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Cancers (Basel). 2019 May 24;11(5). pii: E724. doi: 10.3390/cancers11050724.

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

Author information

1
Department of Internal Medicine, Unit of Endocrinology, National and Kapodistrian University of Athens, Laiko hospital, 11527 Goudi, Greece. a.angelousi@gmail.com.
2
Endocrine Unit, 2nd Department of Internal Medicine Propaedeutic, Research Institute and Diabetes Center, National and Kapodistrian University of Athens, Attikon University Hospital, 12462 Haidari, Greece. moly6592@yahoo.com.
3
Unit of Endocrinology, Theagenio Cancer Hospital, 2 Al Simeonidi Str., 54007 Thessaloniki, Greece. a.chrisoulidou@gmail.com.
4
st Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, Laiko hospital, 11527 Goudi, Athens, Greece. alexandrakik@gmail.com.
5
Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA. annabel.berthon@nih.gov.
6
Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA. fabio.faucz@pucpr.br.
7
Department of Internal Medicine, Unit of Endocrinology, National and Kapodistrian University of Athens, Laiko hospital, 11527 Goudi, Greece. evakassis@gmail.com.
8
Department of Biological Chemistry, Medical School, National and Kapodistrian University of Athens, 11527 Goudi, Athens, Greece. evakassis@gmail.com.
9
st Department of Propaedeutic Internal Medicine, National and Kapodistrian University of Athens, Laiko hospital, 11527 Goudi, Athens, Greece. gkaltsas@endo.gr.

Abstract

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.

KEYWORDS:

ectopic secretion; lL-6; metastatic OR malignant pheochromocytoma; normetanephrines; paraganglioma

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