Teaching Case

A 37-year-old female with no previous comorbidities presented with epigastric pain since 1 month. The pain was intermittent in nature and dull aching in character with no significant radiation of pain. On abdominal examination, there was an ill-defined mass in epigastrium which was non-tender on palpation.

Plain axial CT section of abdomen revealed a well-defined soft tissue density lesion with hyperdense areas within involving left lobe of liver (Fig 1). Coronal contrast-enhanced images of abdomen revealed peripheral enhancement in arterial phase (Fig 2) with progressive filling in venous phase ( Fig 3) and an ill-defined posterior border with an adjacent fluid collection posteriorly suggestive of intraperitoneal rupture (Fig 4). The patient then underwent transarterial chemo-embolisation using Bleomycin and digital subtraction image of the procedure showed peripheral accumulation of lipiodol within the lesion (Fig 5). Follow up plain CT image showed accumulation of lipiodol within the lesion as evidenced by presence of peripheral hyperdense areas of average HU 2000 (Fig 6). Ultrasound 2 months later showed significant decrease in size of the lesion with large central hypoechoic area with uniform internal echoes within (Fig 7).

Haemangioma is the most common benign hepatic tumour. Typical haemangiomas (also called capillary haemangioma) consist of clusters of blood-filled cavities, lined by endothelial cells, which are fed by hepatic artery. They range in size from few mm to up to 40 cm with giant lesions defined as those with size more than 4 cm in diameter[1].  Commonly seen in middle-aged females between 30- 50 years (Female-male ratio = 5:1), typical location is segment IV of left lobe [2].

They classically appear as a well defined focal, homogeneous, hypovascular, echogenic lesions on ultrasound with giant lesions showing heterogenity. The typical CT finding is a well-circumscribed, round or lobular, hypoattenuating lesion, with enhancement in arterial phase similar to that of aorta and hepatic arteries and characterised by presence of a homogenous blood pool density [3]. Central scarring may be seen in giant lesions [4]. They need to be differentiated from hypervascular lesions prone to bleeding like hepatic adenomas which are non-lobulated and hypervascular metastasis which may be multiple, with heterogeneity due to areas of fat and haemorrhage being more common in the former [5]. In the setting of cirrhosis, hepatocellular carcinoma ( HCC) needs to be considered where washout in delayed phases favours HCC over hemangioma [6]. 

Complications are far more common in larger lesions with complication rate varying from 4.5% to 19.7% [7]. Alterations in internal architecture such as coagulation and inflammation, Kasabach-Merritt syndrome, intratumoural haemorrhage and spontaneus rupture with hemoperitoneum are common complications. Trauma needs to be ruled out in all cases before presuming the cause as spontaneous. Intra peritoneal rupture as in this case, though extremely rare, can present with severe symptoms such as severe epigastric pain, vomiting, anaemia, disseminated intravascular coagulation, with haemodynamic instability and signs of hypovolemic shock seen in one-third cases [8]. Anticoagulant therapy, pregnancy, and subcapsular location are risk factors for rupture with imaging findings being hemoperitoneum and intraperitoneal clots near the site of bleed[8]. 

Surgical resection and enucleation is the gold standard of treatment, the drawback being high operative mortality. Preoperative transarterial embolisation ( TAE) using various materials like gel foam, polyvinyl alcohol (PVA) particles, steel coils, as well as isobutyl cyanoacrylate have been tried resulting in reduced intra-op blood loss [9] TAE using bleomycin lipiodol combination ( TACE ) as a replacement for surgery has been proposed with better outcomes than traditional surgery / TAE with post-procedural complications being parenchymal injury and infarction, access site injury, bile duct injury, liver abscess, and post-embolization syndrome [10].

Hemangiomas even though benign, may present unusually in giant cavernous form with complications like intraperitoneal rupture as in this case which can be better managed by procedures like TACE.

Written informed patient consent for publication has been obtained.