A novel SLC8A1/LINC01913 intergenic region-ALK fusion identified by NGS and validated by IHC and FISH in a stage IIB lung adenocarcinoma patient who remains relapse-free during the treatment of crizotinib: a case report.

In resected non-small cell lung cancer (NSCLC), ALK rearrangements are associated with worse recurrence-free survival (RFS) than other driver genes. In addition, the micropapillary pattern of NSCLC is associated with a poor prognosis. In recent years, crizotinib tyrosine kinase inhibitors (TKIs) have been widely used to treat patients with advanced NSCLC with ALK fusion. Patient survival outcomes have become highly promising, reflecting the necessity of exploring the application of ALK-TKIs in resected, early stage NSCLC with ALK rearrangements. A 60-year-old Chinese man was diagnosed with stage IIB lung adenocarcinoma harboring a novel SLC8A1/LINC01913 intergenic region-ALK fusion identified by NGS and validated by immunohistochemical staining (IHC) and fluorescence in situ hybridization (FISH). Crizotinib (250 mg orally once daily) was administered to the patient following surgery. The patient remained relapse-free after four months and seven months. This report provided a valuable treatment plan for early lung adenocarcinoma patients with high risks to prevent a postoperative recurrence.

Early detection of cognitive impairment in patients with insulinoma.

PURPOSE: Long-standing hypoglycemia can cause cognitive impairment, and whether recurrent severe hypoglycemia impacts cognitive function in patients with insulinoma has not been studied. This study focused on exploring the cognitive function in patients with insulinoma. METHODS: A prospective study was conducted to assess cognitive function in patients with insulinoma by administering the Montreal Cognitive Assessment (MoCA) questionnaire between January 2016 and July 2017, and patients with cognitive impairment were followed up to undergo the MoCA test 1 year after surgery. The MoCA scores after surgery were compared with the scores before surgery, and the associations between cognitive impairment and relevant factors were further evaluated by multiple linear regression analysis. RESULTS: Eighteen out of thirty-four patients (53%) with insulinoma were screened positive for cognitive impairment as defined by a MoCA score <26. Performance in certain cognitive domains, including visuospatial and executive functions, delayed memory, attention, language, and abstraction, was significantly worse in patients with cognitive impairment. Multivariate analysis indicated that MoCA scores correlated significantly with tumor grade and years of education. Eight patients with cognitive impairment were lost to follow-up. The remaining ten patients with cognitive impairment showed improvements 1 year postoperatively, and seven patients recovered to normal cognitive function. CONCLUSIONS: Cognitive impairment was found in patients with insulinoma and was reversible in some patients 1 year after surgery. More studies are needed to explore the underlying mechanisms of the existence and reversibility of cognitive impairment in patients with insulinoma.

The prognostic impact of primary tumor resection in pancreatic neuroendocrine tumors with synchronous multifocal liver metastases.

BACKGROUND: Whether primary tumor resection benefits patients with synchronous multifocal liver metastases from pancreatic neuroendocrine tumors remains controversial. We investigated whether primary tumor resection significantly affects survival in this study. METHODS: A retrospective study of patients with synchronous multifocal liver metastases from pancreatic neuroendocrine tumors between 1998 and 2016 was performed. Patient demographics, operation details, adjuvant treatment, and pathological and survival information were collected, and relevant clinical-pathological parameters were assessed in univariate and multivariate survival analyses. RESULTS: Sixty-three patients were included in this study, including 35 who underwent primary tumor resection. The median survival time and 5-year survival rate of this cohort were 50 months and 44.5%, respectively. Median survival time in the resected group was significantly longer at 72 months than that of 32 months in the nonresected group (p = 0.010). Multivariate analysis showed that primary tumor surgery was a significant independent prognostic factor (HR 0.312, 95% CI: 0.128-0.762, p = 0.011). CONCLUSIONS: Primary tumor resection significantly benefits patients with synchronous multifocal liver metastases from pancreatic neuroendocrine tumors.

Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis: A retrospective cohort study of 39 IAC patients.

Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC. The majority of patients were men (82%). Clinical features on presentation included obstructive jaundice in 26 patients (67%) and abdominal pain in 20 (51%). Positive IgG4 immunostaining was seen in 27 patients. The median serum IgG4 level before treatment was 769.4 mg/dL (range, 309.1-1229.7 mg/dL). After the steroid therapy, the median serum IgG4 level in 23 patients was 247.0 mg/dL (range, 139.0-355.0 mg/dL). Cholangiograms were available in 36 (92%) patients. Stenosis of the lower part of the common bile duct was found in 26 of 39 patients. Stenosis was diffusely distributed in the intra- and extrahepatic bile ducts in 14 of 39 patients. Additionally, strictures of the bile duct were detected in the hilar hepatic lesions in 27 of 39 patients. AIP was the most frequent comorbidity (35/39 in this study) of IAC. Other affected organs included eyes (n = 6), salivary glands (sialadenitis, n = 10), lymph nodes (mediastinal and axillary, n = 3), kidneys (n = 2), and the retroperitoneum (retroperitoneal fibrosis, n = 2).Regarding treatment, 29 patients were treated with steroids, of whom one underwent pancreatoduodenectomy, and one underwent choledochojejunostomy. Eight patients were treated with biliary stents. The remaining 19 patients took prednisolone alone. Eight patients achieved spontaneous resolution. Four patients with suspected pancreatic cancer or cholangiocarcinoma underwent surgery, including 2 patients who also received postoperative steroids. All patients were regularly followed up for 9 to 36 months. Only 2 patients in the steroids treatment group relapsed to manifest obstructive jaundice and high serum IgG4 levels. These 2 patients were treated with steroids and biliary stents, resulting in complete remission.We also review the diagnostic and therapeutic management and discuss recent pathophysiological findings, which might aid in understanding the molecular mechanisms contributing to IAC and other manifestations of IgG4-related diseases (IgG4-RD). Biomarkers that are more accurate are needed to correctly diagnose IAC and prevent misdiagnoses and unnecessary therapeutic interventions.