Rais H, Elmansouri F, Belaabidia B, Essadki O, Oussehal A, Sarf I
Service de pathologie, hôpital Ibn-Tofail-Gueliz, CHU Mohammed-VI, 4000 Marrakech, Morocco.
Cancer Radiother. 2010 Apr;14(2):111-4. doi: 10.1016/j.canrad.2009.09.008. Epub 2010 Feb 26.
Desmoplastic small round cell tumors are rare aggressive cancers of adolescence and early adulthood. It has recently been separated from other small round cell tumors because of its pathological characteristics and clinical features. They are usually intra-abdominal tumors affecting young people and have classically been associated with a bad prognosis. However, in recent years there have been reports on desmoplastic small round cell tumors affecting other body regions, including the paratesticular area. We report the case of a 27-year-old male who consulted on a progressive enlargement of the right hemiscrotum. He referred no previous urological symptoms and had no systemic symptomatology. Physical examination revealed a round elastic firm 1-cm mass of the epididymis, which was excised. A computed tomography scan showed a para-aortic mass of 1cm. Histological and immunohistochemical diagnosis confirmed a desmoplastic small round cell tumor. The patient received chemotherapy. Today, 6 months after diagnosis the patient remains well and free of disease. Recent reviews on desmoplastic small round cell tumors affecting the paratesticular area have shown a better prognosis for tumors of this origin compared to abdominal ones. We should include this lesion among the differential diagnosis of paratesticular tumors, mainly in children and adolescents.
促结缔组织增生性小圆细胞肿瘤是青少年和成年早期罕见的侵袭性癌症。由于其病理特征和临床特点,它最近已从其他小圆细胞肿瘤中分离出来。它们通常是影响年轻人的腹腔内肿瘤,传统上与预后不良有关。然而,近年来有关于促结缔组织增生性小圆细胞肿瘤影响其他身体部位的报道,包括睾丸旁区域。我们报告一例27岁男性患者,因右侧阴囊渐进性肿大前来咨询。他既往无泌尿系统症状,也无全身症状。体格检查发现附睾有一个1厘米大小的圆形、有弹性、质地硬的肿块,遂将其切除。计算机断层扫描显示腹主动脉旁有一个1厘米大小的肿块。组织学和免疫组化诊断证实为促结缔组织增生性小圆细胞肿瘤。患者接受了化疗。如今,诊断后6个月,患者情况良好,无疾病迹象。最近关于影响睾丸旁区域的促结缔组织增生性小圆细胞肿瘤的综述表明,与腹部肿瘤相比,这种起源的肿瘤预后较好。我们应将这种病变纳入睾丸旁肿瘤的鉴别诊断中,主要是在儿童和青少年中。