Primary malignant mesenchymal tumors of the liver and the inferior vena cava: a rare tumor entity

Abstract

Background: Malignant mesenchymal tumors (MMT) of the liver and the inferior vena cava are a rare tumor entity. Primary hepatic angiosarcoma (PHA) and hepatic epithelioid hemangioendothelioma (HEH) are of endothelial origin, whereas leiomyosarcoma (LMS) arise from smooth muscle cells. While HEH seems to be a low-grade malignant tumor, PHA is assumed to be an aggressive tumor. In the absence of established treatment guidelines radical surgical resection is considered the best treatment. Methods: All resections of primary MMT of the liver (January 2007 until January 2017) were identified from a database of all liver resections performed at our department. We analyzed the completeness of resection (R0/R1), disease-free (DFS) and overall survivals (OS). Results: 15 resections were performed for MMT. Of these, 7 resections were performed for PHA, 4 for HEH and 4 for LMS. 8 out of 15 (53%) patients are tumor free until last follow-up: 3 patients with LMS relapsed, while 3 patients with HEH were tumor-free during follow-up, as well as 2 patients with PHA, who had received neoadjuvant chemotherapy (Ifosamid/Vincristin/Doxorubicin or Paclitaxel). 3 patients with PHA were multifocal when operated and relapsed within three months. One patient with HEH died independently from tumor. The median OS was 108 months and the median DFS was 45 months. Conclusion: Until now the efficiency of chemotherapy could not be proved. In accordance with the literature the best treatment for MMT is the radical surgical resection.