Resumo

TOBALINA BONIS, Raul et al. Pelvic PEcoma of the broad ligament with lymph node metastasis: Case report and bibliographic review. Arch. Esp. Urol. [online]. 2008, vol.61, n.6, pp.744-748. ISSN 0004-0614.

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 lymphadenectomy 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. Very rare tumors such as epithelioid angiomyolipoma, lymphangioleiomyomatosis, lymphangiomyoma, myelomelanocytic tumor of the falciform ligament, "sugar" clear cell lung tumor and its extrapulmonary variant are all descriptions of what is now a unique tumoral entity named PEcoma. It has not been described yet the normal tissue cell giving origin to this tumor. It affects mainly middle age women, and may appear in any anatomical site. Treatment is surgical, although the role of lymphadenectomy and adjuvant treatment is unknown.

Palavras-chave : PEcoma; Perivascular epithelioid cell; Broad ligament; Sarcoma; ymphangiomatosis; HMB-45.

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