The Hormonal Role in Physiopathology of Inferior Vena Cava Leiomyosarcoma

Abstract

Dear Editor:A recent article by Ohdan et al,1 reported a case of a young woman affected by a leiomyosarcoma of the inferior vena cava (IVC), positive for estrogen receptor and progesterone receptor. They speculated about the pathophysiologic role of these hormones and the interesting therapeutic option in the treatment of patients affected by this rare tumor.In 1991 we reported the results of a review of the world literature from 1871 to 1989, concerning 144 patients affected by IVC leiomyosarcoma. Moreover, in 1992 an International Registry was established at "La Sapienza" University of Rome. At present we have collected more than 190 patients. In most cases the followup was kept up to date through a personal correspondence with the authors.We were interested in several points of the article.First, the upper segment of the IVC is fortunately the less frequent site of tumor origin (18.9% vs 43.4% and 34.7% of middle and lower segments, respectively).Our analysis showed that the tumor involvement of the upper segment is a high-risk factor of death from disease (P < 0.001). A high incidence of lower limb swelling (P < 0.001), Budd-Chiari syndrome (P < 0.001), intraluminal growth of the tumor (P < 0.001), and IVC thrombosis (P <0.001) was noted in these cases. At this location, most tumors were inoperable and the patients' median survival time was one month. Only 5 patients (13.7%) with an upper IVC tumor were radically operated on. Three patients underwent an expanded-polytetrafluoroethylene (ePTFE) prosthetic caval replacement combined with a right hepatic lobe trisegmentectomy in 1 case, a right hepatic lobectomy in the second, and the reimplantation of hepatic veins after mobilization and hypothermic perfusion of the liver in the third case. In 1 patient after a right hepatic lobectomy an ePTFE patch was used to repair the caval wall defect. The fifth patient had an extraluminal