Focal nodular hyperplasia of the liver in children after hematopoietic stem cell transplantation

Abstract

FNH is a non-malignant neoplasia of the liver rarely described in children. A significant percentage of the pediatric cases have been reported in patients with a history of malignant disease treated with chemo-radiation therapy and in children who were given HSCT. Little is known about the pathogenesis of FNH in transplanted children, but many risk factors linked to the HSCT procedure have been hypothesized. The detection of hepatic nodules, particularly in children who underwent HSCT for a previous malignancy, always raises a diagnostic dilemma. To help the physicians in the diagnostic management of this rare entity, we have retrospectively evaluated a series of transplanted children diagnosed with FNH in our Center over the last 15 yr. In this period, we found 10 new diagnoses of FNH. The diagnostic work-up included CEUS, abdominal CT, and MRI. A liver biopsy was performed in two patients. The median FUP time after diagnosing FNH was 3.8 yr, with an abdominal US and no malignant transformation were observed. Possible risk factors and indications for the management of FNH in transplanted children are reported and discussed in a comprehensive review of the literature.

Focal nodular hyperplasia of the liver: an emerging complication of hematopoietic SCT in children

“…[2][3][4][5][6][7][8][9]32 Our study confirmed this trend. Liver tests as well as viral and neoplastic markers are usually normal in FNH patients, although slightly elevated GOT, GPT, ALP and GGT have been reported, [2][3][4][5][6][7][8][9]32 as it happened in 47.1% of our patients. Abbreviations: cGVHD = chronic GVHD; CI = confidential interval; F = female; FNH = focal nodular hyperplasia; HR = hazard ratio; HrT = hormone replacement therapy; HSCT = hematopoietic SCT; M = male; RT = radiotherapy.…”

“…So far, FNH seems to have a benign and uncomplicated course: all the existing studies report that nodules generally do not change their size and characteristics with time. [2][3][4][5][6][7][8][9]32 In our case series, in 53% of patients, one or more additional lesions with the same or increased diameter were discovered on follow-up, without evidence of malignant transformation.…”

“…FNH is a nonmalignant tumor most commonly detected incidentally during ultrasound (US) examination. [1][2][3][4][5][6][7][8] Risk factors are largely unknown and pathogenesis is controversial and not completely understood. Neither complications nor malignant degenerations are described up to now, and the differential diagnosis includes hepatocellular adenoma and fibrolamellar hepatocellular carcinoma.…”

Enhancing the role of paediatric ultrasound with microbubbles: a review of intravenous applications

“…The main purposes include evaluation of vascular patency and complications, transplanted tissue perfusion and identification of necrotic areas, characterization of new focal lesions and evaluation of postoperative complications including fluid collections. 8,17,[59][60][61][62] A study of a mixed adult and paediatric population following liver transplantation assessed CEUS contribution when there was uncertainty on other imaging modalities. CEUS could further characterize suspected findings in all patients while it detected new clinically significant findings in about half of the patients.…”

Hepatic focal nodular hyperplasia after pediatric hematopoietic stem cell transplantation: The impact of hormonal replacement therapy and iron overload

“…Only in the last decade, a growing body of scientific literature has demonstrated that childhood cancer survivors show a remarkably increased risk of FNH, although the specific pathogenesis in this subcohort has not been clarified yet . As regenerative hepatic lesions are mostly asymptomatic, FNH is often detected incidentally when abdominal imaging is performed due to different clinical indications; in particular, the recent advent of diagnostic techniques for the assessment of iron overload (T2*‐MRI) and their systematic use as screening tools in the setting of secondary hemochromatosis have led to an increased awareness that FNH represents a possible incidental finding after hematopoietic stem cell transplantation (HSCT) …”