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Background Solitary fibrous tumors (SFTs) are rare tumors, mostly derived from connective tissue mesenchymal cells that arise from the pleura. There are very few reports of primary pancreatic SFT. Preoperative diagnosis is difficult owing to the lack of distinctive radiological findings. We report a case of pancreatic SFT with particularly rare malignant findings. Case presentation A 60-year-old man was referred to the hospital because of a right upper quadrant mass and abnormal liver function test results. Contrast-enhanced computed tomography (CT) showed a well-defined enhanced tumor measuring approximately 8 cm in the pancreatic head. Magnetic resonance imaging (MRI) showed T1WI hypointensity, T2WI hyperintensity, and DWI hyperintensity. The main pancreatic duct and common bile duct were dilated owing to obstruction by the tumor. The following tumor markers were mildly elevated: carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), SPan-1, and DUPAN-2. The histological diagnosis obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) was negative for pancreatic ductal carcinoma, malignant lymphoma and neuroendocrine tumor, suggesting the possibility of mesenchymal tumor, but the diagnosis was not confirmed. The patient was judged suitable for surgery and underwent subtotal stomach-preserving pancreatoduodenectomy with D2 lymph node dissection. On histopathological examination of the resected specimen, infiltrating spindle-shaped cells had proliferated, containing numerous mitotic figures, with necrotic findings inside the tumor. Immunostaining was positive for cluster of differentiation-34 (CD34), B cell CLL/lymphoma-2 (Bcl-2), and signal transducer and activator of transcription (STAT6). On the basis of these findings, a diagnosis of malignant pancreatic SFT was made. The patient remains free of recurrent disease after 12 months of follow-up without adjuvant therapy and he is being carefully followed up as an outpatient. Conclusions We experienced a case of malignant pancreatic head SFT. Immunohistochemical staining of the extracted specimens was useful for diagnosis.

A66 -year-old man was diagnosed with chronic myeloid leukemia(CML). Imatinib treatment had been initiated, and a major molecular response(MMR)was achieved. The patient had anemia and was diagnosed with descending colon cancer. The patient was surgically treated, and then received postoperative adjuvant chemotherapy with UFT/LV. However, imatinib was not administered during that period. The patient could undergo postoperative adjuvant chemotherapy for 6 months without acute exacerbation of the CML.

We experienced four cases of metachronous double cancer after curative resection for pancreatic adenocarcinoma without the background of intraductal papillary mucinous neoplasm. Case 1, a 67-year-old Japanese female developed tongue cancer 53 months after a pylorus-preserving Whipple resection for pancreatic head adenocarcinoma. Case 2, a 66-year-old female developed multiple breast cancers 52 months after a pylorus-preserving pancreaticoduodenectomy for pancreatic head adenocarcinoma. Case 3, a 59-year-old male developed an adenocarcinoma in the remnant pancreatic head 63 months after a distal pancreatectomy for pancreatic body cancer. Case 4, a 68-year-old male developed lung cancer 92 months after a Whipple procedure for pancreatic head adenocarcinoma. Gemcitabine was administered to three patients as adjuvant chemotherapy at an average administrated dose of 38,199 mg per body surface area. Since primary pancreatic ductal adenocarcinoma is aggressive and always associated with a devastating outcome, metachronous double cancer is scarcely seen. All four cases received curative-intent surgery for each metachronous cancer and were alive for at least 20 months.

We report a rare case of metachronous multiple adenocarcinoma of the pancreas. A 59-year-old Japanese man visited our institute for a routine workup as a hepatitis C virus carrier, resulting in detection of a 3-cm tumor in the pancreatic body by screening echogram. Results from several imaging modalities were consistent with pancreatic carcinoma. Distal pancreatectomy along with dissection of partial gastrectomy, transverse colectomy, and lymph node dissection were performed in November 2003. Histological examination confirmed a pancreatic ductal adenocarcinoma with a clear surgical margin and negative lymph node metastases. Gemcitabine was administered for 5 years, then suspended because no recurrent signs were found. The patient returned to our hospital in March 2009, with obstructive jaundice along with a 2-cm tumor in the head of the remnant pancreas. The condition of the patient was carefully investigated and extra-pancreatic metastatic lesions were not found; a pancreaticoduodenectomy was then carried out. Histological examination revealed a diagnosis of pancreatic adenocarcinoma arising from the remnant pancreas gland.

症例は72歳，男性．下血を主訴に当院を受診し，腹部超音波検査にて偶然骨盤内に境界明瞭な腫瘍を発見された．CT検査で腫瘍は仙骨前面右側に位置し，充実成分と嚢胞性分の混在する腫瘍で周囲組織への浸潤はなく，MRI検査ではT1強調画像で低信号，T2強調画像で不均一な高信号だった．確定診断が得られなかったが腫瘍摘出術を施行した．腫瘍は仙骨前面に強固に癒着しており鋭的に切離した．病理学的に腫瘍は良性変性型神経鞘腫であった．術直後より右下腿のしびれを訴えたが2カ月程度で軽快した．神経鞘腫は特異的な症状や特徴的な画像所見がないため良悪性を含めた術前診断は困難である．また，摘出に伴い神経障害を惹起することがあり手術に際しては充分なインフォームド コンセントが必要である．

症例は50歳，女性．2003年５月に潰瘍性大腸炎（UC）と診断された．内科治療を施行されたが奏効せず，症状の増悪を認めたため2003年７月14日結腸全摘術および回腸瘻，直腸粘液瘻造設術を施行した．同年８月20日頃よりストーマ周囲の皮膚に疼痛を伴う発赤，びらんが出現し同25日には約28×60mmの皮膚潰瘍形成を認めストーマ周囲壊疽性膿皮症（PPG）と診断された．プレドニンの内服およびストーマ管理の工夫によってPPGは一時軽快したが，UCの増悪とともに再度皮膚潰瘍の拡大を認めた．2004年９月22日残存直腸切除術，回腸嚢肛門吻合術，膿皮症部皮膚切除および左下腹部に双孔式回腸瘻造設術を施行した．再手術後，壊疽性膿皮症の再燃は認めていない．

症例は33歳女性．18歳より潰瘍性大腸炎として，年1回の大腸内視鏡検査を施行されていた．平成18年の大腸内視鏡検査にて，横行結腸の生検部位から高中分化腺癌を認め，当科紹介となった．大腸内視鏡再検査にて横行結腸に強い炎症をともなう，不整顆粒状，結節状変化を認め，同部位の生検にて小型類上皮肉芽腫を認めた．以上より，クローン病に合併したcolitic cancerと診断し，腹腔鏡下結腸亜全摘，回腸直腸吻合術を施行した．病変はIIc様であり，病理所見にて，35×18mmの粘液産生の著明な高分化腺癌で，病変部周囲にdysplasiaを認め，深達度はpSS，リンパ節転移はpN0で，クローン病に合併した横行結腸癌の診断となった．今回我々はクローン病病変部に横行結腸癌が合併し，周囲にdysplasiaを認めた症例を経験したので若干の文献的考察を加えて報告する．

We report four cases of Crohn's disease (CD)-associated colorectal cancer (CRC) in our department. CASE 1: A 42-year-old Japanese man had a 14-year history of ileocolon CD. He had a history of an ileocecal resection and a stricture plasty. At the age of 42, sigmoid colostomy was performed because of the deterioration of the anal stenosis. After this operation, the perianal pain had remained, and a magnetic resonance imaging scan revealed a rectal tumor. CASE 2: A 30-year-old Japanese man had a 13-year history of ileocolon CD. He had a history of an ileostomy, a subtotal colectomy, and ileo-rectal anastomosis. At the age of 30, he had perianal pain, and a colonoscopy revealed a rectal cancer. An abdomino-perineal resection of the remnant rectum was performed. CASE 3: A 46-year-old Japanese man had a 9-year history of ileocolon CD. He experienced abdominal fullness. Colonoscopy revealed an ascending colon cancer. He underwent a subtotal colectomy and ileo-rectal anastomosis. CASE 4: A 33-year-old Japanese woman had a 16-year history of ileocolon CD. She had no changes in symptoms of CD. Surveillance colonoscopy revealed a transverse colon cancer. She underwent a subtotal colectomy and ileo-rectal anastomosis.As the number of patients with CD and with CD-associated CRC has increased in Japan, CD-associated CRC, as noted in these patients, should be kept in mind in the management of patients with CD. In addition, a surveillance system of patients with CD should be established and should prompt further study about CD-associated CRC.

今回われわれは31歳の女性で右鼠径部の痛みと腫脹を主訴に受診し,緊急手術を行い子宮円索血栓性静脈瘤と診断した1例を報告する. 腫瘤は妊娠20週に現れ,正常分娩4日後に,急速に増大して,落痛が出現した.鼠径ヘルニア嵌頓を疑い,緊急手術を施行した.術中に血栓性静脈瘤を鼠径管内に認めた.妊娠後期・産褥期の鼠径部腫脹,痔痛などの症状がみられた場合,子宮円索血栓性静脈瘤は念頭に置く必要があると思われる.

今回われわれは直腸癌術後孤立性脳転移に対し腫瘍摘出術および術後照射を施行し,6年3カ月の無再発長期生存例を経験したので報告する.症例は68歳,男性.平成6年8月直腸癌と診断され腹会陰式直腸切断術を施行した.病理組織学的検査では,直腸Rb,腫瘍径は5.0×6.5cm,中分化腺癌,mp,n0,H0,P0,M(-),stage Iであった.平成10年7月頃より失語症,右不全麻痺を認め,脳腫瘍を指摘された.平成10年8月19日脳腫瘍全摘術施行.腫瘍は2.5×1.5cm,組織学的には中分化腺癌であり,直腸癌の脳転移と診断した.術後定位脳放射線治療(50Gy/25回)を施行.直腸癌術後10年3カ月,転移性脳腫瘍術後6年3カ月の現在無再発生存中である.

症例は66歳,男性. 2002年9月より心房細動を指摘され以後,内服薬で加療されていた. 2002年11月16日より,上腹部痛を自覚し当院を受診した.腹痛は,一過性で軽快したが, 12月18日,再び激しい間歇性の腹痛を認め入院となった.精査にて遠位空腸に著しい管状狭窄像を認めた.腫瘍性病変の存在は否定的であり,狭窄型虚血性小腸炎の術前診断にて,原因の確定診断と治療を兼ね, 2003年1月22日,腹腔鏡補助下手術を施行した.病変部小腸は漿膜面の発赤,壁の硬化を認め,小切開を加え小腸部分切除を施行した.その他に異常所見はなかった.術後経過は良好で,術後7病日に退院した.虚血性小腸炎の本邦報告例において腹腔鏡補助下の手術経験は数件のみであるが,腹腔鏡補助下手術は,低侵襲であるだけでなく良好な広い視野が得られ他疾患の鑑別にも有用と考えられた.

Cytomegalovirus (CMV) infection has been reported to be a cause of refractory ulcerative colitis (UC). We herein report a case of refractory ulcerative colitis complicated by CMV infection requiring surgery. A 22-year-old man was admitted to our hospital with lower abdominal pain and bloody diarrhea. Under a diagnosis of acute UC, he was treated with prednisone 60 mg/day and sulfasalazine. Since his symptoms appeared to improve, the prednisone dosage was gradually reduced to 20 mg/day. After 5 months, he had an unexpected flare-up with fever and fresh anal bleeding. Colonoscopy demonstrated a punched out ulcer in the sigmoid colon. Biopsies by colonoscopy revealed cytomegalic inclusion bodies. Serologic and immunologic studies also suggested a recent CMV infection. Under a diagnosis of intractable UC complicated by a CMV infection, ganciclovir therapy was carried out, and the steroid therapy was tapered. Although the serum antigenemia became negative after the antiviral therapy, follow-up colonoscopy confirmed the severe stenosis after the punched-out ulcer healed completely. Since his symptoms did not improve, it was necessary to perform an elective proctocolectomy despite antiviral therapy. He was discharged with an uneventful postoperative course. It is important to recognize CMV colitis as a complication of inflammatory bowel disease, particularly in severe steroid-resistant colitis. Furthermore, in cases which fail to respond to antiviral treatment, the patient may ultimately require surgery.

39歳,男性.1991年より下痢症状あり,大腸型クローン病と診断され,近医に外来通院していた.1993年より臀部に排膿を認め,サラゾスルファピリジン,抗生物質の投与にて加療していたが,軽快傾向なく1997年に当科へ紹介された.両側臀部(右臀部優位)の皮膚は硬く,肥厚し,広範に多数の膿瘍の排出孔.を認めた.メトロニダゾール坐剤の投与にて加療していたが,軽快なく,2002年5月24日,手術を施行した.両側部臀の膿瘍の排出孔をcoring outし,また皮下の病変組織をすべて切除した.術後,排膿は治まり37病日退院となった.クローン病に合併した慢性膿皮症(化膿性汗腺炎)の報告は過去に14例のみと少なく治療方針も明確でない.広範で多数の膿瘍の排出孔をともなった慢性膿皮症に対しては,膿瘍の排出孔のcoring out,皮下組織の全切除により根治可能であると考えられた.

症例は15歳,男性.右下腹部痛を主訴に来院した.来院時,体温37.1°C, Mc Burney点を中心に軽度の自発痛と圧痛および反跳痛が認められた.全身の表在リンパ節の腫脹は認めず,腹部所見では肝・脾ならびに腫瘤は触知しなかった.末梢血検査では白血球数8,900/mm3と白血球数の増多は軽度であった. CRPは陽性であった.腹部超音波検査および腹部CTでは右下腹部,虫垂の部位に腫瘤が認められたため急性虫垂炎の診断にて手術を施行した.虫垂は根部より約3cmの正常な部位と,先端部に8.5×5×5cmの腫瘤を認めた.周囲のリンパ節腫大は認めず,虫垂切除術を施行した.病理組織学的にはびまん性,大細胞型, B細胞性の悪性リンパ腫(LSG分類)であった.術後全身化学療法(CHOP, 6クール)を行い,術後13カ月の現在再発の徴なく社会復帰している.

Malignant pheochromocytoma accounts for approximately 10% of pheochromocytoma cases. The main site of distant metastasis is the liver. Hypertensive crisis due to catecholamine oversecretion is potentially fatal. We present a case of malignant pheochromocytoma with multiple liver metastases. A 60-year-old female with repeated hypertensive episodes was diagnosed with malignant pheochromocytoma. She underwent a left adrenalectomy and partial hepatectomy with resection of segment 6. Catecholamine levels remained high after surgery and she received repeated cycles of chemotherapy. Four months after surgery, multiple liver metastases were detected. In spite of ongoing chemotherapy, catecholamine levels eventually became uncontrollable. Serum and urine noradrenaline and vanillylmandelic acid levels increased, but adrenaline and dopamine levels stayed within the normal range. Preoperative liver imaging revealed multiple metastases in all segments except segment 4. Percutaneous transhepatic portal vein embolization (PTPE) of the right and lateral branches of the portal vein was performed. The functional liver volume of segment 4 increased after PTPE. Right hepatectomy, lateral segmentectomy and partial resection of segment 1 were performed 10 mo after the initial surgery. Intraoperative ultrasonography detected two small tumors in segment 4, which were treated with intraoperative microwave coagulation therapy. Noradrenaline levels normalized immediately after the second hepatectomy. As there was increased telomerase activity in the resected specimen, she received adjuvant chemotherapy. She remained in good health for 2 years. However, further metastases eventually occurred and she subsequently died due to a brain hemorrhage. Hepatectomy may be a therapeutic option for reduction of tumor mass in pheochromocytoma with liver metastases.

A 67-year-old Japanese man complained of a painful lump in his anus. He had a 15-month history of myelodysplastic syndrome (MDS) and had been diagnosed with MDS overt leukemia. A solid lump measuring 1.0 cm in diameter was detected in the anal verge. Under a diagnosis of a thrombosed external hemorrhoid, thrombectomy was performed under local anesthesia. One week after thrombectomy, the wound had not healed, and grayish-green tissue was seen at the bottom. A biopsy of the wound revealed atypical mononuclear cell infiltration. Myeloperoxidase and lysozyme were positive on immunohistochemical staining. Finally, the diagnosis of granulocytic sarcoma (GS) was made. Though it is well known that perianal complications occur quite often in patients with leukemia, it is unusual for a diagnosis of GS of the anus to be definitely established. To our knowledge, there has not been a previous report of GS presenting as a thrombosed external hemorrhoid. The development of GS should be considered during the management of such lesions, especially in patients with bone marrow disorders, such as acute myeloid leukemia (AML) or high-risk MDS.

We report a rare case of ulcerative colitis with sarcoidosis and dermatomyositis. A 33-year old woman had a ten-year history of ulcerative colitis and dermatomyositis. After nine-year maintenance treatment with sulphasalazine and steroids, she underwent a proctocolectomy and ileal pouch-anal anastomosis because of deterioration of ulcerative colitis. One year after the operation, she noticed some subcutaneous masses in the right forearm and left lower leg. A total biopsy of the mass was performed, and noncaseous epithelioid granulomas were found in the specimen. Chest roentgenogram and chest computed tomography showed hilar adenopathy and a diffuse granular shadow. The diagnosis of sarcoidosis was made. Concomitant development of ulcerative colitis and sarcoidosis may not be incidental, and there may be a common factor in the two diseases. Ulcerative colitis also may be a part of a systemic disorder associated with abnormal immune reactions. Complications by disorders associated with other immunologic abnormalities, as noted in this patient, should be kept in mind in the management of ulcerative colitis.

A 77-year-old male was admitted to our hospital complaining of dyschezia. Computed tomography (CT) and colonoscopy (CF) revealed a huge sigmoid colon cancer invading the bladder and seminal vesicle. Chemotherapy with mFOLFOX6 was initiated preoperatively, and the tumor shrunk markedly after seven courses of treatment. Pelvic exenteration with negative margins was carried out. The patient is still alive and disease-free 16 months after surgery. It was suggested that mFOLFOX6 may be useful for advanced colon cancer invading other organs when used as neoadjuvant chemotherapy.

A combination of CPT-11, continuous 5-fluorouracil(5-FU)and leucovorin(LV), the Arbeitsgemeinschaft für Internistische Onkologie(AIO)regimen, is widely used for the treatment of metastatic CRC. The efficacy and toxicity of this regimen, however, have not been determined in Japanese patients with metastatic CRC. Our objective was to evaluate the safety of the AIO regimen plus CPT-11 in Japanese colorectal carcinoma(CRC)patients. We investigated the maximum tolerated dose(MTD), dose-limiting toxicity(DLT), and recommended dose(RD)for CPT-11 and continuous 5-FU. CPT-11, 5-FU, and l-LV were administered on days 1, 8, and 15 of a 28-day cycle. The dose of CPT- 11 was escalated from 40 mg/m2 (level 1)to 80 mg/m2 (level 3). The 5-FU dose was then escalated from 1,000 mg/m2 (level 4)to 2,000 mg/m2 (level 5). If neither level met the criteria for the MTD, the recommended dose was defined as level 5, and the dose escalation was discontinued, because the maximum approved weekly dose of CPT-11 alone in Japan is 80 mg/m2 and the dose of 5-FU in the original AIO regimen was 2,000 mg/m2. A total of 18 patients were enrolled in this study. Hematological and non-hematological toxicity were infrequent and mild. There were no toxicities greater than grade 2 at each dose level. Level 5 did not meet the MTD criteria. Our results confirm that the modified AIO plus CPT-11 regimen is safe for Japanese patients. The recommended doses in the present study were CPT-11 80 mg/m2, 5-FU 2,000 mg/m2, and l-LV 250 mg/m2.

A 54-year-old woman visited our hospital with a chief complaint of lower abdominal pain and melena. The patient was diagnosed with sigmoid colon cancer using colonoscopy. Abdominal CT revealed metastases to para-aortic lymph node, so our diagnosis was unresectable sigmoid colon cancer. She underwent a transverse colostomy to avoid stenosis. Two weeks after surgery, she underwent a 1-week chemotherapy regimen (CPT-11 80 mg/m(2)/week+5-FU 2,000 mg/m(2)/week+l-LV 250 mg/m(2)/week) modified AIO regimen combined irinotecan for 3 weeks, followed by a 1-week rest interval as one course. Throughout the period of treatment, there was no adverse event, and this regimen has been maintained for 5 courses. After 5 courses of chemotherapy, primary tumor and metastases to para-aortic lymph nodes were remarkably reduced on colonoscopy and abdominal CT. So, she could undergo curative resection. Pathological efficacy was Grade 3, a complete response. This combination therapy may well be useful for advanced colon cancer patients.

The exact mechanism of blood vessel thrombus formation remains to be defined. Here, we introduce a new approach to probe thrombus formation in blood vessels of living animals using intravital microscopy in green fluorescent protein (GFP)-transgenic mice to simultaneously monitor platelet aggregation and procoagulant activity. To this end, GFP-expressing platelets and annexin A5 labeled with a fluorescent dye were employed to visualize and analyze platelet aggregation and markers of procoagulant activity (platelet surface phosphatidylserine (PS)). Laser-induced thrombi increased and then decreased in size with time in vessels of living animals, whereas platelet surface PS initiated at the site of injury and then penetrated into the thrombus. PS-positive platelets were predominantly localized in the center of the thrombus, as was fibrin generation. The experimental system proposed here is a valuable tool not only for investigating mechanisms of thrombus formation but also to assess the efficacy of antithrombotic drugs within the vasculature.

Retractile mesenteritis is a rare inflammatory mesenteric disorder with unknown etiology. We reported a resected case of mass-forming retractile mesenteritis. A 64-year-old man noticed a mass with mild tender in the right lower abdomen. Colonoscopy revealed no abnormal findings. Computed tomography of the abdomen showed a solitary tumor of the ileocecal mesentery. Ileocecal resection was performed. Pathological findings were inflammatory change of the mesentery with marked fibrosis. Final diagnosis was retractile mesenteritis. He is doing well for 26 months after the operation.

Paraduodenal hernia is the most common internal hernia. The clinical symptoms of paraduodenal hernia may be intermittent and nonspecific. Therefore, it is difficult to diagnose preoperatively. Abdominal computed tomography (CT) scan currently plays an important role in the evaluation and management of paraduodenal hernia before surgical operation. We report one unique case of preoperatively diagnosed left paraduodenal hernia complicated by advanced ascending colon cancer and reviews of Japanese literature.