Wu Wei, Wu Yibo, Xu Weijue, Liu Jiangbin, Lv Zhibao
Department of General Surgery, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China.
Front Surg. 2022 Feb 2;8:781060. doi: 10.3389/fsurg.2021.781060. eCollection 2021.
One of the most prevalent forms of renal tumors detected among pediatric patients is the Wilms tumor (WT). Teratoid WT is a rare WT subclassification that is characterized by teratoma-like characteristics that include the features of many diverse tissue categories. Less than 70 teratoid Wilms tumor (TWT) cases have been explained up to now.
Between 2010 and 2020, patients with classical WT and TWT admitted to our hospital were included in this study. Clinicopathological characteristics, intraoperative findings, histopathological parameters, and prognostic outcomes were then compared between classical WT and TWT. To compare these variables, TWT and WT cases were matched at a 1:3 ratio.
A total of 67 total WT cases, i.e., five diagnosed with TWT, were enrolled. While no significant differences in analyzed variables were detected between these groups, tumor volumes were notably larger in the TWT group relative to the classical WT group (203.30 ± 109.89 vs. 104.30 ± 66.97 cm) despite similar tumor weight values in both groups (471.00 ± 80.65 vs. 432.67 ± 109.25 g). As for five patients diagnosed with TWT, all were alive during the follow-up, while one of them was diagnosed with pelvic metastasis.
This study is the first to our knowledge to have reported on the incidence of TWT among Chinese children, and our results preliminarily suggest that a combination of surgery and chemotherapy may be appropriate for the treatment of patients with WT, although prognostic outcomes varied substantially among patients with different stages of the disease. TWT tumor density may be lower than classical WT tumor density. Further research regarding the basic biological characteristics of TWT and relevant theranostic markers associated with this tumor type is warranted to better guide the development of individualized treatments for this rare cancer type.
在儿科患者中检测到的最常见的肾肿瘤形式之一是肾母细胞瘤（WT）。畸胎样WT是一种罕见的WT亚分类，其特征是具有畸胎瘤样特征，包括许多不同组织类别的特征。截至目前，已报道的畸胎样肾母细胞瘤（TWT）病例少于70例。
2010年至2020年期间，我院收治的经典WT和TWT患者纳入本研究。然后比较经典WT和TWT之间的临床病理特征、术中发现、组织病理学参数和预后结果。为了比较这些变量，TWT和WT病例按1:3的比例进行匹配。
共纳入67例WT病例，其中5例诊断为TWT。虽然这些组之间在分析变量上未检测到显著差异，但TWT组的肿瘤体积相对于经典WT组明显更大（203.30±109.89 vs. 104.30±66.97 cm），尽管两组的肿瘤重量值相似（471.00±80.65 vs. 432.67±109.25 g）。对于5例诊断为TWT的患者，所有患者在随访期间均存活，其中1例被诊断为盆腔转移。
据我们所知，本研究是首次报道中国儿童TWT的发病率，我们的结果初步表明，手术和化疗相结合可能适合WT患者的治疗，尽管不同疾病阶段患者的预后结果差异很大。TWT肿瘤密度可能低于经典WT肿瘤密度。有必要进一步研究TWT的基本生物学特征以及与这种肿瘤类型相关的相关诊疗标志物，以更好地指导这种罕见癌症类型的个体化治疗的发展。
