Langerhans Cell Histiocytosis of the Head and Neck: Experience at a Rural Tertiary Referral Center

Abstract

Objectives: Retrospectively analyze head and neck Langerhans Cell Histiocytosis at a rural tertiary referral center and compare results with previously published data. Methods: Electronic health record review was performed from 2003 to 2019. Patients with biopsy proven LCH with primary head and neck involvement were included. Demographics, presentation, imaging characteristics, treatment modality, delay in diagnosis (DD, ≥60 days), and outcomes were analyzed and reported. Results: Twenty-four patients were included. The most common presenting symptoms were otorrhea (n = 6) and scalp pain or swelling (n = 6). All patients had bony involvement. The most common site was facial or skull lesions (n = 20). Most skull lesions (75%) demonstrated CNS risk. Six patients were treated with primary surgery, 15 with primary chemotherapy, and 3 with surgery plus adjuvant chemotherapy. Nine patients experienced relapse of disease with median time to documented relapse of 11.4 months; all were treated with salvage chemotherapy to achieve complete remission (median follow-up: 72 months). Patients most likely to relapse were those with multisystem disease (5/7, 71.4%), temporal bone lesions (4/7, 57.1%), and DD (7/12, 58.3%). Of the 9 total patients who experienced relapse, 78% had a delay in diagnosis. Conclusions: LCH is a complex disease process in which diagnosis can be delayed if not considered in the differential. Within the head and neck, the skull, including isolated temporal bone involvement, is the most common site of involvement. Treatment modality does not appear to have an influence on relapse rates. Relapse was more likely to occur in the first year after treatment and close monitoring is required.

Calvarial Langerhans cell histiocytosis in an adult presenting rapid growth

“…[5] Furthermore, patients with delayed diagnoses are likely to relapse after successful initial treatment. [16] erefore, prompt surgical intervention should be performed for histological verification, assuming LCH as a differential diagnosis, in adult patients with osteolytic calvarial lesions. e rapid growth of the present LCH and its lymphocytic infiltration suggests that the inflammatory reaction might be associated with the local pain.…”

“…In general, such LCH is anticipated to have a good prognosis without the need for systemic therapy after successful initial treatment. [2,6,16,17] However, LCH in adults is rare and not well understood. Furthermore, most of our knowledge about its diagnosis and treatment comes from pediatric studies.…”

“…[ 5 ] Furthermore, patients with delayed diagnoses are likely to relapse after successful initial treatment. [ 16 ] Therefore, prompt surgical intervention should be performed for histological verification, assuming LCH as a differential diagnosis, in adult patients with osteolytic calvarial lesions.…”

“…[4] In general, patients with isolated LCH lesions are anticipated to have a good prognosis; however, those with multisystem involvement have a progressive disease course despite treatment. [6,16] LCH commonly develops in the pediatric population and is rare in adulthood. In addition, the differences and similarities in the long-term outcomes of pediatric and adult patients are not well known.…”