smal intestinal carcinoma reached by immunostaining with multiple antibodiesω. The tumor cels in the present case were EMA negative, but only slightly positive for AE1/ AE3, apeared epithelial in nature. They were negative for UCHL-, 1 CD20, CD30, EMA, desmin, thus ruling out malignant lymphoma non-epithelial tumor. Uiferentiated carcinoma s i positive for other cy 陶 to be about 0.2 % of l a gastrointestinal tract cancer. ) Acording to Ikeguchi et ) aj 1 Hiramitsu 6 8 ), large said tokeratins (CK 7, CK 20, CAM 5.2, etc.) 12)13). With smal intestinal cancer. metastasis s i ten times more frequent than primary cancer 凶. In smal intestinal cancer has ben diagnosed, t i s i criticaly important to diferentiate betwen primary cancer metastasis. Since the 2 smal intestinal tumors the gastric tumor of the present case were exposed to the lumen, careful consideration neded to determine the primary site. Generaly, intramural metastasis of the gastrointestinal tract s i when a metastasis forms uer the mucosa or serosa, separated from the primary tumor, via the submucosal layer, lymphatic network, or venous network; the primary tumor the metastasis are histopathologicaly identica, l there should be no continuity betwen the tumors. Macroscopicaly, there said to be submucosal tumor 園 like elevation. When the present case s i considered on the basis of these facts, Together with the gastric tumor with a large number of lymph node metastases, the 3-cm tumor considered to be non-continuous metastasis that had formed in the smal intestine apart from the primary 6-cm tumor that showed identical history had ben perforated. Clinical symptoms of smal intestine malignant tumors overal include abdominal pain, vomiting, bleding, palpable mases, ileus, anemia, abdominal bloating, but in reported cases of smal intestine cancer in J apan, abdominal pain, vomiting, other ileus symptoms as wel as blody stol have caused patients to uergo surgery before a definitive diagnosis can be reached, due to acute abdomen. In the present case, frequent abdominal pain had gone untreated since the year prior, progresed into peritonitis. Blod biochemistry do not include any useful tumor markers for uiferentiated smal intestinal carcinoma, but the present case exhibited high values for both CEA CA Metastasis to the stomach or sigmoid colon s i thought to have lead to the increase in these tumor markers. Elevated soluble IL-2 receptor s i also observed in tumor 町 bearing coitions blod diseases such as malignant lymphoma, therefore s i reports have also noted t i would be an auxiliary diagnosis of uiferentiated smal intestinal carci

6 47 noma 15). In terms of treatment strategies for primary smal intestinal cancer in the future, we fel s i most important that t i be discovered in the early stages, when there s i no lymph node metastasis. Testing generaly includes intraoral or enema contrast examination, eoscopy, u1 t rasou diagnosis, CT, MR, I angiography, the like. However, these tests rarely provide a definite diagnosis before surgery. In CT MRI fiings, smal intestinal cancer often exhibits a 旺 erent wal thickening, with uneven density in the tumor. t I s i considered necessary to confirm the site of ocurence by first diagnosing the presence of a tumor with ultrasou diagnosis, CT, or MRI then identifying the feding vesels by. angiography. Recent improvements in eoscopic technology, such as double-balon eoscopes capsule eoscopes, the spread of PET have raised the frequency of discovery of smal intestinal cancer 玖 Double-balon eoscopy or capsule eoscopy in the smal intestine relies greatly on the practitioner's skils, the examination takes time, but there s i great potential for these new diagnostic procedures. Treatment s i generaly smal intestinal resection including lymph node disection when resection s i posible, but in practice, many cases are advanced they often e up in pa i 1 l ative surgery. Even in the present case, the primary tumor resected in the first surgery, yet there widespread lymph node metastasis, we observed direct infiltration into the sigmoid colon as wel as left adrenal metastasis, thus this not radical surgery. In the seco surgery, as wel, we observed remnant that had become a lump in the abdominal cavity, recurent tumors, surgery confined to bypas colostomy. There s i no regimen at present for chemotherapy for smal intestinal cancer, but there are reports where adjuvant chemotherapy atempted on the basis of regimens for stomach cancer colon cancer anticancer s i an oral agent that bles 5-chloro-24 圃 dihydroxypyridine, a competitive inhibitor of dehydropyrimidine dehydrogenase, which s i a - 5 fluorouracil (5-FU)-degrading enzyme, into tegafur, which s i a prodrug of 5-FU, thus intensifying the stard I7 ). t i S-l a anti-tumor efects of the 5-FU; t i reportedly s i e 旺 'e ctive in stomach cancer colon cancer. Recent years have sen reports where chemotherapy with S-l performed after surgery for smal intestinal mucinous adenocarcinoma or uiferentiated carcinoma yielded recurence-fre survivaf 819 ). S-l has advantages in particular in that t i does not exhibit adverse events alows for medication to be administered on an outpatient basis, without adversely afecting the patien ' t s QO. L On this basis, we obtained informed consent in the present case to perform oral chemotherapy with S-, 1 but this did not lead to long 圃 term surviva. l There are very few reported cases of long-term survival with uiferentiated smal intestinal carcinoma which has a por prognosis, acording to Prid 回 gen et al, the thre-year survival rate, five-year survival rate, 10-year survival rate are reportedly 34.0, % 23.9, % 12.5, % 陀 r especti 討 vely デ戸 72 初制 0ω) Testing methods for early diagnosis have ben established in recent years, reports of cases of radical surgery are expected to increase. References in this paper represent a search from 197 to August 2014 with the keywords smal in 聞 testinal cancer", ui 百 'e rentiated cancer", 1" in the Igaku Chuo Zash. i The authors declare no conflicts of interes. t References Carcinoma Haries primary cases years cancer 1) Ackman FD: f o the smal intestine. Can Med Asoc J 32 : ) 6 ( , ) DJ, Harison CVPR: Primary carcinoma f o the smal intestine. Br Med J 1 (3878): , ) Nakamura T, Otani Y, Kokuba Y t e : l a A case f o uiferentiated smal intestinal carcinoma with neuroeocrine l e c diferentiation observed n i asociation with multiple smal intestine metastases. J Jpn Surg Asoc 61 (12): , 20 4) Ogata S, Kazuto M, Kurayama H t e : l a A study f o five f o malignant smal intestinal tumors n i the clasrom. J Jpn Soc Gastroenterol Surg 19: ) Yao H, Yao K, Matake H t e : l a Sumary f o the last five (195-19) f o Japanese case reports f o smal intestine tumors. Stomach Intestine 36: , 201 6) Kurakane K: Clinical statistical discusion f o primary jejunal l a e l i n i J apan. Saishin Igaku 34: , 1979 often S-

7 48 chief cancer. 7) lkeguchi M, Nishidoi H, Hiroshi K et : l a A case of uiferentiated ileal cancer- A study of 95 reported cases of primary jejunal ileal cancer in Japan. J Jpn Surg Asoc 54: ,193 8) Hiram It su T, Masahi H, N akanishi K et : l a A case of primary ui 旺 'e rentiated jejunal cancer. J Jpn Soc Gastroenterol Surg 42 (12): , 209 9) J apanese Society for Cancer of the Colon Rectum: J apanese Clasification of Colorectal Carcinoma, (8 th edition). Kanehara Shupan, Tokyo (2013) 10) Sasaki M, Hanatate F, Yamamura K et : l a A case of uiferentiated smal intestinal carcinoma with anemia as complain. t J Jpn Soc Gastroenterol Surg 21 (10): ,198 1) Gadoya M, Suzuki Y, Hazama K et : l a A case of uiferentiated primary smal intestine J J pn Soc Gastroenterol Surg 36 (11): , ) Usuda M, Nakano T, Hirano T et : l a A case of uiferentiated smal intestinal carcinoma onset with unidentified fever. J Jpn Surg Asoc 68 (10): ,207 13) Arase K, Hi 伊 lrashi A, Yamada T et : l a A case of primary ui 旺 'e rentiated smal intestinal carcinoma acompanied by bilateral adrenal metastasis. J J pn Surg Asoc 72 : ) 5 ( , ) Matsuoka T, Katsuragi K, Matsuzaki T et : l a A case of uiferentiated smal intestinal carcinoma carcinoma. Gastroenterol therapy treated therapy. acompanied by multiple smal intestinal intramural metastasis. J Jpn Soc Gastroenterol Surg 41 : ) 1 ( ,208 15) Seishima R, Nagase T, Nakagawa M et : l a A case of ui 宜 erentiated smal intestinal carcinoma accompanied by multiple smal intestinal metastases lymph node metastases in the pancreatic head. J J pn Surg Asoc 71 : ) 6 ( , ) ldeguchi K, Obayashi K, Ueda H et : l a Two cases of smal intestinal malignant tumor. J Kyoto Pref Univ Med 12 : ) 4 ( , ) lnaba M, Kataoka M, Yasuhara 1 et al : A case of triplicate cancer including primary ui 旺 erentiated smal intestinal J Jpn Soc Surg 43 (11): , ) Gunji N, Gohongi T, i I da H et : l a A case of jejunal cancer acompanied by lung metastases peritoneal disemination where preoperative TS-l sucesful J J pn Soc Gastroenterol Surg 40 (11): , ) Maruyama T, Horiuchi S, Enomoto T: A case of smal intestinal cancer acompanied by peritoneal disemination with S-1 J Jpn Soc Gastroenterol Surg 37: 1249,204 20) Pridgen JE, Mayo CW, Dockerty MB: Carcinoma of the jejunum ileum exclusive of carcinoid tumors. Surg Gynecol Obstet 90: ,1950 腹膜炎で発症した小揚未分化癌の 1 例 l 伊勢崎佐波医師会病院救急医療科 2 東京女子医科大学東医療センター救急医療科 3 伊勢崎佐波医師会病院病理検査科出口善純 1.2 佐藤孝幸 1. 2 須賀弘泰 1. 2 鈴木忍 3 中川隆雄 1 磯谷栄二 2 小腸腫蕩のなかでも非常に稀で予後不良の小腸未分化癌の 1 例を経験した. 症例は 63 歳男性. 左下腹部の間欠痛が続いていたが放置していた来院時血液検査で炎症反応と腫蕩マーカー高値を認めた. 腹部造影 CT 所見では小腸壁のびまん性腫癌状肥厚があり, 周囲の脂肪織や腹膜の不整と中等量腹水を認め小腸腫蕩による穿孔性腹膜炎と診断した開腹するとトライツ靭帯から約 10cm の空腸に径 3cm 大の腫癌を, その紅側 20cm の部分に径 6cm の腫痛を認めた. 後者は S 状結腸に浸潤しており, ピンホール状の穿孔を伴っていた. 腸間膜や大網にリンパ節腫脹を認めた. 小腸腫蕩をリンパ節とともにー塊に切除し端々吻合した.S 状結腸の腫蕩浸潤部位を切除し大綱のリンパ節切除, 胃体部の腫蕩を部分切除した病理検索では小腸未分化癌が原発巣と考えられた術後経過良好で経口摂取可能となったため 23 日目より経口抗がん剤 S-l を開始し退院したが術後 75 日目に癌性腹膜炎で再入院し術後 18 日目に死亡した. -12-