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Masquerading Primary Liver Leiomyosarcoma as a Hemorrhagic Hepatoma
Image of the Month Masquerading Primary Liver Leiomyosarcoma as a Hemorrhagic Hepatoma CAROL JAMIE MORRIS and RAVI GHANTA Division of Hepatology, Jackson Memorial Hospital and University of Miami Hospital, Miami, Florida
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78-year-old woman presented with 2 months of pleuritic right upper quadrant abdominal pain associated with a 12pound weight loss. Abdominal exam showed moderate right upper quadrant tenderness, with a palpable liver edge up to 3 inches below the right costal margin; no discrete mass was palpated. Computed tomography scan enhanced triple phase of the liver subsequently demonstrated a 13-cm mass in the right lobe, a 3-cm mass in the posterior and inferior subcapsular region of segment 3 of the left hepatic lobe, and a 2-cm focal lesion in segment 3 of the left hepatic lobe near the fissure of the round ligament (Figure A). Follow-up magnetic resonance image with gadolinium showed the largest lesion in right lobe measuring 13.4 ⫻ 11.1 ⫻ 17.4 cm with heterogeneous enhancement and low peripheral and high central T1 and T2 signal, consistent with necrosis or hemorrhage, impressing on, but not originating from, the inferior vena cava (Figure B). Histologic evaluation stained with hematoxylin-eosin from a liver biopsy showed a spindle cell malignant tumor (Figure C). In addition, this tumor immunohistochemically stained positive for desmin, and there was coexpression of keratin, yet it stained negative for DF31, CD 68, S100, and hepatocellular antigen. The tumor was diagnosed as a high-grade sarcoma consistent with primary liver leiomyosarcoma. The patient was referred to oncology for further medical management because she was determined to be a nonsurgical candidate. Leiomyosarcomas are mesenchymal tumors with their origin in the connective tissue and blood vessels of the affected organ. Intestinal leiomyosarcomas are fairly rare, with a frequency of 1.4 cases per 100,000 patients. They consist of spindle-shaped cells and have a high cellularity. With high-grade tumors, necrosis often is present. Approximately 60% of patients are symptomatic, and of these, 70% present with bleeding followed by weight loss.1 The natural history of this tumor involves local
growth initially, with much of its growth being extraluminal; thus, obstruction occurs late. As the size increases, necrosis and bleeding follow. Growth is indolent and late to metastasize; therefore, aggressive surgical resection is feasible, even with a large primary tumor.2 Other local therapy options for unresectable liver metastases include hepatic arterial embolization, microwave ablation, chemoembolization combined with imatinib, as well as radiofrequency ablation, which has been well established as a viable option for nonsurgical candidates.3 Primary liver leiomyosarcomas are rare and have no definite radiologic criteria for diagnosis; however, in any patient presenting with right upper quadrant pain and weight loss in the setting of a hepatoma, leiomyosarcoma should be considered, because multiple treatment options exist even with large tumors. References 1. Evans HL. Smooth muscle tumors of the gastrointestinal tract: a study of 56 cases followed for a minimum of 10 years. Cancer 1985;56:2242–2250. 2. Wilson S, Braitman H, Plested W, et al. Primary leiomyosarcoma of the liver. Ann Surg 1971;174:232–237. 3. Dileo P, Randdhawa R, VanSonnenberg E, et al. Safety and efficacy of percutaneous radiofrequency ablation (RFA) in patients with metastatic gastrointestinal stromal tumor (GIST) with cloan evolution of lesions refractory to imatinib mesylate (IM). J Clin Oncol 2004;22:820a.
Conflicts of interest The authors disclose no conflicts. © 2010 by the AGA Institute 1542-3565/10/$36.00 doi:10.1016/j.cgh.2009.09.021 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2010;8:e26
A
78-year-old woman presented with 2 months of pleuritic right upper quadrant abdominal pain associated with a 12pound weight loss. Abdominal exam showed moderate right upper quadrant tenderness, with a palpable liver edge up to 3 inches below the right costal margin; no discrete mass was palpated. Computed tomography scan enhanced triple phase of the liver subsequently demonstrated a 13-cm mass in the right lobe, a 3-cm mass in the posterior and inferior subcapsular region of segment 3 of the left hepatic lobe, and a 2-cm focal lesion in segment 3 of the left hepatic lobe near the fissure of the round ligament (Figure A). Follow-up magnetic resonance image with gadolinium showed the largest lesion in right lobe measuring 13.4 ⫻ 11.1 ⫻ 17.4 cm with heterogeneous enhancement and low peripheral and high central T1 and T2 signal, consistent with necrosis or hemorrhage, impressing on, but not originating from, the inferior vena cava (Figure B). Histologic evaluation stained with hematoxylin-eosin from a liver biopsy showed a spindle cell malignant tumor (Figure C). In addition, this tumor immunohistochemically stained positive for desmin, and there was coexpression of keratin, yet it stained negative for DF31, CD 68, S100, and hepatocellular antigen. The tumor was diagnosed as a high-grade sarcoma consistent with primary liver leiomyosarcoma. The patient was referred to oncology for further medical management because she was determined to be a nonsurgical candidate. Leiomyosarcomas are mesenchymal tumors with their origin in the connective tissue and blood vessels of the affected organ. Intestinal leiomyosarcomas are fairly rare, with a frequency of 1.4 cases per 100,000 patients. They consist of spindle-shaped cells and have a high cellularity. With high-grade tumors, necrosis often is present. Approximately 60% of patients are symptomatic, and of these, 70% present with bleeding followed by weight loss.1 The natural history of this tumor involves local
growth initially, with much of its growth being extraluminal; thus, obstruction occurs late. As the size increases, necrosis and bleeding follow. Growth is indolent and late to metastasize; therefore, aggressive surgical resection is feasible, even with a large primary tumor.2 Other local therapy options for unresectable liver metastases include hepatic arterial embolization, microwave ablation, chemoembolization combined with imatinib, as well as radiofrequency ablation, which has been well established as a viable option for nonsurgical candidates.3 Primary liver leiomyosarcomas are rare and have no definite radiologic criteria for diagnosis; however, in any patient presenting with right upper quadrant pain and weight loss in the setting of a hepatoma, leiomyosarcoma should be considered, because multiple treatment options exist even with large tumors. References 1. Evans HL. Smooth muscle tumors of the gastrointestinal tract: a study of 56 cases followed for a minimum of 10 years. Cancer 1985;56:2242–2250. 2. Wilson S, Braitman H, Plested W, et al. Primary leiomyosarcoma of the liver. Ann Surg 1971;174:232–237. 3. Dileo P, Randdhawa R, VanSonnenberg E, et al. Safety and efficacy of percutaneous radiofrequency ablation (RFA) in patients with metastatic gastrointestinal stromal tumor (GIST) with cloan evolution of lesions refractory to imatinib mesylate (IM). J Clin Oncol 2004;22:820a.
Conflicts of interest The authors disclose no conflicts. © 2010 by the AGA Institute 1542-3565/10/$36.00 doi:10.1016/j.cgh.2009.09.021 CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2010;8:e26