Abstract

The rare "sex cord tumor with annular tubules" is commonly multifocal and of microscopic size and histologically characterized by the presence of simple and complex annular tubules with hyaline bodies and a tendency to calcification. It is thought to prise from granulosa cells but to grow in a pattern of Sertoli ceIls. It occurs disproportionateIy often in patients with Peutz-Jeghers syndrome. In some cases, estrogenic manifestations are present. There is vigorous histogenic and taxonomic controversy in ovarian stroma and sex cord origin tumors including sex cord tumor with annular tubules. It is due in part to a lack of agreement regarding basic embryologic concepts of gonadogenesis and in part to the potential of genital ridges for differentiating in either testis or ovary. Recently authors experienced a case of sex cord tumor with annular tubules in a 11 year old female who revealed precocious breast enlargement and the emergence of pubic and axillary hairs. The size of the tumor(25×15×15cm) seemed to be the largest among the reported cases and the histology is identical with Scully’s cases. A case report with review of the literature was performed.