[Successful R0 Resection of Hilar Cholangiocarcinoma Invading the Inferior Vena Cava through Left Trisegmentectomy with Combined Resection of the Portal Vein and Inferior Vena Cava after Gemcitabine/Cisplatin Combination Chemotherapy-A Case Report].

A 65-year-old woman was admitted to our institution with sonography results indicating a caudate lobe mass. CT showed a large low-density mass in the caudate lobe, extensively involving the inferior vena cava and main portal vein. Moderately differentiated adenocarcinoma was found on transcutaneous biopsy. We therefore regarded this tumor as a severe locally advanced hilar cholangiocarcinoma and initiated gemcitabine/cisplatin combined chemotherapy. The tumor gradually reduced in size. However, after 28 courses of treatment, CT showed persistent tumor invasion in the left trunk of the portal vein and inferior vena cava invasion in succession in the middle; the tumor had not yet invaded the left hepatic vein. Owing to myelosuppression and general malaise, it was difficult to continue chemotherapy. After 32 courses of treatment, the patient underwent a left trisegmentectomy with combined resection of the portal vein and inferior vena cava. Postoperative microscopic findings revealed no apparent invasion of the tumor in the inferior vena cava, thus suggesting successful R0 resection. The patient is alive without recurrence 18 months postoperatively.

Metastatic primary pulmonary leiomyosarcoma to the pancreas diagnosed by endoscopic ultrasound-guided fine-needle aspiration.

A 77-year-old female patient consulted our hospital for an abnormal shadow observed on chest X-ray. Computed tomography revealed the shadow of a mass in the right lower lung lobe and two shadows of masses in the pancreatic head and body. 18F-fluorodeoxyglucose-positron emission tomography showed an intense uptake only in the fields corresponding to these three masses. Each mass was diagnosed as leiomyosarcoma by transcutaneous needle biopsy of the pulmonary mass and endoscopic ultrasound-guided fine-needle aspiration of the pancreatic masses. The primary site was the lung because the pulmonary lesion was solitary, and no tumor was found in other organs. In English language literature, a case of primary pulmonary leiomyosarcoma with metastasis solely to the pancreas has not yet been reported to the best of our knowledge.

T1b primary remnant cystic duct cancer following cholecystectomy: A case report.

INTRODUCTION: Although primary cystic duct cancer is a rare entity, remnant cystic duct cancer is even more rare. We report a case of early cystic duct cancer following cholecystectomy. PRESENTATION OF THE CASE: A 81 year-old man complained temporary loss of appetite. He had underwent cholecystectomy for acute cholecystitis 5 years prior. Contrast enhanced computed tomography, magnetic resonance image and endoscopic ultrasonography showed remnant cystic duct tumor with protrusion to common bile duct. Endoscopic retrograde cholangiography revealed defect of contrast medium around confluence of the remnant cystic duct and common bile duct. We performed step biopsy by using forceps which revealed adenocarcinoma. Based on these findings, extrahepatic bile duct and remnant cystic duct resection were performed. The histopathology showed adenocarcinoma, pap > tub2, filling in remnant cystic duct, 30 mm in size but showed no lymphovascular or perineural invasion, no lymph node metastasis and negative surgical margin, and was classified as pT1bN0M0. CONCLUSION: This is a rare case of primary carcinoma of remnant cystic duct cancer which is detected during computed tomography follow up for hepatic cell carcinoma recurrence. We confirmed remnant cystic duct cancer and its superficial extension to common bile duct with endoscopic ultrasonography and intraductal ultrasonography. Proper curative surgery was performed.

Small Cystic Pancreatic Neuroendocrine Neoplasm with Huge Liver and Bone Metastases.

Pancreatic neuroendocrine neoplasms occasionally have a cystic component. We herein report a case of multiple hepatic tumors, including a huge one and a 24-mm sized pancreatic cystic lesion. The hepatic tumor showed an enhancement pattern consistent with hepatic adenoma. The pancreatic cystic lesion revealed a thickened outside border and a solid inside component, which was enhanced following a contrast study, suggesting cystic pancreatic neuroendocrine neoplasm. Surgical resection was performed. After the surgery, somatostatin receptor scintigraphy detected an occult lumbar spine metastasis. Huge multiple liver and bone metastases of the neuroendocrine neoplasm G2 component were seen, with a G1 small primary lesion.

Edwardsiella tarda-associated cholangitis associated with Lemmel syndrome.

Edwardsiella tarda is an unusual human pathogen. Gastroenteritis is the most frequently reported manifestation of E.tarda infection and extraintestinal infection including cholangitis has rarely been reported. The overall mortality rate for E.tarda bacteremia is, however, reported to be up to 50% (Janda and Abbott, 1993). We describe a 80-year-old diabetic woman with cholangitis and E.tarda bacteremia with a biliary obstruction associated with a large juxtapapillary duodenal diverticulum (Lemmel syndrome) in the setting of past partial hepatectomy and cholecystectomy. She was successfully treated with endoscopic biliary drainage and antibacterials.

Immunoglobulin G4-related hepatic inflammatory pseudotumor invading the abdominal wall.

A 50-year-old woman presented with epigastralgia. Computed tomography (CT) of the abdomen revealed a 6-cm well-enhanced mass extending from the left lobe of the liver to the abdominal wall, suggestive of cholangiocarcinoma. Liver and skin mass biopsies did not provide evidence of hepatic malignancy but were rich in plasma cells and sclerotic lesions. Subsequent detection of elevated serum immunoglobulin G4 (IgG4) led to a diagnosis IgG4-related inflammatory pseudotumor (IPT) of the liver. Treatment with systemic corticosteroids resulted in rapid clinical improvement. This case is the first report of an IgG4-related hepatic IPT invading the abdominal wall.

[A case report of suspected Stauffer syndrome presenting with fever, extensive inflammation, and abnormal liver function tests].

A man was referred to our hospital because of malaise and abnormal liver function tests. He had had a kidney removed for early renal cell carcinoma. We performed ultrasonography, CT scan, and liver biopsy, all of which were normal. However, FDG-PET revealed abnormal, diffuse uptake in the bone. A bone biopsy showed abnormal clear cells resembling renal cell carcinoma. Because there was no other primary origin, this histopathological finding led to the diagnosis of bone metastasis of renal cell carcinoma, and the abnormal liver function test was thus inferred to be due to Stauffer syndrome.

[CT enterography with polyethylene glycol electrolyte solution for diagnosis of Crohn's disease].

This study aimed to assess the diagnostic usefulness of CT enterography (CTE) in Crohn's disease in Japanese patients. A total of 32 cases with bowel symptoms underwent CT enterography with polyethylene glycol electrolyte solution as oral contrast medium, among which 18 were clinically diagnosed as Crohn's disease and 14 were not: ulcerative colitis 1, Beçhet disease 1, simple ulcer 1, inflammatory bowel disease without definite diagnosis 5 and bowel symptoms of unknown origin 6. The incidence of bowel wall thickening, mural hyperenhancement, "the comb sign", mesenteric lymph nodes swelling, and stenosis were significantly higher in Crohn's disease than in other disease. Moreover, uneven bowel wall thickening and unilateral mural hyperenhancement on CTE were characteristic features of Crohn's disease, which our original quantitative evaluation with imaging analysis could support. Consequently, CTE is useful in Japanese patients with Crohn's disease.

[A case of jejunal adenocarcinoma with serum DUPAN-2 elevation].

A 50-year-old woman was admitted to our hospital because of abdominal pain and vomiting. Ileus with ulcerated jejunal tumor was diagnosed and biopsy revealed adenocarcinoma. Because her serum level of DUPAN-2 was high, she was examined by PET scan, which revealed that she had a left ovarian mass in addition to the jejunal tumor. Surgical resection was performed: both tumors were adenocarcinoma, but the ovarian tumor was considered to be metastatic clinically and histologically. Immunostaining for DUPAN-2 was positive in the both tumors. The serum level of DUPAN-2 returned to normal after the surgery, and has been within normal limits for about 3 years without any additional therapy. This case shows a possible relation between small bowel adenocarcinoma and DUPAN-2.

[A case of pulmonary adenocarcinoma accompanied by minimal change nephrotic syndrome, antiphospholipid syndrome and warm-type autoimmune hemolytic anemia].

A 49-year-old man was urgently admitted due to edema in both leg and left toe pain. A chest radiograph revealed a solitary nodule in the right lung field. Detailed investigations including bronchoscopy and renal biopsy led to a simultaneous diagnosis of clinical stage IIIB pulmonary adenocarcinoma, minimal change nephrotic syndrome, antiphospholipid syndrome, and warm-type autoimmune hemolytic anemia. Prednisolone was administered for nephrotic syndrome, antiphospholipid syndrome and warm-type autoimmune hemolytic anemia, and 6 courses of chemotherapy with 70Gy radio-therapy were performed. The pulmonary nodule significantly decreased in size and the other three autoimmune diseases appeared to be well-controlled. Thirteen months after admission, multiple brain metastases developed along with worsening antiphospholipid syndrome symptoms including lupus anticoagulant. Following whole-brain irradiation, the brain metastases decreased in size and antiphospholipid syndrome symptoms improved. Thirty-nine months after the initial visit, the primary lung cancer, its brain metastasis and the 3 other autoimmune diseases appeared to be well-controlled. The temporal correlation of the lung cancer and the three autoimmune diseases suggests the latter may be paraneoplastic syndrome.