Langerhans cell histiocytosis of the temporal bone in children: 7 cases analysis / 临床耳鼻咽喉头颈外科杂志

ABSTRACT

OBJECTIVE@#To analyze the clinical manifestations, diagnosis, therapy and prognosis of Langerhans cell histiocytosis (LCH) of the temporal bone in children.@*METHOD@#Seven children with LCH of the temporal bone n our hospital were retrospectively summed up from April 2009 to April 2014. The patients were followed up 1-5 years, their clinical manifestations, imaging findings, diagnosis, therapy and prognosis were studied. Correlation between clinical classifications and prognosis was also analyzed.@*RESULT@#Among the 7 patients, 4 were boys and 3 were girls. 5 cases belonged to the single system group and 2 cases belonged to the multisystem group. The most common clinical characters were temporal tumor, otorrhea, otalgia, hearing loss and granulation of external auditory canal. CT of the temporal bones showed extensive osteolytic destructions with diffuse soft tissure density, without border sclerotization. The cases were. received different therapies. Followed up for 1-5 years, 4 cases were regressive, 1 case kept stable, while 2 cases showed progressive. The two boys then received standard treatment combined steroids with vinblastine. The prognosis in the multisystem group was significantly different from the single system group (P < 0.05).@*CONCLUSION@#The clinical manifestations of LCH vary a lot. The diagnosis is based on histological and immunophenotypic examination of lesion tissue. The main therapy includs surgery, chemotherapy and radiotherapy. The prognosis of the single system group is much better than the multisystem group.