Síndrome de Cushing ectópico: descripción de 9 casos

Araujo‐Castro, Marta; García, Nuria Palacios; Pardo, Javier Aller; Álvarez, Cristina Izquierdo; Grao, Laura Armengod; Blas, Javier García

doi:10.1016/j.endinu.2018.02.001

Cited by 13 publications

(2 citation statements)

References 29 publications

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“…A total of 150 cases (Table 3) with ectopic secretion from PCs/PGLs have been reported (data presented in Table 3). ACTH secretion accounted for 33% of all cases (n = 49), whereas CRH, VIP, vasopressin, PTH, renin, aldosterone, insulin or somatostatin, GHRH, and neuropeptide Y secretion have also been described [16,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,…”

Section: Resultsmentioning

confidence: 99%

“…It is probable that ectopic secretion of bioactive compounds from these tumors is often overloooked or clinical manifestations are masked by the hypersecretion of catecholamines. The most frequent ectopically-secreted hormone is ACTH, mostly reported in benign PCs [21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51]. Ectopic secretion of hormones or peptides from malignant PCs/PGLs has been reported even more scarcely.…”

Section: Discussionmentioning

confidence: 99%

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Malignant Pheochromocytomas/Paragangliomas and Ectopic Hormonal Secretion: A Case Series and Review of the Literature

Angelousi

Peppa

Chrisoulidou

et al. 2019

Cancers

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Malignant pheochromocytomas (PCs) and paragangliomas (PGLs) are rare neuroendocrine neoplasms defined by the presence of distant metastases. There is currently a relatively paucity of data regarding the natural history of PCs/PGLs and the optimal approach to their treatment. We retrospectively analyzed the clinical, biochemical, imaging, genetic and histopathological characteristics of fourteen patients with metastatic PCs/PGLs diagnosed over 15 years, along with their response to treatment. Patients were followed-up for a median of six years (range: 1–14 years). Six patients had synchronous metastases and the remaining developed metastases after a median of four years (range 2–10 years). Genetic analysis of seven patients revealed that three harbored succinate dehydrogenase subunit B/D gene (SDHB/D) mutations. Hormonal hypersecretion occurred in 70% of patients; normetanephrine, either alone or with other concomitant hormones, was the most frequent secretory component. Patients were administered multiple first and subsequent treatments including surgery (n = 12), chemotherapy (n = 7), radionuclide therapy (n = 2) and radiopeptides (n = 5). Seven patients had stable disease, four had progressive disease and three died. Ectopic hormonal secretion is rare and commonly encountered in benign PCs. Ectopic secretion of interleukin-6 in one of our patients, prompted a literature review of ectopic hormonal secretion, particularly from metastatic PCs/PGLs. Only four cases of metastatic PC/PGLs with confirmed ectopic secretion of hormones or peptides have been described so far.

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