A 65-year-old woman with past medical history of hypothyroidism presented to the outpatient clinic with progressive abdominal distension of 6 year’s duration. On further questioning, the patient reported progressive early satiety over the same period of time, in addition to fatigue. She reported no other symptoms such as abdominal pain, changes in bowel habits, or weight loss.
Assessment
The patient was afebrile and her vital signs were stable. Physical examination revealed markedly distended abdomen with dullness to percussion and dilated superficial abdominal veins. Laboratory evaluation was significant for normocytic anemia, with hemoglobin of 7.6 g/dL (12-15.5 g/dL), thrombocytopenia with platelet count of 117 × 109/L (150-450 × 109/L), hypofibrinogenemia with fibrinogen level of 112 mg/dL (200-393 mg/dL), and elevated D-dimer at 35,846 ng/mL (<500 ng/mL). Imaging with multiphase abdomen and pelvis computed tomography showed a massive liver mass with progressive enhancement (Figure 1, Figure 2).
Diagnosis
The findings on cross-sectional imaging with the size of the mass and the progressive enhancement, with initial peripheral enhancement followed by subsequent central enhancement, were consistent with a giant liver hemangioma. The patient was diagnosed with Kasabach-Merritt syndrome due to the presence of giant liver hemangioma in combination with thrombocytopenia and consumptive coagulopathy.
Management
The patient was evaluated by surgery, however, surgical excision was not recommended due to the high risk of complications. Hepatic artery embolization was considered, however, it was thought that significant involution is unlikely to occur given the size of the hemangioma. Further treatment options including systemic bevacizumab and liver transplant were offered but the patient declined. The patient was lost to follow-up.
Discussion
Liver hemangiomas (also known as cavernous hemangiomas) are the most common benign mesenchymal liver tumors.1 The majority of patients are asymptomatic, and hemangiomas occur most commonly in females.1 In general, those larger than 5 cm have been referred to as giant liver hemangiomas and are more likely to cause symptoms.2 The Kasabach-Merritt syndrome refers to the combination of giant liver hemangioma, thrombocytopenia, and consumptive coagulopathy, and can be seen in adults with large liver hemangiomas, as in this case.3 Asymptomatic patients with small hemangiomas can be managed conservatively, while surgical resection should be considered for symptomatic patients and those with giant hemangiomas.4
To read this article in its entirety please visit our website.
-Hasan Ahmad Hasan Albitar, MDa,, Vivek Iyer, MD, MPHb
