An Endocrine Picture In Disguise: A Progressive Olfactory Neuroblastoma Complicated With Ectopic Cushing Syndrome
| العنوان: | An Endocrine Picture In Disguise: A Progressive Olfactory Neuroblastoma Complicated With Ectopic Cushing Syndrome |
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| المؤلفون: | Christophe De Block, Marcel Th. B. Twickler, Jan Van Den Brande, Eveline Dirinck, Martin Lammens, Ann Verhaegen, Katrien Clotman, Luc Van Gaal |
| المصدر: | AACE Clinical Case Reports, Vol 3, Iss 3, Pp e278-e283 (2017) AACE clinical case reports |
| بيانات النشر: | Elsevier, 2017. |
| سنة النشر: | 2017 |
| مصطلحات موضوعية: | Pathology, medicine.medical_specialty, Olfactory Neuroblastoma, Metyrapone, business.industry, Muscle weakness, Bone metastasis, 030209 endocrinology & metabolism, General Medicine, Adrenocorticotropic hormone, medicine.disease, RC648-665, Primary tumor, Hypokalemia, Diseases of the endocrine glands. Clinical endocrinology, 03 medical and health sciences, Cushing syndrome, 0302 clinical medicine, 030220 oncology & carcinogenesis, Medicine, Human medicine, medicine.symptom, business, medicine.drug |
| الوصف: | 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 |
| اللغة: | English |
| تدمد: | 2376-0605 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0985eaa0cc83ad1a187b7c24bab1d115 http://www.sciencedirect.com/science/article/pii/S2376060520301930 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....0985eaa0cc83ad1a187b7c24bab1d115 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 23760605 |
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