Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by pigmented lesions of the skin and mucosa, cardiac, cutaneous and other myxomas and multiple endocrine tumors. The disease is caused by inactivating mutations or large deletions of the PRKAR1A gene located at 17q22–24 coding for the regulatory subunit type Ⅰ alpha of protein kinase A (PKA) gene. Most recently, components of the complex have been associated with defects of other PKA subunits, such as the catalytic subunits PRKACA (adrenal hyperplasia) and PRKACB (pigmented spots, myxomas, pituitary adenomas). We reviewed CNC’s clinical features, diagnosis, treatment and molecular etiology.
Objective To summarize the application and advancement of liver transplantation for hepatic metastasis from neuroendocrine tumor. Methods Domestic and overseas publications on the study of liver transplantation for hepatic metastasis from neuroendocrine tumor in recent years were collected and reviewed. Results Liver transplantation can offer good relief of symptoms, long disease-free intervals, and potential cure in individual patients with hepatic metastatic tumor. Important selection criteria are well-differentiated tumors and a low proliferation rate (Ki67＜10%). Conclusion In carefully selected patients with metastatic neuroendocrine tumors, liver transplantation is an appropriate option.
ObjectiveTo investigate value of MSCT imaging on differentiating low grade pancreatic neuroendo-crine neoplasms (pNENs) from non-low grade pNENs. MethodThe clinical and CT data of 32 patients with pNENs,who were confirmed by pathological diagnosis from January 2014 to August 2015,were collected and analyzed retrospec-tively. ResultsThere were 15 patients with grade 1 in the low grade pNENs group,there were 11 patients with grade 2 and 6 patients with grade 3 in the non-low grade pNENs group.Compared with the low grade pNENs,the non-low grade pNENs had the larger diameter of the tumor (P=0.007),irregular tumor shape (P=0.006),obscure tumor margin (P=0.003),peripancreatic tissue or vascular invasion (P=0.036),lymphadenopathy (P=0.003),distant metastasis (P=0.019),lower absolute enhancement of tumor at the arterial (P=0.003) and the relative enhancement of tumor at the arterial (P=0.013). ConclusionThe analysis of MSCT features might help for differentiating low grade pNENs from non-low grade pNENs,so that more timely selection of appropriate treatment strategies would be made.
Objective To discuss the CT imaging differences between hepatic neuroendocrine neoplasms (NENs) and hepatocellular carcinoma (HCC). Methods The clinical and CT data of 42 patients with hepatic NENs (hepatic NENs group) and 49 patients with HCC (HCC group), who were confirmed by pathology in the West China Hospital of Sichuan University from June 2011 to June 2016, were collected and analyzed retrospectively. This study was based on whether the lesions were larger than 3 cm or not, then CT findings of hepatic NENs patients and HCC patients in different stratification were compared. Results When the lesions were less than 3 cm, the location, contour, and enhancement patterns in the portal vein phase of the tumor had significant differences between the hepatic NENs group and the HCC group (P<0.05), multiple liver lesions, the round shape, and prolonged enhancement in the portal vein phase were more often seen in the hepatic NENs group, but there was no significant on diameter of tumor, boundary of lesion, pseudocapsules, scan density, hypervascularity, enhancement degree in arterial phase, enhancement patterns in arterial phase, daughter foci at liver, retraction, neoplastic artery, arteriovenous invaded, portal vein tumor thrombus, diameter of lymph node, and enhancement degree of lymph node between the 2 groups (P>0.05). And when the lesions were greater than or equal to 3 cm, the location, contour, enhancement patterns in the portal vein phase of the tumor, pseudocapsule, neoplastic artery, and arteriovenous invaded had significant differences between the hepatic NENs group and the HCC group (P<0.05), these CT images were often seen in the hepatic NENs group, such as multiple liver lesions, the lobulated shape, the portal venous phase continuous strengthening, no pseudocapsule, no neoplastic artery, and no arteriovenous invaded, but there was no significant difference on the diameter of tumor, boundary of lesion, scan density, hypervascularity, enhancement degree in arterial phase, enhancement patterns in arterial phase, daughter foci at liver, retraction, portal vein tumor thrombus, diameter of lymph node, and enhancement degree of lymph node between the2 groups (P>0.05). Conclusions No matter whether the lesions’ size are larger than 3 cm or not, the location, contour, and enhancement patterns in the portal vein phase could help for differentiating hepatic NENs from HCC. When the lessions are larger than 3 cm, pseudocapsule, neoplastic artery, and arteriovenous invaded may be useful to differentiate.
Objective To approach the recent advances in diagnosis and surgical treatment of pancreatic endocrine tumors （PETs）. Methods Articles relevant to diagnosis and treatment of PETs were collected and reviewed. Results PETs are characterized by their ability to over-produce peptides and hormones， which cause specific clinical syndromes. Because of rare incidence and complex clinical syndromes， there are still impediments to early diagnosis of these tumors. Monitoring of serum hormones and imaging method allow early tumor detection. PETs have been investigated for the past several decades. With the great knowledge of these tumors in molecular genetic level， clinical managements have been greatly changed. Conclusions Avoiding misdiagnosis is important for treatment of PETs. Surgical approach is still considered as the preferred option for curtailing the malignant progression of PETs and controlling the associated biochemical syndromes.
ObjectiveTo explore value of CT imaging features in differentiating pathological grades of pancreatic neuroendocrine neoplasms (pNENs). MethodsThe patients with pNENs admitted to the Sichuan Provincial People’s Hospital from October 2017 to December 2023 were retrospectively collected. The enrolled patients were assigned into a low-grade (G1+G2) pNENs and high-grade (G3+neuroendocrine carcinoma) pNENs. Then, the differences in gender, age, presence of neuroendocrine symptoms, tumor location, tumor diameter, clarity of the tumor boundary, cystic change, capsule, dilation of the biliary and pancreatic ducts, pancreatic parenchymal atrophy, vascular invasion, liver metastasis, lymph node metastasis, tumor enhancement pattern, Ct values in each phase, and the ratio of Ct value of the tumor to that of the normal pancreas (T/N value) between the low-grade pNENs and high-grade pNENs were compared. Subsequently, the multivariate logistic regression analysis was used to screen the CT imaging features with statistical significance and the receiver operating characteristic (ROC) curve was use to differentiate high-grade from low-grade pNENs. ResultsA total of 47 pNENs patients were enrolled, including 36 low-grade and 11 high-grade cases. Compared with the low-grade pNENs, the patients with high-grade pNENs had higher proportions of pancreaticobiliary duct dilation (χ2=9.124, P=0.003) and vascular invasion (χ2=10.967, P=0.001), more mild enhancement (χ2=9.192, P=0.010), larger tumor diameter (Z=–2.378, P=0.017), and lower Ct values and T/N ratios in the arterial and venous phases (P=0.001, P=0.032, P=0.006, P=0.018). The multivariate logistic regression analysis showed that the pancreaticobiliary duct dilation, vascular invasion, and decreased Ct value in the arterial phase were the predictive factors for the high-grade pNENs. The areas under the ROC curves of these three indicators for distinguishing low-grade from high-grade pNENs were 0.760, 0.749, and 0.843, respectively, the Ct value in the arterial phase had the strongest discriminatory ability, and its cutoff value was 78 HU. ConclusionsThe results of this study suggest that pancreatic ductal dilation, vascular invasion, and Ct value in arterial phase play important roles in differentiating high-grade pNENs from low-grade pNENs. Ct value in arterial phase has the greatest diagnostic efficiency.
ObjectiveTo summary the treatment of pancreatic neuroendocrine neoplasms (pNENs). MethodsArticles relevant to pNENs at home and abroad were collected and reviewed. ResultsBecause of rare incidence and non-specific clinical syndromes of pNENs, clinician had no enough cognition about it. For pNENs, surgery was still the preferred option, combining other treatments included chemotherapy, somatostatin analogue, α-interferon, molecular targeted therapy, and peptide receptor radionuclide therapy (PRRT). ConclusionSurgery is still considered as the preferred option for controlling the associated biochemical syndromes and curtailing the malignant progression of pNENs.
ObjectiveTo investigate the hotspots from researches on imaging of pancreatic neuroendocrine tumor in recent five years. MethodsThe bibliographies from research literatures on imaging of pancreatic neuroendocrine tumor from 2010 to 2015 in PubMed database were downloaded. The Bicomb 2.0 bibliographies analysis software was used to count high-frequency of Mesh major topics (MJMEs). SPSS 22.0 statistical software was applied for clustering analysis with MJMEs, then to get the topic hotspots. ResultsA total of 357 literatures were screened out during the years of 2010-2015. The MJMEs which frequency > 13 were 28. Taken the 28 MJMEs into clustering analysis, then three research hotspots were clustered. ConclusionResearches on imaging of the pancreatic neuroendocrine tumor in recent five years are mainly in terms of imaging techniques, a comparative study of pathology and endoscopic ultrasonography-fine needle aspiration, imaging and disease treatment.