[Pelvic PEcoma of the broad ligament with lymph node metastasis: case report and bibliographic review].

Objective: We describe and document the first case of PEcoma published in Spain following the PubMed database. We review the bibliography about these tumors.

Methods/results: 39 year-old female patient with a 9 cm pelvic tumor discovered in a routine gynecologic review. CT scan showed multiple periaortic adenopathies extending up to the left renal vein. The diagnosis of PEcoma was obtained by needle biopsy. Tumor excision including left annex and aortoiliac lymphodenectomy were performed. Pathology confirmed the diagnosis after immunohistochemical study with smooth muscle actin and HMB-45. No adjuvant treatment was given. After one year of follow-up the patient does not show signs of recurrence of the CT scan. A PubMed search was performed finding 73 references about this kind of tumor the conclusions of which are exposed in this article.

Conclusions: Perivascular epithelioid cell tumor, PEcoma, is a very low incidence mesenchymal neoplasia with uncertain malignancy.