Skip to main content
Top
Published in: Pediatric Radiology 6/2014

01-06-2014 | Original Article

Hepatic involvement of Langerhans cell histiocytosis in children—imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

Authors: Yingyan Shi, Zhongwei Qiao, Chunmei Xia, Ying Gong, Haowei Yang, Guoping Li, Mier Pa

Published in: Pediatric Radiology | Issue 6/2014

Login to get access

Abstract

Background

Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor.

Objective

To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP).

Materials and methods

Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement.

Results

Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses.

Conclusion

Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children.
Literature
1.
go back to reference Schmidt S, Eich G, Geoffray A et al (2008) Extraosseous Langerhans cell histiocytosis in children. Radiographics 28:707–726, quiz 910–911PubMedCrossRef Schmidt S, Eich G, Geoffray A et al (2008) Extraosseous Langerhans cell histiocytosis in children. Radiographics 28:707–726, quiz 910–911PubMedCrossRef
2.
go back to reference Weitzman S, Egeler RM (2008) Langerhans cell histiocytosis: update for the pediatrician. Curr Opin Pediatr 20:23–29PubMedCrossRef Weitzman S, Egeler RM (2008) Langerhans cell histiocytosis: update for the pediatrician. Curr Opin Pediatr 20:23–29PubMedCrossRef
3.
go back to reference Schmidt S, Eich G, Hanquinet S et al (2004) Extra-osseous involvement of Langerhans’ cell histiocytosis in children. Pediatr Radiol 34:313–321PubMedCrossRef Schmidt S, Eich G, Hanquinet S et al (2004) Extra-osseous involvement of Langerhans’ cell histiocytosis in children. Pediatr Radiol 34:313–321PubMedCrossRef
4.
go back to reference Abdallah M, Genereau T, Donadieu J et al (2011) Langerhans’ cell histiocytosis of the liver in adults. Clin Res Hepatol Gastroenterol 35:475–481PubMedCrossRef Abdallah M, Genereau T, Donadieu J et al (2011) Langerhans’ cell histiocytosis of the liver in adults. Clin Res Hepatol Gastroenterol 35:475–481PubMedCrossRef
5.
go back to reference Chan YL, Li CK, Lee CY (1997) Sonographic appearance of hepatic Langerhans cell histiocytosis. Clin Radiol 52:761–763PubMedCrossRef Chan YL, Li CK, Lee CY (1997) Sonographic appearance of hepatic Langerhans cell histiocytosis. Clin Radiol 52:761–763PubMedCrossRef
6.
go back to reference Chaudhary A, Debnath J, Thulkar S et al (2006) Imaging findings in hepatic Langerhans’ cell histiocytosis. Indian J Pediatr 73:1036–1038PubMedCrossRef Chaudhary A, Debnath J, Thulkar S et al (2006) Imaging findings in hepatic Langerhans’ cell histiocytosis. Indian J Pediatr 73:1036–1038PubMedCrossRef
7.
go back to reference Kilborn TN, Teh J, Goodman TR (2003) Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol 58:269–278PubMedCrossRef Kilborn TN, Teh J, Goodman TR (2003) Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings. Clin Radiol 58:269–278PubMedCrossRef
8.
go back to reference Kim M, Lyu C, Jin Y et al (1999) Langerhans’ cell histiocytosis as a cause of periportal abnormal signal intensity on MRI. Abdom Imaging 24:373–377PubMedCrossRef Kim M, Lyu C, Jin Y et al (1999) Langerhans’ cell histiocytosis as a cause of periportal abnormal signal intensity on MRI. Abdom Imaging 24:373–377PubMedCrossRef
9.
go back to reference Radin DR (1992) Langerhans cell histiocytosis of the liver: imaging findings. AJR Am J Roentgenol 159:63–64PubMedCrossRef Radin DR (1992) Langerhans cell histiocytosis of the liver: imaging findings. AJR Am J Roentgenol 159:63–64PubMedCrossRef
10.
go back to reference Arakawa A, Matsukawa T, Yamashita Y et al (1994) Periportal fibrosis in Langerhans’ cell histiocytosis mimicking multiple liver tumors: US, CT, and MR findings. J Comput Assist Tomogr 18:157–159PubMedCrossRef Arakawa A, Matsukawa T, Yamashita Y et al (1994) Periportal fibrosis in Langerhans’ cell histiocytosis mimicking multiple liver tumors: US, CT, and MR findings. J Comput Assist Tomogr 18:157–159PubMedCrossRef
11.
go back to reference Wong A, Ortiz-Neira CL, Reslan WA et al (2006) Liver involvement in Langerhans cell histiocytosis. Pediatr Radiol 36:1105–1107PubMedCrossRef Wong A, Ortiz-Neira CL, Reslan WA et al (2006) Liver involvement in Langerhans cell histiocytosis. Pediatr Radiol 36:1105–1107PubMedCrossRef
12.
go back to reference Caruso S, Miraglia R, Maruzzelli L et al (2008) Biliary wall calcification in Langerhans cell histiocytosis: report of two cases. Pediatr Radiol 38:791–794PubMedCrossRef Caruso S, Miraglia R, Maruzzelli L et al (2008) Biliary wall calcification in Langerhans cell histiocytosis: report of two cases. Pediatr Radiol 38:791–794PubMedCrossRef
13.
go back to reference Kaplan KJ, Goodman ZD, Ishak KG (1999) Liver involvement in Langerhans’ cell histiocytosis: a study of nine cases. Mod Pathol 12:370–378PubMed Kaplan KJ, Goodman ZD, Ishak KG (1999) Liver involvement in Langerhans’ cell histiocytosis: a study of nine cases. Mod Pathol 12:370–378PubMed
14.
go back to reference Meyer JS, De Camargo B (1998) The role of radiology in the diagnosis and follow-up of Langerhans cell histiocytosis. Hematol Oncol Clin N Am 12:307–326CrossRef Meyer JS, De Camargo B (1998) The role of radiology in the diagnosis and follow-up of Langerhans cell histiocytosis. Hematol Oncol Clin N Am 12:307–326CrossRef
15.
go back to reference Jaffe R (2004) Liver involvement in the histiocytic disorders of childhood. Pediatr Dev Pathol 7:214–225PubMed Jaffe R (2004) Liver involvement in the histiocytic disorders of childhood. Pediatr Dev Pathol 7:214–225PubMed
16.
go back to reference Ito K, Mitchell DG, Outwater EK et al (1999) Primary sclerosing cholangitis: MR imaging features. AJR Am J Roentgenol 172:1527–1533PubMedCrossRef Ito K, Mitchell DG, Outwater EK et al (1999) Primary sclerosing cholangitis: MR imaging features. AJR Am J Roentgenol 172:1527–1533PubMedCrossRef
17.
go back to reference Konig CW, Pfannenberg C, Trubenbach J et al (2001) MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans’ cell histiocytosis. Eur Radiol 11:2516–2520PubMedCrossRef Konig CW, Pfannenberg C, Trubenbach J et al (2001) MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans’ cell histiocytosis. Eur Radiol 11:2516–2520PubMedCrossRef
18.
go back to reference Siegel MJ, Herman TE (1992) Periportal low attenuation at CT in childhood. Radiology 183:685–688PubMed Siegel MJ, Herman TE (1992) Periportal low attenuation at CT in childhood. Radiology 183:685–688PubMed
19.
go back to reference Braier J, Ciocca M, Latella A et al (2002) Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell Histiocytosis. Med Pediatr Oncol 38:178–182PubMedCrossRef Braier J, Ciocca M, Latella A et al (2002) Cholestasis, sclerosing cholangitis, and liver transplantation in Langerhans cell Histiocytosis. Med Pediatr Oncol 38:178–182PubMedCrossRef
20.
go back to reference Textor HJ, Flacke S, Pauleit D et al (2002) Three-dimensional magnetic resonance cholangiopancreatography with respiratory triggering in the diagnosis of primary sclerosing cholangitis: comparison with endoscopic retrograde cholangiography. Endoscopy 34:984–990PubMedCrossRef Textor HJ, Flacke S, Pauleit D et al (2002) Three-dimensional magnetic resonance cholangiopancreatography with respiratory triggering in the diagnosis of primary sclerosing cholangitis: comparison with endoscopic retrograde cholangiography. Endoscopy 34:984–990PubMedCrossRef
21.
go back to reference Heyn RM, Hamoudi A, Newton WA Jr (1990) Pretreatment liver biopsy in 20 children with histiocytosis X: a clinicopathologic correlation. Med Pediatr Oncol 18:110–118PubMedCrossRef Heyn RM, Hamoudi A, Newton WA Jr (1990) Pretreatment liver biopsy in 20 children with histiocytosis X: a clinicopathologic correlation. Med Pediatr Oncol 18:110–118PubMedCrossRef
22.
go back to reference Mampaey S, Warson F, Van Hedent E et al (1999) Imaging findings in Langerhans’ cell histiocytosis of the liver and the spleen in an adult. Eur Radiol 9:96–98PubMedCrossRef Mampaey S, Warson F, Van Hedent E et al (1999) Imaging findings in Langerhans’ cell histiocytosis of the liver and the spleen in an adult. Eur Radiol 9:96–98PubMedCrossRef
Metadata
Title
Hepatic involvement of Langerhans cell histiocytosis in children—imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography
Authors
Yingyan Shi
Zhongwei Qiao
Chunmei Xia
Ying Gong
Haowei Yang
Guoping Li
Mier Pa
Publication date
01-06-2014
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 6/2014
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-014-2886-3

Other articles of this Issue 6/2014

Pediatric Radiology 6/2014 Go to the issue

Hermes

Hermes