Experience of Ectopic Adrenocorticotropin Syndrome: 88 Cases With Identified Causes

Miao, Hui; Lü, Lin; Zhu, Huijuan; Du, Hanze; Xing, Xiaoping; Zhang, Xiaobo; Song, Lan; Song, Wei; Liu, Qingxing; Cheng, Xin; Zhu, Zhaohui; Chen, Shi; Tong, Anli; Pan, Hui; Wang, Renzhi; Lü, Zhiyong

doi:10.1016/j.eprac.2021.02.015

Cited by 4 publications

(2 citation statements)

References 35 publications

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“…При отсутствии патологии гипоталамо-гипофизарной области требуется проведение дополнительного инструментального обследования. Эктопированный очаг циклической секреции АКТГ может быть различной локализации: в легких, тимусе, поджелудочной железе, щитовидной железе, надпочечнике, яичнике, почке, желудке, аппендиксе, обладать как доброкачественным, так и злокачественным потенциалом [12,13]. Известны также редкие случаи АКТГ-секреции параганглиомой, нейробластомой, хемодектомой [14].…”

Section: Discussionunclassified

Cyclic Cushing’s syndrome: difficulties of diagnostic search and choice of treatment tactics. A clinical case

Dzeranova

Dorovskikh

Pigarova

et al. 2023

Probl Endokrinol (Mosk)

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Cyclic Cushing’s syndrome is a pathological condition characterized by alternating periods of excessive cortisol secretion with corresponding clinical manifestations and periods of spontaneous remission of the disease.To diagnose Cyclic Cushing’s syndrome it is necessary to record at least three episodes of excessive cortisol secretion alternating with periods of normalization of its production.In most cases, this pathology is diagnosed in patients with ACTH-secreting pituitary tumor, however, there are rare cases of cyclic hypercorticism with ectopic ACTH secretion by tumors of different localization and without verification of pathological hormonal secretion focus. In addition, cyclic hyperproduction of cortisol can be also observed in ACTH-independent Cushing’s syndrome associated with the presence of corticosteroma or adrenal hyperplasia. The exact causes and mechanisms of the cyclic hypercorticism are currently insufficiently studied.Due to the atypical course of the disease, the unpredictability of the occurrence of a new «cycle», the variability of its duration and manifestations (not only in different patients, but also in the same patient), verification of the diagnosis and determination of treatment tactics may be difficult in the daily practice of specialists, and the prevalence of this condition can be undervalued.

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Section: Discussionunclassified

Cyclic Cushing’s syndrome: difficulties of diagnostic search and choice of treatment tactics. A clinical case

Dzeranova

Dorovskikh

Pigarova

et al. 2023

Probl Endokrinol (Mosk)

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“…Our center had summarized the clinical spectrum of 88 patients diagnosed with EAS, and it was found that thoracic origins (80.7%) were the most common cause of EAS, comprising pulmonary NETs (43.2%, 38/88), thymic/ mediastinal NETs (33%, 29/88), and small cell lung cancer (SCLC) (3.4%, 3/88). Pancreatic NETs were found in 6 patients (6.8%) (4). An appendiceal NET is such as rare cause of EAS that there were only seven cases published until now worldwide (5)(6)(7)(8)(9).…”

Section: Introductionmentioning

confidence: 98%

Appendiceal Neuroendocrine Tumor Is a Rare Cause of Ectopic Adrenocorticotropic Hormone Syndrome With Cyclic Hypercortisolism: A Case Report and Literature Review

et al. 2022

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ObjectiveEctopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a condition of hypercortisolism caused by non-pituitary tumors secreting ACTH. Appendiceal neuroendocrine tumor as a rare cause of ectopic ACTH syndrome was reported scarcely. We aimed to report a patient diagnosed with EAS caused by an appendiceal neuroendocrine tumor and summarized characteristics of these similar cases reported before.Case Report and Literature ReviewWe reported a case with Cushing’s syndrome who was misdiagnosed as pituitary ACTH adenoma at first and accepted sella exploration. Serum and urinary cortisol decreased, and symptoms were relieved in the following 4 months after surgery but recurred 6 months after surgery. The abnormal rhythm of plasma cortisol and ACTH presented periodic secretion and seemingly rose significantly after food intake. EAS was diagnosed according to inferior petrosal sinus sampling (IPSS). Appendiceal mass was identified by 68Ga-DOTA-Tyr3-octreotate (DOTATATE)-PET-CT and removed. The pathological result was consistent with appendiceal neuroendocrine tumor with ACTH (+). The literature review demonstrated 7 cases diagnosed with EAS caused by appendiceal neuroendocrine tumor with similarities and differences.ConclusionThe diagnosis of an ectopic ACTH-producing tumor caused by the appendiceal neuroendocrine tumor can be a challenging procedure. Periodic ACTH and cortisol secretion may lead to missed diagnosis and misdiagnosis. IPSS is crucial in the diagnosis of EAS and 68Ga-DOTATATE-PET-CT plays an important role in the identification of lesions.

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Paediatric and adolescent ectopic Cushing's syndrome: systematic review

Yami Channaiah,

Karlekar,

Sarathi

et al. 2023

European Journal of Endocrinology

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Objective The data on clinical, biochemical, radiological characteristics and outcomes in pediatric ectopic adrenocorticotropic hormone syndrome (EAS) is limited owing to rarity of the condition. We report three new cases and perform a systematic review of pediatric EAS. Design and Method Case records of pediatric and adolescent EAS patient’s ≤20 years presenting at our center between 1997-2021 were retrospectively reviewed and a systematic review of the literature published between January 1970 to December 2022 was performed. Results A total of 161 patients including three new patients from our center were identified. Bronchial neuroendocrine tumors (NET) (28.5%), thymic NET (22.9%), primitive cell-derived tumors (PCDT, 18.6%), and gastro-entero-pancreatic-NET (13.7%) were the common causes. PCDT were the most common in the first decade (24/45, 53.4%) and were the largest [82 (60-100) mm], whereas bronchial NETs predominated during the second decade (42/116, 36.2%) and were the smallest [15 (10-25) mm]. Computed tomography localized 92.9% (118/127) of pediatric EAS patients. Immediate postoperative remission was attained in 77.9% (88/113) patients whereas 30.4% (24/79) relapsed over a median (IQR) period of 13 (8-36) months. Over a median (IQR) follow-up of 2 (0.6-4.6) years, 31.4% of patients died. The median survival was higher in bronchial NET than in other tumor groups. Distant metastasis and tumor size were independent negative predictors of survival. Conclusions Etiological profile of pediatric and adolescent EAS is distinct from that of adults. Bronchial NETs have the best long-term survival whereas distant metastasis and tumor size predict poor survival.

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Experience of Ectopic Adrenocorticotropin Syndrome: 88 Cases With Identified Causes

Cited by 4 publications

References 35 publications

Cyclic Cushing’s syndrome: difficulties of diagnostic search and choice of treatment tactics. A clinical case

Cyclic Cushing’s syndrome: difficulties of diagnostic search and choice of treatment tactics. A clinical case

Appendiceal Neuroendocrine Tumor Is a Rare Cause of Ectopic Adrenocorticotropic Hormone Syndrome With Cyclic Hypercortisolism: A Case Report and Literature Review

Paediatric and adolescent ectopic Cushing's syndrome: systematic review

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