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We report two cases of carcinoma of the pancreas with anomalous junction of the pancreaticobiliary tracts. A 71-year-old Japanese woman had obstructive jaundice. Ultrasonography showed a hypoechoic mass in the pancreatic head and computed tomography demonstrated a low-density nodule in the pancreatic head. Endoscopic retrograde cholangiopancreatography displayed a double duct sign and an anomalous junction of the pancreaticobiliary tracts. The patient underwent a pancreatoduodenectomy. The histopathologic diagnosis of the resected specimen was adenocarcinoma of the pancreatic head. A 56-year-old Japanese man also developed obstructive jaundice. Ultrasonography and computed tomography showed a huge mass almost replacing the whole pancreas and involving the superior mesenteric artery, splenic artery, splenic vein, and portal vein. Multiple hepatic metastases and peritoneal dissemination were present. Endoscopic retrograde cholangiopancreatography demonstrated an anomalous junction of the pancreaticobiliary tracts. The patient died of hemorrhage from esophageal varices. We discuss the relationship between the anomalous junction of the pancreaticobiliary tracts and pancreatic carcinoma.

A 39-year-old woman was admitted to hospital due to perforated relapsing duodenal ulcer. Clinical, laboratory, and surgical examinations revealed a peripancreatic lymph node gastrinoma as the cause of Zollinger-Ellison syndrome. Further examinations established multiple endocrine neoplasia type 1 (MEN1) with a germline mutation at codon 1153 (T->A) in exon 7, causing an amino-acid change, from isoleucine to asparagine (Ile348Asn), in the MEN1 gene. The following findings strongly supported a diagnosis of primary lymph node gastrinoma: a rapid fall of the serum gastrin level after operation, the continuous normalization of the serum gastrin level before and after secretin stimulation, the lack of any symptoms, and the absence of another tumor for 13 years after surgical resection of the tumor-bearing lymph node. A review of similar cases in the world literature reveals that not all gastrinomas in lymph nodes are the result of metastastic spread. A long-term symptom-free follow-up after the excision of a lymphnode gastrinoma is the only reliable criterion for the diagnosis of a primary lymph node tumor. To our knowledge, this is the only well-documented case of a primary lymph node gastrinoma in a patient with MEN1. Our case supports the idea that any gastrinoma in patients with MEN1 should be surgically resected for cure if possible.

A 24-year-old woman underwent gastrectomy because of a gut autonomic nerve (GAN) tumor originating from the gastric antrum; three synchronous liver metastases were also resected. Liver resection was repeated twice, due to recurring liver metastases. After a disease-free period of 2 years, liver transplantation was felt to be indicated because of the presence of non-resectable, recrudescent liver metastases. Nearly 5 years after orthotopic liver transplantation, there are no signs of clinical or biological tumor recurrence. In view of the literature, liver transplantation may very well be indicated in the presence of liver metastases stemming from GAN tumors which originate from the proximal gastrointestine.

We report a left-hand-assisted laparoscopic resection of hepatocellular carcinoma that developed in an accessory liver in a 47-year-old man. Preoperative assessment of the location of the tumor and the feeder vessels by combined selective angiography and computed tomography studies predicted the feasibility of laparoscopic procedures for complete removal of the tumor. In an attempt to avoid direct contact of the tumor capsule with rigid instruments during the operation, left-hand-assisted procedures were attempted. The encapsulated mass, 6 × 5 × 3 cm in size, was located on the posterior side of the left diaphragm, and a thin stalk between the tumor and the margin of the left lateral segment of the liver proper was recognized. Hand-assisted procedures ensured the complete mobilization of the lesion with an adequate margin, without any unexpected capsular tear. Left-hand-assisted laparoscopic procedures would be feasible for the easy and safe resection of localized hepatocellular carcinoma developing in an accessory liver.

The expression of ectopic pancreatic and duodenal homeobox factor 1 (Pdx1) can transform hepatocytes into pancreatic endocrine cells. Small hepatocytes (SHs) have a high possibility to be a cellular source for islet cell transplantation. However, the efficacy of the transformation of SHs into pancreatic endocrine cells is not fully understood. The focus of our study was to compare the efficacy of the transformation into pancreatic endocrine cells of SHs and mature hepatocytes (MHs). MHs and SHs were cultured for 3 and 10 days, respectively, before Adeno-Pdx1 gene transduction. Western blot analysis was performed for pancreatic transcription factors, and reverse-transcription polymerase chain reaction (RT-PCR) was performed for the gene expression of pancreatic hormones. Confocal laser microscanning analysis was used to observe insulin and glucagon expression. Although the pancreatic transcription factors Pdx1, Ngn3, NeuroD, and Pax6 were induced in both SHs and MHs after Adeno-Pdx1 gene expression, the pancreatic transcription factors Nkx2.2 and Nkx6.1 were induced in SHs more than in MHs. Glucagon mRNA expression was seen in both SHs and MHs, whereas insulin mRNA expression was higher in SHs than in MHs. Confocal laser microscanning analysis showed that SHs expressed both insulin and glucagon, whereas MHs predominantly expressed glucagon. SHs were transformed into both insulin-and glucagon-expressing cells, and the efficacy of the transformation into insulin-expressing cells of SHs was higher than that for MHs. Thus, SHs could be a more suitable source of future cell therapy than MHs.

Eighty cases (82 tumors) of hepatic angiomyolipoma (AML) from the world literature were reviewed, including our 8 cases. The tumors occurred predominantly in females (female: male ratio, 56∶24). Only six patients (7.5%) had associated tuberous sclerosis. The majority of the tumors (95%) occurred in non-cirrhotic, almost normal liver. The tumors usually presented as a hyperechoic mass on ultrasound (US) examination, a hypodense mass on computed tomography (CT) scan, and hyperintense mass on both T1- and T2-weighted magnetic resonance (MR) imagings. However, the imaging features of the tumor were occasionally variable according to the content of fat tissue component, and the tumors could not be clearly differentiated from other hepatic tumors, particularly hepatocellular carcinoma (HCC), based on the imaging features alone. Tumor location was recorded in 81 tumors; 44 were located in the right lobe, 30 in the left, 5 in the caudate, and 2 in both lobes of the liver. The tumor diameter ranged from 0.3 to 36cm (mean, 8.0±7.0cm). Macroscopically, the tumors usually had no fibrous capsule. The cut surface was yellow to dark brown depending on the amount of fat tissue or blood vessels. The histologic features were quite variable and occasionally included atypical and/or pleomorphic cells, which could lead to the erroneous diagnosis of a variety of benign and malignant tumors, including lipoma, leiomyoma, HCC, hepatoblastoma, leiomyosarcoma, and malignant fibrous histiocytoma. Extramedullary hematopoiesis was found in 33% of the tumors. Reactivity of the tumor cells of the liver with HMB-45, a melanoma-specific antibody, can accurately establish the diagnosis of AML.