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Case 9 - Langerhans cell histiocytosis

from Section 1 - Head and neck

Published online by Cambridge University Press:  05 June 2014

By
Vy Thao Tran ,
Thomas Pulling ,
Kristen W. Yeom  and
Bo Yoon Ha
Edited by
Heike E. Daldrup-Link  and
Beverley Newman
Vy Thao Tran
Affiliation:
Stanford University
Thomas Pulling
Affiliation:
Dwight David Eisenhower Army Medical Center
Kristen W. Yeom
Affiliation:
Stanford University
Bo Yoon Ha
Affiliation:
Santa Clara Valley Medical Center
Heike E. Daldrup-Link
Affiliation:
Lucile Packard Children's Hospital, Stanford University
Beverley Newman
Affiliation:
Lucile Packard Children's Hospital, Stanford University
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Summary

Imaging description

A 10-year-old boy presented with pain and soft tissue swelling of the left cheek. A CT scan demonstrated an extensive soft tissue mass centered in the left maxilla with associated cortical erosion of the left alveolar ridge and left maxillary sinus walls. Contralateral involvement of the right alveolar ridge and anterior maxillary sinus wall was also noted. Several teeth appeared to be “floating” within the maxilla. There was absence of adjacent soft tissue fat stranding (Fig. 9.1a–c).

A five-month-old infant presented with bi-parietal scalp swelling. CT of the head showed bi-parietal calvarial lytic lesions with associated soft tissue masses and a characteristic “beveled edge” appearance with greater destruction of the outer table of the calvarium relative to the inner table (Fig. 9.2a, b). On MRI, mixed T1 and T2 intensities and heterogeneous enhancement of the soft tissue mass was seen (Fig. 9.2c–e).

Langerhans cell histiocytosis (LCH) was confirmed pathologically in both cases.

Type
Chapter
Information
Pearls and Pitfalls in Pediatric Imaging
Variants and Other Difficult Diagnoses
 , pp. 25 - 29
Publisher: Cambridge University Press
Print publication year: 2014

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References

Azouz, EM, Saigal, G, Rodriguez, MM, et al. Langerhans’ cell histiocytosis: pathology, imaging and treatment of skeletal involvement. Pediatr Radiol 2005;35(2):103–15.CrossRefGoogle ScholarPubMed
Demaerel, P, Van Gool, S. Paediatric neuroradiological aspects of Langerhans cell histiocytosis. Neuroradiology 2008;50(1):85–92.CrossRefGoogle ScholarPubMed
Hoffbrand, AV, Arceci, RJ, Hann, IM, Smith, OP. Pediatric Hematology. Malden, Massachusetts: Blackwell Publishing, Inc., 2006.Google Scholar
Johnson, K, Hobin, D. Langerhans cell histiocytosis. In: Davies, AM, Sundaram, M, James, SLJ, eds. Imaging of Bone Tumors and Tumor Like Conditions. Heidelberg: Springer, 2009; 447–59.CrossRefGoogle Scholar
Stull, MA, Kransdorf, MJ, Devaney, KO. From the archives of the AFIP Langerhans cell histiocytosis of bone. Radiographics 1992;12(4):801–23.CrossRefGoogle Scholar

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