Abstract

Two patients with hypothalamic hamartoma presented with isosexual precocious puberty. LHRH challenge showed a pubertal LH response in both cases. Serum FSH responses to LHRH were pubertal in case 1, but prepubertal for case 2. Computed tomography revealed isodense noncontrast-enhancing retrosellar mass lesions in both cases. The tumors were composed of mature neurons and neuroglial tissue. Electron microscopy of the lesions failed to demonstrate dense core (neurosecretory) granules in either case. Subtotal removal of the harmartomas resulted in decreased LH responsiveness to LHRH in both cases. Serum FSH responsiveness to LHRH was not significantly suppressed postoperatively in case 1, and FSH responsiveness to LHRH in case 2 showed exaggerated levels, more typical of very young prepubertal girls. Postoperative magnetic resonance imaging (MRI) scans of both patients are also presented.

Original languageEnglish (US)
Pages (from-to)19-26
Number of pages8
JournalPediatric Neurosurgery
Volume13
Issue number1
DOIs
StatePublished - Jan 1 1987

Fingerprint

Gonadotropin-Releasing Hormone
Precocious Puberty
Serum
Electron Microscopy
Tomography
Magnetic Resonance Imaging
Hypothalamic hamartomas
Neurons
Neoplasms

Keywords

  • Hamartoma
  • Hypothalamus
  • Puberty, precocious

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Clinical Neurology

Cite this

Hypothalamic hamartoma : A report of 2 cases. / Markin, Rodney Smith; Leibrock, L. G.; Huseman, C. A.; McComb, Rodney D.

In: Pediatric Neurosurgery, Vol. 13, No. 1, 01.01.1987, p. 19-26.

Research output: Contribution to journalArticle

Markin, Rodney Smith ; Leibrock, L. G. ; Huseman, C. A. ; McComb, Rodney D. / Hypothalamic hamartoma : A report of 2 cases. In: Pediatric Neurosurgery. 1987 ; Vol. 13, No. 1. pp. 19-26.
@article{d775c5ae660d4d07aeafd9913f3a0776,
title = "Hypothalamic hamartoma: A report of 2 cases",
abstract = "Two patients with hypothalamic hamartoma presented with isosexual precocious puberty. LHRH challenge showed a pubertal LH response in both cases. Serum FSH responses to LHRH were pubertal in case 1, but prepubertal for case 2. Computed tomography revealed isodense noncontrast-enhancing retrosellar mass lesions in both cases. The tumors were composed of mature neurons and neuroglial tissue. Electron microscopy of the lesions failed to demonstrate dense core (neurosecretory) granules in either case. Subtotal removal of the harmartomas resulted in decreased LH responsiveness to LHRH in both cases. Serum FSH responsiveness to LHRH was not significantly suppressed postoperatively in case 1, and FSH responsiveness to LHRH in case 2 showed exaggerated levels, more typical of very young prepubertal girls. Postoperative magnetic resonance imaging (MRI) scans of both patients are also presented.",
keywords = "Hamartoma, Hypothalamus, Puberty, precocious",
author = "Markin, {Rodney Smith} and Leibrock, {L. G.} and Huseman, {C. A.} and McComb, {Rodney D}",
year = "1987",
month = "1",
day = "1",
doi = "10.1159/000120296",
language = "English (US)",
volume = "13",
pages = "19--26",
journal = "Pediatric Neurosurgery",
issn = "1016-2291",
publisher = "S. Karger AG",
number = "1",

}

TY - JOUR

T1 - Hypothalamic hamartoma

T2 - A report of 2 cases

AU - Markin, Rodney Smith

AU - Leibrock, L. G.

AU - Huseman, C. A.

AU - McComb, Rodney D

PY - 1987/1/1

Y1 - 1987/1/1

N2 - Two patients with hypothalamic hamartoma presented with isosexual precocious puberty. LHRH challenge showed a pubertal LH response in both cases. Serum FSH responses to LHRH were pubertal in case 1, but prepubertal for case 2. Computed tomography revealed isodense noncontrast-enhancing retrosellar mass lesions in both cases. The tumors were composed of mature neurons and neuroglial tissue. Electron microscopy of the lesions failed to demonstrate dense core (neurosecretory) granules in either case. Subtotal removal of the harmartomas resulted in decreased LH responsiveness to LHRH in both cases. Serum FSH responsiveness to LHRH was not significantly suppressed postoperatively in case 1, and FSH responsiveness to LHRH in case 2 showed exaggerated levels, more typical of very young prepubertal girls. Postoperative magnetic resonance imaging (MRI) scans of both patients are also presented.

AB - Two patients with hypothalamic hamartoma presented with isosexual precocious puberty. LHRH challenge showed a pubertal LH response in both cases. Serum FSH responses to LHRH were pubertal in case 1, but prepubertal for case 2. Computed tomography revealed isodense noncontrast-enhancing retrosellar mass lesions in both cases. The tumors were composed of mature neurons and neuroglial tissue. Electron microscopy of the lesions failed to demonstrate dense core (neurosecretory) granules in either case. Subtotal removal of the harmartomas resulted in decreased LH responsiveness to LHRH in both cases. Serum FSH responsiveness to LHRH was not significantly suppressed postoperatively in case 1, and FSH responsiveness to LHRH in case 2 showed exaggerated levels, more typical of very young prepubertal girls. Postoperative magnetic resonance imaging (MRI) scans of both patients are also presented.

KW - Hamartoma

KW - Hypothalamus

KW - Puberty, precocious

UR - http://www.scopus.com/inward/record.url?scp=0023481538&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0023481538&partnerID=8YFLogxK

U2 - 10.1159/000120296

DO - 10.1159/000120296

M3 - Article

C2 - 3120166

AN - SCOPUS:0023481538

VL - 13

SP - 19

EP - 26

JO - Pediatric Neurosurgery

JF - Pediatric Neurosurgery

SN - 1016-2291

IS - 1

ER -