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Focal nodular hyperplasia of the liver in infancy: a case report
Journal of Pediatric Surgery (2006) 41, 456 – 457
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Focal nodular hyperplasia of the liver in infancy: a case report Giuseppe De Luca, Marcello Zamparelli*, Carlo Fadda, Antonio Martone Department of Oncology , Santobono-Pausilipon National Children’s Hospital, Napoli 80129, Italy Department of Surgery, Santobono-Pausilipon National Children’s Hospital, Napoli 80129, Italy Index words: Focal nodular hyperplasia; Hepatic tumors; Liver mass
Abstract Focal nodular hyperplasia of the liver is a benign disorder usually affecting young women. Its treatment in children is traditionally conservative. Because of its rarity in childhood, its differential diagnosis with other hepatic tumors in children and infants is challenging. We present the case of a 7-month-old infant with large focal nodular hyperplasia affecting the whole left liver lobe. Because of progressive enlargement of the lesion and compression of the portal vein, the baby underwent successful surgical treatment. Different therapeutic options and relative indications are discussed. D 2006 Elsevier Inc. All rights reserved.
1. Case report Focal nodular hyperplasia of the liver (FNH) is a benign liver neoplasia, usually affecting young women [1]. It rarely occurs in childhood [2], anecdotally reported in children younger than 2 years, accounting for 0.02% of all tumors and less than 2% of hepatic tumors in pediatrics [2]. We present here a rare case of FNH in an infant who underwent surgical treatment at the age of 13 months. A male infant, 7 months old, came to our attention for abdominal mass. We performed ultrasound (US) and computed tomography (CT) scans that revealed a solid inhomogeneous mass of the left liver lobe measuring 10 8 6.5 cm (Fig. 1). Serum levels of alpha-fetoprotein were normal.
Presented at the 52nd Annual Congress of British Association of Paediatric Surgeons, Dublin, Ireland, July 12-15, 2005. * Corresponding author. Viale Dei Pini 28-8013, Napoli, Italy. Tel.: +39 347 0897148; fax: +39 81 7444821. E-mail address: [email protected] (M. Zamparelli). 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.11.026
Fig. 1 Radiographic appearance of the infant’s left liver lobe focal nodular hyperplasia at the preoperative abdominal CT scan.
FNH of the liver in infancy
Fig. 2 Histopathology finding of FNH (hematoxylin-eosin staining, original magnification 40).
Laparotomy and biopsy were performed, and the diagnosis of FNH was made (Fig. 2). Because of the reported benignity of this lesion at this age, we planned a conservative treatment and monthly clinical and US examinations. After 6 months the mass had enlarged (12 10 7 cm) and occupied all the left liver lobe. It still appeared inhomogeneous at the US scan and encircled the left portal branch. The splenic and portal veins were patent, but were dislocated inferiorly and compressed. Because of progressive vessel dislocation, we planned a left hepatectomy (II, III, IV, and part of the I segment). Definitive histology confirmed the diagnosis of FNH. The postoperative course was uneventful, and the child is disease-free at 36 months of follow-up.
2. Discussion Focal nodular hyperplasia of the liver is a benign, nodular, noncapsulated, occasionally multifocal (15%-20%) liver mass, most frequently occupying the left liver lobe [7]. Microscopically, it consists of nodules of hyperplastic liver tissue separated by septa originating from a central fibrous scar rich in vascular tissue [2,3,7]. The diagnosis is challenging owing to its rarity, particularly in childhood. On examination the child usually presents with right upper abdominal mass; abdominal pain is occasional [2,8]. Laboratory tests are unremarkable, although the finding of normal levels of alpha-fetoprotein is indicative of a benign lesion. Typical US findings consist of a central hypoechogenic area with bright stripes irradiating in a bstar Q fashion [5,7]. Hemorrhage within a cavernous hemangioma may simulate the US appearance of a FNH [4]. Only 20% to 50% of FNH show the typical CT finding of a fibrous star irradiating from a central hypodense area [4,5]. Cases presenting
457 negative CT and positive magnetic resonance imaging (MRI) have been reported [5]. On the other hand, MRI scan has a high false-negative rate and is not fully diagnostic [6]. Definitive preoperative differential diagnosis between FNH and liver adenoma or hemangioma is particularly challenging in children who may lack the typical central fibrous scar within a liver mass [4]. Definitive diagnosis can be therefore made with a liver biopsy, preferably harvested with an open surgical approach; fine-needle biopsy presents a high risk of bleeding in cases of hepatoblastoma and may be falsely positive for liver cirrhosis [2]. Ultrasound scan and periodical CT or MRI are used to monitor the evolution of FNH [5]. Because a malignant degeneration of FNH has not been demonstrated and the complication rate (rupture, hemorrhage) is very low [2,5,7], the preferred option in adult patients is at the present the conservative treatment [5]. Conversely, in pediatric patients, because of the rarity of the disease, therapeutic indications are not as neat [4,8-10], and surgical excision is an option. In our experience, the involvement of the portal vein and a rapidly enlarging mass are indications for surgery. If, alternatively, the mass grows slowly or even tends to shrink we advice monthly US scans and occasional CT or MRI scans. The unknown and multifactorial etiology of the disease is probably responsible for the various modalities of evolution of the disease [5,9,10], making each case unique.
References [1] Gonzales F, Marks C. Hepatic tumors and oral contraceptives: surgical management. J Surg Oncol 1985;29:193 - 7. [2] Dehner LP, Parker ME, Franciosi RA, et al. Focal nodular hyperplasia and adenoma of the liver. A pediatric experience. Am J Pediatr Hematol Oncol 1979;1:85 - 93. [3] Lack EE, Ornvold K. Focal nodular hyperplasia and hepatic adenoma: a review of eight cases in the pediatric age group. J Surg Oncol 1986; 33:129 - 35. [4] Weinemann A, Ringe B, Klempnauer J, et al. Benign liver tumors: differential diagnosis and indications for surgery. World J Surg 1997; 21:983 - 90. [5] La Conte I, Vaan Beers BE, Lacrosse M, et al. Focal nodular hyperplasia: natural course observed with CT and MRI. J Comput Assist Tomogr 2000;24:61 - 6. [6] Mortele KJ, Praet M, Van Vlierberghe H, et al. CT and MR imaging findings in focal nodular hyperplasia of the liver: radiologicpathologic correlation. AJR Am J Roentgenol 2000;175:687 - 92. [7] Scatarige JC, Elliot K, Fishmann EK, et al. The sonografic bscar signQ in focal nodular hyperplasia of the liver. J Ultrasound Med 1982;1: 275 - 8. [8] Hung PL, Huang SC, Kuo HW, et al. Hepatic focal nodular hyperplasia in children: report of three cases. Chang Gung Med J 2001;24:657 - 62. [9] Altavilla G, Guariso G. Focal nodular hyperplasia of the liver associated with portal vein agenesis: a morphological and immunohistochemical study of one case and review of the literature. Adv Clin Path 1999;3:139 - 45. [10] Kinjo T, Aoki H, Sunagawa H, et al. Congenital absence of portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst. A case report. J Pediatr Surg 2001;36:622 - 5.
www.elsevier.com/locate/jpedsurg
Focal nodular hyperplasia of the liver in infancy: a case report Giuseppe De Luca, Marcello Zamparelli*, Carlo Fadda, Antonio Martone Department of Oncology , Santobono-Pausilipon National Children’s Hospital, Napoli 80129, Italy Department of Surgery, Santobono-Pausilipon National Children’s Hospital, Napoli 80129, Italy Index words: Focal nodular hyperplasia; Hepatic tumors; Liver mass
Abstract Focal nodular hyperplasia of the liver is a benign disorder usually affecting young women. Its treatment in children is traditionally conservative. Because of its rarity in childhood, its differential diagnosis with other hepatic tumors in children and infants is challenging. We present the case of a 7-month-old infant with large focal nodular hyperplasia affecting the whole left liver lobe. Because of progressive enlargement of the lesion and compression of the portal vein, the baby underwent successful surgical treatment. Different therapeutic options and relative indications are discussed. D 2006 Elsevier Inc. All rights reserved.
1. Case report Focal nodular hyperplasia of the liver (FNH) is a benign liver neoplasia, usually affecting young women [1]. It rarely occurs in childhood [2], anecdotally reported in children younger than 2 years, accounting for 0.02% of all tumors and less than 2% of hepatic tumors in pediatrics [2]. We present here a rare case of FNH in an infant who underwent surgical treatment at the age of 13 months. A male infant, 7 months old, came to our attention for abdominal mass. We performed ultrasound (US) and computed tomography (CT) scans that revealed a solid inhomogeneous mass of the left liver lobe measuring 10 8 6.5 cm (Fig. 1). Serum levels of alpha-fetoprotein were normal.
Presented at the 52nd Annual Congress of British Association of Paediatric Surgeons, Dublin, Ireland, July 12-15, 2005. * Corresponding author. Viale Dei Pini 28-8013, Napoli, Italy. Tel.: +39 347 0897148; fax: +39 81 7444821. E-mail address: [email protected] (M. Zamparelli). 0022-3468/$ – see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.11.026
Fig. 1 Radiographic appearance of the infant’s left liver lobe focal nodular hyperplasia at the preoperative abdominal CT scan.
FNH of the liver in infancy
Fig. 2 Histopathology finding of FNH (hematoxylin-eosin staining, original magnification 40).
Laparotomy and biopsy were performed, and the diagnosis of FNH was made (Fig. 2). Because of the reported benignity of this lesion at this age, we planned a conservative treatment and monthly clinical and US examinations. After 6 months the mass had enlarged (12 10 7 cm) and occupied all the left liver lobe. It still appeared inhomogeneous at the US scan and encircled the left portal branch. The splenic and portal veins were patent, but were dislocated inferiorly and compressed. Because of progressive vessel dislocation, we planned a left hepatectomy (II, III, IV, and part of the I segment). Definitive histology confirmed the diagnosis of FNH. The postoperative course was uneventful, and the child is disease-free at 36 months of follow-up.
2. Discussion Focal nodular hyperplasia of the liver is a benign, nodular, noncapsulated, occasionally multifocal (15%-20%) liver mass, most frequently occupying the left liver lobe [7]. Microscopically, it consists of nodules of hyperplastic liver tissue separated by septa originating from a central fibrous scar rich in vascular tissue [2,3,7]. The diagnosis is challenging owing to its rarity, particularly in childhood. On examination the child usually presents with right upper abdominal mass; abdominal pain is occasional [2,8]. Laboratory tests are unremarkable, although the finding of normal levels of alpha-fetoprotein is indicative of a benign lesion. Typical US findings consist of a central hypoechogenic area with bright stripes irradiating in a bstar Q fashion [5,7]. Hemorrhage within a cavernous hemangioma may simulate the US appearance of a FNH [4]. Only 20% to 50% of FNH show the typical CT finding of a fibrous star irradiating from a central hypodense area [4,5]. Cases presenting
457 negative CT and positive magnetic resonance imaging (MRI) have been reported [5]. On the other hand, MRI scan has a high false-negative rate and is not fully diagnostic [6]. Definitive preoperative differential diagnosis between FNH and liver adenoma or hemangioma is particularly challenging in children who may lack the typical central fibrous scar within a liver mass [4]. Definitive diagnosis can be therefore made with a liver biopsy, preferably harvested with an open surgical approach; fine-needle biopsy presents a high risk of bleeding in cases of hepatoblastoma and may be falsely positive for liver cirrhosis [2]. Ultrasound scan and periodical CT or MRI are used to monitor the evolution of FNH [5]. Because a malignant degeneration of FNH has not been demonstrated and the complication rate (rupture, hemorrhage) is very low [2,5,7], the preferred option in adult patients is at the present the conservative treatment [5]. Conversely, in pediatric patients, because of the rarity of the disease, therapeutic indications are not as neat [4,8-10], and surgical excision is an option. In our experience, the involvement of the portal vein and a rapidly enlarging mass are indications for surgery. If, alternatively, the mass grows slowly or even tends to shrink we advice monthly US scans and occasional CT or MRI scans. The unknown and multifactorial etiology of the disease is probably responsible for the various modalities of evolution of the disease [5,9,10], making each case unique.
References [1] Gonzales F, Marks C. Hepatic tumors and oral contraceptives: surgical management. J Surg Oncol 1985;29:193 - 7. [2] Dehner LP, Parker ME, Franciosi RA, et al. Focal nodular hyperplasia and adenoma of the liver. A pediatric experience. Am J Pediatr Hematol Oncol 1979;1:85 - 93. [3] Lack EE, Ornvold K. Focal nodular hyperplasia and hepatic adenoma: a review of eight cases in the pediatric age group. J Surg Oncol 1986; 33:129 - 35. [4] Weinemann A, Ringe B, Klempnauer J, et al. Benign liver tumors: differential diagnosis and indications for surgery. World J Surg 1997; 21:983 - 90. [5] La Conte I, Vaan Beers BE, Lacrosse M, et al. Focal nodular hyperplasia: natural course observed with CT and MRI. J Comput Assist Tomogr 2000;24:61 - 6. [6] Mortele KJ, Praet M, Van Vlierberghe H, et al. CT and MR imaging findings in focal nodular hyperplasia of the liver: radiologicpathologic correlation. AJR Am J Roentgenol 2000;175:687 - 92. [7] Scatarige JC, Elliot K, Fishmann EK, et al. The sonografic bscar signQ in focal nodular hyperplasia of the liver. J Ultrasound Med 1982;1: 275 - 8. [8] Hung PL, Huang SC, Kuo HW, et al. Hepatic focal nodular hyperplasia in children: report of three cases. Chang Gung Med J 2001;24:657 - 62. [9] Altavilla G, Guariso G. Focal nodular hyperplasia of the liver associated with portal vein agenesis: a morphological and immunohistochemical study of one case and review of the literature. Adv Clin Path 1999;3:139 - 45. [10] Kinjo T, Aoki H, Sunagawa H, et al. Congenital absence of portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst. A case report. J Pediatr Surg 2001;36:622 - 5.