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Balbaa A, Thompson A, Forbes KL. Incidental Finding of a Liver Mass in a 4-year-old Girl. Pediatr Rev 2024; 45:652-655. [PMID: 39630991 DOI: 10.1542/pir.2022-005835] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/29/2022] [Revised: 04/13/2023] [Accepted: 04/20/2023] [Indexed: 12/07/2024]
Affiliation(s)
| | - Adrienne Thompson
- Department of Radiology, University of Alberta, Edmonton, Alberta, Canada
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Chambers G, Zarfati A, Aderotimi T, Branchereau S, Humphrey T, Woodley H, Franchi-Abella S. Imaging strategy for focal nodular hyperplasia in children: long-term experience from two specialist European centres. Pediatr Radiol 2023; 53:46-56. [PMID: 35773358 DOI: 10.1007/s00247-022-05420-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/07/2022] [Revised: 05/10/2022] [Accepted: 06/02/2022] [Indexed: 02/07/2023]
Abstract
BACKGROUND Focal nodular hyperplasia (FNH) in children is a rare but benign tumour, which must be differentiated from malignant entities to avoid unnecessary treatment, leading to potential morbidity. OBJECTIVES To provide data on imaging findings of these lesions with a suggested algorithm for diagnosis, sampling and follow-up. MATERIALS AND METHODS This retrospective review evaluated imaging of all patients diagnosed with FNH in two tertiary referral centres in Europe between 1975 and 2018. RESULTS One hundred and four patients with 137 tumours were reviewed. The mean age at presentation was 8.2 years. The median tumour size was 5 cm (range: 0.3-29 cm). Multiple lesions were seen in 16.3% of patients. The male-to-female ratio was 1:2. CONCLUSION FNH with typical features on imaging can be safely followed up once the diagnosis has been established. The use of contrast-enhanced ultrasound and magnetic resonance imaging allows accurate characterisation in most cases. Histological sampling is only advised when there is diagnostic doubt. Atypical arterial enhancement of FNH should prompt the search for a congenital portosystemic shunt.
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Affiliation(s)
- Greg Chambers
- Department of Paediatric Radiology, Leeds General Infirmary, Great George Street, LS1 3EX, Leeds, UK.
| | - Angelo Zarfati
- Department of Pediatric Surgery, Bambino Gesù Pediatric Hospital, Piazza di Sant'Onofrio, 4, Rome, Italy.,Residency School of Pediatric Surgery, Via University of Rome Tor Vergata, Cracovia, 50, Rome, Italy
| | - Tobi Aderotimi
- Royal Alexandra Children's Hospital, University Hospitals Sussex NHS Foundation Trust, Brighton, UK
| | - Sophie Branchereau
- Department of Pediatric Surgery, AP-HP Hôpital Bicêtre, Le Kremlin-Bicêtre, France
| | - Terry Humphrey
- Department of Paediatric Radiology, Leeds General Infirmary, Great George Street, LS1 3EX, Leeds, UK
| | - Helen Woodley
- Department of Paediatric Radiology, Leeds General Infirmary, Great George Street, LS1 3EX, Leeds, UK
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Isolated hepatic lymphangiomas in children: Two case reports. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2020. [DOI: 10.1016/j.epsc.2020.101430] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022] Open
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Akhaladze D, Grachev N, Kachanov D, Talypov S, Merkulov N, Uskova N, Andreev E, Rabaev G, Ivanova N, Varfolomeeva S. Liver resection for atypical giant focal nodular hyperplasia. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2019. [DOI: 10.1016/j.epsc.2019.101202] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022] Open
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Zhuang L, Ni C, Din W, Zhang F, Zhuang Y, Sun Y, Xi D. Huge focal nodular hyperplasia presenting in a 6-year-old child: A case presentation. Int J Surg Case Rep 2016; 29:76-79. [PMID: 27825058 PMCID: PMC5099262 DOI: 10.1016/j.ijscr.2016.10.053] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/21/2016] [Revised: 10/23/2016] [Accepted: 10/23/2016] [Indexed: 11/28/2022] Open
Abstract
FNH is a benign lesion of the liver which is rare in children. We report the case of a 6-year-old child (male) with a huge FNH which size is more than 10 cm. This could be the biggest FNH among all children’s FNH cases ever reported. When pediatric FNH patients accord with (1) clinical symptoms; (2) indefinite diagnosis or hepatitis B virus carriage; (3) tumor size >5 cm, surgical treatment could be considered. Introduction Focal nodular hyperplasia (FNH) is a benign lesion of the liver which is usually found in healthy adults, however, FNH is rare in children, and comprises only 2% of all pediatric liver tumors. Herein, we report the case of a 6-year-old child (male) with a huge FNH which size is more than 10 cm. This could be the biggest FNH among all children’s FNH cases ever reported. Case presentation A 6-year-old boy was found a hepatic space-occupying lesion two years ago. As the time went by, the lesion became bigger gradually. The last CT examination showed the size of the tumor to be 10.5 × 9.9 cm in the right hepatic lobe. This child underwent surgical resection of the tumor which was confirmed as FNH (11 × 8 × 7 cm) by pathology. Conclusion FNH is a benign lesion of the liver, and it is characterized by hepatocyte hyperplasia and a central stellate scar. It is uncommon for FNH to be diagnosed in children. Such huge FNH (about 11 cm) is extremely rare. Surgical operation may be the effective method to cure huge FNH.
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Affiliation(s)
- Lin Zhuang
- Department of General Surgery, Wujin Affiliated Hospital of Jiangsu University, Changzhou, 213161, China
| | - Chuangye Ni
- Department of Liver Surgery, First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Wenbing Din
- Department of Liver Surgery, First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Feng Zhang
- Department of Liver Surgery, First Affiliated Hospital of Nanjing Medical University, Nanjing, 210029, China
| | - Yi Zhuang
- Department of Emergency, Wujin Affiliated Hospital of Jiangsu University, Changzhou, 213161, China
| | - Yawei Sun
- Department of General Surgery, Wujin Affiliated Hospital of Jiangsu University, Changzhou, 213161, China
| | - Dong Xi
- Department of General Surgery, Wujin Affiliated Hospital of Jiangsu University, Changzhou, 213161, China.
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Abstract
BACKGROUND Focal nodular hyperplasia (FNH) is a benign hepatic lesion that is rare in children. The aim of the present study was to review the clinical features of children with FNH and our institution's experience in the management of this rare disease. METHODS A review of the medical records of children diagnosed as having FNH between 1999 and 2013 at West China Hospital of Sichuan University was undertaken. RESULTS Seventy-nine patients with FNH were identified: 68 patients without a history of malignancy and 11 patients with a history of malignancy. Thirty-seven (46.8%) patients were symptomatic at presentation. Patients with a history of malignancy had smaller FNH lesions and were less likely to have a central scar in FNH than do patients without a history of malignancy. Forty-seven patients underwent liver resection because of concern for malignancy, symptoms, and/or large lesions. Three patients were treated by embolization because of compression of the adjacent organs. There was no operative death or severe postoperative complications. Fourteen patients underwent liver biopsy to rule out malignant masses. The remaining 15 patients were managed expectantly with imaging surveillance. On follow-up, 2 patients for whom observation was initially recommended eventually required resection because of a mass effect. CONCLUSIONS The clinical features of pediatric FNH are variable and can be nonspecific. Our data emphasize the therapeutic approach that active management is indicated in symptomatic or progressive FNH and in children with diagnostic difficulties, whereas observation management is recommended in asymptomatic children with a definitive diagnosis.
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Oliveira C, Gil-Agostinho A, Gonçalves I, Noruegas MJ. Transarterial embolisation of a large focal nodular hyperplasia, using microspheres, in a paediatric patient. BMJ Case Rep 2015; 2015:bcr-2014-208879. [PMID: 26163551 DOI: 10.1136/bcr-2014-208879] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023] Open
Abstract
Benign liver tumours are uncommon in children, haemangiomas being the most frequent. Focal nodular hyperplasia (FNH) represents about 2% of paediatric liver tumours. In children, as in adults, a conservative approach is generally recommended. However, large lesions (greater than 5 cm) are more frequent in the paediatric age group, and in these cases, as well as in growing lesions, surgical removal may be advised. Transarterial embolisation (TAE) has been a successful alternative option described in older patients, especially in cases where surgical removal is not possible. This minimally invasive procedure may also become an option in the paediatric group. The authors report the case of a boy with a large FNH treated with TAE using microspheres.
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Affiliation(s)
- Catarina Oliveira
- Medical Imaging Department, Centro Hospitalar e Universitário de Coimbra, Portugal
| | | | - Isabel Gonçalves
- Paediatric Medical unit, Hospital Pediátrico, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
| | - Maria José Noruegas
- Medical Imaging Department, Centro Hospitalar e Universitário de Coimbra, Portugal Radiology unit, Hospital Pediátrico, Centro Hospitalare Universitário de Coimbra, Coimbra, Portugal
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Jung SY, Kang B, Choi YM, Kim JM, Kim SK, Kwon YS, Lee JE. Development of multifocal nodular lesions of a liver mimicking hepatic metastasis, following resection of an insulinoma in a child. KOREAN JOURNAL OF PEDIATRICS 2015; 58:69-72. [PMID: 25774199 PMCID: PMC4357775 DOI: 10.3345/kjp.2015.58.2.69] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/06/2013] [Revised: 09/24/2013] [Accepted: 10/14/2013] [Indexed: 01/01/2023]
Abstract
Insulinoma, which arises from insulin-producing pancreatic beta cells, is a rare tumor in children. Only 5%-10% of insulinomas are malignant and undergo metastasis. We report a case of an 11-year-old girl who experienced hypoglycemia-related seizures induced by an insulinoma; after resection of the primary tumor, she developed hepatic focal nodular hyperplasia (FNH). Laboratory test results indicated marked hypoglycemia with hyperinsulinemia. Abdominal ultrasonography (US) and computed tomography results were normal; however, magnetic resonance imaging (MRI) showed a solid mass in the pancreatic tail. Therefore, laparoscopic distal pancreatectomy was performed. Two months after the surgery, an abdominal MRI revealed multiple nodular lesions in the liver. An US-guided liver biopsy was then performed, and histological examination revealed FNH without necrosis or mitotic activity. The patient has been free of hypoglycemia for 2 years, and recent MRI studies showed a decrease in the size of FNH lesions, without any evidence of metastasis. Even though no metastatic lesions are noted on imaging, close observation and follow-up imaging studies are required in a child with insulinoma that has malignant potential on histopathologic findings.
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Affiliation(s)
- Sook Young Jung
- Department of Pediatrics, Inha University Hospital, Inha University School of Medicine, Incheon, Korea
| | - Ben Kang
- Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
| | - Yoon Mee Choi
- Department of Surgery, Inha University Hospital, Inha University School of Medicine, Incheon, Korea
| | - Jun Mee Kim
- Department of Pathology, Inha University Hospital, Inha University School of Medicine, Incheon, Korea
| | - Soon Ki Kim
- Department of Pediatrics, Inha University Hospital, Inha University School of Medicine, Incheon, Korea
| | - Young Se Kwon
- Department of Pediatrics, Inha University Hospital, Inha University School of Medicine, Incheon, Korea
| | - Ji Eun Lee
- Department of Pediatrics, Inha University Hospital, Inha University School of Medicine, Incheon, Korea
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Ma IT, Rojas Y, Masand PM, Castro EC, Himes RW, Kim ES, Goss JA, Nuchtern JG, Finegold MJ, Thompson PA, Vasudevan SA. Focal nodular hyperplasia in children: an institutional experience with review of the literature. J Pediatr Surg 2015; 50:382-7. [PMID: 25746693 DOI: 10.1016/j.jpedsurg.2014.06.016] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/09/2014] [Revised: 05/13/2014] [Accepted: 06/28/2014] [Indexed: 12/30/2022]
Abstract
BACKGROUND Focal nodular hyperplasia (FNH) is uncommonly diagnosed in pediatric patients and may be difficult to distinguish from a malignancy. We present a review of all children with a tissue diagnosis of FNH at our institution, describe the diagnostic modalities, and provide recommendations for diagnosis and follow-up based on our experience and review of the literature. METHODS A retrospective review of children <18years of age diagnosed with FNH at a single institution was performed from 2000 to 2013. RESULTS Twelve patients were identified with a tissue diagnosis of FNH. Two patients required surgical resection of their lesion owing to concern for malignancy. Ten patients were managed expectantly with imaging surveillance after biopsy confirmed a diagnosis of FNH. All patients who underwent MRI had very typical findings including hypointensity on T1 weighted sequences, hyperintensity on T2, and homogenous uptake of contrast on the arterial phase. On follow-up all patients had either a stable lesion or reduction in size. CONCLUSIONS Focal nodular hyperplasia presents typically in children with liver disease, have undergone chemotherapy, and adolescent females. Young children, particularly <5years of age, without underlying liver disease or history of chemotherapy can pose a diagnostic dilemma. In this unique subgroup of children with FNH, MRI and/or needle biopsy should be adequate diagnostic modalities for these lesions.
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Affiliation(s)
- Irene T Ma
- Department of Surgery, Mayo Clinic Arizona, Phoenix, Arizona
| | - Yesenia Rojas
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Prakash M Masand
- Department of Radiology, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Eumenia C Castro
- Department of Pathology, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Ryan W Himes
- Division of Gastroenterology, Hepatology, and Nutrition, Department of Pediatrics, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Eugene S Kim
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - John A Goss
- Division of Abdominal Transplantation, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Jed G Nuchtern
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Milton J Finegold
- Department of Pathology, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Patrick A Thompson
- Division of Hematology/Oncology, Department of Pediatrics, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas
| | - Sanjeev A Vasudevan
- Division of Pediatric Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital Liver Tumor Center, Dan L. Duncan Cancer Center, Baylor College of Medicine, Houston, Texas.
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Gong Y, Chen L, Qiao ZW, Ma YY. Focal nodular hyperplasia coexistent with hepatoblastoma in a 36-d-old infant. World J Gastroenterol 2015; 21:1028-1031. [PMID: 25624742 PMCID: PMC4299321 DOI: 10.3748/wjg.v21.i3.1028] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2014] [Revised: 07/03/2014] [Accepted: 09/05/2014] [Indexed: 02/06/2023] Open
Abstract
Focal nodular hyperplasia (FNH) is a benign hepatic tumor characterized by hepatocyte hyperplasia and a central stellate scar. The association of FNH with other hepatic lesions, such as adenomas, hemangiomas and hepatocellular carcinoma, has been previously reported, but FNH associated with another hepatic tumor is rare in infants. Here we report a case of FNH coexistent with hepatoblastoma in a 36-d-old girl. Computed tomography (CT) imaging showed an ill-delineated, inhomogeneous enhanced mass with a central star-like scar in the right lobe of the liver. The tumor showed early mild enhancement at the arterial phase (from 40HU without contrast to 52HU at the arterial phase), intense enhancement at the portal phase (87.7HU) and 98.1HU in the 3-min delay scan. A central scar in the tumor presented as low density on non-contrast CT and slightly enhanced at delayed contrast-enhanced scanning. This infant underwent surgical resection of the tumor. Histopathology demonstrated typical FNH coexistent with a focal hepatoblastoma, which showed epithelioid tumor cells separated by proliferated fibrous tissue.
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Darai N, Shu R, Gurung R, Zhang X, Teng G. Atypical CT and MRI Features of Focal Nodular Hyperplasia of Liver: A Study with Radiologic-Pathologic Correlation. ACTA ACUST UNITED AC 2015. [DOI: 10.4236/ojrad.2015.53020] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
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Cho Y, Shimono T, Morikawa H, Shintaku H, Tokuhara D. Hepatic focal nodular hyperplasia with congenital portosystemic shunt. Pediatr Int 2014; 56:e102-e105. [PMID: 25521987 DOI: 10.1111/ped.12456] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/27/2013] [Revised: 06/02/2014] [Accepted: 07/03/2014] [Indexed: 11/30/2022]
Abstract
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.
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Affiliation(s)
- Yuki Cho
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Taro Shimono
- Department of Radiology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Hiroyasu Morikawa
- Department of Hepatology, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Haruo Shintaku
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Daisuke Tokuhara
- Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka, Japan
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Lee J, Molitor M, Alashari M, Winters W, Skarda DE. Exophytic focal nodular hyperplasia torsion: A rare cause of sudden-onset epigastric pediatric abdominal pain. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2014. [DOI: 10.1016/j.epsc.2014.08.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022] Open
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Benign hepatocellular tumors in children: focal nodular hyperplasia and hepatocellular adenoma. Int J Hepatol 2013; 2013:215064. [PMID: 23555058 PMCID: PMC3608344 DOI: 10.1155/2013/215064] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2012] [Accepted: 02/05/2013] [Indexed: 12/11/2022] Open
Abstract
Benign liver tumors are very rare in children. Most focal nodular hyperplasia (FNH) remain sporadic, but predisposing factors exist, as follows: long-term cancer survivor (with an increasing incidence), portal deprivation in congenital or surgical portosystemic shunt. The aspect is atypical on imaging in two-thirds of cases. Biopsy of the tumor and the nontumoral liver is then required. Surgical resection will be discussed in the case of large tumors with or without symptoms. In the case of associated vascular disorder with portal deprivation, restoration of the portal flow will be discussed in the hope of seeing the involution of FNH. HepatoCellular Adenoma (HCA) is frequently associated with predisposing factors such as GSD type I and III, Fanconi anemia especially if androgen therapy is administered, CPSS, and SPSS. Adenomatosis has been reported in germline mutation of HNF1- α . Management will depend on the presence of a predisposing factor and may include metabolic control, androgen therapy withdrawn, or closure of the shunt when appropriate. Surgery is usually performed on large lesions. In the case of adenomatosis or multiple lesions, surgery will be adapted. Close followup is required in all cases.
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Liu QY, Zhang WD, Lai DM, Ou-yang Y, Gao M, Lin XF. Hepatic focal nodular hyperplasia in children: Imaging features on multi-slice computed tomography. World J Gastroenterol 2012; 18:7048-7055. [PMID: 23323007 PMCID: PMC3531693 DOI: 10.3748/wjg.v18.i47.7048] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/13/2012] [Revised: 09/30/2012] [Accepted: 11/13/2012] [Indexed: 02/06/2023] Open
Abstract
AIM: To retrospectively analyze the imaging features of hepatic focal nodular hyperplasia (FNH) in children on dynamic contrast-enhanced multi-slice computed tomography (MSCT) and computed tomography angiography (CTA) images.
METHODS: From September 1999 to April 2012, a total of 218 cases of hepatic FNH were confirmed by either surgical resection or biopsy in the Sun Yat-sen Memorial Hospital of Sun Yat-sen University and the Cancer center of Sun Yat-sen University, including 12 cases (5.5%) of FNH in children (age ≤ 18 years old). All the 12 pediatric patients underwent MSCT. We retrospectively analyzed the imaging features of FNH lesions, including the number, location, size, margin, density of FNH demonstrated on pre-contrast and contrast-enhanced computed tomography (CT) scanning, central scar, fibrous septa, pseudocapsule, the morphology of the feeding arteries and the presence of draining vessels (portal vein or hepatic vein).
RESULTS: All the 12 pediatric cases of FNH had solitary lesion. The maximum diameter of the lesions was 4.0-12.9 cm, with an average diameter of 5.5 ± 2.5 cm. The majority of the FNH lesions (10/12, 83.3%) had well-defined margins. Central scar (10/12, 83.3%) and fibrous septa (11/12, 91.7%) were commonly found in children with FNH. Central scar was either isodense (n = 7) or hypodense (n = 3) on pre-contrast CT images and showed progressive enhancement in 8 cases in the equilibrium phase. Fibrous septa were linear hypodense areas in the arterial phase and isodense in the portal and equilibrium phases. Pseudocapsule was very rare (1/12, 8.3%) in pediatric FNH. With the exception of central scars and fibrous septa within the lesions, all 12 cases of pediatric FNH were homogenously enhanced on the contrast-enhanced CT images, significantly hyperdense in the arterial phase (12/12, 100.0%), and isodense in the portal venous phase (7/12, 58.3%) and equilibrium phase (11/12, 91.7%). Central feeding arteries inside the tumors were observed on CTA images for all 12 cases of FNH, whereas no neovascularization of malignant tumors was noted. In 9 cases (75.0%), there was a spoke-wheel shaped centrifugal blood supply inside the tumors. The draining hepatic vein was detected in 8 cases of pediatric FNH. However, the draining vessels in the other 4 cases could not be detected. No associated hepatic adenoma or hemangioma was observed in the livers of the 12 pediatric cases.
CONCLUSION: The characteristic imaging appearances of MSCT and CTA may reflect the pathological and hemodynamic features of pediatric FNH. Dynamic multi-phase MSCT and CTA imaging is an effective method for diagnosing FNH in children.
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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 Transplant 2012; 16:E265-8. [PMID: 22093884 DOI: 10.1111/j.1399-3046.2011.01603.x] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/06/2023]
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.
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Affiliation(s)
- L Merli
- Transplantation Center, Ospedale Pediatrico Bambino Gesù, Roma, Italy
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Development of hepatic angiomyolipoma accompanied with focal nodular hyperplasia long after treatment of pelvic rhabdomyosarcoma. J Pediatr Surg 2011; 46:1267-70. [PMID: 21683235 DOI: 10.1016/j.jpedsurg.2011.01.021] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2010] [Revised: 01/21/2011] [Accepted: 01/21/2011] [Indexed: 12/11/2022]
Abstract
Survivors of childhood cancer have a higher risk of developing a secondary neoplasm in their lifetime. The increased risk of a second malignant neoplasm is related to treatment of the primary tumor and genetic predisposition. We describe a 19-year-old man with 2 hepatic masses, one of which was diagnosed as a hepatic angiomyolipoma and the other as focal nodular hyperplasia 14 years after the treatment of stage IV pelvic rhabdomyosarcoma. The combination of these tumors has not previously been reported in the literature.
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Towbin AJ, Luo GG, Yin H, Mo JQ. Focal nodular hyperplasia in children, adolescents, and young adults. Pediatr Radiol 2011; 41:341-9. [PMID: 20949264 DOI: 10.1007/s00247-010-1839-8] [Citation(s) in RCA: 46] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/03/2010] [Revised: 08/22/2010] [Accepted: 09/03/2010] [Indexed: 12/22/2022]
Abstract
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. MATERIALS AND METHODS A pathology database was used to identify all cases of FNH diagnosed at our institution. Each patient's imaging was evaluated for the characteristics of FNH lesions. Clinical information was obtained on each patient. RESULTS Thirteen patients with FNH were identified (7 male/6 female, mean age 14.3 years, range 1-27 years). Seven patients (5 male/2 female) had a remote history of childhood malignancy. The time interval between the diagnoses of malignancy and FNH ranged from 9 to 27 years (mean 14.4 years). On imaging, all seven cancer survivors had multiple liver lesions. In the remaining six patients (2 male/4 female), there was no history of malignancy and all but one of these patients had a solitary FNH. CONCLUSION Half of the patients with FNH in this study were long-term cancer survivors and each of these patients had multiple masses. Recognizing the features of FNH will aid in diagnosis and appropriate management.
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Affiliation(s)
- Alexander J Towbin
- Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., ML# 5031, Cincinnati, OH 45229, USA.
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19
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Gobbi D, Dall'Igna P, Messina C, Cesca E, Cecchetto G. Focal nodular hyperplasia in pediatric patients with and without oncologic history. Pediatr Blood Cancer 2010; 55:1420-2. [PMID: 20949588 DOI: 10.1002/pbc.22641] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/28/2023]
Abstract
The diagnosis of FNH is warranted by the possibility of avoiding unnecessary hepatic resections. The 18 patients of our series, 6 of whom were long-term survivors of malignant, non-hepatic tumors, underwent either a biopsy or a complete excision to obtain the diagnosis. The imaging characteristics could not be considered pathognomonic. The lesion remained stable after the biopsy in 8 patients; no complications were observed in other 10 patients who underwent resection of the mass. The outcome of all our patients with or without previous oncological disease was benign.
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Affiliation(s)
- Dalia Gobbi
- Pediatric Surgery Department, University Hospital of Padua, Padua, Italy.
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20
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Chen WH, Peng CM, Chou CM, Chen HC, Jan YJ. Focal nodular hyperplasia of the liver in a 5-year-old girl. J Chin Med Assoc 2010; 73:611-4. [PMID: 21093832 DOI: 10.1016/s1726-4901(10)70133-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2009] [Accepted: 06/03/2010] [Indexed: 11/17/2022] Open
Abstract
Focal nodular hyperplasia of the liver is a benign tumor that usually affects young women. Traditionally, its treatment in children has been conservative. As a result of its rarity in childhood, its differential diagnosis with other liver tumors is challenging. We present the case of a 5-year-old girl with a 1-week history of fever and abdominal pain. No definite diagnosis could be obtained after serial imaging and liver biopsy. As a result of uncertainty in the imaging and needle biopsy results, the patient underwent complete tumor resection. Pathology showed focal nodular hyperplasia that affected the right lobe of the liver. After surgery, the child was doing well at 24 months of follow-up.
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Affiliation(s)
- Wei-Hsin Chen
- Department of Surgery, Taichung Veterans General Hospital, Taichung, Taiwan, ROC
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21
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Lautz T, Tantemsapya N, Dzakovic A, Superina R. Focal nodular hyperplasia in children: clinical features and current management practice. J Pediatr Surg 2010; 45:1797-803. [PMID: 20850623 DOI: 10.1016/j.jpedsurg.2009.12.027] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2009] [Revised: 12/30/2009] [Accepted: 12/31/2009] [Indexed: 02/08/2023]
Abstract
BACKGROUND Although nonoperative management is an accepted practice for most adults with focal nodular hyperplasia (FNH), questions remain about the safety and feasibility of this strategy in children. Our aim was to review the clinical features of children with FNH and determine current management patterns. METHODS We reviewed records of all children and adolescents with FNH managed at our institution from 1999 to 2009 and performed a MEDLINE search to identify all published cases of FNH in the pediatric population. RESULTS A total of 172 patients with FNH were identified, including 11 at our institution. The median age at diagnosis was 8.7 years and 66% were female. Median tumor size was 6 cm, and 25% had multiple lesions. Thirty-six percent were symptomatic at presentation. Twenty-four percent had a history of malignancy. Management included resection (61%), biopsy followed by observation (21%), and observation alone (18%). Indications for resection included symptoms (48%), inability to rule out malignancy (24%), tumor growth (15%), and biopsy-proven concurrent malignancy (9%). CONCLUSIONS Although FNH is a benign lesion that is typically managed nonoperatively in adults, most children with FNH currently undergo resection because of symptoms, increasing size, or inability to confidently rule out malignancy.
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Affiliation(s)
- Timothy Lautz
- Department of Surgery, Children's Memorial Hospital, Feinberg School of Medicine of Northwestern University, Chicago, IL 60614, USA.
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22
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Sugito K, Kusafuka T, Kawashima H, Uekusa S, Furuya T, Ohashi K, Inoue M, Ikeda T, Koshinaga T, Maebayashi T. Usefulness of power Doppler ultrasonography and superparamagnetic iron oxide enhanced magnetic resonance imaging for diagnosis of focal nodular hyperplasia of the liver after treatment of neuroblastoma. Pediatr Hematol Oncol 2010; 27:250-6. [PMID: 20367270 DOI: 10.3109/08880010903464206] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
Focal nodular hyperplasia (FNH) of the liver is rare in children, and it is usually diagnosed through a biopsy of the liver or hepatectomy. The authors report a case of a 10-year-old girl with multiple focal nodular hyperplasia lesions of the liver after the completion of tumor therapy for advanced neuroblastoma, and review the usefulness of the combination of power Doppler ultrasonography (US) and superparamagnetic iron oxide (SPIO) enhanced magnetic resonance imaging (MRI) for the diagnosis of FNH without a biopsy of the liver or hepatectomy.
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Affiliation(s)
- Kiminobu Sugito
- Department of Pediatric Surgery, Nihon University School of Medicine, Tokyo, Japan.
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23
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Amesur N, Hammond JS, Zajko AB, Geller DA, Gamblin TC. Management of unresectable symptomatic focal nodular hyperplasia with arterial embolization. J Vasc Interv Radiol 2009; 20:543-7. [PMID: 19328431 DOI: 10.1016/j.jvir.2009.01.001] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/23/2008] [Revised: 12/19/2008] [Accepted: 01/05/2009] [Indexed: 12/12/2022] Open
Abstract
Symptomatic focal nodular hyperplasia (FNH) of the liver can usually be treated safely with liver resection. However, in those patients in whom resection is not possible because of the location or size of the tumor or other patient factors, selective arterial embolization should be considered. Herein, the authors describe the use of arterial embolization to treat three women with symptomatic FNH and provide a review of the literature.
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Affiliation(s)
- Nikhil Amesur
- Division of Vascular & Interventional Radiology, Department of Radiology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15213, USA
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24
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Turowski C, Feist H, Alzen G, Glüer S, Petersen C. Conversion of a neonatal hepatic hemangioma to focal nodular hyperplasia. Pathol Int 2009; 59:251-4. [PMID: 19351369 DOI: 10.1111/j.1440-1827.2009.02359.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Abstract
Hepatic hemangioma and focal nodular hyperplasia are both frequently observed benign lesions of the liver. Whereas hepatic hemangioma is the most frequent benign liver tumor in children, focal nodular hyperplasia occurs predominantly in adult patients. Concomitance of both entities has been described in adults, suggesting a similar pathogenesis. We report on a 6-month-old child with a continuously shrinking hepatic hemangioma after interventional therapy and a growing hepatic mass 5 years later, which emerged as focal nodular hyperplasia at the site of the former hemangioma. Diagnostic and therapeutic strategies regarding this patient are discussed. The present case supports the theory that these two entities may share a similar pathomechanism.
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Affiliation(s)
- Carmen Turowski
- Department of Pediatric Surgery, Hannover Medical school, Hannover, Germany.
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25
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Gutweiler JR, Yu DC, Kim HB, Kozakewich HP, Marcus KJ, Shamberger RC, Weldon CB. Hepatoblastoma presenting with focal nodular hyperplasia after treatment of neuroblastoma. J Pediatr Surg 2008; 43:2297-300. [PMID: 19040959 DOI: 10.1016/j.jpedsurg.2008.08.069] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/20/2008] [Revised: 08/27/2008] [Accepted: 08/27/2008] [Indexed: 12/17/2022]
Abstract
Focal nodular hyperplasia (FNH) is a benign, poorly understood hepatic tumor that is rare in children. Although there is no evidence for malignant degeneration, FNH can occur adjacent to a malignancy. Here, the case of a 4-year-old boy with a hepatic mass and history of stage IV neuroblastoma is presented. Initial imaging and core-needle biopsy were consistent with FNH. However, after left lateral segmentectomy, pathologic examination revealed a malignant tumor most consistent with small cell undifferentiated hepatoblastoma as well as 3 foci of FNH in the surrounding parenchyma.
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Affiliation(s)
- Jordan R Gutweiler
- Department of Surgery, Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA
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26
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Focal nodular hyperplasia of the liver in patients previously treated for pediatric neoplastic diseases. J Pediatr Hematol Oncol 2008; 30:546-9. [PMID: 18797204 DOI: 10.1097/mph.0b013e3181691709] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
The discovery of a liver nodule during postcancer follow-up suggests malignancy recurrence. However, patients previously treated for pediatric cancers are at greater risk of developing benign hepatic tumors, raising the problems of getting an accurate noninvasive diagnosis. Here we report 3 cases of focal nodular hyperplasia of the liver in patients previously treated for pediatric neoplastic diseases. Radiologic findings and needle-biopsies remained insufficient in 2 cases to rule out metastasis or a potentially threatening tumor. Only surgical resection led to positive diagnosis and prevented complications. However, a review of the literature shows that if the nodule presents with typical features of focal nodular hyperplasia a close imaging follow-up is all that is recommended.
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27
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Okugawa Y, Uchida K, Inoue M, Kawamoto A, Ohtake K, Sakurai H, Uchida K, Isaji S, Miki C, Kusunoki M. Focal nodular hyperplasia in biliary atresia patient after Kasai hepatic portoenterostomy. Pediatr Surg Int 2008; 24:609-12. [PMID: 18084770 DOI: 10.1007/s00383-007-2090-8] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 11/13/2007] [Indexed: 01/11/2023]
Abstract
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.
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Affiliation(s)
- Yoshinaga Okugawa
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Tsu, Mie, 514-8507, Japan.
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28
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Hyperplasie nodulaire multifocale compliquant un cavernome porte. Arch Pediatr 2007; 14:1315-7. [DOI: 10.1016/j.arcped.2007.07.009] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2007] [Accepted: 07/09/2007] [Indexed: 11/21/2022]
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Abstract
In this review we examine the diagnosis and treatment of pediatric liver tumors- both malignant and benign. The two most common malignant tumors are hepatoblastoma and hepatocellular carcinoma. Hepatoblastoma is seen in younger children, hepatocellular carcinoma in older children. Other malignant liver tumors are quite rare and include biliary rhabdomyosarcoma, angiosarcoma, rhabdoid tumor, and undifferentiated sarcoma. The commonly seen benign liver tumors in children are infantile hemangioma, mesenchymal hamartoma, and focal nodular hyperplasia. Rare benign tumors are hepatic adenoma, which is occasionally seen in teenage girls, and teratoma which is a very rare liver tumor in infants.
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Affiliation(s)
- Rebecka L Meyers
- Chief Pediatric Surgery, University of Utah, Primary Children's Medical Center, 100 North Medical Drive, Suite 2600, Salt Lake City, UT 84113, USA.
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30
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Weinsheimer R, Watch L, Azzie G, Rozmiarek A. Laparoscopy in the management of antenatally detected liver masses. J Laparoendosc Adv Surg Tech A 2006; 15:429-31. [PMID: 16108752 DOI: 10.1089/lap.2005.15.429] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
Antenatally detected liver masses that are not clearly benign on postnatal investigation pose a management dilemma. Unless the diagnosis is clear, observation alone is risky. Improvements in radiological diagnosis permit confirmation of the benign nature of these masses in some instances, but it is usually difficult to distinguish them from malignant lesions. Since recent advances in ultrasound facilitate identification of liver masses during prenatal life, differential diagnosis of these masses has become a recurring issue in recent years. Laparoscopy may play a major role in the surgical management of right upper quadrant masses detected antenatally. We describe its use in a patient with an antenatally detected liver mass. No clear diagnosis could be made with radiologic investigation in the neonatal period. Definitive diagnosis was made laparoscopically: focal nodular hyperplasia was confirmed with laparoscopy and biopsy. In cases where the etiology of a liver mass remains unclear after radiologic investigation, laparoscopic intervention may prove beneficial in neonates and infants. We present an algorithm for the management of similar antenatally detected right upper quadrant lesions.
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Affiliation(s)
- Robert Weinsheimer
- Department of Surgery, University of New Mexico Health Sciences Center, Albuquerque, New Mexico 87131-0001, USA
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31
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Bordeianou L, Ryan DP, Goldstein AM. Focal nodular hyperplasia in a child with Beckwith-Wiedemann syndrome. Pediatr Surg Int 2005; 21:742-4. [PMID: 15965690 DOI: 10.1007/s00383-005-1469-7] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 02/23/2005] [Indexed: 12/21/2022]
Abstract
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.
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Affiliation(s)
- Liliana Bordeianou
- Department of Pediatric Surgery, Massachusetts General Hospital, Warren 1153, Boston, MA 02114, USA
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32
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Okada T, Sasaki F, Kamiyama T, Nakagawa T, Nakanishi K, Kobayashi R, Itoh T, Ota S, Todo S. Focal nodular hyperplasia of the liver: usefulness of superparamagnetic iron oxide-enhanced magnetic resonance imaging. J Pediatr Surg 2005; 40:E21-5. [PMID: 15793708 DOI: 10.1016/j.jpedsurg.2004.11.012] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Abstract
This report describes the use of superparamagnetic iron oxide-enhanced magnetic resonance imaging to diagnose fibronodular hyperplasia of the liver in a 12-year-old girl.
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Affiliation(s)
- Tadao Okada
- Department of Pediatric Surgery, 1st Surgery, Pediatrics, Pathology, Hokkaido University School of Medicine, Sapporo, 060-8638, Japan.
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33
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Bouyn CID, Leclere J, Raimondo G, Le Pointe HD, Couanet D, Valteau-Couanet D, Hartmann O. Hepatic focal nodular hyperplasia in children previously treated for a solid tumor. Incidence, risk factors, and outcome. Cancer 2003; 97:3107-13. [PMID: 12784348 DOI: 10.1002/cncr.11452] [Citation(s) in RCA: 79] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
BACKGROUND The detection of hepatic nodules, particularly in patients treated for a previous malignancy, raises a diagnostic dilemma. Focal nodular hyperplasia (FNH) of the liver is an uncommon, benign tumor in children and must be differentiated from malignant hepatic lesions. The etiology of FNH is obscure, and its pathogenesis is poorly understood. FNH may be a reaction to localized vascular abnormalities and circulatory disturbances. The goal of the current study was to identify risk factors for the occurrence of FNH in children who had received prior treatment for a malignant tumor. METHODS The current retrospective study examined 14 cases of FNH in pediatric patients who previously had been treated for a malignancy. Diagnosis was based on clinical and radiologic findings and was proven histologically in four cases. RESULTS FNH lesions were discovered by chance during routine examination in 78% of patients. The incidence of FNH was particularly high in the current series (0.45%) compared with the incidence in the general pediatric population. High doses of alkylating agents (e.g., busulfan or melphalan), venoocclusive disease, and liver radiotherapy may be responsible for injury to the vascular endothelium and subsequent localized circulatory disturbances. FNH is characterized by the absence of complications after its detection; therefore, only close follow-up is recommended. CONCLUSIONS FNH appears to be a late complication of an iatrogenic vascular disease in children with a history of malignancy.
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34
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Santos ROCD, Stracieri LDDS, Vicente YAMVDA, Garrido JJP, Pileggi FDO. Hepatectomia parcial em cirurgia pediátrica. Rev Col Bras Cir 2003. [DOI: 10.1590/s0100-69912003000100009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
OBJETIVO: Embora, atualmente, as indicações de hepatectomias em crianças sejam menos frequentes, em alguns casos elas constituem a melhor opção terapêutica. O objetivo deste trabalho é relatar a experiência de dez anos com grandes ressecções hepáticas em pacientes pediátricos. MÉTODO: Foram analisados os dados de doze pacientes submetidos a lobectomia hepática nos serviços de Cirurgia Pediátrica do Hospital da Clínicas da Faculdade de Medicina da Universidade de São Paulo e do Hospital Santa Lydia, em Ribeirão Preto (SP) de 1985 a 1995. RESULTADOS: Foram realizadas oito lobectomias esquerdas e quatro lobectomias direitas. Dez crianças foram operadas por neoplasia e duas por complicações de traumatismo hepático. A idade das dez crianças portadoras de neoplasia variou de seis dias a dezesseis meses, sendo em média 3,8 meses. O diagnóstico histopatológico foi hemangioendotelioma em cinco (50%), hepatoadenoma em dois (20%), hepatoblastoma em dois (20%) e hepatocarcinoma em um (10%). O peso do tumor correspondeu em média a 7,1% do peso do paciente. A duração média da cirurgia foi de 2 horas e 58 minutos. O seguimento pós-operatório variou até 141 meses, sendo em média 76,5 meses. Sete pacientes receberam transfusão de sangue intra-operatória, correspondente a 23,3% de sua volemia, em média. Um deles apresentou recidiva de tumor, necessitando reoperação. Nenhum dos doze pacientes apresentou complicações pós-operatórias. CONCLUSÃO: A hepatectomia parcial é um procedimento difícil tecnicamente, que, no entanto, pode ser realizado com segurança, mesmo em hospitais que não disponham de recursos tecnológicos sofisticados, desde que o cirurgião esteja bem preparado para enfrentar suas dificuldades.
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Abstract
Advances in the diagnosis and treatment of liver lesions have improved therapy for a broad range of clinical conditions, many of which could not be effectively treated in the recent past. These advances are the result of better surgical techniques as well as diagnostic imaging. This article discusses the anatomy of the liver and the clinical evaluation of patients with liver lesions. Common benign and malignant liver lesions are presented with radiologic characteristics and treatment options.
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Affiliation(s)
- J Nicolas Vauthey
- Division of Surgical Oncology, M.D. Anderson Cancer Center, Houston, Texas, USA
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36
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Kondo F. Benign nodular hepatocellular lesions caused by abnormal hepatic circulation: etiological analysis and introduction of a new concept. J Gastroenterol Hepatol 2001; 16:1319-28. [PMID: 11851827 DOI: 10.1046/j.1440-1746.2001.02576.x] [Citation(s) in RCA: 154] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Problems in definitive diagnosis and etiology of various benign nodular hepatocellular lesions were evaluated. Of these lesions, focal nodular hyperplasia (FNH), nodular regenerative hyperplasia (NRH), nodular lesions associated with idiopathic portal hypertension (IPH), non-cirrhotic large regenerative nodules (LRN), hepatocellular adenoma (HA)-like hyperplastic nodules, and partial nodular transformation (PNT) have been suggested to be related to abnormal hepatic circulation. However, the following points are considered to need further clarification: (i) is the abnormal circulation caused by thrombosis, vasculitis, or congenital anomaly?; (ii) is thrombosis a cause or a result of congestion?; (iii) are impaired blood vessels primarily the portal veins or arteries?; (iv) how are these disorders related to various syndromes, immunological abnormalities and abnormal blood flow of other organs, which are reported to coexist with these lesions often?; and (v) how should non-typical cases, which differ from typical cases, be interpreted? In addition, a concept that may lead to solving these problems (anomalous portal tract syndrome; a hypothesis that congenital vascular anomaly is the origin of these benign nodular hepatocellular lesions) was introduced.
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Affiliation(s)
- F Kondo
- Department of Molecular Pathology, Chiba University School of Medicine, Chiba, Japan.
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37
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38
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Somech R, Brazowski E, Kesller A, Weiss B, Getin E, Lerner A, Rief S. Focal nodular hyperplasia in children. J Pediatr Gastroenterol Nutr 2001; 32:480-3. [PMID: 11396818 DOI: 10.1097/00005176-200104000-00017] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Affiliation(s)
- R Somech
- Department of Pediatrics, Dana Children's Hospital, Tel Aviv, Israel
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39
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40
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Abstract
Liver tumors in children are rare, potentially complex, and encompass a broad spectrum of disease processes. Any age group may be affected, including the fetus. Most present with abdominal distension and/or a mass. Accurate preoperative diagnosis is usually possible using a combination of ultrasound scanning and cross-sectional imaging techniques (CT and/or MR), supplemented by liver biopsy and measurement of tumor markers. The most common benign tumors are hemangiomas, but mesenchymal hamartoma, focal nodular hyperplasia, and adenoma also are found. In Western countries, hepatoblastoma is the most common primary malignant liver tumor; disease-free survival is now possible in more than 80% of affected patients because of advances in combination chemotherapy, improved techniques of surgical resection, and the selective use of liver transplantation. In contrast, there has been less progress in the management of hepatocellular cancer, which still poses many therapeutic challenges.
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Affiliation(s)
- M D Stringer
- Children's Liver Centre, St James University Hospital, Leeds, UK
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41
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Mortelé KJ, Praet M, Van Vlierberghe H, Kunnen M, Ros PR. CT and MR imaging findings in focal nodular hyperplasia of the liver: radiologic-pathologic correlation. AJR Am J Roentgenol 2000; 175:687-92. [PMID: 10954451 DOI: 10.2214/ajr.175.3.1750687] [Citation(s) in RCA: 166] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Affiliation(s)
- K J Mortelé
- Department of Radiology, University Hospital Gent, De Pintelaan 185, B-9000 Gent, Belgium
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42
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Abstract
There is a remarkable diversity of conditions encompassed by benign liver masses in infants and toddlers. The most common benign hepatic tumor in this age group is infantile hepatic hemangioendothelioma. Other commonly seen benign tumors are mesenchymal hamartoma and focal nodular hyperplasia. Hepatic adenoma is almost exclusively a disease of older children; primary hepatic teratoma is exceedingly rare. There are several distinguishing characteristics of these benign tumors on radiographic evaluation; however, imaging techniques such as ultrasound scan, computed tomography, and angiography are not always reliable in differentiating benign from malignant tumors. The differential diagnosis of benign hepatic tumors includes nonneoplastic cystic masses including biliary and simple hepatic cysts, hematoma, parasitic cysts, and pyogenic and amebic liver abscess. Choledochal cyst presents with a classic triad of abdominal pain, cholestatic jaundice, and a palpable abdominal mass. They are classified anatomically into 5 subtypes with the most popular types being type I and type IV. Treatment is with complete cyst excision with hepaticojejunostomy reconstruction.
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Affiliation(s)
- R L Meyers
- Department of Surgery, University of Utah School of Medicine, Primary Children's Medical Center, Salt Lake City, USA
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Chicano Marín F, Torroba Carón A, Aranda García M, Ruiz Jiménez J, Giménez Abadía M. Hiperplasia nodular focal hepática. A propósito de un caso. An Pediatr (Barc) 2000. [DOI: 10.1016/s1695-4033(00)77339-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022] Open
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al-Salem AH, Abu-Srair H, Qaissaruddin S. Focal nodular hyperplasia of the liver with the lumbo-costovertebral syndrome. J Pediatr Surg 1996; 31:1282-4. [PMID: 8887102 DOI: 10.1016/s0022-3468(96)90251-1] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/02/2023]
Abstract
Congenital lumbar hernia is uncommon in children; only 42 cases have been reported. A newborn girl with congenital superior lumbar hernia associated with lumbo-costovertebral syndrome is described. Associated features include focal nodular hyperplasia of the liver, absent right kidney and hydrocephalus.
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Affiliation(s)
- A H al-Salem
- Department of Surgery, Qatif Central Hospital, Saudi Arabia
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