肝脏局灶性结节性增生5例

目的 探讨肝脏局灶性结节性增生(FNH)的诊断和治疗。方法 回顾性分析我院1995~2003年经手术切除及病理证实5例FNH的临床、影像、手术及病理学资料。结果 5例甲胎蛋白(AFP)均为阴性,无肝炎病毒感染依据,术前均未明确诊断而行手术切除,无手术死亡及术后并发症。结论 FNH在临床及影像学上有一定特征,但确诊十分困难;对于无症状确诊病例,可暂不手术,密切随访;有症状、生长迅速或诊断不明确者应积极手术。     

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.     

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.     

Drug induced hepatic focal nodular hyperplasia

Focal nodular hyperplasia, an uncommon benign hepatic tumor, has been reported following ingestion of various drugs and chemical agents. The authors report a case of a young girl who developed such lesion following ingestion of antituberculosis drugs for the treatment of her abdominal tuberculosis.     

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 of the liver in children after hematopoietic stem cell transplantation

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.     

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.     

Focal nodular hyperplasia of the liver in childhood

FNH of the liver is extremely rare during childhood. Based on relevant literature and two of our own cases the diagnosic problems are presented. Unless a clear diagnosis can be obtained by using US (Ultrasonic), CT, Szintegraphy and Angiography, laparotomy should be carried out to avoid confusion with hepatocellular carcinoma or hepatocellular adenome which can become malignant. With the exception of biopsy the higher degree of diagnostic accuracy is obtained with celiacography. Since FNH does not have a tendency towards malignancy, total extsirpation should not be attempted if vital structures are endangered.     

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.     

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.     

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.     

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney

Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported – all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed. Received: 3 December 1996 / Accepted in revised form: 9 September 1997     

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 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.