Department of Clinical Radiology, The Royal Marsden Hospital NHS Foundation Trust, Fulham Road, London, SW3 6JJ, UK,
Insights Imaging. 2013 Feb;4(1):111-8. doi: 10.1007/s13244-012-0212-x. Epub 2013 Jan 10.
Desmoplastic small round cell tumours (DSRCTs) are rare aggressive tumours of young adults that present late and have poor prognosis. This review discusses distinctive radiological features, histopathology and clinical course of this soft-tissue sarcoma.
From 1991 to 2012, the radiology of 20 patients with pathologically proven DSRCT was independently reviewed by two experienced radiologists. The clinical presentation, treatment and outcome were recorded.
16 men, four women; mean age 28.3 years. Computed tomography (CT) demonstrated peritoneal/omental masses without an organ of origin (94 %), with the majority of cases demonstrating large (>5 cm) dominant soft-tissue deposit (80 %) with multiple smaller foci. CT and magnetic resonance imaging (MRI) typically demonstrated heterogeneous soft-tissue enhancement with cystic degeneration. A minority (20 %) demonstrated calcification. Lymph node enlargement occurred in 50 % of cases. Distant metastatic disease occurred in 25 %. Painful abdominal masses were clinically predominant. Treatment strategies include combination chemotherapy with debulking surgery and/or radiotherapy. Median survival from diagnosis was 22.8 months.
Features of multifocal peritoneal/omental masses, usually in combination with a dominant soft tissue deposit, are distinctive in this rare sarcoma. CT/MRI defines the extent of disease and characterises supporting imaging findings. Prolific desmoplastic reaction histologically separates DSRCT from similar subtypes. Combination treatment strategies can infer a survival benefit but prognosis remains poor. TEACHING POINTS : • DSRCTs are rare tumours of young adults (mean age 28.3 years) with a male predominance (4:1). • Painful abdominal masses clinically predominate. Non-specific features of malignancy can be present. • Multifocal peritoneal masses with a dominant soft tissue lesion is a distinctive imaging finding. • A large desmoplastic reaction differentiates DSRCTs from histologically similar round cell subtypes. • Despite debulking surgery with adjuvant chemotherapy, median survival from diagnosis is 22.3 months.
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见的侵袭性软组织肉瘤，主要发生于青年，起病隐匿，预后较差。本文讨论了该肿瘤的影像学特征、组织病理学和临床病程。
1991 年至 2012 年，两位经验丰富的放射科医生对 20 例经病理证实的 DSRCT 患者的影像学资料进行了独立回顾。记录患者的临床表现、治疗方法及转归。
男性 16 例，女性 4 例；平均年龄 28.3 岁。CT 表现为腹膜/网膜肿块，无明确的起源器官（94%），大多数病例表现为大（＞5cm）的软组织肿块（80%），伴多个较小的病灶。CT 和 MRI 表现为不均匀软组织强化，伴囊性变。少数病例（20%）有钙化。50%的病例有淋巴结肿大。25%的病例有远处转移。腹痛伴腹部肿块为最常见的临床表现。治疗策略包括联合化疗、肿瘤减灭术和/或放疗。从诊断到死亡的中位生存时间为 22.8 个月。
弥漫性腹膜/网膜多灶性肿块，通常伴大的软组织肿块，是这种罕见肉瘤的特征性表现。CT/MRI 可明确病变范围，有助于发现特征性影像学表现。丰富的促结缔组织增生反应有助于将 DSRCT 与其他组织学亚型区分开来。联合治疗策略可能有助于改善生存，但预后仍较差。
DSRCT 是一种罕见的软组织肉瘤，主要发生于青年（平均年龄 28.3 岁），男性多见（4:1）。
腹痛伴腹部肿块为最常见的临床表现。恶性肿瘤的非特异性表现可能存在。
弥漫性腹膜肿块伴大的软组织肿块是一种具有特征性的影像学表现。
丰富的促结缔组织增生反应有助于将 DSRCT 与其他组织学亚型区分开来。
尽管进行了肿瘤减灭术和辅助化疗，从诊断到死亡的中位生存时间仍为 22.3 个月。
