Some hypothalamic hamartomas contain transforming growth factor α, a puberty-inducing growth factor, but not luteinizing hormone-releasing hormone neurons

• Published in: The journal of clinical endocrinology and metabolism Vol. 84; no. 12; pp. 4695 - 4701
• Main Authors: JUNG, H, CARMEL, P, OJEDA, S. R, SCHWARTZ, M. S, WITKIN, J. W, BENTELE, K. H. P, WESTPHAL, M, PIATT, J. H, COSTA, M. E, CORNEA, A, MA, Y. J
• Format: Journal Article
• Language: English
• Published: Bethesda, MD Endocrine Society 01.12.1999
• Subjects: Astrocytes - chemistry; Astrocytes - pathology; Biological and medical sciences; Child, Preschool; Endocrinopathies; Female; Follicle Stimulating Hormone - blood; Gonadotropin-Releasing Hormone - analogs & derivatives; Gonadotropin-Releasing Hormone - analysis; Gonadotropin-Releasing Hormone - therapeutic use; Hamartoma - complications; Hamartoma - pathology; Hamartoma - therapy; Human Growth Hormone - deficiency; Human Growth Hormone - therapeutic use; Humans; Hypothalamic Diseases - complications; Hypothalamic Diseases - pathology; Hypothalamic Diseases - therapy; Hypothalamus. Hypophysis. Epiphysis (diseases); Immunohistochemistry; Infant; Luteinizing Hormone - blood; Magnetic Resonance Imaging; Medical sciences; Neurons - chemistry; Neurons - pathology; Non tumoral diseases. Target tissue resistance. Benign neoplasms; Puberty, Precocious - etiology; Transforming Growth Factor alpha - analysis; Endocrinopathy; Human; Pseudotumor; Immunohistochemistry; Hypothalamic diseases; Pathogenesis; Infant; Hypothalamus; Hamartoma; Precocious puberty; Case study; Pathology; Transforming growth factor α; Benign neoplasm; Child; Biological receptor
• ISSN: 0021-972X
• DOI: 10.1210/jcem.84.12.6185

Activation of LH-releasing hormone (LHRH) secretion, essential for the initiation of puberty, is brought about by the interaction of neurotransmitters and astroglia-derived substances. One of these substances, transforming growth factor alpha (TGFalpha), has been implicated as a facilitatory component of the glia-to-neuron signaling process controlling the onset of female puberty in rodents and nonhuman primates. Hypothalamic hamartomas (HH) are tumors frequently associated with precocious puberty in humans. The detection of LHRH-containing neurons in some hamartomas has led to the concept that hamartomas advance puberty because they contain an ectopic LHRH pulse generator. Examination of two HH associated with female sexual precocity revealed that neither tumor had LHRH neurons, but both contained astroglial cells expressing TGFalpha and its receptor. Thus, some HH may induce precocious puberty, not by secreting LHRH, but via the production of trophic factors--such as TGFalpha--able to activate the normal LHRH neuronal network in the patient's hypothalamus.