Lymphangiomas are benign neoplasms regarded as congenital malformations of the lymphatic system (Stavropoulos et al. 1994). Most lymphangiomas are located in the head, neck, and axilla, where the loose connective tissue allows for easy expansion of lymphatic channels. Intraabdominal cases account for less than 5% of all lymphangiomas (Losanoff et al. 2003). Hepatic Lymphangiomas are characterized by cystic dilatation of the lymphatic vessels in the hepatic parenchyma usually observed in children and adolescents (Bertino et al. 2014; Asch et al. 1974; Zhang et al. 2013). In most cases, the hepatic lesion is usually part of multi-organ involvement including the spleen, kidney, skeleton, gastrointestinal tract, mesentery, lung, pleura, pericardium and other tissues (Stavropoulos et al. 1994; Koh & Sheu 2000). A solitary hepatic lymphangioma in an adult is extremely rare and lack of specific clinical symptoms, therefore, it is easy to be misdiagnosed. The aim of this report is to introducing the experience in managing an adult with a giant liver cystic lymphangioma.
Case report
A 41-year-old male with a 6-month history of left upper abdominal pain visited our department. A large, palpable soft mass 8 cm below the left costal margin was found.
Hematology and biochemistry results were normal while Hepatitis B surface antibody (HBsAb) and Hepatitis B core antibody (HBcAb) were positive. Results of kidney function, electrolytes, and all tumor markers related to the liver were negative. Brain computed tomography (CT) scan and chest X-ray did not find other lesions in the patient.
Abdominal ultrasound (US) showed a giant mixed-echoic mass (15 cm × 10 cm) with a pedunculatedextrahepatic growth pattern. An enhanced abdominal CT scan confirmed a giant cystic hepatic lesion with no capsule. The lesion was demonstrated with a heterogeneous enhancement in the arterial phase (Figure 1). The laboratory and image findings were not sufficient to differentiate the benign or malignant nature of the mass.
At laparotomy, a huge, smooth tumor was found in segment II with pedunculated extrahepatic growth, with no other pathology found in the abdomen. The tumor was completely removed bylocal resectionof segment II of the liver using an ultrasonic scalpel and cavitron ultrasonic surgical aspirator after occlusion of the left hemihepatic vascular.
Histology of the resected specimen revealed a giant cystic mass about 16.2 cm × 13 cm × 4.3cmm in size. On macroscopic examination, the tumor was cystic and multilocular. The cyst had a thick, gray-white wall and was filled with serous fluid containing a small amount of blood. Microscopically, the tumor was composed of irregular and expanded lymphatic vessels, lined by flattened endothelial cells (Figure 2). Immunohistochemical study was carried out on formalin-fixed, paraffin-embedded tissues. The endothelial-like cells were almost all positive for CK18, and pCEA, while Hep-1, HBsAg, CK19, CD34, HBcAg, β-catenin, MUC-1, MAT1, GLy-3, and KIAA were all negative. The final histopathologic diagnosis for the specimen was solitary hepatic lymphangioma.
The postoperative course was uneventful and the patient has been followed up for 30 months. Now, he is symptom-free with no evidence of recurrence on subsequent abdominal imaging.