Pediatric gigantism due to ectopic growth hormone releasing hormone secretion from a metastatic bronchial neuroendocrine tumor.
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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.