Management of Langerhans Cell Histiocytosis of the Lateral Skull Base

Langerhans cell histiocytosis (LCH) is a rare neoplastic histiocytic disease of myeloid precursor cells that may be single or multisystemic, often involving skin and bone. Bone involvement is most common in the skull and manifests in multiple sites of focal bone destruction. LCH most commonly occurs in children aged 1 to 3 years old. After biopsy to establish diagnosis, the lesions are typically treated with systemic chemotherapy. Thus, LCH is generally not considered a surgical disease. However, in some rare cases, LCH lesions can be resistant to typical chemotherapeutics and may require surgical intervention beyond biopsy. In this very rare case, LCH occurred in the lateral skull base and was resistant to multiple rounds of chemotherapy, which resulted in significant morbidity including infection. The literature regarding LCH does not explain the therapeutic role of surgery of the lateral skull base.

A 3-year-old male developed recurrent Langerhans cell histiocytosis (LCH) of the skull and temporal bone was evaluated for persistent otorrhea and worsening mastoiditis resistant to antibiotics and in-office external ear debridement. The LCH lesions involved the lateral ear canal, the mastoid air cells, zygomatic arch, and squamosal portion of the temporal bone ([Figs. 1] and [2]). Unfortunately, the patient was also planned to undergo minimal residual disease (MRD) allogenic bone marrow transplant. A multidisciplinary tumor board discussion with various pediatric specialties including oncology, radiation oncology, neurosurgery, otolaryngology, and neurotology discussed the best management strategy.

Due to the risk of worsening mastoiditis and meningitis in a patient set to undergo immunosuppression, the panel recommended surgical management of the temporal bone followed by MRD allogenic bone marrow transplant. Patient underwent left mastoidectomy with local excision of abnormal LCH tissue and culture and debridement of infected tissues ([Fig. 3]). He tolerated the procedure well and was discharged home the same day. The surgery accomplished the goals to minimize the risk of progressive infection and potential serious neurologic complications in the setting of chemotherapy, transplantation and immunosuppression.

We believe this study addresses a gap in the literature examining the role of conservative, therapeutic surgery of LCH lesions to optimize management of infections in immunosuppressed patients.)

Publication History

Article published online:
01 February 2023