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Sertoli-Leydig Cell Tumor of the Ovary, a Rare Cause of Precocious Puberty in a 12-Month-Old Infant

Departments of Pediatric Endocrinology (C.S.C., Y.J., A.M.C.), Pediatrics (R.B., R.S.), Pediatric Surgery (P.B.), and Pathology (F.B., D.P.), Mater Misericordiae Hospitals, South Brisbane, Queensland, Australia 4104; Flinders Cancer Centre (C.S.C.), Flinders Medical Centre, Bedford Park, South Australia, Australia 5042; Southern Cross Pathology Australia (N.D.B.), Clayton, Victoria, Australia 3168; and Prince Henry’s Institute of Medical Research (P.J.F., S.C., D.M.R., H.G.B.), Clayton, Victoria, Australia 3168

Address all correspondence and requests for reprints to: Catherine Choong, Flinders Cancer Center, Flinders Medical Center, Bedford Park, South Australia, Australia 5042. E-mail: catherine.choong{at}flinders.edu.au

We report a 12-month-old infant who presented with a 4-month history of isosexual precocious puberty secondary to an estrogenizing Sertoli-Leydig cell tumor of the ovary. Total serum immunoreactive inhibin and subunits A and B were markedly elevated before surgical resection and subsequently decreased 7 wk later into the normal prepubertal range. Twenty weeks following surgical removal, the patient presented again with central precocious puberty; inhibin B levels were raised on this occasion, a luteinizing releasing hormone stimulation test confirmed central precocious puberty. This is the youngest reported occurrence of this rare sex cord stromal neoplasm. The prognosis of this extremely rare tumor presenting at this early juvenile stage is uncertain. This report illustrates the usefulness of serum inhibin as a tumor marker during therapeutic suppression with leuprorelin acetate for central precocious puberty. Analysis of genomic and tumor DNA revealed a normal nucleotide sequence for the LH receptor and the G_s gene. To understand the molecular pathogenesis of this tumor we analyzed mRNA levels for the inhibin A and B subunits, FSH receptor, LH receptor aromatase, steroidogenic factor-1 and the ER ß genes. Molecular characterization reveals the presence of genes specific for granulosa and Leydig cells; the relative expression of these genes, in addition to its histologic characteristics, suggests that this tumor may result from a dysdifferentiation of a primordial follicle.

This work was supported by the National Health and Medical Research Council of Australia and the Mater Hospital Trust.

Abbreviations: GCT, Granulosa cell tumor; HCG, human CG; MIS, Müllerian inhibiting substance.