A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease
Ectopic ACTH/CRH co-secreting tumors are a very rare cause of Cushing’s syndrome and only a few cases have been reported in the literature. Differentiating between Cushing’s disease and ectopic Cushing’s syndrome may be particularly difficult if predominant ectopic CRH secretion leads to pituitary c...
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| Format: | Article |
| Language: | English |
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Bioscientifica
2017-06-01
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| Series: | Endocrinology, Diabetes & Metabolism Case Reports |
| Online Access: | https://www.edmcasereports.com/articles/endocrinology-diabetes-and-metabolism-case-reports/10.1530/EDM-17-0058 |
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| author | Regina Streuli Ina Krull Michael Brändle Walter Kolb Günter Stalla Marily Theodoropoulou Annette Enzler-Tschudy Stefan Bilz |
| author_facet | Regina Streuli Ina Krull Michael Brändle Walter Kolb Günter Stalla Marily Theodoropoulou Annette Enzler-Tschudy Stefan Bilz |
| author_sort | Regina Streuli |
| collection | DOAJ |
| description | Ectopic ACTH/CRH co-secreting tumors are a very rare cause of Cushing’s syndrome and only a few cases have been reported in the literature. Differentiating between Cushing’s disease and ectopic Cushing’s syndrome may be particularly difficult if predominant ectopic CRH secretion leads to pituitary corticotroph hyperplasia that may mimic Cushing’s disease during dynamic testing with both dexamethasone and CRH as well as bilateral inferior petrosal sinus sampling (BIPSS). We present the case of a 24-year-old man diagnosed with ACTH-dependent Cushing’s syndrome caused by an ACTH/CRH co-secreting midgut NET. Both high-dose dexamethasone testing and BIPSS suggested Cushing’s disease. However, the clinical presentation with a rather rapid onset of cushingoid features, hyperpigmentation and hypokalemia led to the consideration of ectopic ACTH/CRH-secretion and prompted a further workup. Computed tomography (CT) of the abdomen revealed a cecal mass which was identified as a predominantly CRH-secreting neuroendocrine tumor. To the best of our knowledge, this is the first reported case of an ACTH/CRH co-secreting tumor of the cecum presenting with biochemical features suggestive of Cushing’s disease. |
| first_indexed | 2024-12-11T18:33:14Z |
| format | Article |
| id | doaj.art-31ea312ad2c24ec39f285fb031626558 |
| institution | Directory Open Access Journal |
| issn | 2052-0573 2052-0573 |
| language | English |
| last_indexed | 2024-12-11T18:33:14Z |
| publishDate | 2017-06-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrinology, Diabetes & Metabolism Case Reports |
| spelling | doaj.art-31ea312ad2c24ec39f285fb0316265582022-12-22T00:54:51ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732052-05732017-06-01111610.1530/EDM-17-0058A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s diseaseRegina Streuli0Ina Krull1Michael Brändle2Walter Kolb3Günter Stalla4Marily Theodoropoulou5Annette Enzler-Tschudy6Stefan Bilz7Division of Endocrinology and Diabetes, Department of Internal Medicine, St Gallen, SwitzerlandDivision of Endocrinology and Diabetes, Department of Internal Medicine, St Gallen, SwitzerlandDivision of Endocrinology and Diabetes, Department of Internal Medicine, St Gallen, SwitzerlandDepartment of Surgery, Kantonsspital St Gallen, St Gallen, SwitzerlandClinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich, GermanyClinical Neuroendocrinology, Max Planck Institute of Psychiatry, Munich, GermanyInstitute of Pathology, Kantonsspital St Gallen, St Gallen, SwitzerlandDivision of Endocrinology and Diabetes, Department of Internal Medicine, St Gallen, SwitzerlandEctopic ACTH/CRH co-secreting tumors are a very rare cause of Cushing’s syndrome and only a few cases have been reported in the literature. Differentiating between Cushing’s disease and ectopic Cushing’s syndrome may be particularly difficult if predominant ectopic CRH secretion leads to pituitary corticotroph hyperplasia that may mimic Cushing’s disease during dynamic testing with both dexamethasone and CRH as well as bilateral inferior petrosal sinus sampling (BIPSS). We present the case of a 24-year-old man diagnosed with ACTH-dependent Cushing’s syndrome caused by an ACTH/CRH co-secreting midgut NET. Both high-dose dexamethasone testing and BIPSS suggested Cushing’s disease. However, the clinical presentation with a rather rapid onset of cushingoid features, hyperpigmentation and hypokalemia led to the consideration of ectopic ACTH/CRH-secretion and prompted a further workup. Computed tomography (CT) of the abdomen revealed a cecal mass which was identified as a predominantly CRH-secreting neuroendocrine tumor. To the best of our knowledge, this is the first reported case of an ACTH/CRH co-secreting tumor of the cecum presenting with biochemical features suggestive of Cushing’s disease.https://www.edmcasereports.com/articles/endocrinology-diabetes-and-metabolism-case-reports/10.1530/EDM-17-0058 |
| spellingShingle | Regina Streuli Ina Krull Michael Brändle Walter Kolb Günter Stalla Marily Theodoropoulou Annette Enzler-Tschudy Stefan Bilz A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease Endocrinology, Diabetes & Metabolism Case Reports |
| title | A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease |
| title_full | A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease |
| title_fullStr | A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease |
| title_full_unstemmed | A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease |
| title_short | A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease |
| title_sort | rare case of an acth crh co secreting midgut neuroendocrine tumor mimicking cushing s disease |
| url | https://www.edmcasereports.com/articles/endocrinology-diabetes-and-metabolism-case-reports/10.1530/EDM-17-0058 |
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