CLO20-064: A Case Series of Ectopic Cushing Syndrome in Atypical Lung Carcinoid

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  • a Essen Medical Associates, New York, NY
  • | b The Ohio State University, Columbus, OH

Ectopic Cushing syndrome is a rare disease process with approximately 28 – 59% of all cases being from neuroendocrine tumors (NET). Here we present a case series of well differentiated NET with Adrenocorticotropic hormone (ACTH) production. Case1: A 35-year-old woman presented with acute onset of abdominal pain, facial flushing, new hirsutism, and was noted to have a 1.1cm right lower lobe lung nodule that was 18F-FDG-PET avid. Due to patient’s worsening symptoms consistent with ectopic Cushing syndrome she underwent resection of this lesion. Pathology revealed a 1.5cm atypical lung carcinoid with Ki67 <5%. Case 2: A 33-year-old woman presented with new onset of facial acne, hirsutism, worsening muscle weakness, and workup was consistent with ectopic Cushing syndrome. A CT chest revealed a nodular opacity in the left lower lobe of the lung which correlated with a 1.2cm left lower lobe lesion on a Ga68DOTATATE PET scan. Following resection, pathology revealed a 1.3cm atypical lung carcinoid with positive lymph nodes. Case 3: A 27-year-old man presented with new onset diabetes mellitus (DM) and proximal muscle weakness. Initial work-up was consistent with ectopic Cushing syndrome, however all anatomical imaging and octreotide scan were unrevealing. Ga68DOTATATE PET revealed abnormal radiotracer uptake in a nodular opacity in the left lung base. Following resection, pathology revealed a 3.6cm atypical lung carcinoid with Ki67 1%. All three patients had resolution of symptoms following tumor resection. Bronchial carcinoids account for ∼40% of ectopic Cushing syndrome cases secondary to NET. Depending on the tumor grade, the median overall survival varies from 10 months for grade 3 to 16.2 years for grade 1 NET. Often patients with ectopic Cushing syndrome are critically ill who tend to undergo many anatomical and biochemical tests in to localize the lesion which can lead to significant delay in treatment. It is vital to utilize the newer functional imaging modalities such as Ga68DOTATE PET/CT to locate the NET in a timely manner which is known to have high sensitivity and positive predictive value.

Corresponding Author: Neha Verma, MD
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