Giant liver hemangioma with adult Kasabach-Merritt syndrome: Case report and literature review

Abstract

Rationale: Adult Kasabach-Merritt syndrome associated with giant liver hemangioma is rare; to date, most reports have been single-case reports, and no multi-case reports or literature reviews are available.

Diagnoses: We conducted a retrospective analysis of 5 cases of adult Kasabach-Merritt syndrome associated with giant liver hemangioma treated at our hospital between 2011 and 2016. All 5 patients had varying severities of leukopenia, anemia, thrombocytopenia, prolonged prothrombin time, and hypofibrinogenemia.

Interventions: All the patients underwent surgery: 2 patients had left hemihepatectomy; 1 had enucleation; 1 had a right hemihepatectomy; and 1 had a left trisectionectomy.

Outcomes: The 5 patients had an average operative time of 6.9 hours and an average blood loss of 3200 mL. One patient developed a biliary fistula (grade II) after the operation. There was no mortality among 5 patients. The white blood cell counts, hemoglobin, platelets, and prothrombin times of all 5 patients returned to normal after the operation. To date, a total of 11 cases of adult Kasabach-Merritt syndrome associated with giant liver hemangioma have been reported, of which 8 patients underwent surgery, and their platelets and coagulation returned to normal after the operation.

Lessons: Adult Kasabach-Merritt syndrome associated with giant liver hemangioma is uncommon, and surgical treatment is risky. However, resection of the tumor corrected the abnormalities in hematological and coagulative systems.

Figure 1

CT images of a patient with Kasabach-Merritt syndrome associated with giant liver hemangioma. (A) Multiple liver hemangiomas, with the larger one in the left liver; (B) compression of the hepatic artery (black arrow); (C) compression of the portal vein (black arrow); (D) the gastric outlet is obviously compressed (black arrow).

Figure 2

A patient with Kasabach-Merritt syndrome associated with an extremely giant liver hemangioma (50 cm). (A–D) CT images showing an extremely giant liver hemangioma, occupying the entire abdominal cavity and extending to the pelvis, with compression of the right hepatic vein (A, white arrow), right portal vein (B, white arrow), and inferior vena cava (C, white arrow); (E) intraoperative exploration revealed an extremely giant liver hemangioma occupying the entire abdominal cavity.