Management and surgical treatment for focal nodular hyperplasia in children

Focal nodular hyperplasia (FNH) is rare in pediatric liver tumor. To investigate the experience in the appropriate management of pediatric FNH, the records of consecutive children who were managed at our institute from 2000 to 2007were reviewed for FNH. There were four males and nine females whose ages ranged from 26 months to 18 years with a mean of 11.7 years. FNH in most children was detected by medical examination for abdominal pain (61.5%) or other symptoms. All the 13 pediatric patients underwent liver resection. There was no operative death or postoperative complications. The children were regularly followed up ranging from 2 to 85 months and they were healthy without recurrence. For pediatric FNH patients with clinical symptoms or indefinite diagnosis, it is suggested that active surgical treatment by hepatectomy should be performed.     

Focal nodular hyperplasia of the liver imaging by differing modalities

The logistic approach in diagnosis of focal nodular hyperplasia (FNH) of the liver is discussed, based on the experience with three children. In only one child could the diagnosis be made without angiography. In that child the combination of hypervascularity on the radionuclide angiogram and uptake of the radiocolloid by Kupffer cells was sufficient for the diagnosis of FNH. If the radionuclide scintigraphy is inconclusive, angiography has to be done to show the typical features of FNH, seen in all three patients. Only if scintigraphy and angiography are not able to differentiate FNH from hepatic adenoma, biopsy or exploratory laparotomy is indicated as a final diagnostic procedure. Ultrasonography or computerized tomography is of value only in screening for hepatic mass lesions.     

Focal nodular hyperplasia of the liver: a sequela of tumor therapy

Focal nodular hyperplasia (FNH) of the liver occurs with increased frequency in oncology patients after completion of tumor therapy. Its development may be related to the vascular damage induced by such therapy. We present three children who developed FNH after undergoing antineoplastic therapy for non-hepatic primary tumors. Recognition of this association in the appropriate patient population might obviate the need for biopsy.     

Variable MR imaging appearances of focal nodular hyperplasia in pediatric cancer patients

Background  

Focal nodular hyperplasia (FNH) is rare in the general pediatric population but is increasingly found in treated pediatric cancer patients. FNH can be incidentally found on CT and MRI and can be misdiagnosed as liver metastasis in patients with an oncological history.     

Focal nodular hyperplasia in a child with Beckwith–Wiedemann syndrome

Beckwith–Wiedemann Syndrome (BWS) is associated with somatic overgrowth and a high risk for embryonal tumors, including hepatoblastoma, a highly malignant liver tumor of childhood. Focal nodular hyperplasia (FNH), on the other hand, is a benign tumor of the liver that is uncommon in childhood. Herein we describe a case of FNH in a child with BWS, the first such report in the literature.     

Focal nodular hyperplasia in biliary atresia patient after Kasai hepatic portoenterostomy

We present the case of a 10-year-old girl with biliary atresia in whom focal nodular hyperplasia (FNH) of the liver occurred following successful “Kasai” hepatic portoenterostomy at the age of 2 months. Her jaundice completely disappeared a few weeks after the operation. A 4-cm diameter liver tumor in segment IV was found when she was 5-years old. Needle biopsy could not establish a definitive diagnosis. Because the tumor size was not changing, she was conservatively followed by abdominal echo at an outpatient clinic. She showed progressive liver dysfunction and ongoing cirrhosis, and so at the age of 10 years, she received living donor liver transplantation at our institute. The tumor was pathologically diagnosed as a FNH. Though a FNH in biliary atresia patients is extremely rare, there are only two cases reported in the literature; it should be considered in the differential diagnosis for a hepatic nodule during a long follow-up course in patients with biliary atresia.     

Radiological features of focal nodular hyperplasia of the liver in children

Background. Focal nodular hyperplasia (FNH) is an unusual hepatic tumour in children and should be distinguished from other hepatic lesions. Objective. To describe the imaging characteristics of FNH in children. Materials and methods. We examined five patients (three boys and two girls, mean age 9.4 years) with pathologically confirmed FNH. The diagnosis was obtained by tumour resection (n = 4) and percutaneous needle biopsy (n = 1). One patient with multiple FNHs showed recurrent lesions after tumour resection. All patients were studied with US (including colour and power Doppler US [n = 3]) and CT. Dynamic enhanced CT scans were available in three patients. MRI (n = 2) or coeliac angiography (n = 1) was performed in three patients. Results. Seven of eight FNH lesions in five patients were demonstrated by imaging. The average size of the lesions was 6.5 cm. Six lesions detected on US showed variable echogenicity with a central hyperechoic scar (n = 2). On Doppler examination, central or peripheral hypervascular areas were seen (n = 3). Six lesions detected on contrast-enhanced CT showed high attenuation (n = 4) or iso-attenuation (n = 2). On early phase scans, all the lesions (n = 3) showed high attenuation. Irregular linear or ovoid central scars were detected in two patients on CT. MR demonstrated three lesions in two patients, one of which had not been detected by US or CT. A central low signal intensity scar (n = 1) was seen on T2-weighted MRI. Coeliac angiography performed in one patient showed a hypervascular mass with homogeneous staining. Conclusion. FNH in children shows a wide spectrum of imaging findings on various radiological examinations and the typical central scar was not always seen on imaging studies. Dynamic enhanced CT obtained in the early phase and colour Doppler studies may be helpful in the diagnosis of FNH by allowing characterisation of tumour vascularity. FNH should be included in the differential diagnosis of liver mass in children. Received: 10 September 1997 Accepted: 17 April 1998     

Liver transplantation for refractory severe pruritus related to widespread multifocal hepatic focal nodular hyperplasia (FNH) in a child: Case report and review of literature

Merli L, Grimaldi C, Monti L, Nobili V, Francalanci P, de Ville de Goyet J. Liver transplantation for refractory severe pruritus related to widespread multifocal hepatic focal nodular hyperplasia (FNH) in a child: Case report and review of literature.
Pediatr Transplantation 2011. © 2011 John Wiley & Sons A/S. Abstract: FNH is a rare and benign tumor of the liver. It is not a conventional indication for liver transplantation, and no transplant for FNH in a child has been reported to date. Multifocal FNH growing in adolescent age to a widespread tumor invading the whole liver and associated with severe refractory pruritus was an unusual indication for transplantation in a 13‐yr‐old girl. The operation and the follow‐up were uneventful, allowing full recovery and disappearance of pruritus.     

Nodular regenerative hyperplasia and focal nodular hyperplasia of the liver mimicking hepatic metastasis in children with solid tumors and a review of literature

Nodular regenerative hyperplasia (NRH) and focal nodular hyperplasia (FNH) of the liver rarely occur in children after completion of tumor therapy. These lesions mimic hepatic metastasis and they must be distinguished from metastatic lesions. The authors present 2 children, one with NRH and one with FNH, after undergoing antineoplastic therapy for non-hepatic childhood solid tumors and discuss their patients in the context of the literature.     

Focal nodular hyperplasia in children,adolescents, and young adults

Background  

Focal nodular hyperplasia (FNH) is a benign hepatic tumor that is rare in children. In order to understand whether there are differences in the etiology or appearance of FNH in children, we analyzed the clinical information and imaging of pathologically proven cases.     

Orthotopic liver transplantation in glucose-6-phosphatase deficiency--Von Gierke disease--with multiple hepatic adenomas and concomitant focal nodular hyperplasia

Von Gierke disease is occasionally complicated by hepatic adenomas (HAs) causing great concern owing to the current difficulties in monitoring them regarding malignant transformation. Orthotopic liver transplantation (OLT) is proposed as a therapeutic tool when multiple HAs and poor metabolic control are present, owing to the lack of a clear-cut criterion to detect early malignant transformation, whether or not associated with growth retardation. Focal nodular hyperplasia (FNH) has never been described together with multiple adenomas in von Gierke disease. We report a 26-year-old woman with von Gierke disease complicated by multiple HAs concomitant with FNH who underwent OLT and is now free from disease symptoms with good long-term outcome. In conclusion, although FNH could have been managed clinically, when multiple adenomas are present, OLT should be planned for some patients, mainly for those with poor metabolic control.     

Focal nodular hyperplasia of the liver after intensive treatment for pediatric cancer: is hematopoietic stem cell transplantation a risk factor?

Focal nodular hyperplasia (FNH) is a benign hepatic lesion very rarely described in the pediatric population. It has been reported more frequently in patients treated for pediatric cancers with chemotherapy or hematopoietic stem cell transplantation. The use of high dosage of alkylating agents, the occurrence of venous occlusive disease, graft-versus-host disease, and other variables linked to the hematopoietic stem cell transplantation procedure can represent risk factors for the development of FNH in the pediatric age. The discovery of hepatic nodules in the follow-up of patients treated for malignancies suggests recurrence of disease and raises a diagnostic dilemma. Here we describe possible risk factors, clinical and radiological findings of eight pediatric patients who developed focal nodular hyperplasia after hematopoietic stem cell transplantation. The aim of this report is to provide useful diagnostic tools to facilitate accurate diagnosis of FNH and suggest a correct management of this benign lesion during postcancer follow-up.     

Type la glycogen storage disease with focal nodular hyperplasia in siblings

Glycogen storage disease type I (GSD-I) is an inherited disorder that is due to a glucose-6-phosphatase (G6Pase) deficiency. There have been recent reports of hepatocellular tumors in adults with this disease. Hepatic adenoma is the most common tumor described but others, including hepatocellular carcinomas, hepatoblastomas, and focal nodular hyperplasia (FNH) have been reported. FNH of the liver is a rare benign lesion that has been reported in eight patients with GSD-I. Three of these eight patients, in addition to the patient in our study, had been treated with portacaval shunts. When these patients were compared with patients who had not received such treatment, it appeared that the portacaval shunts may have induced the development of FNH and may have been associated with earlier complications. FNH is a benign tumor that may coexist with adjacent fibrolamillar carcinomas and/or adenomas and requires careful follow-up.     

Hepatic focal nodular hyperplasia with congenital portosystemic shunt

Hepatic focal nodular hyperplasia (FNH) is a rare benign tumor in children. Vascular anomalies have been identified as pathological features of FNH, but the etiology remains unclear. We describe a rare case including the time course of formation of hepatic FNH in response to congenital portosystemic shunt (PSS). A 4‐month‐old girl was identified on newborn mass screening to have hypergalactosemia, but no inherited deficiencies in galactose‐metabolizing enzymes were found. Ultrasonography and per‐rectal portal scintigraphy showed intrahepatic PSS of the right lobe as a cause of the hypergalactosemia. At age 12 months, the patient had elevated hepatic enzymes and small hypoechoic hepatic lesions around the shunt. On abdominal contrast‐enhanced ultrasonography spoke‐wheel sign and central stellate scar were seen, which are typical features of hepatic FNH without biopsy. Congenital intrahepatic PSS should be evaluated on abdominal contrast‐enhanced ultrasonography and observed over time because of its potential to develop into hepatic FNH.     

Recurrent abdominal pain caused by nodular focal hyperplasia of the liver. A case report

A case of focal nodular hyperplasia of the liver in childhood (FNH or NRC) is reported; a conservative approach is usually preferred in this disease when the diagnosis is surely identified, and a follow-up for some years is recommended. Despite the increased accuracy of modern imaging methods none of them, singly or together, is able to differentiate a malignant lesion with certainty and histological confirmation is mandatory. In the case described a laparotomy frozen section was performed but it was not possible to make a diagnosis and the liver mass was removed. The patient had a complete relief of symptoms with no evidence of recurrence in the follow-up. A review of the literature has been made, trying to point out this disease and its treatment.     

Gadobenate-dimeglumine-enhanced magnetic resonance imaging for hepatic lesions in children

Background

Magnetic resonance imaging enhanced by hepatocyte-specific contrast media has been found useful to characterize liver lesions in adults and children.

Objective

To present our experience with gadobenate dimeglumine (Gd-BOPTA)-enhanced MRI for evaluation of focal liver lesions in children.

Materials and methods

We retrospectively reviewed gadobenate-dimeglumine-enhanced MR images obtained for evaluation of suspected hepatic lesions in 30 children. Signal characteristics on various sequences including 45- to 60-min hepatobiliary phase images were noted by two radiologists. Chart review identified relevant clinical details including history of cancer treatment, available pathology and stability of lesion size on follow-up imaging.

Results

Of the 30 children who had gadobenate-enhanced MRI, 26 showed focal lesions. Diagnoses in 26 children were focal nodular hyperplasia (FNH) in 15, hemangiomas in 3, regenerating nodules in 3, focal fatty infiltration in 2, indeterminate lesions in 3, and one patient each with adenomas, hepatoblastoma and metastasis. Two patients had multiple diagnoses. All FNH lesions (39), all regenerative nodules (19) and an indeterminate lesion were iso- or hyperintense on hepatobiliary-phase images while all other lesions (28) were hypointense to hepatic parenchyma. The average follow-up period was 21.7 months.

Conclusion

Our experience with gadobenate-enhanced MRI indicates potential utility of gadobenate in the evaluation of pediatric hepatic lesions in differentiating FNH and regenerating nodules from other lesions.     

Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature

Background. Nodular regenerative hyperplasia (NRH) of the liver is a multi-acinar regenerative nodular lesion in a non-cirrhotic liver. It is a rare entity, especially in children, and remains of unknown aetiology.¶Objective. NRH is often seen in association with other diseases or drug intake. In half of patients it is complicated by portal hypertension. Radiologically, its nodular appearance may look like neoplasia.¶Results. We report a case of NRH with enormous hepatomegaly and multiple huge nodules.¶Conclusion. We wish to emphasise the importance of open wedge biopsy to establish diagnosis, since the prognosis of NRH in the absence of portal hypertension is good. Complications such as rupture of a nodule are rare.     

Focal nodular hyperplasia in an adolescent with glycogen storage disease type I with mesocaval shunt operation in childhood: a case report and review of the literature

As patients with glycogen storage disease type I survive longer, cases with hepatic tumor have been increasingly documented. A 16 year old boy with glycogen storage disease type I was evaluated for multiple liver tumors. He was diagnosed on clinical features at 9 months of age and underwent a mesocaval shunt operation at 5 years of age. The biopsy of one of the masses showed focal nodular hyperplasia. This is uncommon in patients with glycogen storage disease type I, compared to those with adenoma or malignant hepatic tumor. The association of a portacaval shunt with focal nodular hyperplasia is significant compared to other tumors. An environment of high estrogen concentration or sex hormone binding globulin accompanied by shunt operation may cause focal nodular hyperplasia to develop in the liver of patients with glycogen storage disease type I.     

Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein

Congenital absence of the portal vein with systemic visceral venous return in a 8-year-old girl with oculoauriculovertebral dysplasia (Goldenhar Syndrome) had been previously reported following its discovery during preoperative evaluation of a liver mass which was diagnosed as focal nodular hyperplasia after open biopsy. Subsequently, an enlarging diffuse hepatic neoplasm developed with associated elevated alpha feto-protein levels. Repeat biopsy and imaging showed the tumor to be a hepatoblastoma involving both lobes of the liver. The patient was treated by hepatic resection and orthotopic liver transplantation and is doing well at 18 months follow-up.     

Management and algorithm for focal nodular hyperplasia of the liver in children.

PURPOSE: The aim of this study was to determine an appropriate management plan for childhood and adolescent FNH, in particular to establish an algorithm for preoperative diagnosis and treatment. PATIENTS AND METHODS: Between 1985 and 2003, 4 children with FNH were diagnosed. Of these 4 patients, 3 (Group A) underwent tumor resection, and 1 (Group B) was treated by conservative management. Clinical data, pathological findings and follow-up were evaluated retrospectively. RESULTS: The 3 patients in Group A were symptomatic, while the 1 patient in Group B was asymptomatic. In 3 of 4 patients, a homogeneous tumor with a central stellate area was noted on abdominal ultrasonography, CT scan and MR imaging. In case 2, SPIO-enhanced MR imaging was useful for differentiating FNH from hepatocellular carcinoma. Though percutaneous needle biopsy was performed in case 3, a pathologically definitive diagnosis was impossible. An open biopsy was performed in case 4 and FNH was diagnosed. In case 4 treated by conservative management, the tumor size did not change during the 7 years after the diagnosis of FNH. CONCLUSION: FNH is usually treated conservatively because of the good evolutionary outcome of the lesion. Surgery is indicated in cases of complications, compressed adjacent organs, lesion progression, or for symptomatic patients. We advocate the use of less invasive SPIO-enhanced MR imaging instead of open biopsy when the diagnosis of focal liver lesions is not clear after contrast-enhanced CT scan and non-enhanced MR imaging.