Hassan Imran, Shyyan Roman, Donohue John H, Edmonson John H, Gunderson Leonard L, Moir Christopher R, Arndt Carola A S, Nascimento Antonio G, Que Florencia G
Department of Surgery, Mayo Clinic, Rochester, MN 55905, USA.
Cancer. 2005 Sep 15;104(6):1264-70. doi: 10.1002/cncr.21282.
Intraabdominal desmoplastic small round cell tumors (IDSRCT) are uncommon but aggressive tumors that occur in young males. To the authors' knowledge, only limited data are available on the natural history and optimal treatment of this disease.
The authors reviewed 12 patients with IDSRCT who were treated at their institution between January 1991 and December 2001.
All patients were males, with a median age of 26 years. All patients were symptomatic at the time of presentation, with a mean duration of symptoms of 2 months. Common presenting symptoms and signs were abdominal pain (67% of patients), palpable abdominal mass (58% of patients), abdominal distension (42% of patients), and hepatomegaly (33% of patients). Six patients (50%) had distant metastases at presentation. Five patients underwent biopsy only. Surgical resection was attempted in seven patients and included macroscopic total resection in three patients and debulking in four patients. All of those patients subsequently developed recurrent or progressive disease, which required a second operation in six patients. Overall, 6 patients (50%) developed symptomatic intestinal obstruction requiring surgical management, and 3 patients (25%) developed ureteral obstruction. All 12 patients received multiagent chemotherapy. Seven patients (55%) also received radiation therapy. The median survival of patients who underwent surgical resection was 34 months, whereas the median survival of patients who underwent biopsy alone was 14 months. One patient remained alive 72 months after he underwent complete resection of primary and recurrent tumors, and 1 patient remained alive with disease 32 months after he underwent complete resection of a primary tumor.
Patients with IDSRCT presented with a short duration of nonspecific symptoms, and the disease was fatal almost uniformly, regardless of the treatment modality used. Surgical resection may prolong survival in some patients.
腹腔促结缔组织增生性小圆细胞肿瘤（IDSRCT）虽不常见，但具有侵袭性，好发于年轻男性。据作者所知，关于该疾病的自然病史和最佳治疗方法仅有有限的数据。
作者回顾了1991年1月至2001年12月在其机构接受治疗的12例IDSRCT患者。
所有患者均为男性，中位年龄26岁。所有患者就诊时均有症状，症状平均持续时间为2个月。常见的症状和体征包括腹痛（67%的患者）、可触及的腹部肿块（58%的患者）、腹胀（42%的患者）和肝肿大（33%的患者）。6例患者（50%）就诊时已有远处转移。5例患者仅接受了活检。7例患者尝试了手术切除，其中3例患者进行了肉眼下全切，4例患者进行了减瘤手术。所有这些患者随后均出现复发或疾病进展，其中6例患者需要再次手术。总体而言，6例患者（50%）出现有症状的肠梗阻需要手术治疗，3例患者（25%）出现输尿管梗阻。所有12例患者均接受了多药化疗。7例患者（55%）还接受了放疗。接受手术切除的患者中位生存期为34个月，而仅接受活检的患者中位生存期为14个月。1例患者在接受原发肿瘤和复发肿瘤全切术后72个月仍存活，1例患者在接受原发肿瘤全切术后32个月带瘤存活。
IDSRCT患者出现非特异性症状的时间较短，无论采用何种治疗方式，该疾病几乎均会致命。手术切除可能会延长部分患者的生存期。
