Uncommon tumors in multiple endocrine neoplasia (MEN) type 1: Do they have a relationship with the prognosis of these pa
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RAPID COMMUNICATION
Uncommon tumors in multiple endocrine neoplasia (MEN) type 1: Do they have a relationship with the prognosis of these patients? B. Febrero1 · P. Segura2 · J. J. Ruiz‑Manzanera1 · E. Teruel3 · I. Ros1 · A. Ríos1 · A. M. Hernández2 · J. M. Rodríguez1 Received: 27 May 2020 / Accepted: 31 August 2020 © Italian Society of Endocrinology (SIE) 2020
Abstract Introduction The prognosis of MEN 1 patients is not only determined by pancreatic disease; it is also related to other uncommon tumors. The objective of this study is to analyze the tumors associated with MEN 1 outside the classic triad and to investigate their relationship with mortality. Materials and methods One hundred and five MEN 1 patients were studied in a tertiary referral hospital (1980–2019). Results With a follow-up of 11 ± 4 years, seven patients died (8%), four as a consequence MEN syndrome. Thirty-three percent had adrenal gland tumors. One patient died of adrenal cancer. Eight percent presented with a neuroendocrine thoracic neoplasm, and one patient died. Another patient died due to cutaneous T-cell lymphoma. A further patient died because of a gastrinoma with liver metastasis. Conclusions To conclude, 75% of MEN-related deaths were the result of an uncommon pathology, and we, therefore, recommend that these tumors should be taken into account in the screening and follow-up of these patients. Keywords Uncommon tumors · MEN 1 · Mortality · Pancreatic neuroendocrine tumors · Prognosis
Introduction It is well-known that patients with Multiple Endocrine Neoplasia (MEN) Type 1 have earlier mortality when treatment is not provided, with a 50% probability of death at 50 years of age [1, 2]. Pancreatic neuroendocrine tumors (PaNETs) have been the ones most widely studied in terms of their effect on the prognosis in these patients. Although prognosis has improved with the introduction of the medical treatment of gastrinoma and PaNETs imaging techniques as a way of screening, they continue to be an important challenge given the amount of tumor progression and/or the development of liver metastasis [1, 3]. * B. Febrero [email protected] 1
General Surgery Service, Endocrine Surgery Unit, Instituto Murciano de Investigación Biosanitaria (IMIB), Virgen de La Arrixaca University Hospital, Crta./Madrid‑Cartagena, S/N, 30120 El Palmar, Murcia, Spain
2
Endocrinology Service, Virgen de la Arrixaca University Hospital, 30120 Murcia, Spain
3
Maxillofacial Surgery Service, Virgen de la Arrixaca University Hospital, 30120 Murcia, Spain
However, other uncommon tumors have been reported that could be aggressive and influence the clinical course of these patients, such as thymic tumors [1]. Some cancers have been gaining importance in recent years, such as adrenal cancer and breast cancer. The objectives of this study were to analyze tumors associated with MEN 1 patients aside from those of the classic triad (hyperparathyroidism, PaNETs, and pituitary disease) related to malignant disease; and also to analyze the mortality rate o
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