Igbaseimokumo Usiakimi, Cartwright Cathy, Lewing Karen, Hutchison Lisa, Habeebu Sultan
University of Missouri Kansas City School of Medicine & Children's Mercy Hospital, Kansas City, Missouri, USA.
University of Missouri Kansas City School of Medicine & Children's Mercy Hospital, Kansas City, Missouri, USA.
World Neurosurg. 2017 Aug;104:1046.e1-1046.e5. doi: 10.1016/j.wneu.2017.03.115. Epub 2017 May 3.
The diagnosis of nephroblastoma outside of the kidneys, in the absence of a renal primary tumor, is known as extrarenal Wilms tumor (ERWT). ERWT is an uncommon entity that typically involves the embryonic path of the developing kidneys and gonads. The occurrence of ERWT in a dysraphic spine is uncommon, with no reported cases of preoperative diagnosis, with all cases diagnosed at pathology. These tumors are malignant and ideally should be completely excised. Thus, preoperative diagnosis would be highly desirable.
A newborn female was found to have a lumbar lipoma. Magnetic resonance imaging (MRI) was performed to rule out lipomyelomeningocele. The MRI showed a dorsal lipoma on the terminal spinal cord, as well as a 2 × 2 cm uniformly enhancing mass abutting the bifid posterior elements of L5. The lesion was completely excised, and the pathological diagnosis was ERWT. We report this case with a review of the literature to raise awareness of this association, illustrate the key imaging findings, and document the clinical outcome.
The lack of pathognomonic radiologic features makes the preoperative diagnosis extremely difficult, but a diagnosis of ERWT should be considered in the context of a soft tissue mass without the typical imaging features of a hemangioma or teratoma.
在没有肾脏原发性肿瘤的情况下，肾脏外肾母细胞瘤的诊断被称为肾外威尔姆斯瘤（ERWT）。ERWT是一种罕见的实体瘤，通常累及发育中的肾脏和性腺的胚胎路径。ERWT发生于脊柱裂的情况并不常见，术前诊断的病例未见报道，所有病例均在病理检查时确诊。这些肿瘤是恶性的，理想情况下应完全切除。因此，术前诊断非常必要。
一名新生女婴被发现患有腰部脂肪瘤。进行了磁共振成像（MRI）以排除脂肪脊髓脊膜膨出。MRI显示终丝上有一个背部脂肪瘤，以及一个2×2cm均匀强化的肿块，毗邻L5的双侧后弓。病变被完全切除，病理诊断为ERWT。我们报告该病例并复习文献，以提高对这种关联的认识，阐明关键的影像学表现，并记录临床结果。
缺乏特征性的影像学表现使得术前诊断极其困难，但在软组织肿块无典型血管瘤或畸胎瘤影像学特征的情况下，应考虑ERWT的诊断。
