Pediatric gigantism due to ectopic growth hormone releasing hormone secretion from a metastatic bronchial neuroendocrine tumor.
- 1Department of Endocrinology and Nutrition, Hospital Universitario Príncipe de Asturias, Madrid 28806, Spain.
- 2Department of Endocrinology and Nutrition, University and Polytechnic Hospital La Fe, Valencia 46026, Spain.
- 3Medicine Department, University of Valencia, Valencia 46010, Spain.
Abstract
Pediatric gigantism due to ectopic secretion of growth hormone-releasing hormone (GHRH) is exceptionally rare. Most pediatric reports have involved pancreatic neuroendocrine tumors in the context of multiple endocrine neoplasia 1 (MEN1). We describe a female patient diagnosed at 9 years of age with a typical bronchial neuroendocrine tumor and subsequently found to have diffuse hepatic and skeletal metastases, accelerated linear growth, and acromegalic features. Evaluation demonstrated markedly elevated plasma GHRH, growth hormone, and insulin-like growth factor 1, with diffuse pituitary hyperplasia on magnetic resonance imaging and no discrete pituitary adenoma. Genetic testing for MEN1 was negative. Management required repeated surgery, first-generation somatostatin analogs, dopamine agonist therapy, pegvisomant, pasireotide, and peptide receptor radionuclide therapy with lutetium-177 DOTA-Tyr3-octreotate. Pasireotide achieved improved biochemical control, while peptide receptor radionuclide therapy contributed to long-term structural stability. After 23 years of follow-up, the patient remains clinically well with stable tumor burden and controlled somatotropic axis. This case highlights the natural history of a pediatric-onset GHRH-secreting neuroendocrine tumor and highlights the efficacy of aggressive, multidisciplinary management in achieving long-term survival.
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BibTeX
@article{lara2026pediatric,
title = {{Pediatric gigantism due to ectopic growth hormone releasing hormone secretion from a metastatic bronchial neuroendocrine tumor.}},
author = {Fernando Vidal-Ostos De Lara and David Sanchis-Pascual and Lorena Hernández-Rienda and María Isabel Del Olmo-García},
journal = {JCEM case reports},
year = {2026},
month = nov,
doi = {10.1210/jcemcr/luag304},
url = {https://europepmc.org/article/MED/42996503},
note = {CC BY},
}
RIS
TY - JOUR
TI - Pediatric gigantism due to ectopic growth hormone releasing hormone secretion from a metastatic bronchial neuroendocrine tumor.
AU - Fernando Vidal-Ostos De Lara
AU - David Sanchis-Pascual
AU - Lorena Hernández-Rienda
AU - María Isabel Del Olmo-García
PY - 2026
DA - 2026/11/06
JO - JCEM case reports
DO - 10.1210/jcemcr/luag304
UR - https://europepmc.org/article/MED/42996503
AB - Pediatric gigantism due to ectopic secretion of growth hormone-releasing hormone (GHRH) is exceptionally rare. Most pediatric reports have involved pancreatic neuroendocrine tumors in the context of multiple endocrine neoplasia 1 (MEN1). We describe a female patient diagnosed at 9 years of age with a typical bronchial neuroendocrine tumor and subsequently found to have diffuse hepatic and skeletal metastases, accelerated linear growth, and acromegalic features. Evaluation demonstrated markedly elevated plasma GHRH, growth hormone, and insulin-like growth factor 1, with diffuse pituitary hyperplasia on magnetic resonance imaging and no discrete pituitary adenoma. Genetic testing for MEN1 was negative. Management required repeated surgery, first-generation somatostatin analogs, dopamine agonist therapy, pegvisomant, pasireotide, and peptide receptor radionuclide therapy with lutetium-177 DOTA-Tyr3-octreotate. Pasireotide achieved improved biochemical control, while peptide receptor radionuclide therapy contributed to long-term structural stability. After 23 years of follow-up, the patient remains clinically well with stable tumor burden and controlled somatotropic axis. This case highlights the natural history of a pediatric-onset GHRH-secreting neuroendocrine tumor and highlights the efficacy of aggressive, multidisciplinary management in achieving long-term survival.
SN - 2755-1520
ER -