Mistaken Oophorectomy in an Adolescent with McCune-Albright Syndrome: A Case Report and Literature Review

Abstract

McCune–Albright syndrome (MAS) is a rare disease characterized by cafe au lait spots, bone fibrous dysplasia, and precocious puberty. Most MAS cases are diagnosed before adolescence. Here, we reported an adolescent girl underwent mistaken oophorectomy for suspected ovarian tumor, and later, she was diagnosed with MAS. An 11-year-old girl was found to have an irregular pelvic mass measuring 9.74 cm × 9.01 cm × 7.30 cm with a cyst–solid component and a clear boundary by magnetic resonance imaging. She underwent right oophorectomy for the suspected ovarian tumor. However, histopathological examination showed ovarian tissue with many antral follicles. One week after the surgery, ultrasonography revealed a left pelvic irregular echo-free mass measuring 60 mm × 53 mm × 48 mm. The patient was then examined by endocrine specialists, and cafe au lait spots were found predominantly located on the right side of her waist, hip, and thigh. She had her first period before the surgery, and her serum concentrations of sex hormones were normal. Thus, MAS was diagnosed. The patient has been taking dydrogesterone 20 mg/d for 10 days from the 14th day of her period for 2 years. Ultrasonography performed every 3–6 months revealed no enlargement of her left ovary. Awareness of MAS and careful physical and imaging examination should be emphasized, even in the absence of full classic triad of syndromes. Hence, unnecessary oophorectomy and irreversible loss of fertility potential can be avoided in these patients. Key words: Adolescent with McCune–Albright Syndrome; Cafe au lait Spots; Diagnosis; Mistaken Oophorectomy