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J Neurosurg Pediatr. 2010 Oct;6(4):393-7. doi: 10.3171/2010.7.PEDS10149.

Langerhans cell histiocytosis in a 5-month-old presenting with biparietal masses.

Author information

1
Division of Pediatric Neurosurgery, Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, California 94305-5327, USA.

Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder that occurs most commonly in the pediatric population as a result of pathological clonal proliferation of Langerhans cells with subsequent damage and destruction to surrounding tissue. Clinically, LCH presents in a variety of ways, which often results in prolonged time to diagnosis and subsequently poorer outcomes. In this case report, the authors describe an unusually early presentation of multisystem LCH in a patient at birth, which resulted in a 5-month delay to diagnosis and treatment. This patient presented both atypically young and with an uncommon initial manifestation of multisystem disease with multiple soft-tissue swellings rather than early skin involvement. Additionally, this patient had an unusual radiographic appearance with biparietal skull destruction on initial skull radiographs and biparietal soft-tissue lesions on CT resembling cephalohematoma at 3 months of age. The clinical and radiological evaluation, pathology, and treatment strategies are discussed, with particular attention paid to the importance of further workup of atypical nonresolving cephalohematomas to prevent disease progression and poorer outcomes.

PMID:
20887116
DOI:
10.3171/2010.7.PEDS10149
[Indexed for MEDLINE]

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