Pancreatic neuroendocrine tumors (PanNETs) are rare in von Hippel-Lindau disease (VHL) but cause serious morbidity and mortality. Management guidelines for VHL-PanNETs continue to be based on limited evidence, and survival data to guide surgical management are lacking. We established the European-American-Asian-VHL-PanNET-Registry to assess data for risks for metastases, survival and long-term outcomes to provide best management recommendations. Of 2330 VHL patients, 273 had a total of 484 PanNETs. Median age at diagnosis of PanNET was 35 years (range 10-75). Fifty-five (20%) patients had metastatic PanNETs. Metastatic PanNETs were significantly larger (median size 5 vs 2 cm; < 0.001) and tumor volume doubling time (TVDT) was faster (22 vs 126 months; = 0.001). All metastatic tumors were ≥2.8 cm. Codons 161 and 167 were hotspots for germline mutations with enhanced risk for metastatic PanNETs. Multivariate prediction modeling disclosed maximum tumor diameter and TVDT as significant predictors for metastatic disease (positive and negative predictive values of 51% and 100% for diameter cut-off ≥2.8 cm, 44% and 91% for TVDT cut-off of ≤24 months). In 117 of 273 patients, PanNETs >1.5 cm in diameter were operated. Ten-year survival was significantly longer in operated vs non-operated patients, in particular for PanNETs <2.8 cm vs ≥2.8 cm (94% vs 85% by 10 years; = 0.020; 80% vs 50% at 10 years; = 0.030). This study demonstrates that patients with PanNET approaching the cut-off diameter of 2.8 cm should be operated. Mutations in exon 3, especially of codons 161/167 are at enhanced risk for metastatic PanNETs. Survival is significantly longer in operated non-metastatic VHL-PanNETs.

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Key Points Question Is cortical-sparing adrenalectomy associated with increased pheochromocytoma-specific morbidity and mortality for patients with bilateral pheochromocytomas compared with total adrenalectomy? Findings In this cohort study of 625 patients with bilateral pheochromocytomas, most had hereditary syndromes, but 36% initially presented with unilateral pheochromocytoma. Bilateral total adrenalectomy resulted in a high rate of adverse effects from glucocorticoid replacement therapy, whereas cortical-sparing surgery was not associated with a worse outcome. Meaning These findings suggest that cortical-sparing surgery may be the preferred approach for patients at risk for, or diagnosed with, bilateral pheochromocytomas, especially those harboring a germline mutation in one of the known predisposition genes.

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Chronic polypous rhinosinusitis does not subject to complete medicinal control; it is difficult to predict the prospects of the pathology development in each individual patient. Therefore, the search of new regimens of treatment of these patients is still in progress. An international consensus published in 2016, considers, inter alia, various groups of drugs for conservative treatment of polypous rhinosinusitis from the viewpoint of evidence-based medicine. We conducted a retrospective analysis of outpatient records and personal data of 1059 patients with polypous rhinosinusitis who underwent outpatient and inpatient treatment at the period from February 2016 to March 2018 in Moscow, Moscow Region and 26 regions of Russia. According to the results, in outpatient treatment, the basic therapy with intranasal glucocorticosteroids was performed in 92.4%, irrigation therapy – in 82.2% of cases, antihistamines were recommended in 79.4%. In case of hospitalization for surgical treatment, 16.6% of patients in Russian regions and 24% in Moscow Region had received no preliminary therapy for 6 months before hospitalization. In general, analyzing the management and treatment regimens of the patients with polypous rhinosinusitis, we found almost no deviations from the present-day domestic and foreign clinical guidelines. However, we observed prescription of some intranasal glucocorticosteroids, the leaflets of which contain no indications for polypous rhinosinusitis treatment.