Diagnosis and management of nasal and pharyngeal dendritic cell tumor-case report and literature review / 临床耳鼻咽喉头颈外科杂志

ABSTRACT

OBJECTIVE@#To investigate the clinical manifestation, pathological characteristics, treatment and prognosis of dendritic cell tumor.@*METHOD@#Four cases of nasal and pharyngeal dendritic cell tumor were described, including two cases of follicular dendritic cell sarcoma (FDCS), one case of Langerhans cell histiocytosis (LCH) and one case of Langer hans cell sarcoma (LCS). One of the patients with FDCS received multimodality therapy (surgery combined with chemotherapy), and the other patient only received chemotherapy and radiotherapy. The patients with LCH or LSC were treated by surgery.@*RESULT@#Of the two FDCS patients, one achieved complete remission after treatment by surgery combined with four cycles of CHOP chemotherapy regimen and concurrent radiotherapy (50 Gy), and the other who only received chemotherapy and radiotherapy survived with tumor for more than seven months of follow up. The patient of LCH was followed up for more than 2 years after surgery without recurrence or metastasis. The patient of LCS did not undergo radiotherapy or chemotherapy after surgery and died after 10 months of follow up.@*CONCLUSION@#Dendritic cell tumor is a group of very rare tumor and can be easily misdiagnosed in clinic, the confirmed diagnosis of which relies on histopathological features, immunohistochemistry combined with electron microscopy. FDCS, LCH and LCS have different pathological features, immunophenotypes and prognosis.