Craniofacial involvement in Langerhans cell histiocytosis: A review of 44 cases at a single medical center

Tzu-En Lin; Yu-Ying Chu; Tsung-Yen Chang; Han-Tsung Liao

doi:10.1016/j.bjps.2023.03.006

Craniofacial involvement in Langerhans cell histiocytosis: A review of 44 cases at a single medical center

J Plast Reconstr Aesthet Surg. 2023 Jul:82:12-20. doi: 10.1016/j.bjps.2023.03.006. Epub 2023 Mar 21.

Authors

Tzu-En Lin¹, Yu-Ying Chu², Tsung-Yen Chang³, Han-Tsung Liao⁴

Affiliations

¹ Division of Trauma Plastic Surgery, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Linkou, Taiwan.
² Division of Trauma Plastic Surgery, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Linkou, Taiwan; Craniofacial Research Center, Chang Gung Memorial Hospital, College of Medicine, Taiwan; Chang Gung University, Taoyuan, Taiwan.
³ Division of Hematology/Oncology, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou, Taiwan.
⁴ Division of Trauma Plastic Surgery, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, Linkou, Taiwan; Craniofacial Research Center, Chang Gung Memorial Hospital, College of Medicine, Taiwan; Chang Gung University, Taoyuan, Taiwan; Department of Plastic Surgery, Xiamen Chang Gung Memorial Hospital, China. Electronic address: lia01211@gmail.com.

PMID: 37148805
DOI: 10.1016/j.bjps.2023.03.006

Abstract

Background: Craniofacial bones are the most commonly involved site of Langerhans cell histiocytosis (LCH). The main purpose of this study was to clarify the relation between subsites of craniofacial bone and clinical presentation, treatment modalities, outcomes, and permanent consequences (PCs) in patients with LCH.

Methods: Forty-four patients diagnosed with LCH involving the craniofacial region presenting at a single medical center during 2001-2019 were collected and divided into four groups: single system with unifocal bone lesion (SS-LCH, UFB); single system with multifocal bone lesions (SS-LCH, MFB); multisystem without risk organ involvement (MS-LCH, RO-); and multisystem with risk organ involvement (MS-LCH, RO+). Data including demographics, clinical presentation, treatments, outcomes, and the development of PC were retrospectively reviewed.

Results: Temporal bone (66.7% versus 7.7%, p = 0.001), occipital bone (44.4% versus 7.7%, p = 0.022), and sphenoid bone (33.3% versus 3.8%, p = 0.041) involvement were more common in SS-LCH, MFB than they were in SS-LCH, UFB. No difference of reactivation rate was noted among the four groups. The most common PC is diabetes insipidus (DI), reported in 9 of the 16 (56.25%) patients with PC. The single system group was reported with the lowest incidence of DI (7.7%, p = 0.035). The reactivation rate was also higher in patients with PC (33.3% versus 4.0%, p = 0.021) or DI (62.5% versus 3.1%, p < 0.001).

Conclusion: An increased risk of multifocal or multisystem lesions was associated with temporal bone, occipital bone, sphenoid bone, maxillary bone, eye, ear, and oral involvement, which may indicate poor outcomes. Longer follow-up may be indicated if there is the presence of PC or DI due to the high risk of reactivation. Therefore, multidisciplinary evaluation and treatment according to risk stratification are vital for patients diagnosed with LCH involving the craniofacial region.

Keywords: Bone diseases; Diabetes insipidus; Facial bones; Histiocytosis; Langerhans cell.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Histiocytosis, Langerhans-Cell* / complications
Histiocytosis, Langerhans-Cell* / diagnosis
Histiocytosis, Langerhans-Cell* / therapy
Humans
Retrospective Studies
Temporal Bone