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Glomus tumor is a rare neoplasm of the distal extremities. It occurs very rarely in the deep visceral organs such as stomach, lung, pancreas, ovary, and liver. Herein, we report a very rare case of glomus tumor of the liver in a 50-year-old woman presenting with abdominal mass which was diagnosed after surgery. The tumor was large and cystic; however, the morphology was similar to the ordinary soft-tissue glomus tumor. Tumor cells were reactive with CD34 and SMA. The patient's follow up failed to show any evidence of malignant behavior or tumor recurrence. This report is the third primary glomus tumor of the liver in the English literature.

Background: Wilson disease is a disorder of copper metabolism characterized by copper overload. A mutation in the ATP7B gene causes dysfunction of ATP7B protein and a reduction in copper excretion into the bile in hepatocytes. Excess copper accumulation leads to liver injury. D-penicillamine primarily can inhibit fibrogenesis and prevent the appearance of scar lesions in the liver. We studied this phenomenon in our patients., Materials and Methods: Pathology slides from the explanted livers of 26 patients diagnosed as having Wilson disease with hepatoneurologic manifestations between 2000 and 2008 who had undergone a liver transplant were investigated retrospectively. Patients were divided into 2 groups according to their history of D-penicillamine use before transplant. The degree of fibrosis and inflammation were classified as mild (1), moderate (2), and severe (3), and were reviewed by an impartial hepatopathologist., Results: Of 26 patients (20 male, 6 female) who had Wilson disease with a mean age of 17.6 -/+ 8.6 years, 69% (18/26) had a history of D-penicillamine use before liver transplant from 6 months to 9 years (mean, 3.4 -/+ 2.7 years). In the D-penicillamine group, 14 patients (77%) had grade 1 fibrosis. Grade 2 and 3 fibrosis was seen in 5.6% and 16% of patients, respectively. In the D-penicillamine group, inflammation was grade 3 in 44% (8/18), grade 2 in 44% (8/18), and grade 1 in 11% of the patients (2/18). In the non- D-penicillamine group (8 patients), grades of fibrosis were grade 3 (62%), grade 2 (25%), and grade 1 (12%); 87% of the patients had grade 2 and 3 inflammation. The degree of fibrosis was significantly lower in the D-penicillamine group than it was in the non-D-penicillamine group (P < .05)., Conclusion: D-penicillamine may reduce the rate of liver fibrogenesis in patients with Wilson disease.

Background: Pediatric hepatic malignancies are rare, accounting for 1-4% of all solid childhood tumors. The histopathology of childhood hepatic tumors guides the treatment and prognosis, and is the cornerstone for precise diagnosis. Until now, there has been no documented study on pediatric liver tumor cases from this center; in this report, we show our experience about the common types of childhood hepatic tumors during five years (2002-2007) and compare them with other studies. Materials and Methods: During five years (2002-2007), all the hepatic tumors of childhood (under 18 year-old) from the pathology file of Namazi Hospital of Shiraz University of Medical Sciences are recorded. This includes both resected specimens and biopsies. All the slides were reviewed and the pathologic diagnosis was confirmed. Results: We detected 53 liver tumor cases in children (below 18 years of age). Among these tumors, 36 (67.9%) were malignant. Male to female ratio was 1.5 to 1. Hepatoblastoma was the most common liver tumor in this age group accounting for 22 patients (41.5%). The second most common primary tumor was hepatocellular carcinoma (HCC), with five patients. Another malignant tumor was embryonal sarcoma. Benign tumors included adenoma, mesenchymal hamartoma, vascular tumors, focal nodular hyperplasia, and inflammatory pseudo tumor. There were also seven metastatic tumors during these five years. Conclusions: The spectrum of hepatic tumors in children is different from that found in the older age group (adults) and also different in different populations.

Background: Progressive familial intrahepatic cholestasis is a disease presenting with severe cholestasis and progressing to the end-stage liver disease later. Liver transplantation is a treatment modality available for progressive familial intrahepatic cholestasis, especially in patients with end-stage liver disease or those who are unsuitable for or have failed biliary diversion. Objectives: To evaluate clinical and pathological characteristics of progressive familial intrahepatic cholestasis patients who had undergone liver transplantation and to determine post-transplant steatosis and steatohepatitis. Methods: We evaluated 111 progressive familial intrahepatic cholestasis patients with normal gamma-glutamyl transferase that performed liver transplantation in Shiraz Transplant Center in Iran between March 2000 and March 2017. Results: The most common clinical manifestations were jaundice and pruritus. Growth retardation and diarrhea were detected in 76.6% and 42.5% of the patients. After transplantation, growth retardation was seen in 31.5% of the patients, and diarrhea in 36.9% of them. Besides, 29.1% of the patients died post-transplant. Post-transplant liver biopsies were taken from 50 patients, and 15 (30%) patients had steatosis or steatohepatitis, five of whom (10%) had macrovesicular steatosis alone, and 10 (20%) had steatohepatitis. Only one patient showed moderate bridging fibrosis (stage III), and none of them showed severe fibrosis. Conclusions: Liver transplantation is the final treatment option for these patients, and it can relieve most clinical manifestations. However, post-transplant mortality rate was relatively high in our center. Diarrhea, growth retardation, and steatosis are unique post-transplant complications in these patients. The rate of post-transplant steatosis and steatohepatitis in patients with liver biopsy in our study was 30%, with a significant difference from previous studies. [ABSTRACT FROM AUTHOR]

FH is a genetic disorder characterized by an increase in serum LDL and total cholesterol values. The afflicted patients are at increased risk of premature atherosclerosis and myocardial infarction. Different treatment modalities are present, including pharmacological agents and surgical procedures. The most effective method of therapy in refractive cases is liver transplantation. Herein, we report our experience on 36 cases of patients with FH undergoing liver transplantation in our center, the main referral center of liver transplantation in Iran. The clinical findings, hospital courses, post-operative complications, and patient follow-up are also described. [ABSTRACT FROM AUTHOR]

Background: Post-transplantation lymphoproliferative disorders (PTLD) are a spectrum of diseases defined as polyclonal or monoclonal proliferations of lymphocytes which occur after solid organ transplants. In this study, we report our first experiences with PTLD following liver transplantation in Iran. Patients and Methods: We retrospectively analyzed five cases of PTLD which followed liver transplantation among more than 550 liver transplants in our center. Of these, three were pediatric cases and two were adults. The underlying causes were tyrosinemia, autoimmune hepatitis, and progressive familial intrahepatic cholestasis (PFIC) in the three pediatric cases. HCV hepatitis was the primary cause for cirrhosis in one of the adults and the other adult was labeled as cryptogenic cirrhosis. All cases, except for one, developed PTLD during the first year following liver transplantation. Results: Patients were diagnosed as PTLD, B-cell, MALT and Hodgkin-like (according to the WHO classification of PTLD). The three pediatric patients died despite discontinuation of immunosuppressive drugs and chemotherapy. Fortunately both adult patients, until now, are still alive. Conclusion: The incidence of PTLD in our center is lower than previous reports from other centers (0.9%), with a 60% mortality rate and worse prognosis in the pediatric age group. [ABSTRACT FROM AUTHOR]

Abstract: Angiosarcomas are rare tumors that predominantly affect adults. Hepatic angiosarcoma in a child is an extremely rare event and is associated with a poor prognosis. Herein, we report our experience in a case of hepatic angiosarcoma in a child who presented with a huge unresectable mass that underwent liver transplantation. [ABSTRACT FROM AUTHOR]

Background: Cirrhosis, the end stage of progressive hepatic fibrosis, is characterized by distortion of the hepatic architecture and the formation of regenerative nodules. Liver transplantation is one of the few available therapies for such patients. However, due to a severe shortage of organ donors, surgical complications, transplant rejection and the high cost of this procedure much interest has focused on research to find new treatment modalities for this disease. There is accumulating evidence for the contribution of bone marrow stem cells to participate in liver regeneration. Methods: Here we report on six patients with end stage liver disease who were subjected to intraportal administration of au- tologous bone marrow-derived CD133* in comparison to mononuclear cells in short-term (6 months) and long-term (24 months) follow up. Results: There were no adverse effects in any of the patients during the short- and long-term follow up period. Moreover, there were no significant alterations of liver function parameters, liver enzymes, serum albumin, creatinine, serum bilirubin and/or liver volume after transplantation of both types of autologous cells in these patients. Conclusion: Our study has shown both the safety and feasibility of this type of liver cell therapy and may be a bridge to liver transplantation. [ABSTRACT FROM AUTHOR]