Bera Goutam, Ara Zeenat, Sengupta Moumita, Chatterjee Uttara, Mukhopadhyay Madhumita
Junior Resident, Department of Pathology, IPGME&R, Kolkata, West Bengal, India.
Assistant Professor, Department of Pathology, IPGME&R, Kolkata, West Bengal, India.
J Clin Diagn Res. 2017 Aug;11(8):ED11-ED12. doi: 10.7860/JCDR/2017/22957.10383. Epub 2017 Aug 1.
Wilms' tumour, a renal malignancy, primarily occurs in children with a peak incidence between 2 to 5 years age group and accounts for approximately 95% of childhood renal malignancies. Though rarely, it may also occur in adults with an incidence rate of less than 0.2 per million per year. Microscopically, there is no difference between Wilms' tumour of paediatric and adult age groups. But the prognosis for adults with Wilms' tumour is thought to be worse than that for children possibly due to more aggressive clinical course in case of adult Wilms' tumour. They are often associated with higher tumour stage at the time of presentation and outcome is often worse than paediatric age group possibly due to difficulty in diagnosis, inappropriate staging, and lack of standard treatment protocol. We report a case of 28-year-old lady presented with flank pain and abdominal lump. Abdominal CT scan showed a right renal mass suggestive of malignancy. A provisional diagnosis of renal cell carcinoma was made based on clinical and radiological findings. Nephrectomy was performed and a final morphological diagnosis of Wilms' tumour was given. Immunohistochemical study showed strong Wilms' Tumour 1(WT1) positivity in both blastemal and epithelial components confirming the morphological diagnosis. In view of its diagnostic difficulty and rare occurrence in adults, this case is being reported.
肾母细胞瘤是一种肾脏恶性肿瘤,主要发生于儿童,发病高峰在2至5岁年龄组,约占儿童肾脏恶性肿瘤的95%。虽然罕见,但也可发生于成人,发病率低于每年百万分之0.2。在显微镜下,儿童和成人年龄组的肾母细胞瘤并无差异。但成人肾母细胞瘤的预后被认为比儿童更差,这可能是因为成人肾母细胞瘤的临床病程更具侵袭性。成人肾母细胞瘤在就诊时往往与更高的肿瘤分期相关,其预后通常比儿童年龄组更差,这可能是由于诊断困难、分期不当以及缺乏标准治疗方案。我们报告一例28岁女性,因胁腹疼痛和腹部肿块就诊。腹部CT扫描显示右肾肿块,提示为恶性肿瘤。根据临床和影像学检查结果,初步诊断为肾细胞癌。行肾切除术,最终形态学诊断为肾母细胞瘤。免疫组化研究显示,在胚芽成分和上皮成分中,肾母细胞瘤1(WT1)均呈强阳性,证实了形态学诊断。鉴于其诊断困难且在成人中罕见,现报告此病例。