A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection
We present a case of a 52-year-old male who developed Cushing’s Syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion from a large esthesioneuroblastoma (ENB) of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Case Reports in Endocrinology |
| Online Access: | http://dx.doi.org/10.1155/2018/6389374 |
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| author | Leslee N. Matheny Sudipa Sarkar Hanyuan Shi Jiun-Ruey Hu Hannah Harmsen Ty W. Abel Shubhada M. Jagasia Shichun Bao |
| author_facet | Leslee N. Matheny Sudipa Sarkar Hanyuan Shi Jiun-Ruey Hu Hannah Harmsen Ty W. Abel Shubhada M. Jagasia Shichun Bao |
| author_sort | Leslee N. Matheny |
| collection | DOAJ |
| description | We present a case of a 52-year-old male who developed Cushing’s Syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion from a large esthesioneuroblastoma (ENB) of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography scan of the head and magnetic resonance imaging showed a 7 cm skull base mass centered in the right cribriform plate without sella involvement. Work-up revealed ACTH-dependent hypercortisolemia, which did not suppress appropriately after high-dose dexamethasone. Subsequent imaging of the chest, abdomen, and pelvis did not reveal other possible ectopic sources of ACTH secretion besides the ENB. His hospital course was complicated by severe hypokalemia and hyperglycemia before successful surgical resection of the tumor, the biopsy of which showed ENB. Postoperatively, his ACTH level dropped below the limit of detection. In the ensuing 4 months, he underwent adjuvant chemoradiation with carboplatin and docetaxel with good response and resolution of hypokalemia and hyperglycemia, with no sign of recurrence as of 30 months postoperatively. His endogenous cortisol production is rising but has not completely recovered. |
| format | Article |
| id | doaj-art-8430af4b7d664454964d808a0bceafd0 |
| institution | Kabale University |
| issn | 2090-6501 2090-651X |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Endocrinology |
| spelling | doaj-art-8430af4b7d664454964d808a0bceafd02025-02-03T01:21:39ZengWileyCase Reports in Endocrinology2090-65012090-651X2018-01-01201810.1155/2018/63893746389374A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after ResectionLeslee N. Matheny0Sudipa Sarkar1Hanyuan Shi2Jiun-Ruey Hu3Hannah Harmsen4Ty W. Abel5Shubhada M. Jagasia6Shichun Bao7Vanderbilt University Medical Center, Division of Endocrinology, Department of Medicine, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 37232, USAJohns Hopkins University School of Medicine, Division of Endocrinology, Diabetes and Metabolism, 5501 Hopkins Bayview Circle, Baltimore, MD 21224, USAVanderbilt University Medical Center, Department of Surgery, Vanderbilt University, 1161 21st Avenue South, Nashville, TN 37232, USAVanderbilt University School of Medicine, 2215 Garland Ave, Nashville, TN 37232, USAVanderbilt University Medical Center, Department of Pathology, Microbiology and Immunology, 1161 21st Avenue South, Nashville, TN 37232, USAVanderbilt University Medical Center, Department of Pathology, Microbiology and Immunology, 1161 21st Avenue South, Nashville, TN 37232, USAVanderbilt University Medical Center, Division of Endocrinology, Department of Medicine, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 37232, USAVanderbilt University Medical Center, Division of Endocrinology, Department of Medicine, Vanderbilt University, 1215 21st Avenue South, Nashville, TN 37232, USAWe present a case of a 52-year-old male who developed Cushing’s Syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion from a large esthesioneuroblastoma (ENB) of the nasal sinuses. The patient initially presented with polyuria, polydipsia, weakness, and confusion. Computed tomography scan of the head and magnetic resonance imaging showed a 7 cm skull base mass centered in the right cribriform plate without sella involvement. Work-up revealed ACTH-dependent hypercortisolemia, which did not suppress appropriately after high-dose dexamethasone. Subsequent imaging of the chest, abdomen, and pelvis did not reveal other possible ectopic sources of ACTH secretion besides the ENB. His hospital course was complicated by severe hypokalemia and hyperglycemia before successful surgical resection of the tumor, the biopsy of which showed ENB. Postoperatively, his ACTH level dropped below the limit of detection. In the ensuing 4 months, he underwent adjuvant chemoradiation with carboplatin and docetaxel with good response and resolution of hypokalemia and hyperglycemia, with no sign of recurrence as of 30 months postoperatively. His endogenous cortisol production is rising but has not completely recovered.http://dx.doi.org/10.1155/2018/6389374 |
| spellingShingle | Leslee N. Matheny Sudipa Sarkar Hanyuan Shi Jiun-Ruey Hu Hannah Harmsen Ty W. Abel Shubhada M. Jagasia Shichun Bao A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection Case Reports in Endocrinology |
| title | A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection |
| title_full | A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection |
| title_fullStr | A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection |
| title_full_unstemmed | A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection |
| title_short | A Case of Cushing’s Syndrome due to Ectopic Adrenocorticotropic Hormone Secretion from Esthesioneuroblastoma with Long Term Follow-Up after Resection |
| title_sort | case of cushing s syndrome due to ectopic adrenocorticotropic hormone secretion from esthesioneuroblastoma with long term follow up after resection |
| url | http://dx.doi.org/10.1155/2018/6389374 |
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