Maria Gabriella Brizi

MR imaging and MRCP of hilar cholangiocarcinoma

Hilar cholangiocarcinoma, or Klatskin tumor, is a primary malignancy arising from the bile duct epithelium, at the confluence of the right and left hepatic ducts, within the porta hepatis [1]. The relative incidence of cholangiocarcinoma among primary liver cancers reported in autopsy series is relatively rare, ranging from 5% to 30% [2]; however, it is the most common primary malignancy of the biliary tree [3]. Hilar cholangiocarcinoma appears most frequently in the sixth and seventh decades of life [4], but most patients with risk factors may develop the neoplasm at younger ages; men are affected more frequently than women. Risk factors for cholangiocarcinoma are primary sclerosing cholangitis, choledochal cysts, familial polyposis, congenital hepatic fibrosis, infection with Clonorchis siniensis (Chinese liver fluke), and a history of chemical and thorium dioxide exposure (Thorotrast). The prognosis is poor, with an overall 5-year survival rate of 1% [5]. Because of this poor prognosis, many patients were treated with palliative drainage rather than with surgery. However, because of technical advances in diagnostic imaging that allow for better patient selection, and because of improved surgical techniques resulting in lower operative morbidity and mortality, more patients are being treated with surgical resection. This has improved overall 5-year survival rates after surgery to 20% [6, 7]. Surgical exploration should be undertaken only when preoperative examination has shown a potential for curative resection because the risks of palliative surgery for malignant obstructive jaundice are high, with surgical mortality rates of 20–30% [8]. Further, accurate preoperative assessment of hilar cholangiocarcinoma resectability has increased its importance because percutaneous and endoscopic palliative techniques for biliary drainage are now available. For these reasons, an accurate preoperative assessment of hilar cholangiocarcinoma is critical in choosing treatment planning.

Evaluation of the Added Value of Diffusion-Weighted Imaging to Conventional Magnetic Resonance Imaging in Pancreatic Neuroendocrine Tumors and Comparison With 68Ga-DOTANOC Positron Emission Tomography/Computed Tomography.

Objectives The aims of this study were to investigate the added value of diffusion-weighted imaging (DWI) in pancreatic neuroendocrine tumor (pNET) evaluation and to compare magnetic resonance imaging (MRI) to 68Ga-DOTANOC positron emission tomography/computed tomography (PET/CT) results. Methods Morphological MRI (T2-weighted [T2-w] + contrast-enhanced [CE] T1-w) and DWI (T2-w + DWI) and 68Ga-DOTANOC PET/CT in 25 patients/30 pNETs were retrospectively evaluated. Per-patient and per-lesion detection rates (pDR and lDR, respectively) were calculated. Apparent diffusion coefficient values were compared among pNET and surrounding and normal pancreas (control group, 18 patients). Apparent diffusion coefficient and standardized uptake value (SUV) values were compared among different grading and staging groups. Results No statistically significant differences in PET/CT and MRI session detection rates were found (morphological MRI and DW-MRI, 88% pDR and 87% lDR; combined evaluation, 92% pDR and 90% lDR; 68Ga-DOTANOC PET/CT, 88% pDR and 80% lDR). Consensus reading (morphological/DW-MRI + PET/CT) improved pDR and lDR (100%). Apparent diffusion coefficient mean value was significantly lower compared with surrounding and normal parenchyma (P < 0.01). The apparent diffusion coefficient and SUV values of pNETs among different grading and staging groups were not statistically different. Conclusions Conventional MRI, DW-MRI + T2-w sequences, and 68Ga-DOTANOC PET/CT can be alternative tools in pNET detection. Diffusion-weighted MRI could be valuable in patients with clinical suspicion but negative conventional imaging findings. However, the consensus reading of the 3 techniques seems the best approach.

The treatment of neuroendocrine tumors with long-acting somatostatin analogs: A single center experience with lanreotide autogel

The aim of this retrospective study was to evaluate the efficacy, safety, and tolerability of lanreotide autogel given to metastatic well-differentiated (WD) neuroendocrine tumors (NET) patients observed in our Institute between 2005 and 2008. Patients with metastatic NET referred to our tertiary referral center were given lanreotide autogel 120 mg/month by deep sc injection for a period of at least 24 months. The efficacy was evaluated by the relief of disease symptoms, behavior of tumor markers and response rate in terms of time to tumor progression. Safety and tolerability were evaluated by assessing the onset of adverse events and treatment feasibility. Twenty-three patients (13 males), median age 62 yr (range 32–87) were considered for the study. All patients were affected by WD metastatic NET and had tumor progression in the last 6 months before the enrolment in the study. Median duration of response was 28 months (range 6–50 months). Fourteen patients (60.9%) showed flushing and diarrhea which improved by 85.7% and 55.6%, respectively, bronchoconstrinction and abdominal pain also ameliorated. A complete, partial or no-changed response in the tumor markers behavior was observed, respectively, in 42.9%, 22.9%, and 17.1% of cases. According to RECIST (Response Evaluation Criteria In Solid Tumors) criteria (version 1.1), there were 2 partial regression (8.7%) and 15 stable disease (65.3%); 6 patients (26.0%) progressed. No patient complained from any severe adverse reaction. The results of our study suggest that lanreotide autogel is effective in the symptoms, biochemical markers, and tumor progression control of WD metastatic NET and confirm that the treatment is well tolerated.

Adult Medullary Cystic Disease of the Kidney and Pancreatic Cystic Disease: a New Association

A rare case of a woman with the adult form of medullary cystic disease associated with pancreatic cysts in pancreas divisum is described, which suggests that specific attention should be paid to computed tomography findings in the presence of pancreatic and renal cysts.

Masking effect of chronic pancreatitis in the interpretation of somatostatin receptor positron emission tomography in pancreatic neuroendocrine tumors.

To the Editor: W e present the rare occurrence of a concurrent pancreatic neuroendocrine tumor (pNET) and pancreatic ductal adenocarcinoma (PDAC) in a patient with multiple endocrine neoplasia 1 (MEN1) syndrome. It is important for clinicians to consider the possibility of PDAC in patients with MEN1 because aggressive early surgical intervention provides the only chance of cure. Multiple endocrine neoplasia 1 is characterized by parathyroid adenomas, pNETs, and anterior pituitary tumors. Multiple endocrine neoplasia 1Yassociated pNETs are usually slowly progressive and associated with low malignant potential. In the context of MEN1, pancreatic NET size correlates with prognosis and the presence of metastases,2 and lesions of more than 2 cm are associated with greater genetic instability and malignant behaviour. The European Neuroendocrine Tumour Society recommendations for management of pancreatic lesions in a patient with MEN1 state that early diagnosis and surgical excision of MEN1-related pNET improve survival, preventing or delaying the development of distant metastases.4 It is mandatory to operate on MEN1-related nonfunctioning pancreatic tumors with metastases, size of more than 2 cm, or yearly increased size of more than 0.5 cm. Management of pNETs of less than 2 cm is controversial, current recommendation being intensive surveillance to avoid repeated intervention where lesions are typically multiple and behave in an indolent fashion.