Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas and account for less than 5% of all primary pancreatic malignancies. Included in this group are insulinomas, gastrinomas, glucagonomas and somatostatinomas. Collectively, these neoplasms are classified as functional PETs. When a PET is not associated with a clinical syndrome due to hormone oversecretion, it is referred to as a non-functional PET. Non-functionalPETs are pancreatic tumors with endocrine differentiation but lack a clinical syndrome of hormone hypersecretion. Although a pancreatic carcinoma shows aggressive biological behavior, a cutaneous metastasis from a pancreas carcinoma is rare. We report a case of a case of a cutaneous metastasis from an endocrine pancreatic carcinoma in a 50-year-old female that clinically manifested as a painful firm nodule on the back.
Female ; Gastrinoma ; Glucagonoma ; Humans ; Insulinoma ; Middle Aged ; Neoplasm Metastasis ; Pancreas ; Pancreatic Neoplasms ; Somatostatinoma

Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in the pancreas, are among the most common neuroendocrine tumors. The genetic causes of familial and sporadic PNETs are somewhat understood, but their molecular pathogenesis remains unknown. Most PNETs are indolent but have malignant potential. The biological behavior of an individual PNET is unpredictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally indicate a less favorable prognosis. Endocrine testing, imaging, and histological evidence are necessary to accurately diagnose PNETs. A 4-pronged aggressive treatment approach consisting of surgery, locoregional therapy, systemic therapy, and complication control has become popular in academic centers around the world. The optimal application of the multiple systemic therapeutic modalities is under development; efficacy, safety, availability, and cost should be considered when treating a specific patient. The clinical presentation, diagnosis, and treatment of specific types of PNETs and familial PNET syndromes, including the novel Mahvash disease, are summarized.
Combined Modality Therapy ; Gastrinoma ; diagnosis ; therapy ; Glucagonoma ; diagnosis ; therapy ; Humans ; Insulinoma ; diagnosis ; therapy ; Multiple Endocrine Neoplasia Type 1 ; diagnosis ; therapy ; Neuroendocrine Tumors ; classification ; diagnosis ; therapy ; Pancreatic Neoplasms ; classification ; diagnosis ; therapy ; Vipoma ; diagnosis ; therapy ; von Hippel-Lindau Disease ; complications

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas accounting for less than 5% of all primary pancreatic malignancies. Insulinomas, gastrinomas, glucagonomas and somatostatinomas is included in PET. PETs are usually classified into functioning and non-functioning tumors and presents with a range of benignity or malignancy. It is very important to accurate diagnose the PET location and to predict the benignity or malignancy of PET in terms of the treatment strategy, because PET have higher respectability, better response to chemotherapy and better prognosis compared to that of pancreatic adenocarcinoma. The utility and reliability of different imaging modalities depends on the characteristics of PETs, specifically their size. Functioning PET tend to be small (less than 2 cm), well circumscribed, homogeneous, and usually shown as strong enhancement on contrast enhanced CT or MR imaging. Non-functioning PET tend to be larger (4~10 cm), heterogeneous, and may contain the cystic areas of degeneration and necrosis. In this article, we present the various imaging findings of PET according to recent WHO classification.
Accounting ; Adenocarcinoma ; Gastrinoma ; Glucagonoma ; Insulinoma ; Magnetic Resonance Imaging ; Necrosis ; Pancreas ; Prognosis ; Somatostatinoma

OBJECTIVETo summarize our experience in the diagnosis and treatment of malignant pancreatic endocrine tumour.

METHODSWe retrospectively reviewed 36 cases of malignant pancreatic endocrine tumours in our hospital from July 1987 to April 2002, and summarized its clinical features.

RESULTSLiver metastasis was the main malignant manifestation of malignant pancreatic endocrine tumours (incidence rate 72.2%). Removals of primary lesion and isolated hepatic metastatic lesion were means of curative therapy. Interventional chemotherapy was an important adjuvant treatment.

CONCLUSIONComprehensive therapy plays an important role in improving the prognosis of malignant pancreatic endocrine tumour.

Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Carcinoma, Islet Cell ; diagnosis ; pathology ; therapy ; Chemotherapy, Adjuvant ; Female ; Follow-Up Studies ; Gastrectomy ; Gastrinoma ; diagnosis ; pathology ; therapy ; Glucagonoma ; diagnosis ; pathology ; therapy ; Humans ; Insulinoma ; diagnosis ; pathology ; therapy ; Liver Neoplasms ; secondary ; therapy ; Lymphatic Metastasis ; Male ; Middle Aged ; Neoplasm Invasiveness ; Pancreas ; surgery ; Pancreatectomy ; Pancreatic Neoplasms ; diagnosis ; pathology ; therapy ; Retrospective Studies

Isolated obstruction of the splenic vein leads to segmental portal hypertension, which is mainly originated from pancreatic disease, such as chronic pancreatitis, pancreatic pseudocyst, pancreatic cancer. The clinical manifestation are devoid of ascites and encephalopathy, but presented with gastric variceal bleeding. We experienced 27-year-old male patient who complained of hematochezia and melena. After work-up with CT and angiography, we performed operation with the impression of pancreatic tumor associated with splenic vein occlusion. It resulted nonfunctioning pancreatic endocrine tumor. Often called islet cell tumor, pancreatic endocrine tumor has various names according to secreted hormones, such as insulinoma, Zollinger-Ellison syndrome and glucagonoma. It was classified as nonfunctioning tumor if there were any evidence of hormone secretion by radioimmunoassay and immunohistochemistry. Surgical excision and adjuvant medical therapy is the mainstay of therapy. We performed tumor resection and splenectomy for control of gastric variceal bleeding due to splenic vein occlusion.
Adenoma, Islet Cell ; Adult ; Angiography ; Ascites ; Esophageal and Gastric Varices* ; Gastrointestinal Hemorrhage ; Glucagonoma ; Humans ; Hypertension, Portal ; Immunohistochemistry ; Insulinoma ; Male ; Melena ; Pancreas* ; Pancreatic Diseases ; Pancreatic Neoplasms ; Pancreatic Pseudocyst ; Pancreatitis, Chronic ; Radioimmunoassay ; Splenectomy ; Splenic Vein ; Zollinger-Ellison Syndrome

BACKGROUND: Neuroendocrine tumors of the pancreas are classified according to the endocrine function as insulinomas gastrinomas somatostatinomas, or nonfunctioning tumors. However, the morphologic features are not different from each other. Therefore, we tried to compare correlations among the morphologic features, endocrine function, and the immunohistochemical reaction with specific monoclonal antibodies to the tumors. METHOD: We reviewed the medical records of seven patients with pancreatic neuroendocrine tumors retrospectively, and analysed the clinical manifestations, the methods of diagnosis, the pathological characteristics and the results of surgery. Additionally, we compared the correlation between the clinical manifestations and the expression of immunohistochemical staining by using six different kinds of monoclonal antibodies to each tumor. RESULTS: The seven pancreatic neuroendocrine tumor patients were treated by surgical excision. Four patients had benign insulinomas, two had nonfunctioning malignant tumor and one patient had a benign nonfunctioning tumor associated with stomach cancer. The pattern of immunohistochemical stain of each tumor was not correlate with the clinical manifestations. CONCLUSION: The morphologic study with H & E stain, even with immunohistochemical staining of pancreatic neuroendocrine tumor, cannot support differentiation of the functional diagnoses, such as insulinoma, gastrinoma, somatostatinoma, nonfunctional tumors and so forth.
Antibodies, Monoclonal ; Diagnosis ; Gastrinoma ; Humans ; Insulinoma ; Medical Records ; Neuroendocrine Tumors* ; Pancreas ; Retrospective Studies ; Somatostatinoma ; Stomach Neoplasms

MicroRNA (miRNA), small non-coding RNA consisted of 19-24 nucleotides, are able to regulate gene expression at the post-transcriptional level. The aberrant expressions of miRNA have been found in various cancers and contribute to carcinogenesis by promoting the expression of proto-oncogenes or by inhibiting the expression of tumor suppressor genes. miRNA are related closely with the oncogenesis, progression, and prognosis of tumors. The discovery of the aberrant expression of miRNA in pancreatic ductal adenocarcinoma (PDA) and its target genes are helpful for the understanding of the pathogenesis of PDA and for the early diagnosis and prediction of this disease. In this paper, we summarize the recent research advances in miRNA expression in PDA and its target genes and discuss the potential role of miRNA in the diagnosis, and treatment of PDA.
Carcinoma, Pancreatic Ductal ; genetics ; Humans ; MicroRNAs ; genetics ; Pancreatic Neoplasms ; genetics

PURPOSE: Cystic pancreatic neoplasms are rare, but interesting, because of their high cure rate. With the exception of pseudocysts and serous cystadenomas, which are always benign, these cystic neoplasms are either premalignant or malignant. However, there is no reliable clinical criteria for differential diagnosis, and the treatment plan may be confusing. METHODS: From October 1994 to November 1999, 60 cases, diagnosed as a cystic neoplasm preoperatively were reviewed retrospectively. The clinical findings of benign lesions (benign group) and those for malignant or premalignant tumors (malignant group) were compared. RESULTS: The postoperative pathology results indicate 10 serous cystadenomas, 13 mucinous cystic neoplasms, 11 solid and papillary neoplasms, 10 duct ectasias, 2 cystic islet cell tumors, 1 metastatic papillary carcinoma, 1 lymphepithelial cyst, 2 simple cysts, 6 pseudocysts, and 4 retension cysts without malignancy. The mean age of the patients was 48.6 years, and the male-to-female ratio was 5 to 7. The accuracy of CT for diagnosing the malignancy of malignant tumors was 37.8% (14/37) and that of US was 22.2% (4/18). The rate of tumors having malignant potential was 71.7% (37/60). The mean size of the tumors in the benign group was smaller than that in the malignant group (p=0.014). There was a higher proportion of females in the malignant group than in the benign group (p=0.001). Heavy alcohol consumption was found more frequently in the benign group (p=0.021). There were no differences in the other clinical findings. The mortality rate of the operations was 0%, and the morbidity rate was 18%. CONCLUSION: Since it is difficult to determine the precise tumor type of a cystic pancreatic neoplasm preoperatively, all these lesions should be treated with surgical resection in order to identify and remove the malignant or premalignant neoplasms early. However, if the operative risk is high, malignant risk factors having large sized tumor, especially more than 6 cm, female, and having no history of heavy alcohol consumption may be useful for deciding the treatment plan.
Adenoma, Islet Cell ; Alcohol Drinking ; Carcinoma, Papillary ; Cystadenoma, Serous ; Diagnosis, Differential ; Dilatation, Pathologic ; Female ; Humans ; Mortality ; Mucins ; Pancreatic Cyst* ; Pancreatic Neoplasms ; Pathology ; Retrospective Studies ; Risk Factors

We present our surgical experiences with functioning neuroendocrine neoplasms of the pancreas to define its natural history, and to suggest its proper management. From June 1990 to June 2005, patients with diagnosis of functioning neuroendocrine (islet cell) neoplasms of the pancreas were retrospectively reviewed. Fourteen patients (5 men and 9 women) with a median age of 49 years (range, 12 - 68 years) were identified. Twelve patients (86%) had insulinoma, two (14%) had gastrinoma. One (7%) with pancreatic insulinoma was multiple endocrine neoplasia type 1. Intraoperative ultrasound scan (sensitivity, 83%) was the most powerful modality for tumor localization. Fifteen neoplasms with median tumor size 1 cm (range 0-3 cm) were resected. Four insulinomas (26.7%) were located in the head of the pancreas and 5 (36%), in the tail. Another 5 (36%) insulinomas and 1 (7%) gastrinoma were located around the neck area near the SMV or PV. Eleven patients (79%) underwent enucleation, and 2 patients (14%), distal pancreatectomy with splenectomy. 100% of patients with functioning neuroendocrine neoplasms of the pancreas have survived. The overall disease free 10-year survival was found to be about 81%. Exact localization of tumor by intraoperative ultrasound and surgical removal are promising for good prognosis.
Retrospective Studies ; Prognosis ; Postoperative Complications ; Pancreatic Neoplasms/*surgery/ultrasonography ; Neoplasm Metastasis ; Middle Aged ; Male ; Insulinoma/*surgery/ultrasonography ; Humans ; Gastrinoma/surgery/ultrasonography ; Female ; Child ; Aged ; Adult ; Adolescent

We present our surgical experiences with functioning neuroendocrine neoplasms of the pancreas to define its natural history, and to suggest its proper management. From June 1990 to June 2005, patients with diagnosis of functioning neuroendocrine (islet cell) neoplasms of the pancreas were retrospectively reviewed. Fourteen patients (5 men and 9 women) with a median age of 49 years (range, 12 - 68 years) were identified. Twelve patients (86%) had insulinoma, two (14%) had gastrinoma. One (7%) with pancreatic insulinoma was multiple endocrine neoplasia type 1. Intraoperative ultrasound scan (sensitivity, 83%) was the most powerful modality for tumor localization. Fifteen neoplasms with median tumor size 1 cm (range 0-3 cm) were resected. Four insulinomas (26.7%) were located in the head of the pancreas and 5 (36%), in the tail. Another 5 (36%) insulinomas and 1 (7%) gastrinoma were located around the neck area near the SMV or PV. Eleven patients (79%) underwent enucleation, and 2 patients (14%), distal pancreatectomy with splenectomy. 100% of patients with functioning neuroendocrine neoplasms of the pancreas have survived. The overall disease free 10-year survival was found to be about 81%. Exact localization of tumor by intraoperative ultrasound and surgical removal are promising for good prognosis.
Retrospective Studies ; Prognosis ; Postoperative Complications ; Pancreatic Neoplasms/*surgery/ultrasonography ; Neoplasm Metastasis ; Middle Aged ; Male ; Insulinoma/*surgery/ultrasonography ; Humans ; Gastrinoma/surgery/ultrasonography ; Female ; Child ; Aged ; Adult ; Adolescent