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腹部促结缔组织增生性小圆细胞肿瘤:一例报告。

Desmoplastic small round cell tumor of the abdomen: A case report.

机构信息

Department of Gastrointestinal Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

出版信息

Medicine (Baltimore). 2023 Nov 10;102(45):e35965. doi: 10.1097/MD.0000000000035965.

Abstract

RATIONALE

Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor with poor prognosis, usually involving the peritoneum. There are currently no standardized treatment approaches. This study helped to further advance our understanding of DSRCT, and help to guide therapy.

PATIENT CONCERNS

The patient, a 19-year-old male, presented with left-sided back pain with no obvious cause and occasional abdominal pain, and underwent abdominal electron computed tomography examination in our hospital suggesting consideration of small bowel mesenchymal tumor with possible multiple implantation metastasis in the abdominopelvic cavity.

DIAGNOSES

After surgical treatment, the pathology report suggested a DSRCT, and immunohistochemistry and fluorescence in situ hybridization revealed EWSR1-WT1 gene rearrangement. Lung computer tomography and abdominal magnetic resonance imaging performed half a month later showed multiple solid nodules on the proximal septal surface of the right lung base, right posterior cardiac/right anterior inferior vena cava nodules, and multiple nodules in the abdominopelvic cavity, omenta, peritoneum, and around the liver or liver, all of which were considered as metastatic foci.

INTERVENTIONS AND OUTCOMES

Patient received 5 cycles of chemotherapy after surgery. The review results showed a smaller size than before. Currently, he continues to receive treatment.

LESSONS

The reported case has raised awareness of the importance of DSRCT in the treatment of chemotherapy, including its role in the differential diagnosis of abdominal tumors.

摘要

背景

促结缔组织增生性小圆细胞肿瘤(DSRCT)是一种罕见的恶性肿瘤,预后较差,通常累及腹膜。目前尚无标准化的治疗方法。本研究有助于进一步提高我们对 DSRCT 的认识,并有助于指导治疗。

患者情况

患者为 19 岁男性,表现为左侧腰痛,无明显原因,偶尔腹痛,在我院行腹部电子计算机断层扫描检查提示考虑小肠间叶性肿瘤,可能伴有腹腔盆腔多发种植转移。

诊断

经手术治疗后,病理报告提示 DSRCT,免疫组化和荧光原位杂交显示 EWSR1-WT1 基因重排。术后半个月行肺计算机断层扫描和腹部磁共振成像显示右肺基底近隔面多发实性结节,右心后/下腔静脉右前结节,以及腹腔、网膜、腹膜和肝周或肝内多发结节,均考虑为转移灶。

干预及结果

患者术后接受了 5 个周期的化疗。复查结果显示肿瘤较前缩小。目前,他仍在继续接受治疗。

启示

本报告病例提高了对 DSRCT 在化疗治疗中的重要性的认识,包括其在腹部肿瘤鉴别诊断中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/086b/10637438/45addd8a2145/medi-102-e35965-g001.jpg

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