Carcinoid crisis after injection of 6-F-18-fluorodihydroxyphenylalanine in a patient with metastatic carcinoid

carcinoid crisis is a severe complication of the carcinoid syndrome that can arise in patients with advanced metastatic neuroendocrine tumors. It can be initiated by stress, catecholamines, and tumor manipulation. In this article, we report a case in which an injection with the catecholamine tracer 6-F-18-fluorodihydroxyphenylalanine, used for PET, induced a carcinoid crisis. Octreotide can be used for treatment and should be available. Tracer injection should be slow

Health-Related Quality of Life in Patients with Multiple Endocrine Neoplasia Type 1

Introduction: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary endocrine tumor syndrome characterized by the triad of primary hyperparathyroidism, duodenopancreatic neuroendocrine tumors (pNETs), and pituitary tumors. Patients are confronted with substantial morbidity and are consequently at risk for an impaired quality of life (QOL). Meticulous assessment of QOL and associated factors in a representative population is needed to understand the full spectrum of the burden of the disease. Patients and Methods: A cross-sectional study was performed using the national Dutch MEN1 cohort. Patients with a confirmed MEN1 mutation received the SF-36 Health Related Quality of Life questionnaire and questions regarding sociodemographic and medical history. Results: A total of 227 of 285 (80%) eligible MEN1 patients returned the questionnaires. Health-related QOL scores (HRQOL) in MEN1 patients were significantly lower for the majority of subscales of the SF-36 in comparison with the general Dutch population. The most consistent predictor for HRQOL was employment status, followed by the presence of a pituitary tumor. 16% of patients harboring a pNET and 29% of patients with a pituitary tumor according to the medical records, reported that they were unaware of such a tumor. These subgroups of patients had several significant better QOL scores than patients who were aware of their pNET or pituitary tumors. Conclusion: Patients with MEN1 have an impaired QOL in comparison with the general Dutch population warranting special attention within routine care. For daily practice, physicians should be aware of their patients' impaired QOL and of the impact of unemployment on QOL

Natural Course and Survival of Neuroendocrine Tumors of Thymus and Lung in MEN1 Patients

Context: The natural course and survival of neuroendocrine tumors (NETs) of thymus (Th) and lung in multiple endocrine neoplasia type 1 (MEN1) patients are still unknown. Objective: Our objective was to assess prevalence, tumor growth, and survival of Th and lung NETs in an unselected MEN1 population with long-term follow-up. Design: This was an observational study. Patients and Methods: A longitudinal study was performed using the Dutch national MEN1 database, including >90% of the Dutch MEN1 population >16 years of age. Patients under care of the Dutch University Medical Centers (1990-2011) (n = 323) were included. Main Outcome Measures: The prevalence and survival of Th and lung NETs were assessed. Linear mixed-models analysis was applied to assess tumor growth with age as a possible confounder and gender, genotype and baseline tumor size as possible effect modifiers. Results: Th NETs occurred in 3.4% of patients, almost exclusively in males with a 10-year survival of 25% (95% confidence interval = 8%-80%). Athoracic computed tomographys can was available in 188 patients (58.2%). A lung NET was identified in 42 patients (13.0%) with a 10-year survival of 71.1% (95% confidence interval = 51%-100%). Tumor volume of lung NETs increased 17% per year (P = 1 cm). Conclusion: In MEN1 patients, Th NETs almost exclusively occurred in males and had a very low prevalence and a high mortality. Lung NETs occurred more often than previously thought, had an indolent course, and occurred equally in both sexes. Tumor growth in males was double compared with female patients

MEN1-dependent breast cancer: indication for early screening? Results from the Dutch MEN1 study group.

Objective: Multiple Endocrine Neoplasia type 1 (MEN1) is associated with an early onset elevated breast cancer risk. This finding potentially has implications for breast cancer screening for females with MEN1. Considering the impact for females with MEN1, regarding distress and anxiety, it is necessary to assess if other risk factors are involved to identify those at greatest risk. Design: A cross-sectional case control study was performed using the Dutch MEN1 cohort, including >90% of the Dutch MEN1 population of 18 years and older. All females with a confirmed MEN1 mutation received a questionnaire regarding breast cancer related endocrine risk factors, cancer family history and other known risk factors for breast cancer. Results: A total of 138 of 165 (84%) eligible females with MEN1 completed the questionnaire. Eleven of the 138 females had breast cancer. Another 34 relatives with breast cancer were identified in the families of the included females, of whom 11 were obligate MEN1 carriers, 14 had no MEN1 mutation and nine had an unknown MEN1 status. The median age at breast cancer diagnosis of females with MEN1 (n=22) was 45 (range 30-80) years in comparison with 57.5 (range 40-85) years in female relatives without MEN1 (n= 14) (p=0.03) and 61.2 years in the Dutch reference population. Known endocrine risk factors as age at menarche, oral contraception use, pregnancy, age at first birth, parity and breast-feeding were not different for females with and without breast cancer. In addition, smoking and alcohol consumption did not differ between respondents with and without breast cancer. Conclusion: The increased breast cancer risk in MEN1 carriers was not related to other known breast cancer risk factors or familial cancer history and therefore breast cancer surveillance from the age of 40 years for all females with the MEN1 is justifiable