Pimiento Figueroa Jonathan, Royero-Arias Mónica, Mejia Marcia, Garcia Elkin E
Radiology, Servicios de Salud San Vicente Fundación, Medellín, COL.
Pediatric Radiology, Servicios de Salud San Vicente Fundación, Medellín, COL.
Cureus. 2024 Apr 11;16(4):e58037. doi: 10.7759/cureus.58037. eCollection 2024 Apr.
Desmoplastic small round cell tumor (DSRCT) is a rare multifocal peritoneal sarcoma, typically found in adolescent and young adult males. Symptoms are nonspecific and vary depending on tumor involvement. Diagnosis is primarily histopathological, although imaging results can assist in the diagnostic process. Although not pathognomonic, certain radiologic findings can help narrow down potential diagnoses and sometimes suggest the condition, as seen in our cases. Treatment options are not well-established or effective, and despite employing various therapeutic approaches, the prognosis remains poor. We present two cases of boys aged 11 and 10 with a final diagnosis of DSRCT, emphasizing the imaging findings.
促结缔组织增生性小圆细胞肿瘤(DSRCT)是一种罕见的多灶性腹膜肉瘤,多见于青少年男性和青年男性。症状不具特异性,因肿瘤累及情况而异。诊断主要依靠组织病理学检查,不过影像学检查结果有助于诊断过程。虽然某些影像学表现并非具有确诊意义,但如我们病例所示,某些影像学发现可帮助缩小潜在诊断范围,有时还能提示该病。治疗方案尚未明确且效果不佳,尽管采用了多种治疗方法,预后仍然很差。我们报告两例分别为11岁和10岁男孩最终诊断为DSRCT的病例,重点介绍其影像学表现。