Ektopowy zespół Cushinga u pacjentów z nowotworem śródpiersia lub płuc — doniesienie z ośrodka trzeciego stopnia referencyjności w Iranie

Abstract

Introduction: Ectopic Cushing`s syndrome (ECS) secondary to neuroendocrine tumours (NETs) of the lung and mediastinum are rarely encountered. In this study, we present our experience in Iran on 15 patients with ECS secondary to lung and mediastinal tumours over a period of 27 years. Material and methods: Since 1985, 15 patients with ECS secondary to lung and mediastinal tumours have been diagnosed and prospectively followed by the endocrinology team of Taleghani Hospital, Tehran, Iran. The clinical signs and symptoms, laboratory findings, radiological features, immunohistochemical characteristics, management strategies and outcome data are here presented. Results: There were six women and nine men, aged 26-70 years, all presenting with typical features of Cushing's syndrome. Based on histopathologic evaluations, four patients had small cell lung cancer (SCLC) and seven patients had pulmonary NETs, one patient had a thymic NET, and one case was diagnosed as a lung tumourlet. The mean ± SD serum cortisol, 24h urine free cortisol and plasma ACTH were 47.2 ± 20.5 µg/dL, 2,702 ± 5,439 µg/day, and 220 ± 147 pg/mL, respectively. Pulmonary lesions ranged in diameter from 1.1 to 4 cm (mean 1.9 ± 1.1 cm). One patient had a 10 cm mediastinal mass. The duration of follow up in these cases was between one month and seven years (mean 29.9 ± 27.5 months). The four patients with SCLC died within three months of diagnosis. Conclusion: Our data demonstrates the protean clinical and laboratory manifestations of ECS secondary to lung and mediastinal tumours, the problems encountered in diagnosis, and the need for a multidisciplinary approach. This study confirms other series from Western Europe and North America that, unlike the SCLC patients who show a poor outlook, ECS secondary to lung carcinoids has a more favourable prognosis. (Endokrynol Pol 2015; 66 (1): 2-9)

Klotho expression and nodal involvement as predictive factors for large cell lung carcinoma

“…Patients usually present with non-specific symptoms. Occasionally, the tumour may cause carcinoid, Cushing syndrome or acromegaly [22–24]. Most data concerning this entity derive from small retrospective studies, and there are no ongoing prospective clinical trials concerning the best treatment options [25].…”

Ectopic Cushing syndrome in small cell lung cancer: A case report and literature review

“…Ectopic Cushing’s syndrome secondary to lung cancer is rare and limited papers have reported this syndrome since it was first described by Brown in 1928 4. ECS in SCLC does not usually exhibit the classic signs of CS and CS could also appear during effective chemotherapy 8, 9.…”

“…Up to 50% of ECS cases are lung tumors, including carcinoid tumors (30–46% ECS cases) and SCLC (8–20% ECS cases) 2, 3, 4. SCLC patients with ECS have a poorer prognosis because of their advanced stage, poor response to chemotherapy, increased susceptibility to severe infections, and greater incidence of thromboembolic phenomena 5, 6.…”

Zespół Cushinga w przebiegu ektopowego wydzielania ACTH

“…The incidence of malignancies arising from cells other than endocrine is 6-8% [7,8,11]. Further, in many patients the origin of malignancy is unknown (even up to 20%; the most commonly finally diagnosed tumour is a bronchial NEN) [5,12,13]. Causes of EAS are mentioned in Table II [6,7,11,[14][15][16][17].…”

“…Do najczęstszych przyczyn EAS należy: NEN oskrzeli (25%), raka płuc (20%), NEN grasicy (11%), NEN trzustki (8%), rak rdzeniasty tarczycy (6%), guz chromochłonny nadnerczy (5%), a nowotwory wywodzące się z innych narządów niż gruczoły dokrewne i płuca stanowią 6-8% [7,8,11]. Dodatkowo dużą grupę stanowią pacjenci z nieznanym punktem wyjścia choroby nowotworowej, których odsetek w zależności od piśmiennictwa ocenia się na 20% przypadków (najczęściej ostateczne rozpoznanie to NEN oskrzeli) [5,12,13]. Wszystkie możliwe przyczyny EAS przedstawiono w tabeli II [6,7,11,[14][15][16][17].…”