A distinctive ovarian sex cord-stromal tumor causing sexual precocity in the Peutz-Jeghers syndrome.

Two distinctive ovarian tumors that caused sexual precocity in young girls with the Peutz-Jeghers syndrome are described. Each of the tumors varied in its histologic pattern, with diffuse areas, foci of tubular differentiation, microcysts and papillae, and contained two unusual cell types. The light-microscopic and ultrastructural findings were consistent with a hitherto undescribed form of sex cord-stromal tumor. The unique microscopic appearance of this tumor and its association with the Peutz-Jeghers syndrome in both cases suggest that it is a third type of gynecological neoplasm related to that disorder.