Clinical and pathological features of desmoplastic small round cell tumor
- VernacularTitle:促结缔组织增生性小圆细胞瘤的临床病理特征
- Publication Type:Journal Article
- Keywords: desmoplastic small round cell tumor; pathology
- From:China Oncology 2001;0(02):-
- CountryChina
- Language:Chinese
- Abstract: Purpose: To study the clinical and pathological features of desmoplastic small round cell tumor. Methods: Clinicopathologic data of 4 cases of desmoplastic small round cell tumor were presented with review of the literature, immunohistochemical staining ( EnVision method) for CK, EMA, Desmin, Vim, NSE, Actin, etc. were performed. Results: All cases were young males. The clinical presentation was intra-abdominal in three patients and para-testicular in one patients. The connective tissue was significantly proliferated around the tumor cell nests. Immunohistochemical staining revealed the tumor exepresses CK, EMA, Desmin, Vim and NSE. The two patients without chemotherapy died 10 months and 15 months respectively after the diagnosis. The tumors of the other two patients with chemotherapy had no progress after two cycles. Conclusions: Desmoplastic small round cell tumor is a malignant sarcoma occurring usually in young males. Desmin perhaps is the most useful diagnostic marker with a perinuclear dot-like pattern. Surgical resection with chemotherapy and radiotherapy might be the proper approach to this rare malignancy.