Tumor desmoplásico de células pequeñas y redondas. Diagnóstico y tratamiento

Q4 Medicine

Gaceta Medica de Bilbao Pub Date : 2012-07-01 DOI:10.1016/j.gmb.2012.04.003

Izaskun Markinez Gordobil, Inmaculada Ruiz, Raúl Jiménez, Eloisa Villarreal, Aintzane Lizarazu, Nerea Borda, Xabier Arteaga, Miguel Ángel Medrano, Esther Guisasola, Adolfo Beguiristain, José María E. Navascués

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引用次数: 2

Abstract

Desmoplastic small-round-cell tumor is an uncommon highly aggressive type of cancer that affects young men. We present the case of a 31-year-old man with stage iv desmoplastic small-round-cell tumor, diagnosed in the context of abdominal pain and hemoperitoneum. Surgery confirmed extensive hemoperitoneum and revealed multiple peritoneal nodules. Hemostasis was performed, a biopsy was taken, and chemotherapy was initiated. Seven months after surgery, the patient is still alive. Desmoplastic small-round-cell tumor usually occurs in the abdominal or pelvic peritoneum and is associated with nonspecific signs and symptoms but with a unique cytogenetic profile [a translocation t(11;22)(p13;q12) affecting the EWS and WT1 genes]. The best outcomes are produced by a combination of chemotherapy, radiotherapy and surgery. Nevertheless, the prognosis of desmoplastic small-round-cell tumor is poor, with an overall 5-year survival rate of 15%.

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小圆形细胞的去瘤性肿瘤。诊断与治疗

结缔组织增生小圆细胞瘤是一种罕见的高侵袭性癌症，多发于年轻男性。我们提出的情况下，31岁的男子与第四期结缔组织增生小圆细胞肿瘤，诊断的背景下腹痛和腹膜出血。手术证实有广泛的腹膜出血，并发现多个腹膜结节。止血，活检，并开始化疗。手术后七个月，病人仍然活着。结缔组织增生小圆细胞瘤通常发生在腹部或盆腔腹膜，与非特异性体征和症状相关，但具有独特的细胞遗传学特征[易位t(11;22)(p13;q12)影响EWS和WT1基因]。化疗、放疗和手术相结合的治疗效果最好。然而，结缔组织增生小圆细胞瘤的预后较差，总5年生存率为15%。

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来源期刊

Gaceta Medica de Bilbao Medicine-Medicine (all)

CiteScore

0.10

自引率

0.00%

发文量