| Popis: |
Purpose: Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant cancer syndrome characterized by the development of multiple neuroendocrine tumors. The condition requires lifelong surveillance and multiple medical and surgical therapies throughout the patient’s life. For all these reasons, a diagnosis of MEN1 can be a psychological shock for the patient, as well as his/her relatives. Over the past two decades, clinicians have started to consider the emotional, psychological, relational and social aspects of MEN1 patients’ lives, as these may be important considerations in the clinical and therapeutic management of these patients. Methods: This paper reviews and critically analyzes perceptions of MEN1-related quality of life (QoL) in patients diagnosed with the syndrome and in relatives, highlighting the unique features of MEN1 syndrome compared with a single tumor diagnosis. Results: Interestingly, studies in MEN1 patients have shown that a relatively high percentage of them, despite having a complex multi-tumor syndrome, were moderately optimistic (50%), self-reporting a normal QoL. This positive response correlated with the fact that these patients were cared for at dedicated referral centers, where personalized care and constant follow-up provide them with reassurance that they are receiving high quality of management of their disorder. Conclusions: The possibility of having access to a clinical referral center for this complex rare disease, together with the support of a dedicated patient association, emerged as the ideal model for the management of post-diagnosis shock, and appeared to contribute to the preservation of good health-related quality of life in MEN1 patients. |
