TY  - JOUR
TI  - Sporadic Adrenocorticotropic Hormone-Secreting  Lung Carcinoids: Outcomes of Long-Term Clinical  Follow-Up at a Single Center
AB  - Objective: Lung carcinoids represent a small portion of all lung tumors and about 1%-5% are associ ated with ectopic adrenocorticotropic hormone secretion, representing 1%-10% of Cushing’s syn drome. They occur both sporadically and rarely in association with multiple endocrine neoplasia type  1. MEN1 variations were detected in approximately 16% of sporadic cases with seldom reports about  disease prognosis. This study aimed to identify MEN1 variants in our cohort of sporadic lung carci noids to associate with disease outcome following surgery.  Methods: Pathologically confirmed 5 lung carcinoid cases were retrospectively analyzed in terms of  age, gender, imaging studies, clinicopathologic features, and long-term disease outcome. Genetic  analysis was performed to detect copy number variations and point mutations in MEN1. Results: Totally 1 female and 4 males with cushingoid features underwent thoracoscopic lobectomy  for ectopic Cushing’s syndrome. Histopathological examinations revealed 2 atypical (males) and 3 typ ical (1 female and 2 males) carcinoids. One underwent Wedge resection, the others underwent lobec tomy; none of them had any post-operative complications. Median follow-up period was 11 years  (range, 5-19); all patients were alive with no recurrence or metastases, up to date. Genetic analysis  revealed a novel MEN1 variant [c.1623G>T, p.(Gln541His)] in only 1 atypical carcinoid. Despite this  variant, this case did not develop other components of MEN1 syndrome during long-term follow-up.  Conclusion: We detected the frequency of MEN1 variation as 20%. Further studies are required to  clarify the role of this variant.
AU  - yarman, sema
AU  - Soyluk Selcukbiricik, Ozlem
AU  - Tuncer, Feyza
AU  - SERBEST, ESİN
DO  - 10.5152/tjem.2022.22022
PY  - 2022
JO  - Turkish Journal of Endocrinology and Metabolism
VL  - 26
IS  - 3
SN  - 1301-2193
SP  - 109
EP  - 114
DB  - TRDizin
UR  - http://search/yayin/detay/1124133
ER  -