Turkish Journal of
Endocrinology and Metabolism
Original Article

Sporadic Adrenocorticotropic Hormone-Secreting Lung Carcinoids: Outcomes of Long-Term Clinical Follow-Up at a Single Center


Division of Endocrinology and Metabolism, Department of Internal Medicine, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey


Department of Genetics, Aziz Sancar Institute of Experimental Medicine, İstanbul University, İstanbul, Turkey


Department of Hematology, Ankara University Faculty of Medicine, Ankara, Turkey

Turk J Endocrinol Metab 2022; 26: 109-114
DOI: 10.5152/tjem.2022.22022
Read: 517 Downloads: 193 Published: 06 September 2022

Objective: Lung carcinoids represent a small portion of all lung tumors and about 1%-5% are associated with ectopic adrenocorticotropic hormone secretion, representing 1%-10% of Cushing’s syndrome. 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 carcinoids 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 typical (1 female and 2 males) carcinoids. One underwent Wedge resection, the others underwent lobectomy; 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.

Cite this article as: Yarman S, Serbest E, Soyluk Selçukbiricik Ö, Tuncer FN. Sporadic adrenocorticotropic hormone-secreting lung carcinoids: Outcomes of long-term clinical follow-up at a single center. Turk J Endocrinol Metab. 2022;26(3):109-114.

EISSN 1308-9846