Precocious puberty associated with a pineal cyst: is it disinhibition of the hypothalamic-pituitary axis?
Accelerated development of secondary sexual characteristics or sexual precocity is a well-known entity. Most authors recognize two groups of patients, those described as having central precocious puberty (CPP) and those with precocious pseudopuberty. CPP results from premature activation of the hypothalamic-pituitary-gonadal axis and pseudopuberty is caused by lesions that secrete gonadotropin-like substances or hormones. The onset of CPP is usually before age 8 in females and age 9 in males; however, there is contention that the age of onset is much earlier and also differs depending on the patients' race. Previously reported causes of CPP include intracranial neoplasm, infection, trauma, hydrocephalus and Angelman's syndrome. Pineal cysts are usually asymptomatic incidental findings, but have been associated with CPP. We present an interesting case of a patient with CPP and an associated pineal cyst. We review the literature on the pathogenesis of CPP and associated pineal cyst, the neuroendocrine relationship between the pineal gland and puberty and the neurosurgical role in these cases.
Dickerman
Rob D
RD
Department of Neurosurgery, North Shore University-Long Island Jewish Medical Center, New Hyde Park, NY 11004, USA. drrdd@yahoo.com
Stevens
Qualls E
QE
Steide
Jean-Alex
JA
Schneider
Steven J
SJ
D002648:Child; D003560:Cysts / Q000601:surgery*; D005260:Female; D006801:Humans; D007986:Luteinizing Hormone / Q000097:blood; D010870:Pineal Gland / Q000601:surgery*; D011629:Puberty, Precocious / Q000209:etiology*
D007986:Luteinizing Hormone