INTRAABDOMINAL DESMOPLASTIC - SMALL-CELL TUMOR IN A PATIENT WITH PEUTZ-JEGHERS SYNDROME

A surgical case of intra-abdominal desmoplastic small cell tumour with divergent differentiation (IDSCT) in a patient with Peutz-Jeghers syn drome (PJS) is reported. The patient was a 23-year-old woman who under went surgery for a tumour in the left paraovarian region. Widespread p eritoneal metastases were noted. Histopathological examination reveale d solid cell nests composed of medium-sized cells demarcated by desmop lastic stroma. Gland-like spaces were found within many cell nests. Tu mour cells were positive for cytokeratin and smooth muscle actin, but negative for desmin. Both ovaries contained minute foci of a sex cord tumour with annular tubules. This is the first documentation of the as sociation of IDSCT with PJS. Since patients with PJS have an increased risk of cancer in various organs, the occurrence of IDSCT in the pres ent patient might not be fortuitous.