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Diagnosis and Treatment > Signs and Symptoms

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  • Facial plethora x
  • Lymphadenitis x
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Lima Lawrence Departments of Endocrinology, Diabetes & Metabolism, Cleveland Clinic, Cleveland, Ohio, USA

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Peng Zhang Departments of Pulmonary Medicine & Critical Care, Cleveland Clinic, Cleveland, Ohio, USA

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Humberto Choi Departments of Pulmonary Medicine & Critical Care, Cleveland Clinic, Cleveland, Ohio, USA

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Usman Ahmad Departments of Thoracic and Cardiovascular Surgery, Cleveland Clinic, Cleveland, Ohio, USA

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Valeria Arrossi Departments of Anatomic Pathology, Cleveland Clinic, Cleveland, Ohio, USA

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Andrei Purysko Departments of Diagnostic Radiology, Cleveland Clinic, Cleveland, Ohio, USA

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Vinni Makin Departments of Endocrinology, Diabetes & Metabolism, Cleveland Clinic, Cleveland, Ohio, USA

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Summary

Ectopic adrenocorticotropic hormone (ACTH) production leading to ectopic ACTH syndrome accounts for a small proportion of all Cushing’s syndrome (CS) cases. Thymic neuroendocrine tumors are rare neoplasms that may secrete ACTH leading to rapid development of hypercortisolism causing electrolyte and metabolic abnormalities, uncontrolled hypertension and an increased risk for opportunistic infections. We present a unique case of a patient who presented with a mediastinal mass, revealed to be an ACTH-secreting thymic neuroendocrine tumor (NET) causing ectopic CS. As the diagnosis of CS from ectopic ACTH syndrome (EAS) remains challenging, we emphasize the necessity for high clinical suspicion in the appropriate setting, concordance between biochemical, imaging and pathology findings, along with continued vigilant monitoring for recurrence after definitive treatment.

Learning points:

  • Functional thymic neuroendocrine tumors are exceedingly rare.

  • Ectopic Cushing’s syndrome secondary to thymic neuroendocrine tumors secreting ACTH present with features of hypercortisolism including electrolyte and metabolic abnormalities, uncontrolled hypertension and hyperglycemia, and opportunistic infections.

  • The ability to undergo surgery and completeness of resection are the strongest prognostic factors for improved overall survival; however, the recurrence rate remains high.

  • A high degree of initial clinical suspicion followed by vigilant monitoring is required for patients with this challenging disease.

Taxonomy:
Clinical Overview, Gland/Organ, Adrenal, Pituitary, Thymus, Topic, Adrenal, Hormone, ACTH, Cortisol, Condition/ Syndrome, Bilateral adrenal hyperplasia, Cushing's syndrome, Ectopic ACTH syndrome, Ectopic Cushing's syndrome, Hypokalaemia, Lymphadenopathy, Neuroendocrine tumour, Rhabdomyolysis, Patient Demographics, Age, Adult, Gender, Male, Ethnicity, White, Country of Treatment, United Kingdom, Diagnosis and Treatment, Signs and Symptoms, Buffalo hump, Chest pain, Chest pain (pleuritic), Dyspnoea, Facial fullness, Facial plethora, Hypercortisolaemia, Hyperglycaemia, Hyperpigmentation, Hypertension, Hypokalaemia, Hypotension, Leukocytosis, Lymphadenitis, Metabolic alkalosis, Myasthaenia, Obesity, Photosensitivity, Rhabdomyolysis, Supraclavicular fat pads, Syncope, Weight gain, Investigation, ACTH, ACTH stimulation, Bicarbonate, Biopsy, Blood pressure, Chromogranin A, Cortisol, Cortisol, free (24-hour urine), Creatine kinase, CT scan, Dexamethasone suppression, Glucose (blood), Histopathology, Immunohistochemistry, MRI, PET scan, Potassium, Synaptophysin, White blood cell count, X-ray, Intervention, Resection of tumour, Medication, Dexamethasone, Glucocorticoids, Ketoconazole, Steroids, Publication Details, Case Report Type, Unique/unexpected symptoms or presentations of a disease
DOI:
https://doi.org/10.1530/EDM-19-0002
Volume/Issue:
Volume 2019: Issue 1
Open access
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