Yonsei Med J.  2020 Mar;61(3):257-261. 10.3349/ymj.2020.61.3.257.

Adrenocorticotropic Hormone-Secreting Esthesioneuroblastoma with Ectopic Cushing's Syndrome

Affiliations
  • 1Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea. euihyunkim@yuhs.ac
  • 2Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
  • 3Pituitary Tumor Center, Severance Hospital, Seoul, Korea.
  • 4Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
  • 5Brain Tumor Center, Severance Hospital, Seoul, Korea.

Abstract

Esthesioneuroblastoma as a source of ectopic Cushing's syndrome is rare, and to the best of our knowledge, only 20 cases have been reported worldwide. A 46-year-old healthy man visited a local clinic for general weakness and hyposmia, and underwent examination with serial endocrinological workup and brain imaging. ⁶⁸Gallium-DOTA-TOC positron emission tomography scan was helpful where diagnosis of sellar MRI and inferior petrosal sinus sampling were discordant. Combined transcranial and endoscopic endonasal approach surgery was performed, and a diagnosis of esthesioneuroblastoma was given.

Keyword

Cushing's disease; ectopic ACTH syndrome, esthesioneuroblastoma; positron emission tomography

MeSH Terms

Cushing Syndrome*
Diagnosis
Esthesioneuroblastoma, Olfactory*
Humans
Magnetic Resonance Imaging
Middle Aged
Neuroimaging
Petrosal Sinus Sampling
Positron-Emission Tomography

Figure

  • Fig. 1 Preoperative magnetic resonance imaging (MRI) and 68Ga-DOTA-congugated octreotide positron emission tomography (PET)/computed tomography (CT) scan. Axial and sagittal views of T1-weighted post-contrast MRI show a heterogeneously enhanced mass in the nasal cavity, sphenoid sinus, and frontal base (A and B). 68Ga-DOTA-congugated octreotide PET/CT scan reveals high somatostatin uptake in the tumor, which is suggestive of a neuroendocrine tumor (C). Twelve-month postoperative MRI shows no evidence of a remnant or recurrent tumor (D).

  • Fig. 2 High-power view of H&E staining shows small- to medium-sized neoplastic cells with lobulation. Necrosis and mitosis are not seen. Immunohistochemistry (IHC) staining for various cell markers revealed positive staining for adrenocorticotropic hormone (ACTH), neuron-specific enolase (NSE), and chromogranin A and negative staining for T-Pit. (A) H&E ×200, (B) ACTH-IHC ×200, (C) NSE-IHC ×200, (D) Chromogranin A-IHC ×200, and (E) T-Pit-IHC ×200.


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