Tamoxifen treatment of progressive precocious puberty in a patient with McCune-Albright syndrome

Erica Eugster, Ravi Shankar, Lori K. Feezle, Ora H. Pescovitz

Research output: Contribution to journalArticle

36 Citations (Scopus)

Abstract

Treatment of progressive precocious puberty in patients with McCune- Albright syndrome (MAS) has traditionally been with aromatase inhibitors, such as testolactone. However, the use of these agents has been characterized by problems with both efficacy and compliance. We report a case of MAS in which tamoxifen proved to be a successful alternative in the treatment of progressive precocious puberty. An African-American female presented with MAS at 2-5/12 years. Frequent menses, skeletal maturation and growth acceleration prompted initiation of therapy with testolactone at 22 mg/kg/d. Over the next 13 months, the patient's puberty advanced unchecked, despite progressive increases in the dose of testolactone. At age 4 years, medication was discontinued due to treatment failure. At 4-6/12 years, bone age was 10 years, predicted adult height was 137 cm, and monthly bleeding continued. Tamoxifen was then begun on an experimental basis. In response, the patient experienced immediate cessation of menses, and had an abrupt decrease in the rates of pubertal progression and linear growth. This patient has now been maintained on tamoxifen for over three years with no apparent adverse effects. GnRH analogue therapy was begun when the onset of central precocious puberty was noted. Predicted adult height has improved to 154 cm and growth velocity and skeletal maturation remain stable. Our results suggest that tamoxifen may have a valuable role in the treatment of precocious puberty in patients with MAS and may lead to superior results compared with those achieved with aromatase inhibitors.

Original languageEnglish
Pages (from-to)681-686
Number of pages6
JournalJournal of Pediatric Endocrinology and Metabolism
Volume12
Issue number5
StatePublished - 1999

Fingerprint

Polyostotic Fibrous Dysplasia
Precocious Puberty
Testolactone
Tamoxifen
Aromatase Inhibitors
Menstruation
Growth
Therapeutics
Puberty
Treatment Failure
Gonadotropin-Releasing Hormone
African Americans
Compliance
Hemorrhage
Bone and Bones

Keywords

  • McCune-Albright syndrome
  • Precocious puberty
  • Tamoxifen

ASJC Scopus subject areas

  • Endocrinology
  • Pediatrics, Perinatology, and Child Health

Cite this

Tamoxifen treatment of progressive precocious puberty in a patient with McCune-Albright syndrome. / Eugster, Erica; Shankar, Ravi; Feezle, Lori K.; Pescovitz, Ora H.

In: Journal of Pediatric Endocrinology and Metabolism, Vol. 12, No. 5, 1999, p. 681-686.

Research output: Contribution to journalArticle

@article{543cff4c923744b097406bbb15d4cf83,
title = "Tamoxifen treatment of progressive precocious puberty in a patient with McCune-Albright syndrome",
abstract = "Treatment of progressive precocious puberty in patients with McCune- Albright syndrome (MAS) has traditionally been with aromatase inhibitors, such as testolactone. However, the use of these agents has been characterized by problems with both efficacy and compliance. We report a case of MAS in which tamoxifen proved to be a successful alternative in the treatment of progressive precocious puberty. An African-American female presented with MAS at 2-5/12 years. Frequent menses, skeletal maturation and growth acceleration prompted initiation of therapy with testolactone at 22 mg/kg/d. Over the next 13 months, the patient's puberty advanced unchecked, despite progressive increases in the dose of testolactone. At age 4 years, medication was discontinued due to treatment failure. At 4-6/12 years, bone age was 10 years, predicted adult height was 137 cm, and monthly bleeding continued. Tamoxifen was then begun on an experimental basis. In response, the patient experienced immediate cessation of menses, and had an abrupt decrease in the rates of pubertal progression and linear growth. This patient has now been maintained on tamoxifen for over three years with no apparent adverse effects. GnRH analogue therapy was begun when the onset of central precocious puberty was noted. Predicted adult height has improved to 154 cm and growth velocity and skeletal maturation remain stable. Our results suggest that tamoxifen may have a valuable role in the treatment of precocious puberty in patients with MAS and may lead to superior results compared with those achieved with aromatase inhibitors.",
keywords = "McCune-Albright syndrome, Precocious puberty, Tamoxifen",
author = "Erica Eugster and Ravi Shankar and Feezle, {Lori K.} and Pescovitz, {Ora H.}",
year = "1999",
language = "English",
volume = "12",
pages = "681--686",
journal = "Journal of Pediatric Endocrinology and Metabolism",
issn = "0334-018X",
publisher = "Walter de Gruyter GmbH & Co. KG",
number = "5",

}

TY - JOUR

T1 - Tamoxifen treatment of progressive precocious puberty in a patient with McCune-Albright syndrome

AU - Eugster, Erica

AU - Shankar, Ravi

AU - Feezle, Lori K.

AU - Pescovitz, Ora H.

PY - 1999

Y1 - 1999

N2 - Treatment of progressive precocious puberty in patients with McCune- Albright syndrome (MAS) has traditionally been with aromatase inhibitors, such as testolactone. However, the use of these agents has been characterized by problems with both efficacy and compliance. We report a case of MAS in which tamoxifen proved to be a successful alternative in the treatment of progressive precocious puberty. An African-American female presented with MAS at 2-5/12 years. Frequent menses, skeletal maturation and growth acceleration prompted initiation of therapy with testolactone at 22 mg/kg/d. Over the next 13 months, the patient's puberty advanced unchecked, despite progressive increases in the dose of testolactone. At age 4 years, medication was discontinued due to treatment failure. At 4-6/12 years, bone age was 10 years, predicted adult height was 137 cm, and monthly bleeding continued. Tamoxifen was then begun on an experimental basis. In response, the patient experienced immediate cessation of menses, and had an abrupt decrease in the rates of pubertal progression and linear growth. This patient has now been maintained on tamoxifen for over three years with no apparent adverse effects. GnRH analogue therapy was begun when the onset of central precocious puberty was noted. Predicted adult height has improved to 154 cm and growth velocity and skeletal maturation remain stable. Our results suggest that tamoxifen may have a valuable role in the treatment of precocious puberty in patients with MAS and may lead to superior results compared with those achieved with aromatase inhibitors.

AB - Treatment of progressive precocious puberty in patients with McCune- Albright syndrome (MAS) has traditionally been with aromatase inhibitors, such as testolactone. However, the use of these agents has been characterized by problems with both efficacy and compliance. We report a case of MAS in which tamoxifen proved to be a successful alternative in the treatment of progressive precocious puberty. An African-American female presented with MAS at 2-5/12 years. Frequent menses, skeletal maturation and growth acceleration prompted initiation of therapy with testolactone at 22 mg/kg/d. Over the next 13 months, the patient's puberty advanced unchecked, despite progressive increases in the dose of testolactone. At age 4 years, medication was discontinued due to treatment failure. At 4-6/12 years, bone age was 10 years, predicted adult height was 137 cm, and monthly bleeding continued. Tamoxifen was then begun on an experimental basis. In response, the patient experienced immediate cessation of menses, and had an abrupt decrease in the rates of pubertal progression and linear growth. This patient has now been maintained on tamoxifen for over three years with no apparent adverse effects. GnRH analogue therapy was begun when the onset of central precocious puberty was noted. Predicted adult height has improved to 154 cm and growth velocity and skeletal maturation remain stable. Our results suggest that tamoxifen may have a valuable role in the treatment of precocious puberty in patients with MAS and may lead to superior results compared with those achieved with aromatase inhibitors.

KW - McCune-Albright syndrome

KW - Precocious puberty

KW - Tamoxifen

UR - http://www.scopus.com/inward/record.url?scp=0032854049&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0032854049&partnerID=8YFLogxK

M3 - Article

C2 - 10703542

AN - SCOPUS:0032854049

VL - 12

SP - 681

EP - 686

JO - Journal of Pediatric Endocrinology and Metabolism

JF - Journal of Pediatric Endocrinology and Metabolism

SN - 0334-018X

IS - 5

ER -