NEURAL VARIANT OF FETAL RHABDOMYOMA AND NEVOID BASAL-CELL CARCINOMA SYNDROME

A 15-year-old boy with the characteristic features of the naevoid basa l cell carcinoma syndrome presented with a retroperitoneal mass. The t umour showed morphological features of a still ill-defined variant of fetal rhabdomyoma, characterized by well-differentiated nerve fibres a dmired with immature striated muscle cells, similar to neuromuscular c horistoma. Four cases of fetal rhabdomyoma and naevoid basal cell carc inoma syndrome have been previously reported. The behaviour of this tu mour has been benign, although a complete excision was impossible due to its close relation with abdominal vascular trunks.