Cardiothoracic Surgery: Global Case Report Poster - Cardiothoracic Surgery |

Ectopic Cushing Syndrome Due to ACTH Secreting Atypic Thymic Carcinoid Tumor FREE TO VIEW

Cevdet Duran, MD; Meryem Eren Karanis, MD; Suleyman Bakdik, MD; Uysaler Aslan, MD; Mustafa Calik, MD; Saniye Calik, MD
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Konya Education and Research Hospital, Konya, Turkey

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):50A. doi:10.1016/j.chest.2016.08.057
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SESSION TITLE: Global Case Report Poster - Cardiothoracic Surgery

SESSION TYPE: Global Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Cushing's syndrome (CS) due to ectopic adrenocorticotropic hormone (ACTH) and corticotrophin releasing hormon (CRH) secretion from non-pituitary tumors comprise of 10-20% of all endogen CS. Thymic carcinoids account for less than 5% of all carcinoid tumors and 1% of all cases with endogen CS (1). In this text, a case with CS due to ectopic ACTH secreting atypical thymic carcinoid tumor is presented.

CASE PRESENTATION: A 50-year-old male patient was admitted to our emergency department with the complaint of fatigue and weight loss (nearly 8 kg for the last 2 years). In his history, he has been hypothyroid and hypertensive for three years and treated with l-thyroxine, candesartan 16 mg with hydrochloride 12.5 mg in combination, and amlodipin 5 mg per day. On the physical examination, he was 180 cm in height and 80 kg in weight, body mass index was 24.6 kg/m2, blood pressure was 130/80 mmHg. He had central obesity, plethora, acnea, moon face and dorsal fat pad. Chest X-ray showed upper mediastinal widening. Considering CS, 24-h urine free cortisol, plasma ACTH and serum cortisol were measured and found as 953 µg/day (normal 36-137 µg/day), 257.9 pg/ml and 32.8 µg/dl, respectively. Low and high dose overnight dexametasone test (DST) were unable to suppress serum cortisol levels. Sella MR was normal. Because he had upper mediastenal widening, thorax computerized tomography was performed and showed a macrolobulary mass in size of 67x49 mm with irregular border and calcification,and also invasion to brachio-cephalic vein and paratracheal, subcarinal and hiler lyphadenopathy in a size up to 45 mm were detected (Figure 1). Biopsy was performed and pathological examination revealed atypical thymic carcinoid.

DISCUSSION: ACTH secretion from thymic carcinoid is a very rare condition and has been described only in limited numbers (1). ACTH-dependent CS may result from ectopic ACTH production; and, it is usually difficult to distinguish from hypophyseal-ACTH secretion. Underlying slow growing tumor, such as carcinoids, can mimic Cushing's disease. Rarely can these tumors be cyclic secretuar and to carry out dynamic endocrine tests during this period is often inconclusive. Additionally, most of these tumors are occult and it is difficult to detect the source of ACTH production. In this case, higher plasma ACTH levels and no suppression were detected during low and high dose DST. IPSS was compatible with ectopic ACTH secretion and thorax CT revealed a thymic mass, as we considered ectopic ACTH syndrome due to thymic cancer, biopsy was performed and atypical carcinoid was detected. To confirm the ACTH source from this tissue, ACTH staining was performed and positive staining was detected. Treatment options of thymic carcinoids are surgical excision, chemotherapy, SRA (somatostatin receptors analogues) and radiotherapy. Due to aggressivity of the atypical carcinoid tumor, surgical cure can be achieved only in limited patients. Likewise, although different chemotherapy regimens are recommended, success rate is lower than 30%. As carcinoid tumors have expressed SRs, analogues can be used for suppression of tumor growth and ectopic hormone secretion (2,3). In our case, because of vascular invasion and multipl lympadenopathy, surgery was not preferred for initial therapy instead lanreotide, cisplatin and etoposide treatments were started and conventional radiotherapy was planned.

CONCLUSIONS: Ectopic CS can be derived from athypical thymic carcinoid. In this case, we used ACTH staining in thymic tissue to confirm ACTH secretion from these tissues and positive staining was detected. Routinely, ACTH staining was not performed for extrahypophysial tissue tumours. In difficult cases, ACTH staining can be helpful to confirm the presence of ACTH secretion in tumour tissues.

Reference #1: Ferone D, Albertelli M. Ectopic Cushing and other paraneoplastic syndromes in thoracic neuroendocrine tumors. Thoracic surgery clinics. 2014;24(3):277-83.

Reference #2: Sato H, Kajiya H, Kanai G, Hirukawa T, Tanaka H, Kakuta T, et al. Atypical thymic carcinoid associated with Cushing's syndrome. The Tokai journal of experimental and clinical medicine. 2010;35(2):78-84.

Reference #3: Hofland LJ. Responsiveness to somatostatin analog treatment and potentials of novel somatostatin analog. Journal of endocrinological investigation. 2003;26(8 Suppl):8-13.

DISCLOSURE: The following authors have nothing to disclose: Cevdet Duran, Meryem Eren Karanis, Suleyman Bakdik, Uysaler Aslan, Mustafa Calik, Saniye Calik

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