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Diagnosis and Treatment > Medication

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Ayanthi A Wijewardene Departments of Medicine

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Sarah J Glastras Departments of Endocrinology, Diabetes & Metabolism, Royal North Shore Hospital, Sydney, Australia
Kolling Institute of Medical Research
Sydney Medical School, University of Sydney, Sydney, Australia

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Diana L Learoyd Departments of Endocrinology, Diabetes & Metabolism, Royal North Shore Hospital, Sydney, Australia
Sydney Medical School, University of Sydney, Sydney, Australia

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Bruce G Robinson Departments of Endocrinology, Diabetes & Metabolism, Royal North Shore Hospital, Sydney, Australia
Sydney Medical School, University of Sydney, Sydney, Australia

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Venessa H M Tsang Departments of Endocrinology, Diabetes & Metabolism, Royal North Shore Hospital, Sydney, Australia
Sydney Medical School, University of Sydney, Sydney, Australia

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Summary

Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour that originates from the parafollicular cells of the thyroid gland. The most common presentation of MTC is with a single nodule; however, by the time of diagnosis, most have spread to the surrounding cervical lymph nodes. Cushing’s syndrome is a rare complication of MTC and is due to ectopic adrenocorticotrophic hormone (ACTH) secretion by tumour cells. Cushing’s syndrome presents a challenging diagnostic and management issue in patients with MTC. Tyrosine kinase inhibitors (TKI) previously used for the management of metastatic MTC have become an important therapeutic option for the management of ectopic ACTH in metastatic MTC. The article describes three cases of ectopic ACTH secretion in MTC and addresses the significant diagnostic and management challenges related to Cushing’s syndrome in metastatic MTC.

Learning points:

  • Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour.

  • Cushing’s syndrome is a rare complication of MTC that has a significant impact on patients’ morbidity and mortality.

  • Tyrosine kinase inhibitors (TKI) provide an important therapeutic option for the management of ectopic ACTH in metastatic MTC.

Taxonomy:
Clinical Overview, Gland/Organ, Thyroid, Topic, Thyroid, Hormone, ACTH, Calcitonin, Condition/ Syndrome, Cushing's syndrome, Medullary thyroid cancer, Neuroendocrine tumour, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, White, Country of Treatment, Australia, Diagnosis and Treatment, Signs and Symptoms, Abdominal distension, Back pain, Bone fracture(s), Chest pain, Facial plethora, Fatigue, Hypercortisolaemia, Hypertension, Lymphadenitis, Myopathy, Striae, Weight gain, Investigation, ACTH, Blood pressure, Calcitonin, Carcinoembryonic antigen, Chromogranin A, Cortisol, free (24-hour urine), Creatinine, CT scan, Dexamethasone suppression (high dose), Dexamethasone suppression (low dose), Electrolytes, Fine needle aspiration biopsy, Haemoglobin A1c, Histopathology, Immunohistochemistry, Liver function, Molecular genetic analysis, PET scan, Ultrasound scan, X-ray, Intervention, Lymph node dissection, Radiotherapy, Thyroidectomy, Medication, Indapamide, Ketoconazole, Metoprolol, Metyrapone, Mitotane, Perindopril, Potassium chloride, Thyroxine (T4), Tyrosine-kinase inhibitors, Vandetanib, Related Disciplines, Oncology, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-16-0110
Volume/Issue:
Volume 2017: Issue 1
Open access