A rare case of Cushing syndrome by cyclic ectopic-ACTH

Farage, Mariana; Costa, Mario Alberto da Dantas Loures; Godoy-Matos, Amélio F.

doi:10.1590/s0004-27302012000500008

Cited by 13 publications

(7 citation statements)

References 39 publications

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“…Cyclic Cushing syndrome has been reported especially in ACTH-independent Cushing syndrome and rarely in ACTH-dependent Cushing syndrome, both in cases of microadenomas and ectopic ACTH secretion. 8,18 In the cyclic form of ACTH-dependent Cushing syndrome, the hypersecretion of ACTH shows a cyclic or intermittent pattern. As a result, serum ACTH levels together with cortisol serum levels normalize in the clinical remission phase of the disease.…”

Section: Discussionmentioning

confidence: 99%

ACTH adenomas transforming their clinical expression: report of 5 cases

Zoli¹,

Faustini‐Fustini

Mazzatenta³

et al. 2015

FOC

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OBJECT Adrenocorticotropic hormone (ACTH) adenomas have been recognized as a more aggressive and invasive subtype of pituitary adenomas. An additional and clinically relevant peculiarity of these tumors is their ability to modify their clinical expression from a silent form to Cushing disease or vice versa. The aim of this study was to review a series of patients with pituitary adenomas and analyze the clinical implications of the transformation of clinical expression in 5 cases that showed this phenomenon. METHODS The authors retrospectively reviewed a series of patients with pituitary adenoma and collected clinical, biohumoral, and neuroradiological data of those who presented with a transformation from silent ACTH adenomas to functioning tumors or vice versa. In all the cases, preoperative assessment consisted of brain MRI, ophthalmological examination, and complete baseline endocrinological investigation. In patients with clinical and/or biochemical findings suspicious for Cushing syndrome, a low-dose dexamethasone suppression test was performed to rule in or out this diagnosis. Endocrinological evaluations were repeated 1 month after surgery, 3 months after surgery, and every 6 months or annually thereafter. Ophthalmological evaluations and brain MRIs were repeated after 3 months and then every 6 or 12 months thereafter. RESULTS Five patients (2 men and 3 women) included in this series had corticotropic tumors that showed transformation from an endocrinologically silent form to manifest Cushing disease and vice versa. The mean age at presentation was 40 years (range 18–51 years). In 3 of these patients, a transformation from silent to functioning ACTH adenoma with manifest Cushing disease occurred. In 1 patient, the authors observed the transition from a functioning to a silent adenoma with spontaneous resolution of hypercortisolism. Another patient's silent adenoma “shifted” to a functioning adenoma and then regressed back to a silent form with spontaneous resolution of Cushing disease. This patient again developed hypercortisolism, which finally resolved spontaneously. In this series, the transformation occurred after a mean of 3.5 years (range 6 months to 7 years). The shift from an ACTH-silent to a functioning adenoma was observed in 9% of the ACTH-silent adenomas in this series (4 of 44 cases), and the spontaneous remission of Cushing disease to a silent corticotroph cell adenoma occurred in 1.5% of cases of this series (2 of 132 functioning ACTH adenomas). At follow-up (mean 107 months; range 60–177 months), cortisol levels were within normal limits in all 5 cases. However, 1 patient required Gamma Knife radiosurgery and eventually adrenalectomy for disease control to be achieved. CONCLUSIONS The ability of silent ACTH adenomas to transform their secretion pattern poses a challenge for neurosurgeons and endocrinologists. Because the transformation is often unexpected, the clinical and biochemical data can be underestimated. Furthermore, this bizarre and unpredictable postoperative tumor behavior can lead to misinterpretation of clinical and endocrinological outcomes. Even if these cases are very rare, they are not anecdotal in large series. Thus, ACTH adenomas require careful biohumoral and neuroradiological follow-up to detect possible transformations.

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Section: Discussionmentioning

confidence: 99%

ACTH adenomas transforming their clinical expression: report of 5 cases

Zoli¹,

Faustini‐Fustini

Mazzatenta³

et al. 2015

FOC

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“…Neuroendocrine tumors of the thymus are the most frequently represented, followed by neuroendocrine bronchial tumors [4, 19–26]. Other tumors reported as responsible of cyclic ectopic CS are pancreatic, renal and gastric NET [27–29], epithelial thymoma [30], phaeochromocytoma [31], carotid glomus tumor [32] and ectopic pituitary adenoma [33]. Surprisingly, although small cell lung carcinoma is frequent in patients with ectopic CS, biochemical episodes of cycling seem to be rare.…”

Section: Discussionmentioning

confidence: 99%

A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome

Albani¹,

Berr²,

Beuschlein

et al. 2019

BMC Endocr Disord

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BackgroundClinical care of patients with cyclic Cushing’s syndrome (CS) is challenging. Classical pitfalls include incorrect subtyping, unnecessary surgical procedures and delayed definite treatment.Case presentationA 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.ConclusionsWe 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.

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“…МРЩЖ является одним из самых злокачественных видов опухолей эндокринной системы [9,10]. Большая часть этих опухолей характеризуется агрессивным течением со склонностью к раннему метастазированию и рецидивированию.…”

Section: заключениеunclassified

ACTH-producing medullary thyroid cancer. Clinical case.

Марова¹,

Рожинская²,

Воронкова³

et al. 2016

Probl Endokrinol (Mosk)

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Medullary thyroid cancer with ectopic ACTH production - the disease is extremely rare. The literature describes only a few cases of this disease. Rare ectopic ACTH syndrome caused by medullary thyroid cancer, and the diversity of the clinical picture are responsible for numerous diagnostic errors leading to ineffective treatment. In this regard, we consider it expedient to share our own experience in this area.

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A rare case of Cushing syndrome by cyclic ectopic-ACTH

Cited by 13 publications

References 39 publications

ACTH adenomas transforming their clinical expression: report of 5 cases

ACTH adenomas transforming their clinical expression: report of 5 cases

A pitfall of bilateral inferior petrosal sinus sampling in cyclic Cushing’s syndrome

ACTH-producing medullary thyroid cancer. Clinical case.

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