Calidad de vida y preocupación por desarrollar cáncer en pacientes con neoplasia endocrina múltiple tipo 1

Abstract

Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) syndrome is characterized by having varying degrees of tumor aggression, which can affect the quality of life and daily activity of patients with this disease. The objective of this work is to determine the level of QoL and the degree of concern for developing cancer presented by patients with MEN1, analyze the relationship between both components, and compare the results with a control group (CG) of a healthy population, and also analyzing the relationship between both components. Likewise, the influence of different socio-personal (SP) and clinical variables on QoL and on the concern about developing cancer is analyzed. Patients and methods A cross-sectional study was carried out in MEN1 patients under follow-up in a tertiary care hospital between June 2018 and June 2020. SP and clinical variables were analyzed. QoL was assessed using the generic SF-36 questionnaire, a specific questionnaire for parathyroid pathology (PHPQoL), and two specific questionnaires for oncological pathology (EORTC QLQ-C30) and neuroendocrine tumors (QLQ-GINET-21). The degree of concern about developing cancer was estimated using the Cancer Concern Scale (CPS). The scores of the SF-36 and EPC questionnaires were compared with those of a CG of a healthy population matched for age and sex. Statistical analysis: SPSS v.28. Descriptive analysis, t-Student/ANOVA or Mann Whitney/KruskalWallis tests. A multivariate analysis of multiple linear regression was carried out with the objective of evaluating the repercussion of the SP and clinical variables on the Physical Component Summary (CSF) and Mental Summary Component (CSM) of the SF-36 questionnaire. p<0,05 was considered statically significant. Results MEN1 patients had lower levels of general health (47.03 vs 69.8), vitality (57.15 vs 64.31), mental health (62.36 vs 67.29), and physical summary component (PSC) (46.42 vs 51.71) compared to the CG (p<0.05). Differences in QoL were found according to age, educational level, work activity and offspring. In addition, patients with a higher educational level, younger age, active employment status, and no offspring had significantly higher SF-36 CSF scores (p<0.05). Differences in HRQoL associated with pancreatic pathology, adrenal pathology, adrenal surgery and carcinoid tumors were also observed (p<0.05). 34 patients with current PHPT (primary hyperparathyroidism) had a mean PHPQoL score of 68,08. Patients with pancreatic pathology presented a worsening of most of the spheres of the SF-36 questionnaire. They also presented a worsening of the PSC and the MSC (p<0.05). In addition, pancreatic surgery influenced 3/9 spheres and the MSC (p<0.05). Pancreatic surgery and advanced tumor stage were associated with a worse QoL in the EORTC QLQ-C30 and GINET21 questionnaires (p<0.05). In the multivariate analysis, employment status and pancreatic surgery were the variables most related with the QoL of patients with MEN, both in the PSC and MSC analyzes (p<0.05); in addition to the carcinoid tumor, which influenced the PSC (p<0.05) MEN1 patients presented a greater concern for developing cancer than the CG (p<0.001). In addition, MEN1 patients with lower concern for developing cancer (EPC <14) presented better mean scores in the SF-36 domains compared to MEN1 patients with EPC>14 (p<0.001). Patients with pancreatic pathology presented greater concern about developing cancer (p=0.007), mainly patients with pancreatic surgery (p=0.037). Conclusions MEN1 patients have a worse QoL than the general population, mainly in the dimensions related to general health, vitality and mental health; as well as increased concern about developing cancer. Various SP variables could influence QoL, fundamentally an inactive employment situation. Of the clinical variables, pancreatic or carcinoid disease can worsen QoL. Different variables related to pancreatic pathology, and, mainly, surgery in these patients, are aspects that decisively influence both QoL and concern about developing cancer. All these clinical and sociodemographic aspects should be taken into account and included in the clinical assessment of these patients.