A unique case of ectopic Cushing’s syndrome from a thymic neuroendocrine carcinoma

Lawrence, Lima; Zhang, Peng; Choi, Humberto; Ahmad, Usman; Arrossi, Valeria; Purysko, Andrei S.; Makin, Vinni

doi:10.1530/edm-19-0002

Cited by 6 publications

(5 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Steroidogenesis inhibitors have also been studied in combination with pituitary-directed agents (pasireotide and cabergoline); however, such therapy is used in patients with persistent or recurrent CD rather than for the purpose of controlling SH [31][32][33][34]. Lawrence et al reported a patient with severe EAS, due to a thymic neuroendocrine tumor, who was successfully controlled with a combination regimen of ketoconazole and mifepristone [35]. However, it is difficult to monitor the efficacy and safety of such treatment because measuring serum cortisol and/or UFC is not objective in that case [36].…”

Section: Discussionmentioning

confidence: 99%

Cushing’s syndrome: a combined treatment with etomidate and osilodrostat in severe life-threatening hypercortisolemia

Dzialach

Sobolewska

Respondek³

et al. 2022

Hormones

View full text Add to dashboard Cite

Endogenous Cushing’s syndrome (CS) is associated with increased morbidity and mortality. Early diagnosis and initiation of therapy are essential, but effective treatment remains a challenge. In a long-term follow-up, biochemical control of hypercortisolemia, especially when severe, is difficult to achieve. Life-threatening hypercortisolemia is difficult to control due to the limitations of pharmacotherapy, including its side effects, and may require etomidate infusion in the intensive care unit (ICU) to rapidly lower cortisol levels. The effectiveness of hypercortisolemia management can be increased by a dual blockade of cortisol production. We report the efficacy, safety, and tolerability of combined therapy with two steroidogenesis inhibitors, etomidate, and osilodrostat, in a 32-year-old woman diagnosed with severe ACTH-dependent hypercortisolemia, subsequently maintaining a stable level of cortisol with osilodrostat monotherapy. This approach enabled achievement of relatively rapid control of the hypercortisolemia while using an etomidate infusion and concomitant increasing doses of oral osilodrostat applying a “titrations strategy.” Our experience shows that it is worth taking advantage of the synergistic anticortisolic action of etomidate with osilodrostat.

show abstract

Section: Discussionmentioning

confidence: 99%

Cushing’s syndrome: a combined treatment with etomidate and osilodrostat in severe life-threatening hypercortisolemia

Dzialach

Sobolewska

Respondek³

et al. 2022

Hormones

View full text Add to dashboard Cite

show abstract

“… 6 Thymic NETs are usually rare and account for a small percentage of malignancies, occurring more in male individuals at a ratio of 3:1. 7 Functional thymic tumors tend to have a more complicated course and rapid onset of the development of clinical symptoms when associated with endocrinopathy. Suspicion of such tumors should be raised and sought when diagnosing an ectopic Cushing's syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…There is little evidence in the literature describing Cushing's syndrome with a neuroendocrine thymic tumor 6 . Thymic NETs are usually rare and account for a small percentage of malignancies, occurring more in male individuals at a ratio of 3:1 7 . Functional thymic tumors tend to have a more complicated course and rapid onset of the development of clinical symptoms when associated with endocrinopathy.…”

Section: Discussionmentioning

confidence: 99%

A rare initial presentation of a thymic neuroendocrine tumor as Cushing's syndrome

matarneh

Hamad

et al. 2021

Clinical Case Reports

View full text Add to dashboard Cite

Thymic neuroendocrine tumors represent a rare group of neoplasms, with only a few cases described in the literature. They are considered malignant which have the propensity to metastasize. The presentation can be variable as it can present with local symptoms, or if functional, it can secrete hormones, leading to paraneoplastic syndromes such as acromegaly and Cushing's syndrome. Cushing's syndrome is characterized by the signs and symptoms of exposure to excess glucocorticoids. Establishing the diagnosis is often challenging as the signs and symptoms may not always be clear. Among several benign and malignant causes, ectopic secretion of ACTH by nonpituitary tumors accounts for about 10-15 percent of Cushing's syndrome. Most ectopic cases are caused by neuroendocrine tumors arising from the lung, pancreas, or thymus. Due to the diagnostic and presentation challenges, it can often be missed. We wrote this report to highlight a diagnostic dilemma we encountered and the association of thymic neuroendocrine tumors with Cushing's syndrome, along with the patient's outcome. We report a 24-year-old Filipina woman who was presented to our hospital with

show abstract

“…Thymic carcinoid can also secrete ACTH, resulting in ectopic ACTH syndrome, and causing electrolyte and metabolic abnormalities in patients. Its clinical manifestations can include typical Cushing's syndrome manifestations such as central obesity, moon-shaped face, skin ecchymosis, purple skin striae, skin pigmentation, hypertension, hyperglycemia, hypokalemia, and others (7)(8)(9)(10). 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 and can be manifested as insomnia, memory disorders and so on.…”

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

View full text Add to dashboard Cite

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.

show abstract

A unique case of ectopic Cushing’s syndrome from a thymic neuroendocrine carcinoma

Cited by 6 publications

References 10 publications

Cushing’s syndrome: a combined treatment with etomidate and osilodrostat in severe life-threatening hypercortisolemia

Cushing’s syndrome: a combined treatment with etomidate and osilodrostat in severe life-threatening hypercortisolemia

A rare initial presentation of a thymic neuroendocrine tumor as Cushing's syndrome

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

Contact Info

Product

Resources

About