An Endocrine Picture In Disguise: A Progressive Olfactory Neuroblastoma Complicated With Ectopic Cushing Syndrome

Katrien Clotman, MD; Marcel Twickler, MD, PhD; Eveline Dirinck, MD, PhD; Jan Van Den Brande, MD; Martin Lammens, MD, PhD; Ann Verhaegen, MD; Christophe De Block, MD, PhD; Luc Van Gaal, MD, PhD

AACE Clinical Case Reports (Jan 2017)

An Endocrine Picture In Disguise: A Progressive Olfactory Neuroblastoma Complicated With Ectopic Cushing Syndrome

Katrien Clotman, MD,
Marcel Twickler, MD, PhD,
Eveline Dirinck, MD, PhD,
Jan Van Den Brande, MD,
Martin Lammens, MD, PhD,
Ann Verhaegen, MD,
Christophe De Block, MD, PhD,
Luc Van Gaal, MD, PhD

Affiliations

Katrien Clotman, MD: From the 1Department of Endocrinology, Diabetology and Metabolism, Antwerp University Hospital, Edegem, Belgium.; Address correspondence to Dr. Katrien Clotman, Antwerp University Hospital, Wilrijkstraat 10, 2650 Edegem, Belgium.
Marcel Twickler, MD, PhD: From the 1Department of Endocrinology, Diabetology and Metabolism, Antwerp University Hospital, Edegem, Belgium.
Eveline Dirinck, MD, PhD: From the 1Department of Endocrinology, Diabetology and Metabolism, Antwerp University Hospital, Edegem, Belgium.
Jan Van Den Brande, MD: Department of Oncology, Antwerp University Hospital, Edegem, Belgium.
Martin Lammens, MD, PhD: Department of Pathology, Antwerp University Hospital, Edegem, Belgium.
Ann Verhaegen, MD: From the 1Department of Endocrinology, Diabetology and Metabolism, Antwerp University Hospital, Edegem, Belgium.
Christophe De Block, MD, PhD: From the 1Department of Endocrinology, Diabetology and Metabolism, Antwerp University Hospital, Edegem, Belgium.
Luc Van Gaal, MD, PhD: From the 1Department of Endocrinology, Diabetology and Metabolism, Antwerp University Hospital, Edegem, Belgium.

Journal volume & issue: Vol. 3, no. 3
pp. e278 – e283

Abstract

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ABSTRACT: Objective: To describe a unique case of a 61-year-old patient presenting with ectopic Cushing syndrome as a paraneoplastic phenomenon due to adrenocorticotropic hormone (ACTH)-secreting bone metastasis of an olfactory neuroblastoma (ON).Methods: We report a detailed case, with clinical, biochemical, and imaging findings and compare it with existing literature.Results: A patient with an ON, with subsequent 10-year stable disease after initial craniofacial surgery, presented with paraparesis due to progressively metastasized ON disease located in the thoracic spine. During palliative chemotherapy, severe hypokalemia and muscle weakness developed, being part of paraneoplastic Cushing syndrome. Examination showed that metastatic bone lesions were mostly responsible for the ectopic secretion of ACTH, with just a few ACTH-secreting tumor cells located in the primary tumor. She was treated with a combination of ketoconazole and metyrapone that controlled cortisol levels. However, low potassium levels persisted. Finally, she underwent a bilateral adrenal resection, after which most cushingoid symptoms disappeared. She died 1 month later.Conclusion: Our case shows a growing ON with several bone metastases, which is a rare clinical condition. Moreover, this is the first report that ON is associated with bone metastases that express excessive ACTH secretion (and suggesting dedifferentiation of the primary tumor during chemotherapy treatment). Ectopic Cushing syndrome (with ACTH secreted by ON bone metastasis) can present late in the clinical trajectory. Finally, a bilateral adrenalectomy was needed to correct the life-threatening hypokalemia as part of the ectopic Cushing syndrome.Abbreviations: ACTH adrenocorticotropic hormone; CRH corticotropin-releasing hormone; IPSS inferior petrosal sinus smapling; ON olfactory neuroblastoma

Published in AACE Clinical Case Reports

ISSN: 2376-0605 (Online)
Publisher: Elsevier
Country of publisher: United States
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Diseases of the endocrine glands. Clinical endocrinology
Website: https://www.journals.elsevier.com/aace-clinical-case-reports

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