Primary Fibrosarcoma of the Liver Infiltrating the Right Atrium of the Heart

Abstract

A rare case of a primary fibrosarcoma of the liver infiltrating the right heart is described in a 72-year-old woman. The patient presented with a history of progressive dyspnea and ascites and her general condition was poor. Preoperative cardiac magnetic resonance (CMR) imaging revealed a large mass, which originated from the liver and had infiltrated the right atrium via the inferior vena cava. The patient underwent tumor resection yet died shortly afterwards. Histologically the mass was classified as a fibrosarcoma with positive immunostaining for vimentin. We report the CMR imaging characteristics in this uncommon case. Preoperative CMR proved to be useful for clinical decision making and the planning of surgery.

State of the art paper Sarcomas of the heart as a difficult interdisciplinary problem

“…Kelle et al . reported the case of a 72-year-old female with a fibrosarcoma of the liver right lobe, which extensively infiltrated the inferior vena cava and then spread to the right atrium and tricuspid valve [ 83 ]. Another example is the case of a 23-year-old woman with an osteosarcoma, which arose through the left femoral vein via the external iliac vein, common iliac vein, the vena cava inferior and reached the right atrium stretching up to the right ventricle.…”

Primary Fibrosarcoma of the Liver: We Don’t Know Much

“…Though few reports recommend chemotherapy [9] in liver metastasis of retroperitoneal fibrosarcoma consisting of cyclophosphamide, vincristine, farmorubicin, and dacarbazine, there is no definite role as treatment for primary lesion of liver. Overall the prognosis reported by most of the authors is dismal [1, 2, 3, 10]. …”

Fibroblastoid and Myofibroblastoid Tumors of the Liver and Bile Ducts

“…As in other locations, hepatic fibrosarcoma is markedly vimentin positive (Nakahama et al 1989;Ito et al 1990;Kelle et al 2005). A subset of the tumor cells may be reactive for alpha-SMA.…”

“…In one case of hepatic fibrosarcoma with hypoglycemia, production of insulin-like growth factor II (IGF-II) was proposed to have induced hypoglycemia, based on the detection of IGF-II reactivity in the Golgi area of the neoplastic cells (Kotani et al 1993). Shallow and Wagner 1947;Simpson et al 1955;Steiner 1960;Ojima et al 1964;Snapper et al 1964;Totzke and Hutcheson 1965;Balouet and Destombes 1967;Cavallo et al 1968;Smith and Rele 1972;Walter et al 1972;Chudacek 1973;Alrenga 1975;Papazian et al 1978;Bodker and Boiesen 1981;Gen et al 1983;Kameya et al 1985;Bremondy et al 1989;Nakahama et al 1989;Ito et al 1990;Kotani et al 1993;Tameda and Shiraki 1995;Kelle et al 2005;Ali et al 2008;Zhao et al 2008.…”