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Ectopic Cushing’s syndrome from a corticotropin-releasing hormone-secreting medullary thyroid carcinoma: a rare pitfall of inferior petrosal sinus sampling

Vivi-Nelli Mäkinen Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark
Department of Internal Medicine, Regional Hospital, Horsens, Denmark

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Stine Horskær Madsen Department of Pathology, Aarhus University Hospital, Aarhus, Denmark

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Mette Ji Riis-Vestergaard Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark
Department of Internal Medicine, Gødstrup Hospital, Herning,Denmark

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Mette Bjerre Department of Clinical Medicine, Aarhus University, Aarhus University Hospital, Aarhus, Denmark

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Steen Bønløkke Pedersen Steno Diabetes Center Aarhus, Aarhus University Hospital, Aarhus, Denmark

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Sylvia L Asa Department of Pathology, University Health Network, Toronto,Canada

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Lars Rolighed Department of Otorhinolaryngology, Head and Neck Surgery, Aarhus University Hospital, Aarhus, Denmark

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Jens Otto Lunde Jørgensen Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark
Department of Clinical Medicine, Aarhus University, Aarhus University Hospital, Aarhus, Denmark

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Marie Juul Ornstrup Department of Endocrinology and Internal Medicine, Aarhus University Hospital, Aarhus, Denmark

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Summary

This case report describes a rare presentation of ectopic Cushing’s syndrome (CS) due to ectopic corticotropin-releasing hormone (CRH) production from a medullary thyroid carcinoma (MTC). The patient, a 69-year-old man, presented with symptoms of muscle weakness, facial plethora, and easy bruising. An inferior petrosal sinus sampling test (IPSS) demonstrated pituitary adrenocorticotrophic hormone (ACTH) secretion, but a whole-body somatostatin receptor scintigraphy (68Ga-DOTATOC PET/CT) revealed enhanced uptake in the right thyroid lobe which, in addition to a grossly elevated serum calcitonin level, was indicative of an MTC. A 18F-DOPA PET/CT scan supported the diagnosis, and histology confirmed the presence of MTC with perinodal growth and regional lymph node metastasis. On immunohistochemical analysis, the tumor cell stained positively for calcitonin and CRH but negatively for ACTH. Distinctly elevated plasma CRH levels were documented. The patient therefore underwent thyroidectomy and bilateral adrenalectomy. This case shows that CS caused by ectopic CRH secretion may masquerade as CS due to a false positive IPSS test. It also highlights the importance of considering rare causes of CS when diagnostic test results are ambiguous.

Learning points

  • Medullary thyroid carcinoma may secrete CRH and cause ectopic CS.

  • Ectopic CRH secretion entails a rare pitfall of inferior petrosal sinus sampling yielding a false positive test.

  • Plasma CRH measurements can be useful in selected cases.