Bilateral Borderline Serous Tumor of Fallopian Tube in a Child With Klippel-Trenaunay Syndrome: An Exceptionally Rare Combination.

Abstract

Klippel-Trenaunay syndrome (KTS) is a rare overgrowth disorder characterized by capillary malformations, vascular anomalies, and limb length discrepancies. It is a congenital, mostly sporadic disorder with unknown pathogenesis, though recent studies have shown an association with somatic mosaic-activating mutations in the PIK3CA gene. The prognosis is variable, depending on the clinical presentation. Visceral involvement in KTS is rare, usually in the form of hemangiomas or venous malformations. Varied neoplastic pathologies have been reported in KTS; however, unlike other overgrowth syndromes, no clear association between KTS and malignancy has so far been elucidated. We report herein an account of a 2-yr-old female child with KTS who presented with abdominal distention and was diagnosed to have a serous borderline tumor (SBT) of bilateral fallopian tubes. Fallopian tube SBT is exceptionally rare and, to the best of our knowledge, has only been reported once previously in a premenarchal patient, who, incidentally, also had KTS. Bilateral fallopian tube involvement in a pediatric SBT has not been described hitherto.