Department of Gynecological Oncology, Fudan University Shanghai Cancer Center, 270 Dong-an Road, Shanghai, 200032, China.
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, 200032, China.
Sci Rep. 2022 Jun 25;12(1):10820. doi: 10.1038/s41598-022-14917-0.
Clear cell carcinoma (CCC) of the abdominal wall is a rare and agressive disease. We aim to elucidate the clinical and prognostic characteristics of this disease. Medical records of ten patients diagnosed with CCC of the abdominal wall at Fudan University Shanghai Cancer Center were reviewed. We illustrate the clinical characteristics, treatment modality, and development of local recurrence or distant metastasis, as well as the survival outcome. The median (range) age of patients was 47 (39-61) years old. All patients had a history of cesarean section and abdominal wall endometriosis. All patients had primary surgery before referred to our center. Seven patients had only tumor resection, while two patients had lymph node metastasis at primary diagnosis. Four patients underwent supplementary surgery, and all postoperative pathology were negative. Genetic analyses had also been performed. The median (range) follow-up time was 20 (12-59) months. Local recurrence and lymph node metastasis were the most common recurrence types. The median (95% confidence interval) PFS was 11 (8.08-13.92) months. In summary, primary surgery should consider wide tumor resection and lymph node dissection. Adjuvant chemotherapy and radiotherapy should be recommended for potential benefits. More cases are still needed to elucidate the clinical management of this disease.
腹壁透明细胞癌（CCC）是一种罕见且侵袭性的疾病。我们旨在阐明这种疾病的临床和预后特征。回顾了复旦大学附属肿瘤医院诊断为腹壁 CCC 的 10 例患者的病历。我们说明了临床特征、治疗方式、局部复发或远处转移的发展情况以及生存结果。患者的中位（范围）年龄为 47（39-61）岁。所有患者均有剖宫产和腹壁子宫内膜异位症病史。所有患者在转诊至我们中心之前均接受了初次手术。7 例患者仅行肿瘤切除术，而 2 例患者在初次诊断时即有淋巴结转移。4 例患者接受了补充手术，所有术后病理均为阴性。还进行了基因分析。中位（范围）随访时间为 20（12-59）个月。局部复发和淋巴结转移是最常见的复发类型。中位（95%置信区间）PFS 为 11（8.08-13.92）个月。总之，初次手术应考虑广泛的肿瘤切除术和淋巴结清扫术。应推荐辅助化疗和放疗以获得潜在益处。还需要更多的病例来阐明这种疾病的临床管理。
