Ectopic Adrenocorticotropic Hormone Syndrome Caused by Thoracic Neuroendocrine Tumors: Surgical Treatment and Prognosis Factors

    Objective       To discuss the surgical management and prognosis factors of ectopic adrenocorticotropic hormone syndrome (EAS) caused by thoracic neuroendocrine tumors(NETs).

    Methods       The clinical manifestations, radiological findings, outcome of surgical treatment and follow-up of consecutive 33 cases(20 males and 13 females) of EAS caused by thoracic NETs from December 1983 to December 2013 in Peking Union Medical College Hospital were retrospectively analyzed.

    Results       The median age was 33 years old (13~65 years)and the median duration of disease was 12 months(1~156 months). Cushing's syndrome was demonstrated in all cases by clinical and biochemical evidences. All the thoracic NETs were located by computed tomography (CT) eventually. The serum cortisol and adrenocorticotropic hormone levels decreased significantly after surgery(both P < 0.05).One patient(3.0%) died after surgery and two patients(6.1%) had postoperative complications.The overall 5-year survival rate was 65.8%.Pulmonary diseases had better survival rate versus thymic diseases(84.2% vs. 36.3%, P < 0.05).The 5-year survival rate was 76.2% in typical carcinoid and 45.7% in atypical carcinoid(P=0.056).The 5-year survival rate was 80.0% in stage Ⅰ and Ⅱ, 50.5% in stage Ⅲ and Ⅳ respectively(P=0.072).

    Conclusions       Due to the varity and atypical clinical features, early diagnosis and therapy in EAS is very challenging for clinicians. CT scan plays a key role in localization of the thoracic NETs. Pulmonary diseases have better prognosis than thymic diseases.Pathological type and stage of tumors might be prognostic factors for this rare syndrome.