Leiomyoma Of Vulva And Esophagus

Wahlen and Astedt (1965) described this combination in mother and daughter. The esophageal tumor was an obstructing lesion in the lower portion. In both women the presenting complaint with reference to the vulval lesions was enlargement of the clitoris due to growth of the tumor at its base. Chromosome and endocrinologic studies showed nothing abnormal. The authors emphasized that leiomyoma of the vulva should prompt x-ray studies of the esophagus, and leiomyoma of the esophagus should prompt search for vulval leiomyoma. Schapiro and Sandrock (1973) reported 2 unrelated women in their 20s who for years had been thought to have achalasia. It is possible that the disorder in these 2 reports was in fact the leiomyomatosis nephropathy syndrome (308940), an entity in which expression is milder in females, and which appears to be a contiguous gene deletion syndrome (Antignac et al., 1992) that in many cases also includes congenital cataract.