Pancreatic neuroendocrine tumors (PNETs) are relatively rare; however, the incidence has increased over the last few decades. They are classified as functional or non-functional tumors according to the presence of associated clinical symptoms. The majority are non-functional tumors. For classification and staging, the World Health Organization 2010 classification system is the most commonly accepted. Chromogranin A is the most sensitive marker but has insufficient specificity. In general, PNETs are hypervascular tumors, and multiphasic contrast-enhanced computed tomography is considered the first choice for imaging study. Multiphasic magnetic resonance imaging can detect PNETs smaller than 2 cm and small liver metastasis compared with other modalities. Somatostatin receptor scintigraphy is often used in cases where functional PNETs are suspected. Positron emission tomography (PET) scan with 18F-fluorodeoxyglucose cannot visualize PNETs, but PET with 68-Ga DOTATATE can. Endoscopic ultrasonography can characterize smaller PNETs using contrast and confirm histology through fine needle aspiration or biopsy. In this article, we review the characteristics of grading systems and diagnostic modalities commonly used for PNETs.
Biopsy ; Biopsy, Fine-Needle ; Chromogranin A ; Classification ; Diagnosis* ; Endosonography ; Incidence ; Liver ; Magnetic Resonance Imaging ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors* ; Positron-Emission Tomography ; Radionuclide Imaging ; Receptors, Somatostatin ; Sensitivity and Specificity ; World Health Organization

BACKGROUND/AIMS: 18F-Fluorodeoxyglucose positron-emission tomography (18F-FDG PET) has been used to assess the biological behavior of hepatocellular carcinoma (HCC). In this study, we investigated the usefulness of 18F-FDG PET for predicting tumor progression and survival in patients with intermediate Barcelona Clinic Liver Cancer (BCLC) intermediate-stage HCC treated by transarterial chemoembolization (TACE). METHODS: From February 2006 to March 2013, 210 patients treated with TACE, including 77 patients with BCLC intermediate-stage HCC, underwent examination by 18F-FDG PET. 18F-FDG uptake was calculated based on the tumor maximum (Tmax) standardized uptake value (SUV), the liver mean (Lmean) SUV, and the ratio of the Tmax SUV to the Lmean SUV (Tmax/Lmean). RESULTS: The mean follow-up period for the 77 patients (52 males, 25 females; average age, 63.3 years) was 22.2 months. The median time to progression of HCC in patients with a low Tmax/Lmean (< 1.83) and high Tmax/Lmean (> or = 1.83) was 17 and 6 months, respectively (p < 0.001). The median overall survival time of patients with a low and high Tmax/Lmean was 44 and 14 months, respectively (p = 0.003). Multivariate analysis revealed that the Tmax/Lmean was an independent predictor of overall survival (hazard ratio [HR], 1.96; 95% confidence interval [CI], 1.210 to 3.156; p = 0.006) and tumor progression (HR, 2.05; 95% CI, 1.264 to 3.308; p = 0.004). CONCLUSIONS: 18F-FDG uptake calculated by the Tmax/Lmean using PET predicted tumor progression and survival in patients with BCLC intermediate-stage HCC treated by TACE.
Aged ; Carcinoma, Hepatocellular/mortality/*radionuclide imaging/*surgery ; *Chemoembolization, Therapeutic/adverse effects/mortality ; Disease Progression ; Female ; *Fluorodeoxyglucose F18 ; Humans ; Kaplan-Meier Estimate ; Liver Neoplasms/mortality/*radionuclide imaging/*surgery ; Male ; Middle Aged ; Multimodal Imaging ; Neoplasm Staging ; *Positron-Emission Tomography ; Predictive Value of Tests ; Proportional Hazards Models ; *Radiopharmaceuticals ; Retrospective Studies ; Risk Factors ; Time Factors ; Tomography, X-Ray Computed ; Treatment Outcome

We present a rare case of functional stenosis of the jejunal loop following left hepatectomy and hepaticojejunostomy long after pylorus-preserving pancreaticoduodenectomy (PPPD), which was successfully managed by balloon dilation. A 70-year-old Korean man had undergone PPPD 6 years before due to 1.8 cm-sized distal bile duct cancer. Sudden onset of obstructive jaundice led to diagnosis of recurrent bile duct cancer mimicking perihilar cholangiocarcinoma of type IIIb. After left portal vein embolization, the patient underwent resection of the left liver and caudate lobe and remnant extrahepatic bile duct. The pre-existing jejunal loop and choledochojejunostomy site were used again for new hepaticojejunostomy. The patient recovered uneventfully, but clamping of the percutaneous transhepatic biliary drainage (PTBD) tube resulted in cholangitis. Biliary imaging studies revealed that biliary passage into the afferent jejunal limb was significantly impaired. We performed balloon dilation of the afferent jejunal loop by using a 20 mm-wide balloon. Follow-up hepatobiliary scintigraphy showed gradual improvement in biliary excretion and the PTBD tube was removed at 1 month after balloon dilation. This very unusual condition was regarded as disuse atrophy of the jejunal loop, which was successfully managed by balloon dilation and intraluminal keeping of a large-bore PTBD tube for 1 month.
Aged ; Bile Duct Neoplasms ; Bile Ducts, Extrahepatic ; Cholangiocarcinoma ; Cholangitis ; Choledochostomy ; Constriction ; Constriction, Pathologic* ; Diagnosis ; Drainage ; Extremities ; Follow-Up Studies ; Hepatectomy* ; Humans ; Jaundice, Obstructive ; Liver ; Muscular Disorders, Atrophic ; Pancreaticoduodenectomy* ; Portal Vein ; Radionuclide Imaging

Von Hippel-Lindau (VHL) syndrome is a rare neoplastic disorder characterized by central nervous system (CNS) and visceral tumors. We here present 68Ga-labelled [1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid]-1-Nal3-Octreotide positron emission tomography computed tomography findings in a 52 year old female with VHL syndrome, demonstrating both CNS and visceral tumors.
Brain Diseases/radionuclide imaging ; Female ; Humans ; Kidney Diseases/radionuclide imaging ; Liver Diseases/radionuclide imaging ; Middle Aged ; Multimodal Imaging/*methods ; Organometallic Compounds/diagnostic use ; Pancreatic Diseases/radionuclide imaging ; Positron-Emission Tomography/*methods ; Tomography, X-Ray Computed/*methods ; von Hippel-Lindau Disease/*radionuclide imaging

Basaloid squamous cell carcinoma is a rare and aggressive variant of squamous cell carcinoma, which mostly occurs in the upper aerodigestive tracts. Basaloid squamous cell carcinoma also typically arises in the anal canal, but is extremely rare in the lower gastrointestinal tract. A 70-year-old man presented with loose stool and intermittent hematochezia 2 months ago. Colonoscopy showed an ulceroinfiltrative mass on the rectosigmoid colon from 16 cm to 18 cm above the anal verge. Conventional colonoscope could not pass through the lesion but it was possible with pediatric colonoscope. Abdominal CT scan showed 1.6 cm sized wall thickening with circumferential luminal narrowing in the rectosigmoid colon and multiple ill-defined low density masses in both lobes of the liver. Therefore, colon cancer with liver metastasis was suspected. However, basaloid cells were noted on histologic examination, and they were weakly positive for synaptophysin on immunohistochemical study. After palliative lower anterior resection, histologic examination of the resected specimen revealed basaloid differentiation with keratin pearls, and tumor cells were positively stained with high molecular weighted cytokeratin (34BE12) and CK 5/6. Thus, the patient was finally diagnosed with basaloid squamous cell carcinoma of rectosigmoid colon with distant metastases.
Aged ; Carcinoma, Squamous Cell/*diagnosis/pathology/surgery ; Colonoscopy ; Colorectal Neoplasms/*diagnosis/pathology/surgery ; Humans ; Immunohistochemistry ; Keratins/metabolism ; Liver Neoplasms/radiography/secondary ; Lung Neoplasms/radionuclide imaging/secondary ; Male ; Positron-Emission Tomography ; Synaptophysin/metabolism ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Few studies have assessed the prognostic value of the primary tumor maximum standardized uptake value (SUVmax) measured by 2-[18F]-fluoro-2-deoxy-D-glucose PET-CT for patients with bile duct and gallbladder cancer. METHODS: A retrospective analysis of 61 patients with confirmed bile duct and gallbladder cancer who underwent FDG PET-CT in Kangbuk Samsung Medical Center (Seoul, Korea) from April 2008 to April 2011. Prognostic significance of SUVmax and other clinicopathological variables was assessed. RESULTS: Twenty-three patients were diagnosed as common bile duct cancer, 17 as hilar bile duct cancer, 12 as intrahepatic bile duct cancer, and nine as gallbladder cancer. In univariate analysis, diagnosis of intrahepatic cholangiocarcinoma and gallbladder cancer, mass forming type, poorly differentiated cell type, nonsurgical treatment, advanced American Joint Committee on Cancer (AJCC) staging and primary tumor SUVmax were significant predictors of poor overall survival. In multivariate analysis adjusted for age and sex, primary tumor SUVmax (hazard ratio [HR], 4.526; 95% CI, 1.813-11.299), advanced AJCC staging (HR, 4.843; 95% CI, 1.760-13.328), and nonsurgical treatment (HR, 6.029; 95% CI, 1.989-18.271) were independently associated with poor overall survival. CONCLUSIONS: Primary tumor SUVmax measured by FDG PET-CT is an independent and significant prognostic factor for overall survival in bile duct and gallbladder cancer.
Aged ; Bile Duct Neoplasms/*diagnosis/mortality/radionuclide imaging ; Cholangiocarcinoma/diagnosis/mortality/radionuclide imaging ; Female ; Fluorodeoxyglucose F18/diagnostic use/metabolism/standards ; Gallbladder Neoplasms/*diagnosis/mortality/radionuclide imaging ; Humans ; Kaplan-Meier Estimate ; Liver Neoplasms/diagnosis/mortality/radionuclide imaging ; Male ; Middle Aged ; Neoplasm Staging ; Positron-Emission Tomography/standards ; Prognosis ; Proportional Hazards Models ; Radiopharmaceuticals/diagnostic use/metabolism/standards ; Retrospective Studies ; Tomography, X-Ray Computed/standards

Zollinger-Ellison syndrome (ZES) is characterized by gastrinoma and resultant hypergastrinemia, which leads to recurrent peptic ulcers. Because gastrinoma is the most common pancreatic endocrine tumor seen in multiple endocrine neoplasia type I (MEN 1), the possibility of gastrinoma should be investigated carefully when patients exhibit symptoms associated with hormonal changes. Ureteral stones associated with hyperparathyroidism in the early course of MEN 1 are known to be its most common clinical manifestation; appropriate evaluation and close follow-up of patients with hypercalcemic urolithiasis can lead to an early diagnosis of gastrinoma. We report a patient with ZES associated with MEN 1, and urolithiasis as the presenting entity. A 51-year-old man visited the emergency department with recurrent epigastric pain. He had a history of calcium urinary stone 3 years ago, and 2 years later he had 2 operations for multiple jejunal ulcer perforations; these surgeries were 9 months apart. He was taking intermittent courses of antiulcer medication. Multiple peripancreatic nodular masses, a hepatic metastasis, parathyroid hyperplasia, and a pituitary microadenoma were confirmed by multimodal imaging studies. We diagnosed ZES with MEN 1 and performed sequential surgical excision of the gastrinomas and the parathyroid adenoma. The patient received octreotide injection therapy and close follow-up.
Gastrinoma/metabolism/pathology/ultrasonography ; Gastrins/metabolism ; Humans ; Immunohistochemistry ; Liver/radiography ; Magnetic Resonance Imaging ; Male ; Mesenteric Artery, Superior/radiography ; Middle Aged ; Multimodal Imaging ; Multiple Endocrine Neoplasia Type 1/complications/*diagnosis/radiography ; Pancreas/radiography/radionuclide imaging ; Pituitary Gland/radiography ; Positron-Emission Tomography ; Radiopharmaceuticals/diagnostic use ; Thyroid Gland/ultrasonography ; Tomography, X-Ray Computed ; Urolithiasis/*diagnosis/etiology ; Zollinger-Ellison Syndrome/complications/*diagnosis

No abstract available.
Amyloidosis/*radionuclide imaging ; Female ; Fluorodeoxyglucose F18/*diagnostic use ; Humans ; Liver Diseases/*radionuclide imaging ; Multiple Myeloma/*complications ; *Positron-Emission Tomography and Computed Tomography ; Radiopharmaceuticals/*diagnostic use

No abstract available.
Adult ; Carcinoma, Hepatocellular/radiography/radionuclide imaging/ultrasonography ; Granuloma, Plasma Cell/pathology/*radiography/radionuclide imaging ; Humans ; Liver Diseases/pathology/*radiography/radionuclide imaging ; Liver Neoplasms/radiography/radionuclide imaging/ultrasonography ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

Although the adrenal gland is a common site of metastasis from hepatocellular carcinoma (HCC), adrenal metastases are rarely seen in clinical practice because of its lower metastatic potential compared to the other malignancies. Adrenal metastases usually were detected at the time of diagnosis of primary HCC or simultaneously with intrahepatic recurrence after curative management of HCC. It is very rare that only metastatic HCC is detected without evidence of intrahepatic recurrence. Hereby, we report two cases of adrenal metastasis from HCC without intrahepatic recurrence after hepatic resection.
Adrenal Gland Neoplasms/*diagnosis/radionuclide imaging/secondary ; Aged, 80 and over ; Carcinoma, Hepatocellular/*pathology/surgery ; Humans ; Liver Neoplasms/*pathology/surgery ; Male ; Positron-Emission Tomography ; Tomography, X-Ray Computed