A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome
Abstract Background Clinical care of patients with cyclic Cushing’s syndrome (CS) is challenging. Classical pitfalls include incorrect subtyping, unnecessary surgical procedures and delayed definite treatment. Case presentation A 43-year-old female suffered from a rapidly cycling ectopic CS. She exp...
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| Format: | Article |
| Language: | English |
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BMC
2019-10-01
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| Series: | BMC Endocrine Disorders |
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| Online Access: | http://link.springer.com/article/10.1186/s12902-019-0433-9 |
| _version_ | 1818388054783557632 |
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| author | Adriana Albani Christina M. Berr Felix Beuschlein Marcus Treitl Klaus Hallfeldt Jürgen Honegger Günter Schnauder Martin Reincke |
| author_facet | Adriana Albani Christina M. Berr Felix Beuschlein Marcus Treitl Klaus Hallfeldt Jürgen Honegger Günter Schnauder Martin Reincke |
| author_sort | Adriana Albani |
| collection | DOAJ |
| description | Abstract Background Clinical care of patients with cyclic Cushing’s syndrome (CS) is challenging. Classical pitfalls include incorrect subtyping, unnecessary surgical procedures and delayed definite treatment. Case presentation A 43-year-old female suffered from a rapidly cycling ectopic CS. She experienced six cycles of severe hypercortisolism within a 2 year period (maximum plasma cortisol 5316 nmol/L, normal range 124.2–662.4 nmol/L; maximum urinary free cortisol 79,469 nmol/24 h, normal range < 414 nmol/24 h) lasting 2–9 weeks. The episodes were associated with pronounced hypokalemia (lowest K+ value recorded 2.4 mmol/l) and progressive signs and symptoms of CS. A bilateral inferior petrosal sinus sampling (BIPSS) performed during a trough phase was false positive for pituitary ACTH overproduction resulting in unnecessary transsphenoidal surgery while a second BIPSS performed during an active phase was indicative for ectopic CS. The 18F-DOPA PET/CT showed a pancreatic lesion, which was subsequently partially removed. Surprisingly, the histopathology was conclusive for ACTH-positive lymph node metastasis located in the retro-duodenal tissue of an occult neuroendocrine tumor WHO grade II. The primary tumor has not been identified so far and, because of the persistent hypercortisolism, the patient underwent bilateral adrenalectomy. Two years later, ACTH levels started to increase progressively. Percutaneous biopsy of a newly identified suspected lesion in the fifth thoracic vertebra revealed a metastasis with positive staining for ACTH, synaptophysin and chromogranin A. Therapy with carboplatin and etoposide was started and, since then, the patient underwent 12 cycles of chemotherapy. Conclusions We report the challenging case of a rapidly cycling CS secondary to ACTH-secreting neuroendocrine intestinal tumor of unknown primary. We highlight the importance of performing diagnostic tests only during the phases of active cortisol secretion and as soon as first symptoms appear to avoid pitfalls. |
| first_indexed | 2024-12-14T04:19:45Z |
| format | Article |
| id | doaj.art-4decb3efde31482a8f5362f32839a40f |
| institution | Directory Open Access Journal |
| issn | 1472-6823 |
| language | English |
| last_indexed | 2024-12-14T04:19:45Z |
| publishDate | 2019-10-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Endocrine Disorders |
| spelling | doaj.art-4decb3efde31482a8f5362f32839a40f2022-12-21T23:17:25ZengBMCBMC Endocrine Disorders1472-68232019-10-011911610.1186/s12902-019-0433-9A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndromeAdriana Albani0Christina M. Berr1Felix Beuschlein2Marcus Treitl3Klaus Hallfeldt4Jürgen Honegger5Günter Schnauder6Martin Reincke7Medizinische Klinik und Poliklinik IV, Ludwig Maximilian University MünchenMedizinische Klinik und Poliklinik IV, Ludwig Maximilian University MünchenMedizinische Klinik und Poliklinik IV, Ludwig Maximilian University MünchenKlinik und Poliklinik für Radiologie, Klinikum der Universität München, LMU MünchenChirurgische Klinik und Poliklinik–Innenstadt Klinikum der Ludwig-Maximilians-Universität MünchenDepartment of Neurosurgery, Eberhard Karls University TubingenDepartment of Internal Medicine, Endocrinology and Diabetology, University of TubingenMedizinische Klinik und Poliklinik IV, Ludwig Maximilian University MünchenAbstract Background Clinical care of patients with cyclic Cushing’s syndrome (CS) is challenging. Classical pitfalls include incorrect subtyping, unnecessary surgical procedures and delayed definite treatment. Case presentation A 43-year-old female suffered from a rapidly cycling ectopic CS. She experienced six cycles of severe hypercortisolism within a 2 year period (maximum plasma cortisol 5316 nmol/L, normal range 124.2–662.4 nmol/L; maximum urinary free cortisol 79,469 nmol/24 h, normal range < 414 nmol/24 h) lasting 2–9 weeks. The episodes were associated with pronounced hypokalemia (lowest K+ value recorded 2.4 mmol/l) and progressive signs and symptoms of CS. A bilateral inferior petrosal sinus sampling (BIPSS) performed during a trough phase was false positive for pituitary ACTH overproduction resulting in unnecessary transsphenoidal surgery while a second BIPSS performed during an active phase was indicative for ectopic CS. The 18F-DOPA PET/CT showed a pancreatic lesion, which was subsequently partially removed. Surprisingly, the histopathology was conclusive for ACTH-positive lymph node metastasis located in the retro-duodenal tissue of an occult neuroendocrine tumor WHO grade II. The primary tumor has not been identified so far and, because of the persistent hypercortisolism, the patient underwent bilateral adrenalectomy. Two years later, ACTH levels started to increase progressively. Percutaneous biopsy of a newly identified suspected lesion in the fifth thoracic vertebra revealed a metastasis with positive staining for ACTH, synaptophysin and chromogranin A. Therapy with carboplatin and etoposide was started and, since then, the patient underwent 12 cycles of chemotherapy. Conclusions We report the challenging case of a rapidly cycling CS secondary to ACTH-secreting neuroendocrine intestinal tumor of unknown primary. We highlight the importance of performing diagnostic tests only during the phases of active cortisol secretion and as soon as first symptoms appear to avoid pitfalls.http://link.springer.com/article/10.1186/s12902-019-0433-9ACTHCortisolHypercortisolismCushing diseaseNeuroendocrine tumor |
| spellingShingle | Adriana Albani Christina M. Berr Felix Beuschlein Marcus Treitl Klaus Hallfeldt Jürgen Honegger Günter Schnauder Martin Reincke A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome BMC Endocrine Disorders ACTH Cortisol Hypercortisolism Cushing disease Neuroendocrine tumor |
| title | A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome |
| title_full | A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome |
| title_fullStr | A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome |
| title_full_unstemmed | A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome |
| title_short | A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome |
| title_sort | pitfall of bilateral inferior petrosal sinus sampling in cyclic cushing s syndrome |
| topic | ACTH Cortisol Hypercortisolism Cushing disease Neuroendocrine tumor |
| url | http://link.springer.com/article/10.1186/s12902-019-0433-9 |
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