Bayindir Petek, Guillerman Robert Paul, Hicks M John, Chintagumpala M Murali
Department of Radiology, Ege University Medical Faculty, AD Bornova, Izmir 35100, Turkey.
Pediatr Radiol. 2009 Oct;39(10):1066-74. doi: 10.1007/s00247-009-1348-9. Epub 2009 Jul 21.
Cellular mesoblastic nephroma has been associated with a more aggressive course than classic mesoblastic nephroma, including local recurrences and metastases.
To define the clinicopathologic and imaging features distinguishing cellular from classic mesoblastic nephroma.
Retrospective review of clinical charts and imaging studies of ten children with mesoblastic nephroma from 1996 to 2007 at a large children's hospital.
In six children the mesoblastic nephroma was pure cellular, in two mixed, and in two classic. The mean ages at diagnosis were 107 days for those with the cellular form, and 32 days for those with the classic form. Hypoechoic or low-attenuation regions representing necrosis or hemorrhage were found in all children with the cellular form and in none of those with the classic form. Hypertension was present in 70% and hypercalcemia in 20% of the children and resolved following nephrectomy. Two cellular tumors encased major abdominal vessels. Local recurrence and metastases occurred within 6 months of tumor resection in two children with the cellular form. Intraspinal extension and intratumoral pseudoaneurysm were seen in one child with the cellular form. The cellular tumors shared histopathologic features with infantile fibrosarcoma (IFS), and RT-PCR testing in two children with the cellular form revealed the t(12;15) ETV6-NTRK3 gene fusion common to IFS.
Distinct from the classic form, cellular mesoblastic nephroma is more heterogeneous in appearance on imaging, tends to be larger and present later in infancy, and can exhibit aggressive behavior including vascular encasement and metastasis. Intraspinal extension and intratumoral pseudoaneurysm are previously unreported findings encountered in our cellular mesoblastic nephroma series. The shared histopathology and translocation gene fusion support the concept of cellular mesoblastic nephroma as the renal form of IFS.
与经典型中胚叶肾瘤相比，细胞型中胚叶肾瘤病程更具侵袭性，包括局部复发和转移。
明确区分细胞型中胚叶肾瘤与经典型中胚叶肾瘤的临床病理及影像学特征。
回顾性分析1996年至2007年在一家大型儿童医院就诊的10例中胚叶肾瘤患儿的临床病历及影像学检查资料。
6例患儿为单纯细胞型中胚叶肾瘤，2例为混合型，2例为经典型。细胞型中胚叶肾瘤患儿的平均诊断年龄为107天，经典型患儿为32天。所有细胞型中胚叶肾瘤患儿均发现代表坏死或出血的低回声或低衰减区域，而经典型患儿均未发现。70%的患儿出现高血压，20%出现高钙血症，肾切除术后均缓解。2例细胞型肿瘤包绕腹部主要血管。2例细胞型中胚叶肾瘤患儿在肿瘤切除后6个月内出现局部复发和转移。1例细胞型中胚叶肾瘤患儿出现脊髓内扩展和瘤内假性动脉瘤。细胞型肿瘤与婴儿纤维肉瘤（IFS）具有共同的组织病理学特征，2例细胞型中胚叶肾瘤患儿的逆转录聚合酶链反应（RT-PCR）检测显示存在IFS常见的t(12;15) ETV6-NTRK3基因融合。
与经典型不同，细胞型中胚叶肾瘤在影像学上表现更不均匀，往往体积更大且在婴儿期较晚出现，可表现出侵袭性行为，包括血管包绕和转移。脊髓内扩展和瘤内假性动脉瘤是我们的细胞型中胚叶肾瘤系列中以前未报道过的发现。共同的组织病理学和易位基因融合支持细胞型中胚叶肾瘤是IFS肾型的概念。
