Focal nodular hyperplasia-like lesion of the liver in a child previously treated for nephroblastoma

Described herein is the case of an 8-year-old boy who developed a hepatic nodular lesion after having received anti-neoplastic therapy for nephroblastoma. Histological examination of the excised specimen indicated a disordered arrangement of hepatic components with hemangioma-like features. This case was diagnosed as a variant of focal nodular hyperplasia. It is speculated that this hepatic lesion is a late complication of treatment for nephroblastoma and might develop from congenital malformative area of the liver.     

B超、CT和静脉肾盂造影诊断肾母细胞瘤的价值

目的：比较B超、CT和IVP诊断肾母细胞瘤的价值。方法：肾母细胞瘤98例,年龄3个月～9岁,中位年龄2．5岁,98例均行B超检查,50例行CT检查,89例行静脉肾盂造影(IVP)。比较3种方法的检查结果并与术中所见及病理检查结果进行对照分析。结果：B超、CT及IVP诊断肾母细胞瘤的符合率分别为93．8％(92／98),88．0％(44／50)和78．0％(70／89)。常见误诊原因是把肾母细胞瘤误诊为肾外肿物,手术和病理检查发现被误诊的肿瘤常位于肾上极或肾下极。B超联合CT或IVP均能显著提高肾母细胞瘤的诊断符合率(98％与97％)。结论：3种影像学检查诊断肾母细胞瘤均有一定的误诊率。B超操作灵活,诊断符合率高,应为诊断小儿肾母细胞瘤的首选方法。     

肾母细胞瘤组织中DNA倍体、SPF、增殖细胞指数的测定

目的：探讨肾母细胞瘤组织中DNA倍体、SPF、增殖细胞指数(PI)与临床分期及病理分级间的关系。方法：应用流式细胞仪(FCM)测定35例肾母细胞瘤标本的DNA倍体、SPF、PI值,并与正常肾组织比较。结果：与正常肾组织相比,I～Ⅱ期与Ⅲ～Ⅳ期肿瘤的异倍体率、SPF、PI值均升高,但肿瘤各临床分期I～Ⅱ期与Ⅲ～Ⅳ期之间,及病理FH型与UH型之间差异无统计学意义。结论：测定肾母细胞瘤的DNA倍体、SPF、PI值能了解其某些分子生物学特性,从分子水平上了解其增殖活性,对肿瘤的诊断及评估预后有一定的意义。     

Cystic partially differentiated nephroblastoma in an adult: an immunohistochemical, lectin histochemical and ultrastructural study

AIMS: Cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal multicystic tumour, usually affecting early infants. To our knowledge, this report describes the first case of CPDN occurring in an adult. METHODS AND RESULTS: A 45-year-old man was found incidentally to have a left renal cystic tumour, measuring 20 mm in diameter, at the lower pole far from the pelvis. The tumour was composed of multilocular cystic spaces of variable size and intervening septa without solid nodular areas. The cysts were lined by a single layer of flattened, hobnail, or columnar epithelium. The septa were made of mesenchymal cells, which were admixed with small numbers of loosely aggregated blastemal cells, occasional tubular structures in various stages of development, and a few glomeruloid structures. The tumour cells had no anaplasia, and mitoses were rare. Immunohistochemical and lectin histochemical studies revealed that the cyst lining epithelium and the tubular structures in the septa expressed predominantly the markers for distal tubules and collecting ducts. Ultrastructurally, the cyst lining cells closely resembled collecting duct cells while some tubular structures showed an immature nephrogenic morphology. The patient was alive and well without evidence of recurrence 11 months after surgery. CONCLUSIONS: CPDN does occur in adults, as experienced in Wilms' tumour, though its incidence is extremely low. This study suggests that CPDN may show maturation intermediate between cystic nephroma and Wilms' tumour, even in adult cases.     

THE EXPRESSION PATTERN OF WILMS' TUMOUR GENE (WT1) PRODUCT IN NORMAL TISSUES AND PAEDIATRIC RENAL TUMOURS

WT1 , the Wilms' tumour suppressor gene located at chromosome 11p13, plays an important role in the development of the kidney. It is mutated in 10 per cent of Wilms' tumours (WTs) and their putative precursors called nephrogenic rests (NRs). A sensitive immunohistochemical technique was established to localize the WT1 gene product in archival normal tissues and paediatric renal tumour samples. Strong nuclear signal was seen in the various sites such as the kidneys, gonads, and decidua. Nuclear immunoreactivity of variable intensity was also seen in the skeletal muscle, smooth muscle of urinary bladder, ureter, and arteries. All 28 WTs (including the anaplastic variant) showed the WT1 gene product in a varying proportion of the blastema, epithelium, and stroma. The anaplastic nuclei in six WTs showed an intensity of staining comparable to their adjacent favourable histology counterparts. All the intralobar and perilobar types of NR demonstrated the WT1 gene product. All three malignant rhabdoid tumours were positive, while three of four mesoblastic nephromas and a clear cell sarcoma were negative. These findings provide an insight into the interrelationships of these renal tumours.     

The COVID‐19 pandemic: A rapid global response for children with cancer from SIOP,COG, SIOP‐E,SIOP‐PODC,IPSO, PROS,CCI, and St Jude Global

The COVID‐19 pandemic is one of the most serious global challenges to delivering affordable and equitable treatment to children with cancer we have witnessed in the last few decades. This Special Report aims to summarize general principles for continuing multidisciplinary care during the SARS‐CoV‐2 (COVID‐19) pandemic. With contributions from the leadership of the International Society for Pediatric Oncology (SIOP), Children's Oncology Group (COG), St Jude Global program, and Childhood Cancer International, we have sought to provide a framework for healthcare teams caring for children with cancer during the pandemic. We anticipate the burden will fall particularly heavily on children, their families, and cancer services in low‐ and middle‐income countries. Therefore, we have brought together the relevant clinical leads from SIOP Europe, COG, and SIOP‐PODC (Pediatric Oncology in Developing Countries) to focus on the six most curable cancers that are part of the WHO Global Initiative in Childhood Cancer. We provide some practical advice for adapting diagnostic and treatment protocols for children with cancer during the pandemic, the measures taken to contain it (e.g., extreme social distancing), and how to prepare for the anticipated recovery period.     

Management of Wilms' tumour

Wilms' tumour (WT) is the commonest renal tumour of childhood and its treatment is regarded as one of the success stories of paediatric oncology with over 90% cure rate. Most patients present with localized unilateral tumours. Histology and stage are important prognostic factors, and children with stage IV and diffuse anaplasia have poorer outcomes. Whilst there are differences in the treatment approaches around the world, outcomes are excellent for most subgroups of Wilms' tumour. The European Société Internationale d'Oncologie Pédiatrique (SIOP) recommends pre-operative chemotherapy to children over 6 months whilst the North American Children's Oncology Group offers primary nephrectomy and uses biomarkers for treatment stratification. Overall, 15% patients experience a relapse and their management depends on the initial treatment they had received. The focus of research is to decrease long term side effects, reduce treatment for selected subgroups, and improve our understanding of prognostic biomarkers to help tailor treatments. This article provides an overview for paediatricians giving an update on the important recent advances.     

Cystic poorly differentiated nephroblastoma: A case report and review of literature

BackgroundCystic poorly differentiated nephroblastoma (CPDN) is a rare variant of nephroblastoma which follows a benign clinical course.Case diagnosis/treatmentIn this report, we document a case of CPDN in a 2 year old boy who presented with recurrent gross painless hematuria and progressive abdominal distension. Abdominal ultrasound showed a multicystic lesion and CT scan features of Stage III Wilms tumour. Nephrectomy was done after two cycles of chemotherapy according to the SIOP Nephroblastoma therapeutic protocols. Histology showed blastemal cells in the wall of only one of the cysts, with no solid expansile nodules. The patient had to have five more cycles of chemotherapy and also radiotherapy for residual tumour.ConclusionsSurgery is curative in Stage I CPDN and adjuvant therapy is not required. Adequate sampling is critical to ensure accurate diagnosis and appropriate management. We suggest that a minimum of 2–3 tissue sections should be taken per centimetre of tumour diameter. Related entities including cystic nephroma, cystic Wilms tumour and completely necrotic nephroblastoma are discussed in the differential diagnosis.     

小儿肾胚胎瘤综合治疗观察（附42例报告）

采用手术加化疗和放疗综合措施治疗肾胚胎瘤患儿42例，使瘤体高分化组织结构的患儿存活率达90．48％，对该病的诊断和治疗进行了初步讨论。