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Langerhans cell histiocytosis in a 5-month-old presenting with biparietal masses
Katie L Pricola1, Jason Karamchandani, Hannes Vogel
1Division of Pediatric Neurosurgery, Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, California 94305-5327, USA.
Insights
This case report details an infant diagnosed with multisystem Langerhans cell histiocytosis (LCH) at birth. Early recognition of atypical presentations is crucial for timely treatment and improved outcomes in pediatric LCH.
Area of Science:
- Pediatric Pathology
- Histiocytic Disorders
- Rare Childhood Diseases
Background:
- Langerhans cell histiocytosis (LCH) is a rare pediatric disorder involving clonal Langerhans cell proliferation and tissue damage.
- Clinical presentations of LCH are diverse, often leading to delayed diagnosis and poorer prognoses.
Observation:
- This report describes an infant with an unusually early, at-birth presentation of multisystem LCH.
- The patient exhibited atypical initial symptoms including soft-tissue swellings and unusual skull lesions resembling cephalohematoma, delaying diagnosis by 5 months.
Findings:
- Multisystem LCH can present atypically in neonates, challenging early diagnosis.
- Radiographic findings like biparietal skull destruction and soft-tissue lesions require thorough investigation.
Implications:
- Emphasizes the need for comprehensive workup of non-resolving cephalohematomas in infants.
- Highlights the importance of recognizing atypical LCH presentations for prompt intervention and better patient outcomes.
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.