An ectopic ACTH-producing small cell lung carcinoma associated with enhanced corticosteroid biosynthesis in the peritumoral areas of adrenal metastasis

Satoh, Hironori; Saito, Ryoko; Shu, Hu; Shiihara, Jun; Taniuchi, Shinji; Nakamura, Yasuhiro; Nukiwa, Toshihiro; Ebina, Masahito; Sasano, Hironobu

doi:10.1016/j.lungcan.2011.12.007

Cited by 5 publications

(4 citation statements)

References 30 publications

(33 reference statements)

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“…Very rarely, ACTH-positive cells in the adrenocortical tissue can reveal adrenal micrometastases of an ACTH-secreting cancer. In the published cases, plasma ACTH levels were strongly elevated, as a result of ACTH secretion by the primary tumor ( 87 – 89 ). More surprisingly, a subpopulation of adrenocortical cells has been shown to produce detectable amounts of ACTH in various types of adrenal neoplasms.…”

Section: Paracrine Control Of Adrenal Steroidogenesis By Intraadrenalmentioning

confidence: 97%

Role of ACTH in the Interactive/Paracrine Regulation of Adrenal Steroid Secretion in Physiological and Pathophysiological Conditions

et al. 2016

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In the normal human adrenal gland, steroid secretion is regulated by a complex network of autocrine/paracrine interactions involving bioactive signals released by endothelial cells, nerve terminals, chromaffin cells, immunocompetent cells, and adrenocortical cells themselves. ACTH can be locally produced by medullary chromaffin cells and is, therefore, a major mediator of the corticomedullary functional interplay. Plasma ACTH also triggers the release of angiogenic and vasoactive agents from adrenocortical cells and adrenal mast cells and, thus, indirectly regulates steroid production through modulation of the adrenal blood flow. Adrenocortical neoplasms associated with steroid hypersecretion exhibit molecular and cellular defects that tend to reinforce the influence of paracrine regulatory loops on corticosteroidogenesis. Especially, ACTH has been found to be abnormally synthesized in bilateral macronodular adrenal hyperplasia responsible for hypercortisolism. In these tissues, ACTH is detected in a subpopulation of adrenocortical cells that express gonadal markers. This observation suggests that ectopic production of ACTH may result from impaired embryogenesis leading to abnormal maturation of the adrenogonadal primordium. Globally, the current literature indicates that ACTH is a major player in the autocrine/paracrine processes occurring in the adrenal gland in both physiological and pathological conditions.

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Section: Paracrine Control Of Adrenal Steroidogenesis By Intraadrenalmentioning

confidence: 97%

Role of ACTH in the Interactive/Paracrine Regulation of Adrenal Steroid Secretion in Physiological and Pathophysiological Conditions

et al. 2016

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“…2 Some studies also suggest that SCLC patients with adrenal metastases, such as our patient, may have a greater propensity to develop hypercortisolism, as there is increased cortisol production in areas adjacent to the ACTH-producing adrenal tumor. 5 Aggressive cancers like SCLC produce ACTH at much higher rates than pituitary adenomas. 2 Many patients will not have had time to develop the exam findings of chronic Cushing's, such as truncal obesity, buffalo hump, moon facies, and proximal muscle wasting.…”

Section: Discussionmentioning

confidence: 99%

Ectopic Cushing’s Syndrome as the First Presenting Sign of Small Cell Lung Carcinoma

Srivillibhuthur,

Yu,

et al. 2023

Journal of Brown Hospital Medicine

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A 67-year-old man with a history of hypertension, hyperlipidemia, coronary artery disease, and former smoking presented with fatigue, lower extremity edema, and a new tremor over the course of one month. Workup was notable for hypokalemia, metabolic alkalosis, and extremely elevated adrenocorticotropic hormone (ACTH) and cortisol levels. He was ultimately diagnosed with ectopic Cushing’s syndrome (CS) secondary to new metastatic small cell lung carcinoma. About 5-10% of CS cases are caused by ectopic ACTH production. The most commonly implicated malignancies are small cell lung cancer (SCLC) and neuroendocrine tumors, with SCLC accounting for up to three-fourths of cases. ACTH production is a poor prognostic sign in SCLC, associated with more extensive disease and decreased response to first-line treatment. Tumor-associated ACTH production is often so rapid that patients do not have time to develop classic Cushingoid physical exam findings, instead presenting with acute-onset hyperglycemia, pseudohyperaldosteronism, and generalized weakness. Prompt diagnosis and treatment are essential to improve outcomes.

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“…Aniszewski et al 24 reported that the diagnoses of CS and ACTH-producing neoplasm were usually concurrent, although there were remarkable cases in which the 2 conditions were diagnosed several years apart. 25 With regard to bronchopulmonary carcinoid, they reported that the diagnosis of bronchial carcinoid ranged from 15 years before the onset of CS to 21 years after CS diagnosis. In this case, the time from the onset of CS to tumor localization was 12 years.…”

Section: Discussionmentioning

confidence: 99%

A Case of Ectopic ACTH-Producing Pulmonary Carcinoid Arising in an Extralobar Pulmonary Sequestration

et al. 2015

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Ectopic adrenocorticotrophic hormone (ACTH)-producing bronchopulmonary carcinoid arising in a bronchopulmonary sequestration is extremely rare. The case of a 67-year-old woman with a 1.7-cm nodule in the mediastinal side of the left lower lobe is presented. At 52 years of age, she was diagnosed as having ACTH-dependent Cushing's syndrome (CS). However, no ectopic source of ACTH-secretion was detected. Seven years later, she underwent a bilateral adrenalectomy because of aggravation of her health condition. This time, tumor excision was performed by thoracoscopic surgery. The tumor adhered sparsely to the mediastinal pleura and the left lower lobe and was bluntly separated from these tissues. Pathologically, the tumor was a typical carcinoid arising in an extralobar pulmonary sequestration. Immunohistochemical staining confirmed the secretion of ACTH by bronchopulmonary carcinoid tumor cells. After surgery, the serum ACTH level was almost normalized, and the dexamethasone (1 mg) suppression test showed significant suppression of ACTH.

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An ectopic ACTH-producing small cell lung carcinoma associated with enhanced corticosteroid biosynthesis in the peritumoral areas of adrenal metastasis

Cited by 5 publications

References 30 publications

Role of ACTH in the Interactive/Paracrine Regulation of Adrenal Steroid Secretion in Physiological and Pathophysiological Conditions

Role of ACTH in the Interactive/Paracrine Regulation of Adrenal Steroid Secretion in Physiological and Pathophysiological Conditions

Ectopic Cushing’s Syndrome as the First Presenting Sign of Small Cell Lung Carcinoma

A Case of Ectopic ACTH-Producing Pulmonary Carcinoid Arising in an Extralobar Pulmonary Sequestration

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