Cadaveric Liver Transplantation for a Giant Mass

Abstract

Question: A 44-year-old woman was admitted to our institution complaining of abdominal fullness, evident shortness of breath, weight loss, anorexia, petechial hemorrhages, ecchymosis, and swelling of feet and ankles that have been present for 3 years. There was no significant property at her medical history besides the absence of family history for hemangioma, and she was not taking any medications. At physical examination, increased abdominal circumference was noticed, and the edge of the liver was palpated well at right lower quadrant. Laboratory tests revealed abnormal hepatic functions, albumin (3.3 g/dL; control, 3.5–5.2), prolonged prothrombin time (20 s; control, 10–15.8), prolonged International Normalized Ratio (1.92; control, 0.9–1.3), and borderline low platelet count (119/mm3), which were compatible with Kasabach–Merritt syndrome. On coronal abdominal computed tomography and 3-dimensional volume-rendered imaging, a 22 × 18 × 23-cm mass in the right hepatic lobe which nearly replaced the right hepatic lobe was showed. The right diaphragm was elevated, and there was a mass effect on right atrium, and right ventricle. The inferior vena cava, main and left portal veins, and gastric outlet were compressed by the lesion, and then truncus coliacus was deviated to the left. Right kidney displaced inferomedially, and rotated 90° around its long axis (Figure A, B). What is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The dynamic hepatic computed tomography of the patient revealed that the mass had peripheral nodular centripetal enhancement with persistent nonenhancement areas in the central part of the mass might be secondary to fibrous scar (Figure C). She was considered to have cadaveric liver transplantation because of respiratory distress, risk of bleeding owing to trauma, and the presence of Kasabach–Merritt syndrome. The patient underwent transplantation, and the removed liver weighed 4.8 kg (dry weight). Pathologic examination of the surgical specimen affirmed the diagnosis of cavernous hemangioma with peripheral sponge-like matrix full of blood products, and central fibrous scar tissue (Figure D). Her clinical status gradually improved posttransplantation and platelet count (193/mm3), prothrombin time (13 s), International Normalized Ratio (1.2), and albumin (3.8 g/dL) levels all returned to normal the 1-month follow-up. Giant cavernous hemangiomas are a minor atypical subgroup of hepatic hemangiomas, which are defined as ≥1 diameter >5 cm.1Vilgrain V. Boulos L. Vullierme M.P. et al.Imaging of atypical hemangiomas of the liver with pathologic correlation.RadioGraphics. 2000; 20: 379-397Crossref PubMed Scopus (331) Google Scholar Giant cavernous hemangiomas are symptomatic with their complications, which are Kasabach–Merritt syndrome (consumptive coagulopathy with intravascular coagulation, clotting, and fibrinolysis within the hemangioma), compression of adjacent organs, inflammatory changes, and potential volvulus of a pedunculated lesion.2Prasanna P.M. Fredericks S.E. Winn S.S. et al.Best cases from the AFIP: giant cavernous hemangioma.RadioGraphics. 2010; 30: 1139-1144Crossref PubMed Scopus (14) Google Scholar The pathognomonic imaging characteristics of hemangioma are peripheral nodular puddling with progressive centripetal enhancement on dynamic studies.1Vilgrain V. Boulos L. Vullierme M.P. et al.Imaging of atypical hemangiomas of the liver with pathologic correlation.RadioGraphics. 2000; 20: 379-397Crossref PubMed Scopus (331) Google Scholar When hemangiomas are >3 cm, central scarring is typically seen in lesions, owing to intralesional ischemia or hemorrhage.2Prasanna P.M. Fredericks S.E. Winn S.S. et al.Best cases from the AFIP: giant cavernous hemangioma.RadioGraphics. 2010; 30: 1139-1144Crossref PubMed Scopus (14) Google Scholar Management of very large (>10 cm) hepatic hemangiomas include surgical resection, enucleation, arterial embolization, hepatic irradiation, and liver transplantation.3Sood D. Mohan N. Singh A. et al.Living donor liver transplantation for giant cavernous hemangioma of liver in a child.Pediatr Transplant. 2011; 15: E135-138Crossref PubMed Scopus (4) Google Scholar