Ovarian Fibromata

Dumont-Herskowitz et al. (1978) reported ovarian fibromata in 6 women in 5 sibships of 4 generations of a family. In most, presentation was in childhood. The pedigree was consistent with either autosomal or X-linked dominant inheritance with, of course, sex limitation. Ovarian tumors occur with the Peutz-Jeghers syndrome (175200) and with the basal cell nevus syndrome (109400). In the latter condition the ovarian tumors are fibromata and undergo calcification. No stigmata of basal cell nevus syndrome was found in the family reported by Dumont-Herskowitz et al. (1978).