In members of a family there appeared to be an association between the development of cutaneous pigmented keratoses in sun-exposed sites, and the later evolution of internal malignancies, particularly carcinoma of the uterus. Affected individuals were not clinically photosensitive, but their fibroblasts demonstrated gross cytopathic changes, low survival indices and an increased frequency of DNA single strand breaks following exposure to long-wave ultraviolet radiation (UVA). These clinical and cellular features appear to identify an unrecognized syndrome that may not be uncommon. Recognition of this syndrome in a family may prevent certain malignancies developing in those affected.

No abstract

A woman in her sixth decade presented with several months of abdominal cramping, decreased appetite, bloating, and increased constipation. Radiologic imaging revealed a 28 cm, multilocular, heterogeneous cystic neoplasm involving the right adnexa. An intraoperative frozen section showed mucinous glandular epithelium, with and without foci of goblet cells, embedded in apparent ovarian stroma. The findings were concerning at least borderline mucinous cystadenoma with possible invasion. Subsequent surgical management and staging were performed. Permanent sections showed a moderately to poorly differentiated Sertoli-Leydig cell tumor (SLCT) with retiform foci and heterologous elements. The discrepancy between frozen and permanent sections was attributable to solely sampling a focus of heterologous elements during intraoperative consultation. The rarity of SLCT and even rarer presence of both heterologous and retiform elements make this concerning frozen section diagnostic pitfall.