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Skull Base 2008; 18(1): 073-076
DOI: 10.1055/s-2007-994291
CASE REPORT

© Thieme Medical Publishers

Cushing's Syndrome: An Unusual Presentation of Olfactory Neuroblastoma

Lydia Josephs1 , Lamont Jones2 , Lawrence Marenette2 , Paul McKeever3
  • 1University of Michigan Medical School, Ann Arbor, MI
  • 2Department of Otolaryngology, University of Michigan Health System, Ann Arbor, Michigan
  • 3Department of Pathology, University of Michigan Health System, Ann Arbor, Michigan
Further Information

Publication History

Publication Date:
16 November 2007 (online)

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ABSTRACT

A 48-year-old man presented with symptoms consistent with Cushing's syndrome. Subsequent laboratory studies revealed markedly elevated adrenocorticotropic (ACTH) and cortisol levels, as well as a hypoklemic metabolic alkalosis. A pituitary MRI was performed, which revealed a normal pituitary; however, a large mass was seen centered in the ethmoid and paranasal sinuses with a significant amount of extension into surrounding structures. A biopsy was performed and pathology of the specimen was consistent with esthesioneuroblastoma. Immunohistochemical staining further defined the tumor as an ACTH-secreting esthesioneuroblastoma. After total resection of the mass and further treatment with adjuvant radiation therapy, the patient's symtoms completely resolved and the ACTH and cortisol levels were also greatly reduced. This case demonstrates the successful diagnosis and treatment of a rare neoplasm. Ectopic ACTH syndrome due to esthesioneuroblastoma is extremely uncommon with only five other cases being discussed in the literature.

KEYWORDS

Ectopic ACTH syndrome - Cushing's syndrome - olfactory Neuroblastoma - esthesioneuroblastoma

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Lydia Josephs

University of Michigan Health System

1500 E. Medical Center Drive, Room 1904, Ann Arbor, MI 48109

Email: ljosephs@med.umich.edu

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