Primary hepatic leiomyosarcoma is exceedingly rare accounting for less than 1% of the hepatic tumors. Close to 45 cases have been reported in the English literature. Presentation is usually nonspecific and diagnosis is often delayed until tumors reach a large size. This leads to a dismal prognosis. The tumors are not yet fully understood, hence the standard of care is not well defined. Curative resection remains the mainstay of management. Close association of Epstein Barr virus (EBV) induced soft tissue sarcomas is proven, especially in the presence of immunosuppression encountered in HIV/AIDS patients and in posttransplant patients. We herein present a case report of a 54-year-old man diagnosed to have HIV/AIDS and EBV infection admitted to our hospital with complaints of intractable hiccups for more than a week. Extensive workup revealed primary leiomyosarcoma of the liver.
Sarcomas are rare mesenchymal tumors and account for 1% of all adult tumors, of which 5–10% are leiomyosarcomas. Hepatic leiomyosarcomas are unusual tumors. Most of them are metastatic leiomyosarcomas. Primary hepatic leiomyosarcoma is a very rare entity. Primary leiomyosarcomas are known to occur in uterus, retroperitoneum, genital organs, major blood vessels of the body, lungs, and liver [
A 54- year- old African American man presented to our hospital with intractable hiccups that started about a week prior to admission. He also reported intermittent low grade fever with poor oral intake and weight loss of 9 kilograms for the last 3 months. He denied abdominal pain, nausea, vomiting, or irregular bowel habits. His comorbidities included HIV/AIDS, seizure disorder, and hypertension. His CD4 count was 24 at the time of admission. He had no history of liver disease or alcohol abuse. He never had any surgery. Family history was negative for any malignancies or liver disease. On physical examination he appeared cachectic. He had no palpable lymph nodes. Abdominal examination revealed minimal tenderness in the right upper quadrant and hepatomegaly of about 6 cm below the right costal margin. Laboratory analysis revealed alanine aminotransferase 81 IU/L (0–34 IU/L), aspartate aminotransferase 56 IU/L (0–40 IU/L), alkaline phosphatase 160 IU/L (28–94 IU/L), serum albumin 2.8 g/dL (3.4–5 g/dL). Serum bilirubin, prothrombin time, white blood cells, and platelets were normal. Septic workup including blood and urine cultures and chest radiograph were normal. Abdominal computed tomography (CT) of the chest and abdomen showed a hypodense rim enhancing lesion about 3.5 cm by 2.5 cm in size with a necrotic center within segment VII (Figure
Computer tomography scan with contrast medium showing the liver mass with in the Segment VII of the right lobe with peripheral rim enhancement with central hypodensity.
CT chest showing the pulmonary nodules.
Histopathology of the hepatic mass. (a) Spindle shaped cells distributed in a collagenous stroma. (b) Spindle cells proliferation in interlacing pattern.
Immunohistochemical staining of the hepatic tumor. (a) Cytoplasm of the spindle shaped tumor cells stain positive for smooth muscle actin. (b) Neoplastic cells stain positive with nuclear in situ hybridization for EBV.
Sarcomas constitute about 1-2% of malignant tumors of the liver [
Mean age of presentation as identified from various case reports is 58 years [
The findings of hepatic leiomyosarcoma on CT scan and MRI are non-specific. These tumors are found to be hypervascular on imaging. CT may reveal a large, well-defined, heterogeneous hypodense mass with internal and peripheral enhancement or cystic mass with an enhancing thick wall [
Association of EBV infection and increased incidence of leiomyosarcoma in immunosuppressed patients such as HIV/AIDS and posttransplant is clearly documented and well researched in the literature. In situ hybridization studies of leiomyoma and leiomyosarcoma in HIV patients demonstrate EBV in the tumor cell, but not the adjoining normal cells. In AIDS patients the EBV receptor (CD21/C3d) may be upregulated allowing EBV to enter into muscles cells, contributing to malignant transformation. Similar pathogenesis of EBV induced leiomyosarcoma in iatrogenic immunosuppressed patients, especially in post-transplant patients, has been reported. There are isolated reports of decreased incidence of recurrence of leiomyosarcoma in EBV infected patients, in whom immunosuppression is decreased after transplantation.
If liver lesion appears malignant and deemed to be surgically resectable, there is no need for preoperative biopsy. In our case there was concern for abscess and hence CT guided aspiration was performed. Biopsy will reveal intersecting bundles of spindle cells with immunoreactivity to desmin, actin, and SMA but negative for keratin, S-100 protein, and neuron-specific enolase [
Therapeutic interventions include surgical resection, systemic chemotherapy, and liver transplantation. Best treatment modality is surgical resection with tumor-free margins which has good long term survival [
Patterns of sites of recurrence of these tumors are similar to other sarcomas. Hepatic leiomyosarcoma tends to metastasize hematogenously to the lung, but lymphatic metastasis and peritoneal seeding have been described. Adjuvant chemoradiotherapy had been recommended for eradication of microscopic residual disease. However the success of this therapy in achieving local control and prolonged patient survival needs to be further studied although a meta-analysis has shown a small benefit [
The role of liver transplantation remains controversial. In the study by Husted et al., six patients with primary hepatic angiosarcoma and thirteen patients with metastatic sarcoma underwent liver transplantation and the 1-, 3-, and 5-year survival was 47%, 15%, and 5%, respectively. Recurrence rate was 95% after a median interval of 6 months. Given the high tumor recurrence rate and poor survival, Husted et al. discouraged liver transplantation for the management of primary or metastatic sarcoma of the liver [
High index of suspicion and prompt investigation will help to diagnose primary hepatic leiomyosarcoma at an earlier stage. Surgical resection without any residual tumor has clearly been demonstrated for prolonging patient’s survival. Use of neoadjuvant, adjuvant chemoradiotherapy, and hepatic artery chemoembolization needs to be further evaluated.