Pulmonary embolism as the presenting symptom and a confounder in ACTH-secreting bronchial carcinoid

• Published in: Endocrinology, diabetes & metabolism case reports Vol. 2019; no. 1; pp. 1 - 6
• Main Authors: Yang, Wei, Pham, David, Vierra, Aren T, Azam, Sarah, Gui, Dorina, Yoon, John C
• Format: Journal Article
• Language: English
• Published: England Bioscientifica Ltd 26.07.2019; Bioscientifica
• Subjects: ACTH; Adrenal; Adult; Amenorrhoea; Amlodipine; Angiography; Black - African; Calcitonin; Chromogranin A; Core needle biopsy; Cortisol; Cortisol (9am); Cortisol, free (24-hour urine); CT scan; CTPA scan; Cushing's syndrome; Dexamethasone suppression (high dose); Dexamethasone suppression (low dose); Dyspnoea; Ectopic Cushing's syndrome; Facial plethora; Fatigue; Female; FSH; Furosemide; Gallium scan; Glucose (blood); Haematoxylin and eosin staining; Heparin; Histopathology; Hypercortisolaemia; Hypertension; Hypokalaemia; Immunohistochemistry; Insulin; Lanreotide; Lisinopril; Metastatic tumour; Metformin; Microadenoma; Nausea; Neuroendocrine tumour; Obesity; Octreotide; Oedema; Oncology; Pituitary; Potassium; Potassium chloride; Radiology/Rheumatology; Somatostatin analogues; Spironolactone; Striae; Synaptophysin; Tachycardia; Thrombophilia; Ultrasound-guided biopsy; Unique/Unexpected Symptoms or Presentations of a Disease; United States; Immunohistochemistry; Somatostatin analogues; Facial plethora; ACTH; Octreotide; Cortisol; Histopathology; Lanreotide; CTPA scan; FSH; July; Dexamethasone suppression (low dose); Obesity; Synaptophysin; Gallium scan; Neuroendocrine tumour; Ultrasound-guided biopsy; Lisinopril; Striae; Fatigue; Cushing's syndrome; Nausea; Adrenal; Potassium chloride; Insulin; Furosemide; Thrombophilia; Amlodipine; Metformin; Unique/unexpected symptoms or presentations of a disease; Angiography; United States; Oedema; Black - African; Oncology; Metastatic tumour; Core needle biopsy; Radiology/Rheumatology; Dyspnoea; Spironolactone; Amenorrhoea; Calcitonin; Adult; Female; CT scan; Hypertension; Ectopic Cushing's syndrome; Glucose (blood); Cortisol (9am); Chromogranin A; Dexamethasone suppression (high dose); Haematoxylin and eosin staining; Hypercortisolaemia; Cortisol, free (24-hour urine); Microadenoma; 2019; Tachycardia; Hypokalaemia; Pituitary; Heparin; LH; Potassium
• ISSN: 2052-0573; 2052-0573
• DOI: 10.1530/EDM-19-0033

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.