Hepatic angiosarcoma five years following spontaneous intraperitoneal bleed of a hepatic mass

Abstract

Primary hepatic angiosarcoma is a rare and rapidly fatal disease. We present the highly unusual identification of this lesion five years after the initial clinical presentation.In 2003, a 32-year-old man presented with abdominal pain, tachycardia, and evidence of hemorrhage. A CT scan showed a hepatic mass with intralesional hemorrhage, intraperitoneal blood, and splenomegaly. The patient was stabilized clinically. Laparoscopic core biopsies demonstrated no malignancy, only findings consistent with an old hemorrhage. Contralateral lobe biopsies revealed normal liver tissue. A metastatic workup was negative and the decision was made to observe the patient clinically with radiographic follow-up, given his suspected portal hypertension based on thrombocytopenia and splenomegaly.Sequential imaging demonstrated a decrease in the size of the mass from 12.0 cm in 2003 to 3.0 cm in 2007. Subsequent newly identified esophageal varices prompted a reevaluation of the case. A repeat biopsy demonstrated a neoplasm of vascular etiology and uncertain malignant potential. By early 2008 the lesion had increased to 4.8 cm and was resected via a left hepatic lobectomy. An extremely vascular lesion with surrounding dense fibrosis was identified and pathologic examination demonstrated a high-grade angiosarcoma.We are unaware of any previous reports suggesting such a prolonged natural history of hepatic angiosarcoma. This case may represent the possibility of malignant transformation of a lower grade vascular neoplasm such as hepatic epithelioid hemangioendothelioma to an angiosarcoma.

Hepatic Angiosarcoma: A Challenging Diagnosis

“…Radiologically, HAs vary in their appearance. Grossly, the tumor may appear with one of four different growth patterns: multinodular, a single dominant mass, mixed patterns of a dominant mass with smaller nodules, or an infiltrating micronodular tumor [ 2 ]. Metastases are common at the time of presentation, with the lungs, spleen, or bone being the most frequent secondary lesion sites.…”

“…Though historically, HA has been linked with environmental toxins, including thorotrast, arsenic, radiation, vinyl chloride, anabolic steroids, and exogenous estrogens, most cases of HA never yield a causative factor. It should be noted that there is no difference in gross or microscopic pathology between the toxin-related HAs with their 20- to 30-year latency periods and idiopathic HAs [ 2 ]. On pathology, HAs range in appearance from epithelioid to spindled neoplasms, demonstrating various patterns of vascular channels [ 6 ].…”

“…Treatment for HA has yielded generally poor outcomes due to the late stage and profound metastasis at diagnosis. Better prognosis is seen in patients that present with a single tumor mass, small tumor size, lack of metastases, low-grade lesion, and negative surgical resection margins [ 2 ]. For those without metastasis, surgical resection is the definitive treatment [ 3 ].…”

“…Demographically, the malignancy has a male to female predominance of 3:1 with the majority of patients diagnosed in their sixth decade of life [ 1 ]. Though HA has been historically linked to environmental toxins, including thorotrast, arsenic, radiation, vinyl chloride, anabolic steroids, and exogenous estrogens, most cases of HA do not yield a causative factor [ 2 ]. In this report, we describe the case of a previously healthy male whose initial presentation of dyspnea lead to the diagnosis of HA.…”