A Rare Case of Primary Hepatic Leiomyosarcoma Presenting as Hemangioma

Abstract

Purpose: Leiomyosarcoma is a rare malignant mesenchymal tumor originating from the smooth musculature. Common sites include uterus and intestines. Primary hepatic leiomyosarcoma (PHL) is extremely rare and probably originates from the smooth muscle layer in the walls of intrahepatic blood vessels and bile ducts. We present a case of PHL initially presenting as hemangioma on CT scan. 48 year old African American female with history of depression, iron deficiency anemia and menorrhagia presented with a 1-month history of right upper quadrant (RUQ) abdominal pain. Ultrasound and subsequent CT scan of the abdomen showed a lobulated mass, measuring 9.7 x 8.2 x 5.6 cm in size, in the right hepatic lobe, occupying segments 5 through 8, with arterial enhancement, which was felt to be mostly consistent with a giant hemangioma. She had no history of liver disease or any risk factors for chronic liver disease. Physical exam was positive only for RUQ tenderness. Her blood work up was significant only for hemoglobin of 7.3 g/dL and ferritin of 4 ng/mL. The patient underwent right hepatic lobectomy since she was symptomatic. The pathology of the liver mass revealed leiomyosarcoma, 11 cm in size and with negative resection margins. It was felt that the primary tumor site might be the uterus since the patient was found to have multiple uterine nodules on pelvic imaging and had a history of severe menorrhagia. She underwent hysterectomy but the pathology of the uterine nodules was consistent with adenomyosis and leiomyoma without features of malignancy. She was started on adjuvant chemotherapy with epirubicin and ifosfamide by Oncology. Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumor (0.1-2% of all primary liver cancers). The patients may be asymptomatic or have abdominal pain, nausea, vomiting, jaundice and weight loss. Commonly these tumors are diagnosed in the 6th decade of life, but recently there has been a trend for diagnosis earlier in life (even in the 3rd decade). PHL usually presents on CT as a large well-defined heterogeneous lesion enhancing internally and peripherally, sometimes with hemorrhage or necrosis. The most important differential diagnosis of PHL is hemangioma - the most common benign liver tumor which is also hypervascular but more homogenous and rarely associated with any symptoms other than RUQ pain. Early diagnosis and treatment of PHL with surgery and chemotherapy is very important as this is an aggressive tumor with poor prognosis. Misdiagnosis and a decision for interval follow up may allow the PHL to grow further, metastasize and become untreatable. Therefore, the possibility of PHL should always be considered in patients presenting with a “giant hemangioma”.