Multifocal Unisystem LCH
Multifocal unisystem Langerhans cell histiocytosis (LCH) involves multiple sites within a single organ system. It includes most cases of Hand-Schuller-Christian disease and usually occurs in young children as multiple destructive bone lesions with adjacent soft tissue involvement. Skull and mandible are usually the affected sites. About 50% of patients develop diabetes insipidus due to hypothalamic-posterior pituitary mass lesion.
The Hand-Schuller-Christian triad consists of classic combination of a osteolytic skull lesions, diabetes insipidus, and exophthalmos. It is present in only a few patients with chronic disseminated disease. Involvement of mastoid or petrous temporal bone may lead to chronic otitis externa. Many patients go into spontaneous remission. The rest can be treated with a chemotherapy and/or BRAF inhibitors like vemurafenib.
Slide courtesy of Piero Picci, M.D., Director, Laboratory of Experimental Oncology, Instituto Ortopedico Rizzoli, Bologna, Italy. Used with permission.