EXTRAMAMMARY PAGETS-DISEASE OF THE VULVA - METASTASES TO THE BONE-MARROW IN THE ABSENCE OF AN UNDERLYING ADENOCARCINOMA - CASE-REPORT AND LITERATURE-REVIEW

A case of Paget's disease of the vulva is presented which, over a peri od of 6 years, was characterized by local recurrences and metastases t o the bone marrow. Detailed examination of multiple sections of the pr imary tumor and of the bone marrow biopsy specimen failed to demonstra te an underlying adenocarcinoma in any of the sites. The routine histo logic staining and immunohistochemical staining demonstrated that the metastatic cells strongly correlated with cells from the vulvar lesion . Specifically, immunohistochemical staining of the specimens from the primary and metastatic sites was positive for the gross cystic diseas e fluid protein-15, which has been associated with extramammary Paget' s disease uncomplicated by underlying malignancies. Finally, during se veral years of close follow-up, no other primary site of Paget's disea se declared itself clinically or was found by radiographic studies or analysis of serum tumor markers. (C) 1997 Academic Press.