444 Cutaneous langerhans cell histiocytosis in adults: Clinical features, disease course, and management among patients treated at the Dana-Farber Cancer Institute between 2003-2017

Abstract

Langerhans Cell Histiocytosis (LCH) is a rare inflammatory neoplasm originating from myeloid dendritic cells1. Empiric treatment for LCH in adults has been described as a “roulette wheel” as there lack guidelines on staging, surveillance, and management1. Patients with cutaneous LCH may develop systemic disease and secondary hematologic malignancies; however, incidence and time to progression remains to be studied2. This is a retrospective cohort study of 31 patients (median age 53) with cutaneous LCH at the Dana-Farber Cancer Institute between 2003 to 2017 and literature review. 20 (64.5%) had cutaneous only disease. 5 (16.1%) had cutaneous LCH and developed extra-cutaneous LCH, with average time to progression of 84.2 months (range 46-131). 3 (9.7%) had extra-cutaneous LCH and then developed cutaneous LCH, with average time to onset of 45.7 months (8-84). 3 (9.7%) presented with cutaneous and extra-cutaneous LCH. 8 (26%) developed a secondary malignancy including diffuse large B cell lymphoma (n=2), breast cancer (n=1), chronic myelomonocytic leukemia (n=1), cutaneous T-cell lymphoma (n=1), melanoma (n=1), and thyroid cancer (n=2), with average time to diagnosis of 102 months (45-262). Patients were treated with a variety of skin-directed and/or systemic therapies. Literature review of cutaneous LCH in adults revealed 47 papers with 60 total cases. Our tertiary center experience represents the largest known retrospective cohort of cutaneous LCH in adults and may guide staging, surveillance, and treatment. 1. Allen CE et al. Langerhans-Cell Histiocytosis. N Engl J Med. 2018; 2. Edelbroek JR et al. Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy. Br J Dermatol. 2012.