Farhat F, Culine S, Lhomme C, Duvillard P, Terrier-Lacombe M J, Michel G, Soulie P, Theodore C, Lotz J P, Droz J P
Département de médecine, institut Gustave-Roussy, Villejuif, France.
Bull Cancer. 1995 Aug;82(8):665-73.
Desmoplastic small round cell tumor is a recently recognized clinicopathologic entity with distinctive morphologic and immunohistochemical features. Moreover, specific cytogenetic and molecular characteristics have been described. We report here five new adult cases. The median age was 22 years. The clinical presentation was intra-abdominal in four patients and para-testicular in one patient. In patients with intra-abdominal disease, the initial surgery consisted of suboptimal debulking in three patients and only biopsies in the fourth. The patient with para-testicular tumor underwent an orchiectomy. Chemotherapy including cisplatin, etoposide, cyclophosphamide and doxorubicin or epirubicin (PAVEP/PEVEP regimen) was delivered after initial surgery in patients with intra-abdominal presentation or at relapse in the patient with para-testicular tumor. Only one persistent complete response was obtained and no salvage treatment was active in the other patients who died of progressive disease. A review of the literature concerning the clinicopathologic, cytogenetic and molecular features of this new entity is exposed.
促纤维组织增生性小圆细胞肿瘤是一种最近才被认识的具有独特形态学和免疫组化特征的临床病理实体。此外，还描述了其特定的细胞遗传学和分子特征。我们在此报告5例新的成人病例。中位年龄为22岁。临床表现为4例患者为腹腔内病变，1例患者为睾丸旁病变。腹腔内疾病患者中，3例患者的初始手术为减瘤不彻底，第4例仅进行了活检。睾丸旁肿瘤患者接受了睾丸切除术。腹腔内病变患者初始手术后或睾丸旁肿瘤患者复发时，给予包括顺铂、依托泊苷、环磷酰胺和多柔比星或表柔比星的化疗（PAVEP/PEVEP方案）。仅获得1例持续完全缓解，其他死于疾病进展的患者未发现有效的挽救治疗方法。本文对有关这一新实体的临床病理、细胞遗传学和分子特征的文献进行了综述。
