S5852 To Transplant or Not to Transplant: A Case of Epithelioid Hemangioendothelioma

Introduction: Gastrointestinal malignancies encompass different cancers within the digestive tract/related organs and therapies are tailored accordingly. In some instances, liver transplant is a viable option for treatment. One malignancy in particular known as a rare vascular sarcoma called epithelioid hemangioendothelioma (EHE). This vascular tumor arises from the lining of blood vessels and occurs in various tissues and organs. In the liver, it can present as multifocal lesions mimicking metastatic disease or sometimes be misdiagnosed as cirrhosis. Usually, the findings are incidental on imaging. Due to the unpredictability of EHE, patients can experience indolent disease progression, while others suffer from aggressive metastasis or complications. We present a patient with EHE who underwent liver transplant. Case Description/Methods: A 72-year-old man with presumed cryptogenic cirrhosis, esophageal varices, and lung nodules presented for further evaluation of liver disease. He was found to have abnormal liver biochemistries on routine labs. Comprehensive liver workup was unremarkable. US showed a lobulated liver with multiple areas of heterogeneity. Subsequent magnetic resonance imaging (MRI) showed multiple hepatic masses concerning for malignancy. Recent bidirectional endoscopy reported small esophageal varices and a hyperplastic colon polyp. PET-CT showed innumerable hypermetabolic hypoattenuating hepatic masses concerning for malignancy and multiple pulmonary nodules measuring up to 7 mm. Recommendation was for liver biopsy to establish tissue diagnosis and discuss management at multidisciplinary tumor board. Biopsy showed EHE. Prior MRI was reviewed again with radiology which demonstrated a confluent process with well-defined MRI characteristics of EHE consistent with biopsy results. Standard treatment was for liver transplant due to risks of proliferation of disease with other treatment options. Additional staging with CT chest showed stable small lung nodules. The transplant committee listed the patient as active for transplant. He underwent an uncomplicated deceased donor transplant 12 months later. One-year post-transplant, a surveillance CT showed new and enlarging pulmonary nodules. He underwent wedge resection. Final pathology showed metastatic EHE. Discussion: Liver transplant remains the primary treatment for localized unresectable EHE. The unpredictable clinical course and variable prognosis underscores the importance of further prospective analysis on EHE and liver transplant.