Ectopic Cushing’s syndrome due to thymic neuroendocrine tumours: a systematic review

Guerrero-Pérez, Fernando; Peiró, Inmaculada; Marengo, Agustina Pia; Teulé, Àlex; Ruffinelli, J.C.; Llatjós, Roger; Serrano, Teresa; Macı́a, Iván; Vilarrasa, Núria; Iglesias, Pedro; Villabona, Carles

doi:10.1007/s11154-021-09660-2

Cited by 11 publications

(16 citation statements)

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“…Thymic neuroendocrine neoplasms have a significant male preponderance and are reported to occur in heavy smokers in contrast to our case ( 9 ). In a review of 21 cases of thymic neuroendocrine neoplasms, 18 were identified as atypical carcinoid, with three associated with ectopic Cushing’s syndrome (CS) ( 10 ). A systematic review and narrative synthesis published in 2021 included 105 cases of sporadic thymic carcinoids.…”

Section: Discussionmentioning

confidence: 99%

Multiple electrolyte disturbances as the presenting feature of multiple endocrine neoplasia type 1 (MEN-1)

Sathyanarayan

Díaz‐Cano

et al. 2022

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary A 49-year-old teacher presented to his general physician with lethargy and lower limb weakness. He had noticed polydipsia, polyuria, and had experienced weight loss, albeit with an increase in central adiposity. He had no concomitant illnesses and took no regular medications. He had hypercalcaemia (adjusted calcium: 3.34 mmol/L) with hyperparathyroidism (parathyroid hormone: 356 ng/L) and hypokalaemia (K: 2.7 mmol/L) and was admitted for i.v. potassium replacement. A contrast-enhanced CT chest/abdomen/pelvis scan revealed a well-encapsulated anterior mediastinal mass measuring 17 × 11 cm with central necrosis, compressing rather than invading adjacent structures. A neck ultrasound revealed a 2 cm right inferior parathyroid lesion. On review of CT imaging, the adrenals appeared normal, but a pancreatic lesion was noted adjacent to the uncinate process. His serum cortisol was 2612 nmol/L, and adrenocorticotrophic hormone was elevated at 67 ng/L, followed by inadequate cortisol suppression to 575 nmol/L from an overnight dexamethasone suppression test. His pituitary MRI was normal, with unremarkable remaining anterior pituitary biochemistry. His admission was further complicated by increased urine output to 10 L/24 h and despite three precipitating factors for the development of diabetes insipidus including hypercalcaemia, hypokalaemia, and hypercortisolaemia, due to academic interest, a water deprivation test was conducted. An 18flurodeoxyglucose-PET (FDG-PET) scan demonstrated high avidity of the mediastinal mass with additionally active bilateral superior mediastinal nodes. The pancreatic lesion was not FDG avid. On 68Ga DOTATE-PET scan, the mediastinal mass was moderately avid, and the 32 mm pancreatic uncinate process mass showed significant uptake. Genetic testing confirmed multiple endocrine neoplasia type 1. Learning points In young patients presenting with primary hyperparathyroidism, clinicians should be alerted to the possibility of other underlying endocrinopathies. In patients with multiple endocrine neoplasia type 1 (MEN-1) and ectopic adrenocorticotrophic hormone syndrome (EAS), clinicians should be alerted to the possibility of this originating from a neoplasm above or below the diaphragm. Although relatively rare compared with sporadic cases, thymic carcinoids secondary to MEN-1 may also be associated with EAS. Electrolyte derangement, in particular hypokalaemia and hypercalcaemia, can precipitate mild nephrogenic diabetes insipidus.

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Section: Discussionmentioning

confidence: 99%

Multiple electrolyte disturbances as the presenting feature of multiple endocrine neoplasia type 1 (MEN-1)

Sathyanarayan

Díaz‐Cano

et al. 2022

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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“…CS is a rare endocrine disorder, presenting a cluster of symptoms caused by an excessive level of glucocorticoids [ 14 ]. It is often clinically noticeable; however, in some instances, the spectrum is wide and nonspecific, coinciding with several medical disorders such as type 2 diabetes, obesity, and PCOS [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

“…These tumors exhibit an aggressive response in conjunction with common endocrinopathies. According to a recent study on ectopic CS caused by thymic neuroendocrine tumors, fewer than 20% of such patients just had a single tumor in mediastinum at the time of presentation [ 14 ]. In addition, 88% of the patients with mediastinal tumors had surgery, and histological subtypes of the tumors were unusual in almost half of the cases [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

“…According to a recent study on ectopic CS caused by thymic neuroendocrine tumors, fewer than 20% of such patients just had a single tumor in mediastinum at the time of presentation [ 14 ]. In addition, 88% of the patients with mediastinal tumors had surgery, and histological subtypes of the tumors were unusual in almost half of the cases [ 14 ]. In our case, at the time of presentation, the differential diagnosis included CS and hyperaldosteronism.…”

Section: Discussionmentioning

confidence: 99%

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Successful resolution of ectopic Cushing syndrome by minimally invasive thoracoscopic resection of the neuroendocrine tumor of the thymus: a rare case report

et al. 2022

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Background Ectopic Cushing syndrome (ECS) is a sporadic condition. Even uncommon is an ECS that derives from a carcinoid tumor of the thymus. These tumors may pose several diagnostic and therapeutic conundrums. This report discusses the differential diagnosis, clinicopathological findings, and effective treatment of a rare case of ECS using a minimally invasive approach. Case presentation A 29-year-old woman with Cushing syndrome presented with facial flushing. Physical examination revealed hypertension (blood pressure: 141/100 mmHg). A mediastinal tumor was discovered to be the cause of the patient’s chronic hypokalemia and hypercortisolemia. Cortisol levels increased in the morning, reaching 47.7 ug/dL. The levels of the hormones ACTH, aldosterone, and renin were determined to be 281 pg/mL, 3.0 ng/dL, and 2.1 pg/mL, respectively. The presence of hypertension, hypokalemia, and alkalinity suggested Cushing’s syndrome, which was proven to be ACTH-dependent ECS by a dexamethasone suppression test. A chest CT scan revealed inflammation in the posterior basal region of the right lower lobe. The superior anterior mediastinum was characterized by round-shaped isodensity lesions with distinct borders. She underwent thoracoscopic anterior mediastinal tumor excision via the subxiphoid technique (R0 resection); following surgery, her blood pressure returned to normal, and the hypernatremia/hypopotassemia resolved. The tumor was determined to be a thymic carcinoid. Most notably, cortisol levels fell to half of their presurgical levels after one hour of surgery, and other abnormalities corrected substantially postoperatively. Conclusion Thoracoscopic excision of thymic tumors by subxiphoid incision may be a useful treatment option for ECS caused by neuroendocrine tumors of the thymus

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“…Thymic tumors can secrete a variety of bioactive substances, which can result in different paraneoplastic syndromes, including myasthenia gravis, limbic encephalitis, pure red-cell anemia, or Cushing's syndrome, among others. Surgical resection of primary tumors is the best treatment for paraneoplastic syndromes (5,6). Thymic carcinoid is a rare thymic tumor, which is usually attributed to large tumors, also known as highly differentiated neuroendocrine neoplasms.…”

Section: Introductionmentioning

confidence: 99%

Ectopic adrenocorticotrophic hormone syndrome initially presenting as abnormal mental behavior caused by thymic carcinoid: a case report

Han¹,

Liu²,

Yan³

et al. 2022

Gland Surg

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Background: Thymic carcinoid is a rare highly differentiated neuroendocrine neoplasm, which can manifest as endocrine disorders caused by ectopic adrenocorticotrophic hormone (ACTH) syndrome.Although clinical manifestations such as hypertension and hypokalemia are common manifestations in patients with ectopic ACTH syndrome, clinicians should also be aware of the mental and behavioral abnormalities that may initially appear in patients. It is extremely rare for patients with ectopic ACTH syndrome caused by thymic carcinoid to concurrently exhibit abnormal mental behavior, especially as the initial clinical manifestation of the tumor. Studies have suggested that abnormal mental behavior may be related to elevated blood cortisol levels.Case Description: A patient was admitted to hospital due to abnormal mental behavior, manifesting as hyperphasia involving gibberish and illogical language, trance, and a state of suspicion. The patient had experienced persecutory delusion. Auxiliary examination revealed elevated cortisol and ACTH.Chest computed tomography (CT) showed right anterior mediastinal tumor. After discussion, the multidisciplinary team (MDT) concluded that ectopic ACTH syndrome derived from the thymus should be considered. After excluding surgical contraindications, a thymic tumor was resected, and the postoperative pathology confirmed that it was thymic carcinoid. At 6 postoperative months, the results were as follows: cortisol at 8:00 am 196.50 nmol/L; and ACTH at 8:00 am 28.63 pg/mL. The patient's mental behavior had returned to normal, and normal communication was possible. The postoperative symptoms and signs of the patient were improved, which reiterated the presence ectopic ACTH syndrome caused by thymic carcinoid.Conclusions: Thymic carcinoid with ectopic ACTH syndrome is very rare in clinical practice, and it is easily missed and misdiagnosed. Although clinical manifestations such as hypertension and hypokalemia are common manifestations in patients with Cushing's syndrome, clinicians should be aware that patients with Cushing's syndrome may initially exhibit abnormal mental behavior. Clinically, if the patient exhibits abnormal mental behavior accompanied by symptoms such as hypokalemia, hypertension, and diabetes, blood cortisol and ACTH hormone levels should be screened without delay. If the levels are found to be significantly increased, ACTH syndrome should be highly suspected.

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Ectopic Cushing’s syndrome due to thymic neuroendocrine tumours: a systematic review

Cited by 11 publications

References 137 publications

Multiple electrolyte disturbances as the presenting feature of multiple endocrine neoplasia type 1 (MEN-1)

Multiple electrolyte disturbances as the presenting feature of multiple endocrine neoplasia type 1 (MEN-1)

Successful resolution of ectopic Cushing syndrome by minimally invasive thoracoscopic resection of the neuroendocrine tumor of the thymus: a rare case report

Ectopic adrenocorticotrophic hormone syndrome initially presenting as abnormal mental behavior caused by thymic carcinoid: a case report

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