Uncommon tumors in multiple endocrine neoplasia (MEN) type 1: Do they have a relationship with the prognosis of these patients?

被引：5

作者：

Febrero, B. ^{[1
]}

Segura, P. ^{[2
]}

Ruiz-Manzanera, J. J. ^{[1
]}

Teruel, E. ^{[3
]}

Ros, I. ^{[1
]}

Rios, A. ^{[1
]}

Hernandez, A. M. ^{[2
]}

Rodriguez, J. M. ^{[1
]}

机构：

[1] Virgen Arrixaca Univ Hosp, Inst Murciano Invest Biosanitaria IMIB, Endocrine Surg Unit, Gen Surg Serv, Crta Madrid Cartagena S-N, Murcia 30120, Spain

[2] Virgen Arrixaca Univ Hosp, Serv Endocrinol, Murcia 30120, Spain

[3] Virgen Arrixaca Univ Hosp, Maxillofacial Surg Serv, Murcia 30120, Spain

来源：

JOURNAL OF ENDOCRINOLOGICAL INVESTIGATION | 2021年 / 44卷 / 06期

关键词：

Uncommon tumors; MEN; 1; Mortality; Pancreatic neuroendocrine tumors; Prognosis;

D O I：

10.1007/s40618-020-01414-2

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1327 / 1330

页数：4

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