Murray J C, Minifee P K, Trautwein L M, Hicks M J, Langston C, Morad A B
Texas Children's Cancer Center and Hematology Service, Houston, USA.
J Pediatr Hematol Oncol. 1996 Aug;18(3):289-92. doi: 10.1097/00043426-199608000-00011.
An atypical case of childhood intraabdominal desmoplastic small round cell tumor (DSRCT) is presented.
An 11-year-old boy presented with progressive nausea and vomiting, abdominal pain, hepatomegaly, and an epigastric mass. Computed tomographic scanning as well as findings at gastroscopy and laparotomy revealed a large gastric mural tumor accompanied by multiple large intrahepatic masses. Histopathologic examination of biopsy samples revealed evidence of a polyphenotypic neoplasm diagnostic of DSRCT.
Unlike most reported cases of DSRCT, no evidence of peritoneal involvement or ascites was detected. Despite an excellent initial response to multiagent chemotherapy, the patient eventually died of progressive tumor.
Though the radiographic and surgical findings in DSRCT usually suggest a mesenteric, peritoneal, or retroperitoneal site of tumor origin, this case demonstrates that intraabdominal DSRCT can present with a primary visceral lesion without evidence of peritoneal or mesenteric involvement or ascites. Combination chemotherapy using vincristine, doxorubicin, cyclophosphamide, cisplatin, and 5-fluorouracil may be of some benefit to patients with this rare tumor.
报告一例儿童腹腔内促纤维增生性小圆细胞肿瘤（DSRCT）的非典型病例。
一名11岁男孩，表现为进行性恶心、呕吐、腹痛、肝肿大及上腹部肿块。计算机断层扫描以及胃镜和剖腹手术结果显示，有一个巨大的胃壁肿瘤，并伴有多个肝内大肿块。活检样本的组织病理学检查显示有证据支持诊断为DSRCT的多表型肿瘤。
与大多数报道的DSRCT病例不同，未检测到腹膜受累或腹水的证据。尽管对多药化疗最初反应良好，但患者最终死于肿瘤进展。
虽然DSRCT的影像学和手术结果通常提示肿瘤起源于肠系膜、腹膜或腹膜后部位，但该病例表明，腹腔内DSRCT可表现为原发性内脏病变，而无腹膜或肠系膜受累或腹水的证据。使用长春新碱、阿霉素、环磷酰胺、顺铂和5-氟尿嘧啶的联合化疗可能对患有这种罕见肿瘤的患者有一定益处。
