Ectopic <scp>C</scp>ushing syndrome in small cell lung cancer: <scp>A</scp> case report and literature review

Zhang, Hangyu; Zhao, Jun

doi:10.1111/1759-7714.12403

Cited by 20 publications

(21 citation statements)

References 17 publications

(16 reference statements)

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“…It is the second most common paraneoplastic syndrome in SCLC patients, after SIADH, occurring frequently in those with metastases to adrenal glands with a prevalence of 1–5% due to excess cortisol secretion in this area (7). …”

Section: Discussionmentioning

confidence: 99%

“…It is rare, appearing in 50% of cases in lung cancer (30–46% in carcinoid tumors from which 80% are intrabronchial, 8–20% in SCLC and rarely in adenocarcinoma), in addition to thymic neoplasms in 15%, pancreatic neuroendocrine tumors, medullary thyroid cancers, pheochromocytomas and unidentified tumors in 12–19% of cases as in our last case. Less commonly, it is associated with paragangliomas, breast, kidney, prostate and esophagus cancer (3, 5, 7). …”

Section: Discussionmentioning

confidence: 99%

“…When arising from SCLC, diagnosis can be difficult especially when it presents without the classical signs of CS, and later during chemotherapy (7). Final diagnosis is done after resection of the primary tumor and confirmation by histology of the neuroendocrine type or the positivity of ACTH staining.…”

Section: Discussionmentioning

confidence: 99%

“…SCLC associated with ECS has the worst prognosis because of several features related to the disease itself like advance stage, poor response to chemotherapy in addition to consequences of the paraneoplastic syndrome as increased susceptibility to infections (opportunistic infections and sepsis) due to the immunosuppressive effect and increased thromboembolic events due to the hypercoagulable state (7). …”

Section: Discussionmentioning

confidence: 99%

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Case-series of paraneoplastic Cushing syndrome in small-cell lung cancer

Richa

Saad

Halabi³

et al. 2018

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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SummaryThe objective of this study is to report three cases of paraneoplastic or ectopic Cushing syndrome, which is a rare phenomenon of the adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome. Three cases are reported in respect of clinical presentation, diagnosis and treatment in addition to relevant literature review. The results showed that ectopic ACTH secretion can be associated with different types of neoplasm most common of which are bronchial carcinoid tumors, which are slow-growing, well-differentiated neoplasms with a favorable prognosis and small-cell lung cancer, which are poorly differentiated tumors with a poor outcome. The latter is present in two out of three cases and in the remaining one, primary tumor could not be localized, representing a small fraction of patients with paraneoplastic Cushing. Diagnosis is established in the setting of high clinical suspicion by documenting an elevated cortisol level, ACTH and doing dexamethasone suppression test. Treatment options include management of the primary tumor by surgery and chemotherapy and treating Cushing syndrome. Prognosis is poor in SCLC. We concluded that in front of a high clinical suspicion, ectopic Cushing syndrome diagnosis should be considered, and identification of the primary tumor is essential.Learning points:Learning how to suspect ectopic Cushing syndrome and confirm it among all the causes of excess cortisol.Distinguish between occult and severe ectopic Cushing syndrome and etiology.Providing the adequate treatment of the primary tumor as well as for the cortisol excess.Prognosis depends on the differentiation and type of the primary malignancy.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Case-series of paraneoplastic Cushing syndrome in small-cell lung cancer

Richa

Saad

Halabi³

et al. 2018

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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“…Well differential lung NETs (typical and atypical carcinoids) account for the majority of PN-CS, approximately 36-46%, producing a clinical and biochemical syndrome resembling pituitary dependent CS (Li et al 2016, Zhang & Zhao 2016. Symptoms and biochemical manifestations typically evolve gradually and usually do not produce a severe CS phenotype (Li et al 2016).…”

Section: Cushing's Syndrome (Cs)mentioning

confidence: 99%

Paraneoplastic endocrine syndromes

Dimitriadis

Angelousi

Weickert

et al. 2017

Endocrine-Related Cancer

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The majority of neoplasms are responsible for symptoms caused by mass effects to surrounding tissues and/or through the development of metastases. However, occasionally neoplasms, with or without endocrine differentiation, acquire the ability to secrete a variety of bioactive substances or induce immune cross-reactivity with the normal tissues that can lead to the development of characteristic clinical syndromes. These syndromes are named endocrine paraneoplastic syndromes when the specific secretory components (hormones, peptides or cytokines) are unrelated to the anticipated tissue or organ of origin. Endocrine paraneoplastic syndromes can complicate the patient's clinical course, response to treatment, impact prognosis and even be confused as metastatic spread. These syndromes can precede, occur concomitantly or present at a later stage of tumour development, and along with the secreted substances constitute the biological 'fingerprint' of the tumour. Their detection can facilitate early diagnosis of the underlying neoplasia, monitor response to treatment and/or detect early recurrences following successful initial management. Although when associated with tumours of low malignant potential they usually do not affect long-term outcome, in cases of highly malignant tumours, endocrine paraneoplastic syndromes are usually associated with poorer survival outcomes. Recent medical advances have not only improved our understanding of paraneoplastic syndrome pathogenesis in general but also enhanced their diagnosis and treatment. Yet, given the rarity of endocrine paraneoplastic syndromes, there is a paucity of prospective clinical trials to guide management. The development of well-designed prospective multicentre trials remains a priority in the field in order to fully characterise these syndromes and provide evidence-based diagnostic and therapeutic protocols.

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