Fernandes E T, Kumar M, Douglass E C, Wilimas J, Parham D M, Rao B N
Department of Surgery, LeBonheur Children's Medical Center, Memphis TN.
J Pediatr Surg. 1989 May;24(5):483-5. doi: 10.1016/s0022-3468(89)80407-5.
The extrarenal location of Wilms' tumor is extremely rare. These tumors can arise from other neoplasms, generally teratomas, or they can present without any associated teratomatous elements. We encountered only 19 well-documented cases of isolated extrarenal nephroblastoma, and we describe three previously unreported patients with this disease. Two of the three children presented also with horseshoe kidneys, an association that may have clinical and embryologic significance. The presence of tumor cephalad to a horseshoe kidney is easier to explain when we consider that they originate from primitive mesodermal tissue, probably mesonephric rests, and not from metanephric remnants. Also, this association should alert physicians to the possible diagnosis of extrarenal nephroblastoma in patients with a retroperitoneal mass and horseshoe kidneys. These patients should be treated according to the same protocols as those for patients with intrarenal Wilms' tumor.
肾母细胞瘤的肾外定位极为罕见。这些肿瘤可起源于其他肿瘤，通常为畸胎瘤，或者也可在无任何相关畸胎瘤成分的情况下出现。我们仅遇到19例有充分记录的孤立性肾外肾母细胞瘤病例，并且我们描述了3例此前未报道的患有该疾病的患者。这3名儿童中有2名还伴有马蹄肾，这种关联可能具有临床和胚胎学意义。当我们考虑到它们起源于原始中胚层组织，可能是中肾残余，而非后肾残余时，就更容易解释马蹄肾上方出现肿瘤的情况。此外，这种关联应提醒医生，对于患有腹膜后肿块和马蹄肾的患者，要警惕肾外肾母细胞瘤的可能诊断。这些患者应按照与肾内肾母细胞瘤患者相同的方案进行治疗。
