Anne J Klompenhouwer1, Roy S Dwarkasing2, Michael Doukas3, Silvia Pellegrino4, Valerie Vilgrain4, Valerie Paradis5, Olivier Soubrane6, Joal D Beane7, David A Geller7, Michael A Nalesnik8, Verena Tripke9, Hauke Lang9, Moritz Schmelzle10, Johann Pratschke10, Wenzel Schöning10, Eliza Beal11, Steven Sun11, Timothy M Pawlik11, Robert A de Man12, Jan N M Ijzermans13. 1. Department of Surgery, Erasmus MC University Medical Center, Rotterdam, the Netherlands. 2. Department of Radiology, Erasmus MC University Medical Center, Rotterdam, the Netherlands. 3. Department of Pathology, Erasmus MC University Medical Center, Rotterdam, the Netherlands. 4. Department of Radiology, Hôpitaux Universitaires Paris Nord Val de Seine, Beaujon, APHP, Clichy, France. 5. Department of Pathology, Hôpitaux Universitaires Paris Nord Val de Seine, Beaujon, APHP, Clichy, France. 6. Department of Surgery, Hôpitaux Universitaires Paris Nord Val de Seine, Beaujon, APHP, Clichy, France. 7. Department of Surgery, Division of Hepatobiliary and Pancreatic Surgery, University of Pittsburgh Medical Center Pittsburgh, USA. 8. Department of Pathology, University of Pittsburgh Medical Center Pittsburgh, USA. 9. Department of General, Visceral and Transplant Surgery, University Hospital of Mainz, Germany. 10. Department of Surgery, Campus Virchow-Klinikum and Campus Mitte, Charité-Universitätsmedizin Berlin, Germany. 11. Department of Surgery, Wexner Medical Center, The Ohio State University, OH, USA. 12. Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center, Rotterdam, the Netherlands. 13. Department of Surgery, Erasmus MC University Medical Center, Rotterdam, the Netherlands. Electronic address: j.ijzermans@erasmusmc.nl.
Abstract
BACKGROUND: Hepatic angiomyolipoma (HAML) may easily be misdiagnosed as a malignancy. The study aim was to assess diagnostic dilemmas, clinical management and outcome of this rare tumor. METHODS: This retrospective international multicenter study included all patients with pathologically proven HAML diagnosed between 1997 and 2017. Data on patient characteristics, diagnostic work-up, management and follow-up were analyzed. RESULTS: Thirty-eight patients were included, 32 female. Median age was 56yrs (i.q.r. 43-64) and median HAML-diameter was 57.5 mm (i.q.r. 38.5-95.3). Thirty patients had undergone CT and 27/38 MRI of the liver, diagnostic biopsy was performed in 19/38. Initial diagnosis was incorrect in 15/38 patients, of which 13 were thought to have malignancy. In 84% biopsy resulted in a correct preoperative diagnosis. Twenty-nine patients were managed with surgical resection, 4/38 with surveillance and 3/38 with liver transplantation. Recurrence after resection occurred in two cases. No HAML related deaths or progression to malignancy were documented. CONCLUSION: HAML diagnosis proved problematic even in hepatobiliary expertise centers. Biopsy is indicated and may provide valuable additional information when HAML diagnosis is considered on cross-sectional imaging, especially when surgical resection imposes a risk of complications. Conservative management with regular imaging follow-up might be justified when biopsy confirms (classic type) HAML.
BACKGROUND:Hepatic angiomyolipoma (HAML) may easily be misdiagnosed as a malignancy. The study aim was to assess diagnostic dilemmas, clinical management and outcome of this rare tumor. METHODS: This retrospective international multicenter study included all patients with pathologically proven HAML diagnosed between 1997 and 2017. Data on patient characteristics, diagnostic work-up, management and follow-up were analyzed. RESULTS: Thirty-eight patients were included, 32 female. Median age was 56yrs (i.q.r. 43-64) and median HAML-diameter was 57.5 mm (i.q.r. 38.5-95.3). Thirty patients had undergone CT and 27/38 MRI of the liver, diagnostic biopsy was performed in 19/38. Initial diagnosis was incorrect in 15/38 patients, of which 13 were thought to have malignancy. In 84% biopsy resulted in a correct preoperative diagnosis. Twenty-nine patients were managed with surgical resection, 4/38 with surveillance and 3/38 with liver transplantation. Recurrence after resection occurred in two cases. No HAML related deaths or progression to malignancy were documented. CONCLUSION:HAML diagnosis proved problematic even in hepatobiliary expertise centers. Biopsy is indicated and may provide valuable additional information when HAML diagnosis is considered on cross-sectional imaging, especially when surgical resection imposes a risk of complications. Conservative management with regular imaging follow-up might be justified when biopsy confirms (classic type) HAML.
Authors: Cong Long Nguyen; Ham Hoi Nguyen; Tuan Hiep Luong; Nghe Tinh Nguyen; Van Khang Le; Truong Khanh Vu Journal: Int J Surg Case Rep Date: 2022-04-18
Authors: Paul Calame; Gaëlle Tyrode; Delphine Weil Verhoeven; Sophie Félix; Anne Julia Klompenhouwer; Vincent Di Martino; Eric Delabrousse; Thierry Thévenot Journal: World J Gastroenterol Date: 2021-05-21 Impact factor: 5.742