Letters to the Editor - ISCIII
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Letters to the Editor 1130-0108/2014/106/7/494-496 REVISTA ESPAÑOLA DE ENFERMEDADES DIGESTIVAS COPYRIGHT © 2014 ARÁN EDICIONES, S. L. REV ESP ENFERM DIG (Madrid Vol. 106, N.º 7, pp. 494-496, 2014 Hepatic adenomatosis: A rare cause of liver transplant Key words: Hepatic adenomatosis. Liver transplant. Dear Editor, Hepatocellular adenoma is the most common benign epi- thelial liver tumour, with an estimated average incidence of 3 cases/million people/year. It was first described by Edmond- son in 1958 (1,2). Hepatic adenomatosis (HA) was described by Flejou et al. in 1985, who reported 13 patients with multiple hepatocellular adenomas. The diagnostic features were: a) Presence of multi- ple hepatic nodules; b) similar distribution in both sexes; c) no confirmed association with prolonged oral contraceptive intake; d) presence of elevated serum alkaline phosphatase and GGT. Fewer than 4 % of all HA cases require orthotopic liver trans- plantation (OLT) (3). Case report A female, 31 years-old, with a long history of oral contra- ceptive use, presented in the emergency room with acute ab- dominal pain and dyspepsia. Physical examination revealed moderate hepatomegaly. She presented moderate anaemia and liver function tests were normal. An abdominal CT identified a large liver mass measuring 17 x 16 cm, with a heterogeneous appearance and signs of bleeding, located in the right hepatic lobe (Fig. 1A). A fine needle aspiration was performed, which revealed no evidence of malignancy. The patient underwent a right hepatectomy. The removed piece of liver measured 22 x 18 x 14 cm and weighed 2,300 g. Gross multiple nodular lesions with intratu- moral haemorrhagic areas were observed (Fig. 1B). Microscop- ic examination revealed hepatocytes with foamy cytoplasm, with round nuclei and multiple uniform adenomatous nodules with varying degrees of steatosis (Fig. 1C). Taken together, the clinico-pathological findings were consistent with an HA with extensive involvement of the right hepatic lobe. Two years later, two new small lesions were observed in the left hepatic lobe. These were also resected (Fig. 1D). After four asymptomatic years, the patient presented again with a clinical picture of acute abdominal pain. Abdominal CT revealed two large liver tumour lesions (Fig. 1E). Finally, the patient underwent an OLT (Fig. 1F). Three years later, she con- tinues to be healthy and disease-free. Discussion The customary treatment of HA with localised lesions in one lobe is partial surgical resection by segmentectomy, hemi-hepa- tectomy or extended lobectomy (4-6). Patients with multifocal lesions should be periodically mon- itored by serial measurements of serum alpha-fetoprotein and the periodic use of imaging techniques, principally magnetic resonance imaging, carried out at least, annually. Disease progression is confirmed by the presence of large subcapsular adenomas (> 4 cm). The risk of malignancy and the presence of persistent symptoms are indications for performing partial resections of multifocal lesions. Partial resection is the preferred option, unless this is technically impossible (7,8). Liver transplantation is the last therapeutic option. The in- dications for carrying this out include continuously elevated serum AFP, suspicion of malignancy from imaging techniques and/or PAF or BAF positivity in highly symptomatic patients, or those with marked hepatomegaly, or a history of repeated complications of adenomas, as occurred in the case described here (9,10).
Transcript of Letters to the Editor - ISCIII
Letters to the Editor
1130-0108/2014/106/7/494-496Revista española de enfeRmedades digestivasCopyRight © 2014 aRán ediCiones, s. l.
Rev esp enfeRm dig (MadridVol. 106, N.º 7, pp. 494-496, 2014
Hepatic adenomatosis: A rare cause of liver transplant
Key words: Hepatic adenomatosis. Liver transplant.
Dear Editor,
Hepatocellular adenoma is the most common benign epi-thelial liver tumour, with an estimated average incidence of 3 cases/million people/year. It was first described by Edmond-son in 1958 (1,2).
Hepatic adenomatosis (HA) was described by Flejou et al. in 1985, who reported 13 patients with multiple hepatocellular adenomas. The diagnostic features were: a) Presence of multi-ple hepatic nodules; b) similar distribution in both sexes; c) no confirmed association with prolonged oral contraceptive intake; d) presence of elevated serum alkaline phosphatase and GGT. Fewer than 4 % of all HA cases require orthotopic liver trans-plantation (OLT) (3).
Case report
A female, 31 years-old, with a long history of oral contra-ceptive use, presented in the emergency room with acute ab-dominal pain and dyspepsia. Physical examination revealed moderate hepatomegaly. She presented moderate anaemia and liver function tests were normal. An abdominal CT identified a large liver mass measuring 17 x 16 cm, with a heterogeneous appearance and signs of bleeding, located in the right hepatic lobe (Fig. 1A). A fine needle aspiration was performed, which
revealed no evidence of malignancy. The patient underwent a right hepatectomy.
The removed piece of liver measured 22 x 18 x 14 cm and weighed 2,300 g. Gross multiple nodular lesions with intratu-moral haemorrhagic areas were observed (Fig. 1B). Microscop-ic examination revealed hepatocytes with foamy cytoplasm, with round nuclei and multiple uniform adenomatous nodules with varying degrees of steatosis (Fig. 1C). Taken together, the clinico-pathological findings were consistent with an HA with extensive involvement of the right hepatic lobe. Two years later, two new small lesions were observed in the left hepatic lobe. These were also resected (Fig. 1D).
After four asymptomatic years, the patient presented again with a clinical picture of acute abdominal pain. Abdominal CT revealed two large liver tumour lesions (Fig. 1E). Finally, the patient underwent an OLT (Fig. 1F). Three years later, she con-tinues to be healthy and disease-free.
Discussion
The customary treatment of HA with localised lesions in one lobe is partial surgical resection by segmentectomy, hemi-hepa-tectomy or extended lobectomy (4-6).
Patients with multifocal lesions should be periodically mon-itored by serial measurements of serum alpha-fetoprotein and the periodic use of imaging techniques, principally magnetic resonance imaging, carried out at least, annually.
Disease progression is confirmed by the presence of large subcapsular adenomas (> 4 cm). The risk of malignancy and the presence of persistent symptoms are indications for performing partial resections of multifocal lesions. Partial resection is the preferred option, unless this is technically impossible (7,8).
Liver transplantation is the last therapeutic option. The in-dications for carrying this out include continuously elevated serum AFP, suspicion of malignancy from imaging techniques and/or PAF or BAF positivity in highly symptomatic patients, or those with marked hepatomegaly, or a history of repeated complications of adenomas, as occurred in the case described here (9,10).
Vol. 106, N.º 7, 2014 LETTERS TO THE EDITOR 495
Rev esp enfeRm Dig 2014; 106 (7): 494-496
Fig. 1. CT and pathological studies. A. Contrast-enhanced CT at the onset of the disease, showing a huge mass (white arrow) in the right hepatic lobe. Zones are observed with intratumoral bleeding (white arrowheads). B. Right hepatectomy. Several nodules yellowish-brown in colour, well defined and different sizes and shapes (black arrows). Presence of intratumoral bleeding (arrowhead). C. Microscopy of the liver corresponding to the right hepatic lobe resection. To the left is the normal parenchyma adjacent to the tumour, showing no remarkable changes (black asterisk). To the right, there is a well-defined area corresponding to one of the hepatic tumours. Steatostic changes occur with preserved architecture (white asterisk). Methenamine silver stain, 100x. D. Contrast-enhanced CT prior to the two lumpectomies. Presence of two isodense subcapsular nodular lesions (white arrows). E. Contrast-enhanced CT prior to OLT. Liver enlarged, occupying most of the abdominal cavity. Presence of two large liver tumour lesions (white arrows) with areas of intratumoral haemorrhage (white arrowhead). F. Post-contrast CT of OLT. Presence of post-surgical changes.
A B
C D
E F
496 LETTERS TO THE EDITOR Rev esp enfeRm Dig (maDRiD)
Rev esp enfeRm Dig 2014; 106 (7): 494-496
4. Vetelainen R, Erdogan D, de Graaf W, ten Kate F, Jansen PL, Gouma DJ, et al. Liver adenomatosis: Re-evaluation of aetiology and manage-ment. Liver Int 2008;28:499-508.
5. Hagiwara S, Takagi H, Kanda D, Sohara N, Kakizaki S, Katakai K, et al. Hepatic adenomatosis associated with hormone replacement therapy and hemosiderosis: A case report. World J Gastroenterol 2006;12:652-5.
6. Adam R, Karam V, Delvart V, O’Grady J, Mirza D, Klempnauer J, et al. Evolution of indications and results of liver transplantation in Europe. A report from the European Liver Transplant Registry (ELTR). J Hepatol 2012;57:675-88.
7. Ribeiro A, Burgart LJ, Nagorney DM, Gores GJ. Management of liver adenomatosis: Results with a conservative surgical approach. Liver Transpl Surg 1998;4:388-98.
8. Bambha K, Nagorney D, Sanderson S, Gores GJ. Hepatic adenoma-tosis in a young woman with glucose intolerance. Nat Clin Pract Gas-troenterol Hepatol 2006;3:526-31; quiz (following 31).
9. Foster JH, Berman MM. The malignant transformation of liver cell adenomas. Arch Surg 1994;129:712-7.
10. Chiche L, Dao T, Salame E, Galais MP, Bouvard N, Schmutz G, et al. Liver adenomatosis: reappraisal, diagnosis, and surgical management: Eight new cases and review of the literature. Ann Surg 2000;231:74-81.
Iván Fernández-Vega1, Jorge Santos-Juanes1, Carmen García-Pravia1, Manuel F. Fresno-Forcelledo1
and Luis Rodrigo2
1Pathology Department and 2Gastroenterology Service. Hospital Universitario Central de Asturias. Oviedo, Spain
References
1. Rooks JB, Ory HW, Ishak KG, Strauss LT, Greenspan JR, Hill AP, et al. Epidemiology of hepatocellular adenoma. The role of oral contracep-tive use. JAMA 1979;242:644-8.
2. Edmondson HA. Tumors of the liver and intrahepatic bile ducts. Sec-tion 7, fascicle 25, Atlas of Tumor Pathology. Washington: Armed Forces Institute of Pathology; 1958.
3. Flejou JF, Barge J, Menu Y, Degott C, Bismuth H, Potet F, et al. Liver adenomatosis. An entity distinct from liver adenoma? Gastroenterol-ogy 1985;89:1132-8.
