Ectopic adrenocorticotropic hormone (ACTH)-dependent Cushing is a rare syndrome. We present a case that illustrates the diagnostic and therapeutic challenges of ectopic Cushing. A 35-year-old woman presented to the outpatient clinic for evaluation of progressive weight gain, muscle weakness, easy bruising, uncontrolled hypertension, and hyperglycemia. Biochemical workup revealed elevated salivary cortisol and 24-hour urine cortisol; the baseline ACTH was elevated, consistent with ACTH-dependent hypercortisolemia. Imaging showed a pituitary microadenoma and a lung nodule. Inferior petrosal sinus sampling was suggestive of an ectopic source. Medical treatment was employed to manage acute hypercortisolemia with a resolution of symptoms. A biopsy of the lung nodule showed the neuroendocrine tumor. Surgical treatment with pulmonary wedge resection did not alleviate hypercortisolemia, leading to repeat surgery with radical lymph node dissection, which resulted in the resolution of hypercortisolemia. This case illustrates that radical lymph node dissection, along with tumor resection, has a high likelihood of cure.
Keywords: acth-dependent cushing syndrome; hypercortisolemia; lung surgery; lymph node dissection; pulmonary carcinoids.
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