Multiple endocrine neoplasia type 1 (MEN1) and quality of life

Abstract

Patients with multiple endocrine neoplasia type 1 (MEN1) represent among cancer patients a particular group of hereditary tumour syndrome with a long duration of disease and multiple follow-ups. MEN1 is characterized by predisposition mainly to tumours of the parathyroid glands and (enteropancreatic) neuroendocrine tumours but also involvement of anterior pituitary, adrenals and other endocrine glands. Only one report regarding quality of life (QoL) in these patients is available so far (Berglund et al., Fam Cancer 2003). Patients and Methods: 23 patients (13 males, 10 females, age range 23–67yrs) with MEN1, followed up by the interdisciplinary team (internists and surgeons) in our university hospital. Patients answered three questionnaires: the Nottingham Health Profile (NHP – a generic healthy survey), the questionnaire for Quality of life – Assessment of Growth Hormone Deficiency in Adults (QoL-AGHDA – designed to evaluate adult patients with growth hormone deficiency) and the European Organisation for Research and Treatment of Cancer (EORTC) Quality of Life Questionnaire Core 30 (QLQ-C30) Version 3.0– used to asses the health related quality of life in cancer patients (22 patients). The results for the NHP and the EORTC QLQ-C30 were matched to reference data published for theses questionnaires for the German population. Differences ≥ +2 SD (standard deviation) were defined as pathological, ≤+1 as normal and between +1SD and +2 SD as marginal/intermediate. Results: Most pathological scorings in the NHP were for energy lost (26%) and emotional reactions (22%). Only 13% reported pains. 26% of patients showed low QoL as evaluated with the QoL-AGHDA (47% of the studied patients hat pituitary involvement but none of them pituitary hormones deficiencies). Scorings on functional scales of the EORTC QLQ-C30 were comparable and higher for the cognitive and role scales (better QoL), the lowest ratings were achieved for the social, emotional and physical scales. On the symptoms scale more problems were reported with fatigue (41%), less with pain and with nausea/vomiting. The global health/QoL scale reflected only in 14% patients pathological results, but indicated marginal results in 32%. Conclusions: Patients with MEN1 perceives their global/health-related QoL as reduced but still relatively good; items as energy lost/fatigue rises as the most important aspects, probably influencing physical, emotional and social functioning.