title: Dendritic cell sarcoma: a pooled analysis including 462 cases with presentation of our case series.
abstract: Dendritic cell tumors are extremely rare and current knowledge on these tumors is limited. The characteristics of three dendritic cell sarcoma subtypes and their optimal treatment approaches are not fully clarified. We aimed to make a systematic review of the literature and enrich the current data with five new cases. Pooled analysis of 462 reported cases revealed that the tumor had no age, gender or racial predilection. Our analysis suggests that the young age, advanced stage, intraabdominal involvement and unfavorable histological features (i.e. large tumor size, absence of lymphoplasmacytic infiltration, coagulative necrosis, high mitotic count) may predict poor prognosis. Subtypes of this tumor have different clinical behaviors with interdigitating dendritic cell sarcoma being the most aggressive form. In general, surgery is the most effective treatment modality and adjuvant radiotherapy has no significant effect on overall survival of patients. The role of chemotherapy for the management of advanced disease is controversial.
authors.affiliation: Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey. Electronic address: csaygin@yahoo.com.
authors.forename: Caner
authors.initials: C
authors.lastname: Saygin
authors.forename: Didem
authors.initials: D
authors.lastname: Uzunaslan
authors.forename: Mustafa
authors.initials: M
authors.lastname: Ozguroglu
authors.forename: Mustafa
authors.initials: M
authors.lastname: Senocak
authors.forename: Nukhet
authors.initials: N
authors.lastname: Tuzuner
mesh_terms: D000328:Adult; D003713:Dendritic Cells / Q000473:pathology*; D005260:Female; D015620:Histiocytic Disorders, Malignant / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012509:Sarcoma / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016896:Treatment Outcome; D047368:Tumor Burden
keywords: Clinical features; Cytokeratin-positive reticulum cell; Fibroblastic reticular cell tumor; Follicular dendritic cell sarcoma; Interdigitating dendritic cell sarcoma; Prognosis; Survival; Therapy