Langerhan’s cell histiocytosis: the great imitator—a clinical report and literature review

Background and objectives: Langerhan’s cell histiocytosis is a destructive disease ranging from solitary to generalised osteolytic lesions. This aims to emphasise awareness since most frequent presentations are within the oral and maxillofacial domain. Diagnostic challenges are described to educate clinicians on need for osseous biopsies, as evident in an initially misdiagnosed case.

Methods: The Langerhan’s spectrum in children, complications and current treatments are reviewed. A case is described of a 2 year old male with 12 month history of an enlarging left hemimandibular mass.

Results: This child underwent multiple investigations, including ultrasound which suggested a reactive enlarged lymph node and MRI which reported a mandibular lytic lesion consistent with hamartoma. Mucosal biopsy by an ENT specialist reported rhabdomyomatous hamartoma and multidisciplinary recommendation was conservative management with biannual imaging. Parental request for referral to the Oral and Maxillofacial department was made and intraosseous biopsies were taken. Intraoperatively, pathological fracture of the left mandible and exfoliation of developing tooth buds were observed. Histological analysis revealed Langerhans cell histiocytosis.

Conclusions: This case highlights inclusion of Langerhan’s cell histiocytosis in differential diagnoses of gnathic lesions and importance of appropriate tissue sampling to avoid incorrect management of this troublesome disease.

Key words: Langerhan’s; biopsy; diagnosis

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Jan 21, 2018 | Posted by in Oral and Maxillofacial Surgery | Comments Off on Langerhan’s cell histiocytosis: the great imitator—a clinical report and literature review
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