Bahador Ali, Dehghani Seyed Mohsen, Geramizadeh Bita, Nikeghbalian Saman, Bahador Mohsen, Malekhosseini Seyed Ali, Kazemi Kurosh, Salahi Heshmatolah
Department of Surgery, Transplant Ward, Shiraz University of Medical Sciences, Shiraz, Iran.
Exp Clin Transplant. 2015 Aug;13(4):329-32. doi: 10.6002/ect.2013.0158. Epub 2014 Mar 28.
This study sought to determine the prevalence of hepatocellular carcinoma and other premalignant lesions in children with hereditary tyrosinemia type 1 who had undergone an orthotopic liver transplant at the Shiraz Transplant Center, in Shiraz, Iran.
Between September 2006, and June 2011, thirty-six patients with hereditary tyrosinemia type 1 received a liver transplant from a deceased (whole or split) or a living-related donor. Clinical records and pathologic specimens, before and after surgery, for each case were reviewed. In addition, ultrasound, abdominal computed tomographic imaging scan findings, and levels of alpha-fetoprotein were recorded.
Twenty-two patients with hepatic nodules larger than 10 mm underwent a Tru-Cut needle biopsy before their liver transplant. In 2 patients, a diagnosis of hepatocellular carcinoma was made by pathologic examination; in the other 20, cirrhosis was confirmed with no evidence of malignancy. After pathologic examination of the explanted livers, the largest nodules in the 36 patients were 35 mm. Five cases had at least 1 nodule of hepatocellular carcinoma. Three of the other patients had small cell dysplasia in some of nodules. All 5 cases with hepatocellular carcinoma were patients older than 2 years of age (19 patients were older than 2 years of age). All patients with hepatocellular carcinoma received pretransplant nitisinone treatment. All patients with hepatocellular carcinoma after their liver transplant are alive at the time of this writing.
The prevalence of cell dysplasia and hepatocellular carcinoma in children with hereditary tyrosinemia type 1 in our study is not as high as that reported previously, so it appears that patients older than 2 years of age require a liver transplant.
本研究旨在确定在伊朗设拉子的设拉子移植中心接受原位肝移植的1型遗传性酪氨酸血症患儿中肝细胞癌及其他癌前病变的患病率。
2006年9月至2011年6月期间，36例1型遗传性酪氨酸血症患者接受了来自已故（全肝或劈离）或活体亲属供体的肝移植。回顾了每个病例手术前后的临床记录和病理标本。此外，记录了超声、腹部计算机断层扫描成像结果以及甲胎蛋白水平。
22例肝结节大于10mm的患者在肝移植前接受了粗针活检。2例经病理检查诊断为肝细胞癌；另外20例确诊为肝硬化，无恶性证据。对切除的肝脏进行病理检查后，36例患者中最大的结节为35mm。5例至少有1个肝细胞癌结节。另外3例患者的一些结节中有小细胞发育异常。所有5例肝细胞癌患者均为2岁以上（19例患者年龄大于2岁）。所有肝细胞癌患者在移植前均接受了尼替西农治疗。撰写本文时，所有肝移植后发生肝细胞癌的患者均存活。
在我们的研究中，1型遗传性酪氨酸血症患儿中细胞发育异常和肝细胞癌的患病率并不像之前报道的那么高，因此似乎2岁以上的患者需要进行肝移植。
