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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
Shinobu Takayasu Departments of Endocrinology and Metabolism

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Shingo Murasawa Departments of Endocrinology and Metabolism

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Satoshi Yamagata Departments of Endocrinology and Metabolism

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Kazunori Kageyama Departments of Endocrinology and Metabolism

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Takeshi Nigawara Departments of Endocrinology and Metabolism

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Yutaka Watanuki Departments of Endocrinology and Metabolism

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Daisuke Kimura Departments of Endocrinology and Metabolism

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Takao Tsushima Departments of Endocrinology and Metabolism

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Yoshiyuki Sakamoto Departments of Endocrinology and Metabolism

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Kenichi Hakamada Departments of Endocrinology and Metabolism

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Ken Terui Departments of Endocrinology and Metabolism

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Makoto Daimon Departments of Endocrinology and Metabolism

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Summary

Patients with Cushing’s syndrome and excess exogenous glucocorticoids have an increased risk for venous thromboembolism, as well as arterial thrombi. The patients are at high risk of thromboembolic events, especially during active disease and even in cases of remission and after surgery in Cushing’s syndrome and withdrawal state in glucocorticoid users. We present a case of Cushing’s syndrome caused by adrenocorticotropic hormone-secreting lung carcinoid tumor. Our patient developed acute mesenteric ischemia after video-assisted thoracoscopic surgery despite administration of sufficient glucocorticoid and thromboprophylaxis in the perioperative period. In addition, our patient developed hepatic infarction after surgical resection of the intestine. Then, the patient was supported by total parenteral nutrition. Our case report highlights the risk of microthrombi, which occurred in our patient after treatment of ectopic Cushing’s syndrome. Guidelines on thromboprophylaxis and/or antiplatelet therapy for Cushing’s syndrome are acutely needed.

Learning points:

  • The present case showed acute mesenteric thromboembolism and hepatic infarction after treatment of ectopic Cushing’s syndrome.

  • Patients with Cushing’s syndrome are at increased risk for thromboembolic events and increased morbidity and mortality.

  • An increase in thromboembolic risk has been observed during active disease, even in cases of remission and postoperatively in Cushing’s syndrome.

  • Thromboprophylaxis and antiplatelet therapy should be considered in treatment of glucocorticoid excess or glucocorticoid withdrawal.

Taxonomy:
Clinical Overview, Gland/Organ, Liver, Topic, Pituitary, Condition/ Syndrome, Carcinoid tumour, Cushing's syndrome, Diabetes - steroid-induced, Iatrogenic disorder, Patient Demographics, Age, Adult, Gender, Female, Ethnicity, Asian - Japanese, Country of Treatment, Japan, Diagnosis and Treatment, Signs and Symptoms, Acute stress reaction, Central obesity, Facial plethora, Facies - moon, Hypertension, Myasthaenia, Investigation, ACTH, Biopsy, Cortisol, Cortisol, free (24-hour urine), C-reactive protein, Creatinine, CT scan, Glucose (blood), Haemoglobin A1c, PET scan, Radionuclide imaging, Intervention, Laparotomy, Resection of tumour, Medication, Heparin, Hydrocortisone, Metyrapone, Mitotane, Warfarin, Publication Details, Case Report Type, Insight into disease pathogenesis or mechanism of therapy
DOI:
https://doi.org/10.1530/EDM-16-0144
Volume/Issue:
Volume 2017: Issue 1
Open access