Barroca Helena Maria, Costa M J, Carvalho José Luis
Department of Anatomic Pathology, Hospital de S. João, Al. Hernani Monteiro-4200 Porto, Portugal.
Acta Cytol. 2003 Nov-Dec;47(6):1055-8. doi: 10.1159/000326646.
Malignant rhabdoid tumor of the kidney (MRTK) is a rare malignant neoplasm that usually presents as an abdominal mass. The histogenesis is uncertain, and cases outside the kidney have been reported. An association with separate primary tumors of primitive neuroepithelial origin occurring in the midline of the posterior or middle cranial fossa has been reported in approximately 15% of cases.
Three patients, a 7-month-old girl and two boys, aged of 6 and 2 months of age, underwent fine needle aspiration biopsy (FNAB) for the diagnosis of renal masses. In 2 cases the smears revealed round to polygonal cells, singly or arranged in irregularly shaped clusters. The neoplastic cells did not differ much in shape and exhibited clear, empty nuclei with prominent nucleoli; the cytoplasm was abundant and sometimes eosinophilic. In the remaining case the aforementioned characteristics of the nuclei and cytoplasm were not as prominent, and sheets of fibrovascular stroma, with attached neoplastic cells, were seen. Diagnosis of MRTK was suspected in every case based upon morphology and immunocytochemistry; the diagnosis was histologically confirmed in the surgical specimens.
MRTK may pose diagnostic problems due to its broad morphologic spectrum. Distinction from Wilms' tumor and clear cell sarcoma of the kidney is essential for therapeutic proposes. The results obtained in the FNAB study of these 3 cases demonstrate that diagnosis of MRTK may be proposed from fine needle aspiration smears using conventional methods together with ancillary ones (immunocytochemistry and electron microscopy).
肾恶性横纹肌样瘤(MRTK)是一种罕见的恶性肿瘤,通常表现为腹部肿块。其组织发生尚不确定,肾外病例也有报道。据报道,约15%的病例与发生在后颅窝或中颅窝中线的原始神经上皮来源的独立原发性肿瘤有关。
三名患者,一名7个月大的女孩和两名男孩,年龄分别为6个月和2个月,接受了细针穿刺活检(FNAB)以诊断肾肿块。2例涂片显示圆形至多边形细胞,单个或呈不规则形状的簇状排列。肿瘤细胞在形状上差异不大,细胞核清晰、空泡状,核仁突出;细胞质丰富,有时呈嗜酸性。在其余病例中,上述细胞核和细胞质的特征不那么明显,可见成片的纤维血管间质以及附着的肿瘤细胞。根据形态学和免疫细胞化学,每例均怀疑为MRTK;手术标本经组织学证实诊断。
MRTK因其广泛的形态学谱可能会带来诊断问题。为了治疗目的,将其与肾母细胞瘤和肾透明细胞肉瘤区分开来至关重要。这3例细针穿刺活检研究结果表明,使用传统方法并结合辅助方法(免疫细胞化学和电子显微镜),可从细针穿刺涂片中提出MRTK的诊断。