Oophorectomy in McCune-Albright syndrome

a case of mistaken identity

Zeina Nabhan, Karen W. West, Erica Eugster

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

Objective: The objective of the study was to determine the incidence of oophorectomy in girls presenting with precocious puberty and vaginal bleeding who are subsequently diagnosed with McCune-Albright syndrome (MAS). Study Design: Medical records of girls diagnosed with MAS between 1988 and 2005 were reviewed. Variables analyzed included presenting features, presence of café au lait macules, presence of fibrous dysplasia, radiographic studies, estradiol levels, tumor markers, surgery, and pathology reports. Results: Nine girls with MAS were identified. Average age at initial presentation was 3.2 ± 2.1 years (range, 0.6-7 years). All patients presented with sudden onset of vaginal bleeding. Eight (88%) also had breast development and 2 (22%) had associated pubic hair. Four (44%) girls underwent salpingo-oophorectomy before the diagnosis of MAS was made. Of these, 3 had café au lait macules on initial presentation, and 3 were later diagnosed with fibrous dysplasia. Surgical pathology revealed benign ovarian cysts in all 4 patients. Conclusion: Unnecessary oophorectomy is common in girls with MAS who are taken to the operating room for a presumed ovarian tumor. This highlights the need for increased awareness of MAS among pediatricians, pediatric surgeons, and emergency room physicians. Distinguishing features, which can be helpful in differentiating these 2 conditions, are often present.

Original languageEnglish
Pages (from-to)1578-1583
Number of pages6
JournalJournal of Pediatric Surgery
Volume42
Issue number9
DOIs
StatePublished - Sep 2007

Fingerprint

Polyostotic Fibrous Dysplasia
Ovariectomy
Uterine Hemorrhage
Precocious Puberty
Surgical Pathology
Ovarian Cysts
Operating Rooms
Tumor Biomarkers
Hair
Medical Records
Hospital Emergency Service
Estradiol
Breast
Pediatrics
Pathology
Physicians
Incidence

Keywords

  • McCune-Albright syndrome
  • Oophorectomy
  • Ovarian tumors

ASJC Scopus subject areas

  • Surgery

Cite this

Oophorectomy in McCune-Albright syndrome : a case of mistaken identity. / Nabhan, Zeina; West, Karen W.; Eugster, Erica.

In: Journal of Pediatric Surgery, Vol. 42, No. 9, 09.2007, p. 1578-1583.

Research output: Contribution to journalArticle

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