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肾脏原发性间叶性软骨肉瘤:一例报告并文献复习

Primary mesenchymal chondrosarcoma of the kidney: A case report and review of literature.

作者信息

Valente Pedro, Macedo-Dias José António, Lobato Carlos, Reis Mário, Pina Francisco

机构信息

Department of Urology, Hospital de São João, Porto; Department of Urology, Centro Hospitalar do Tâmega e Sousa, Penafiel, Portugal.

Patology Laboratory Dr. Macedo Dias, Porto, Portugal.

出版信息

J Cancer Res Ther. 2018 Apr-Jun;14(3):694-696. doi: 10.4103/0973-1482.172121.

Abstract

Primary mesenchymal chondrosarcoma of the kidney is extremely rare, with only nine cases reported in the English literature. We report a new case of this disease. A 35-year-old man, presented with flank pain, episodic gross hematuria and a painless palpable mass in left abdominal quadrant. Computed tomography scan identified a left renal tumor with 20 cm, with no evidence of regional or metastatic spread disease. The patient underwent radical nephrectomy. The immunohistopathological diagnosis was mesenchymal chondrosarcoma of the kidney. At 18 months of follow-up, there was no evidence of recurrence or distant metastasis. Primary renal chondrosarcoma is so rare that its prognosis is unknown. Disease recurrence is unpredictable and when it is detected, the prognosis is poor. The radical nephrectomy with complete resection of the tumor with wide resection free margins is recommended, and the patients need long-term and close surveillance, with particular attention to local recurrence and uncommon sites of metastization.

摘要

肾脏原发性间叶性软骨肉瘤极为罕见,英文文献中仅报道过9例。我们报告1例该疾病的新病例。一名35岁男性,表现为胁腹疼痛、间歇性肉眼血尿以及左腹象限可触及的无痛性肿块。计算机断层扫描发现一个20厘米的左肾肿瘤,无区域或转移性扩散疾病的证据。患者接受了根治性肾切除术。免疫组织病理学诊断为肾脏间叶性软骨肉瘤。随访18个月时,无复发或远处转移的证据。原发性肾软骨肉瘤非常罕见,其预后尚不清楚。疾病复发不可预测,一旦发现,预后较差。建议行根治性肾切除术,完整切除肿瘤且切缘宽阔,患者需要长期密切监测,尤其要注意局部复发和不常见的转移部位。

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