Pulmonary embolism as the presenting symptom and a confounder in ACTH-secreting bronchial carcinoid. (26th July 2019)

• Record Type: Journal Article
• Authors: Yang, Wei; Pham, David; Vierra, Aren T; Azam, Sarah; Gui, Dorina; Yoon, John C
• Is Part Of: Endocrinology, diabetes & metabolism case reports. (2019)
• Publication Date: 2019-07-26
• Subjects: Adult -- Female -- Black - African -- United States; Adrenal -- Pituitary -- Adrenal -- ACTH -- Cortisol -- Cushing's syndrome -- Ectopic Cushing's syndrome -- Metastatic tumour -- Microadenoma -- Neuroendocrine tumour; Thrombophilia -- Hypertension -- Hypokalaemia -- Dyspnoea -- Oedema -- Amenorrhoea -- Facial plethora -- Obesity -- Striae -- Tachycardia -- Hypercortisolaemia -- Nausea -- Fatigue -- Core needle biopsy -- Dexamethasone suppression (high dose) -- Dexamethasone suppression (low dose) -- Gallium scan -- Histopathology -- CT scan -- Angiography -- Ultrasound-guided biopsy -- Immunohistochemistry -- Haematoxylin and eosin staining -- Chromogranin A -- Synaptophysin -- Potassium -- Glucose (blood) -- Cortisol (9am) -- Cortisol, free (24-hour urine) -- ACTH -- FSH -- LH -- Calcitonin -- CTPA scan -- CT scan -- Somatostatin analogues -- Lisinopril -- Potassium chloride -- Furosemide -- Amlodipine -- Heparin -- Metformin -- Insulin -- Octreotide -- Lanreotide -- Spironolactone; Oncology -- Radiology/Rheumatology; Unique/unexpected symptoms or presentations of a disease -- July -- 2019; Endocrinology -- Periodicals; Diabetes -- Periodicals; Diabetes Mellitus; Endocrinology; Diabetes; Endocrinology; Case Reports; Periodicals; Periodicals; 616.4
• DOI: 10.1530/EDM-19-0033
• Languages: English
• ISSNs: 2052-0573

Abstract

Summary: Ectopic ACTH-secreting pulmonary neuroendocrine tumors are rare and account for less than 5% of endogenous Cushing's syndrome cases. We describe an unusual case of metastatic bronchial carcinoid tumor in a young woman presenting with unprovoked pulmonary emboli, which initially prevented the detection of the primary tumor on imaging. The source of ectopic ACTH was ultimately localized by a Gallium-DOTATATE scan, which demonstrated increased tracer uptake in a right middle lobe lung nodule and multiple liver nodules. The histological diagnosis was established based on a core biopsy of a hepatic lesion and the patient was started on a glucocorticoid receptor antagonist and a somatostatin analog. This case illustrates that hypercogulability can further aggravate the diagnostic challenges in ectopic ACTH syndrome. We discuss the literature on the current diagnosis and management strategies for ectopic ACTH syndrome. Learning points: In a young patient with concurrent hypokalemia and uncontrolled hypertension on multiple antihypertensive agents, secondary causes of hypertension should be evaluated. Patients with Cushing's syndrome can develop an acquired hypercoagulable state leading to spontaneous and postoperative venous thromboembolism. Pulmonary emboli may complicate the imaging of the bronchial carcinoid tumor in ectopic ACTH syndrome. Imaging with Gallium-68 DOTATATE PET/CT scan has the highest sensitivity and specificity in detecting ectopic ACTH-secreting tumors. A combination of various noninvasive biochemical tests can enhance the diagnostic accuracy in differentiating Cushing's disease from ectopic ACTH syndrome provided they have concordant results. Bilateral inferior petrosal sinus sampling remains the gold standard.