Clinicopathological characteristics and differential diagnosis of interdigitating dendritic cell sarcoma
10.3760／cma.j.issn.0253?3766.2019.06.010
- VernacularTitle:指状突树突细胞肉瘤的临床病理特征和鉴别诊断
- Author:
Yuelu ZHU
1
;
Tian QIU
;
Haifeng ZHANG
;
Jianming YING
;
Hongtu ZHANG
Author Information
1. 国家癌症中心 国家肿瘤临床医学研究中心 中国医学科学院北京协和医学院肿瘤医院病理科100021
- Keywords:
Interdigitating dendritic cell sarcoma;
Clinical pathological features;
Diagnosis;
Diagnosis,differential
- From:
Chinese Journal of Oncology
2019;41(6):449-453
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinicopathological features and differential diagnosis of interdigitating dendritic cell sarcoma (IDCS). Methods The clinical pathological features of 7 IDCS were analyzed. Among them, the follow?up results of 6 cases were available. Results Among the 7 IDCS patients, 4 cases were male and 3 were female. The age of the patients ranged from 26 to 69 years.Three cases were originated from lymph nodes and 4 cases were originated from skin, stomach, adrenal gland and mesentery, respectively. Microscopically, the tumor cells presented as fascicular and storiform proliferation and infiltrated by lymphocytes. The tumor cells were short?spindle or ovoid, with indistinct border of cytoplasm. The immunohistochemistry results showed that tumor cells were S?100, Vim, CD68 and CD163 positive, and AE1／AE3, EMA, CD117, CD34, Desmin, SMA, CD1α, CD21, CD23, CD35, HMB45, Melan?A, MelanPan and ALK negative.The BRAF mutation and clonal rearrangement of T and B cells were not detected. Among the follow?up period of 7 IDCS patients, 3 occurred disease progressions. Conclusions IDCS is extremely rare with unique pathological features, and its lesion is not limited to the lymph node. The IDCS patients with extensive lesions may have worse prognose. The differential diagnosis of IDCS includes other histiocytic and dendritic cell neoplasms, malignant melanoma and soft tissue neoplasms.
