Survival of children with a Wilms tumor in Blantyre,Malawi

Abstract

Wilms tumor (WT) has a survival rate above 90% in high income countries. Reported survival rates in sub-Saharan Africa are much lower and long-term outcome is not well known as follow-up is challenging. In Blantyre, Malawi, an adapted WT treatment guideline with preoperative chemotherapy, supportive care, and strategies to enable children and parents to complete treatment was introduced in 2006. Between 2006 and 2011, 73 children with a unilateral WT were treated. Follow-up, including home visits when needed, was done. Median follow-up time is 5 years (range 14–95 months). Two and five-year event free survivals are 46 and 42%. Causes of treatment failure are: 7% (5/73) abandonment of treatment, 15% (11/73) death during treatment and 30% (22/73) disease-related deaths (persistent disease and relapse). Long-term follow-up is challenging but necessary to be able to assess outcome and the true impact of interventions.     

Precursor lesions of Wilms tumor: Clinical and biological implications

Nephrogenic rests (NR) are persistent embryonal remnants in the kidney that are apparent precursors of Wilms tumor (WT). Nephroblastomatosis (Nbl) denotes multiple or diffuse NR. Two major categories of NR have been recognized to date, perilobar (PLNR) and intralobar (ILNR). A dynamic classification of NR according to their recognized developmental fates is presented. Dormancy, maturation, involution, hyperplastic overgrowth, and neoplastic induction are the common fates of NR. Hyperplastic NR are far more common than formerly recognized, and are frequently confused with WT, especially in cases of multicentric and bilateral tumors. Biopsy is of limited value in distinguishing hyperplastic NR from WT, and the use of surgery in cases of Nbl requires careful consideration, as its role can in many cases be reduced or supplanted due to the effectiveness of modern imaging techniques and chemotherapy. An understanding of the natural history of NR and Nbl is essential for rational patient care decisions, and is important for understanding the molecular biology of WT. © 1993 Wiley-Liss, Inc.     

Perirenal hemorrhage as first presentation of Wilms tumor

Wilms tumor typically presents as an abdominal mass, though occasionally patients present with other manifestations. We report a case of a child presenting with a perirenal hemorrhage and an initially occult Wilms tumor, found only on subsequent renal arteriography. Symptoms in this patient were caused by the presence of perirenal and subcapsular hemorrhage rather than the tumor itself. Despite an unusual presentation, we need to consider underlying neoplasia in children with renal hemorrhage and the absence of a history of trauma. Follow-up studies might help clarify initial negative imaging results.     

The diagnosis and therapy of bilateral Wilms tumor in children (11 cases report)

目的 评估双侧肾母细胞瘤的诊治及长期存活质量.方法 回顾性分析1997～2010年收治的肾母细胞瘤315例,其中双侧11例(3.5％);8例同期性(synchronous),3例不同期性(meta-chronous)均于一侧瘤肾切除后1～2.5年经B超检出.男7例,女4例.同期性平均年龄:17月龄;不同期者第二次肿瘤被检出年龄分别为4岁、4岁3月龄及20月龄.并发畸形中有1例左睾缺如、1例右睾下降不全、1例尿道下裂Ⅲ度,1例双侧虹膜缺如.11例的病理组织均属良好型;同期性中1例双侧均有肾母细胞瘤病;另1例右侧有肾母细胞瘤病.术前均接受化疗,最少4周,长春新碱(VCR)或加放线菌素D(ACTD).手术以肿瘤分期剜除为主,术后用VCR+ ACTD+ ADR(阿霉素)15个月,定期随诊最少5年.结果 8例随访20个月至14年(平均4.2年)无瘤健康存活,1例不同期者失访,1例做右瘤肾切除、同期探查左肾门肿瘤,术后无尿死亡.1例11个月女婴死于肿瘤复发.结论 双侧肾母细胞瘤就诊时患儿年龄小,病理组织多属良好型,术前化疗使肿瘤缩小,便于完整挖除,保留肾单位.伴随的畸形和肾母细胞瘤病并未影响预后.     

Late relapsing Wilms tumor with rhabdomyomatous differentiation

The authors report a patient with abdominally relapsed Wilms tumor with rhabdomyomatous differentiation leading to renal failure and death 9 years after the initial diagnosis. The patient was treated with intensive chemotherapy because of inoperable tumor but no response was obtained. The prognosis of children with Wilms tumor relapsed in abdomen and in previously irradiated fields is poor and intensive chemotherapy protocols for differentiated tumors after chemotherapy will increase the risk of complications without obvious benefit. Pediatr Blood Cancer 2009;52:675–677. © 2008 Wiley‐Liss, Inc.     

Intracranial relapse in Wilms tumor patients

BACKGROUND: In children with nephroblastoma, recurrence with metastases in the central nervous system is rare. Recently, previous reports (NWTSG and UKCCSG) reported brain metastases with an incidence of respectively 0.5% and 0.6% in Wilms tumor (WT) patients (respectively n = 30/5,852 and n = 7/1,249). PROCEDURE: We retrospectively investigated the incidence and survival of patients with central nervous system relapse in WT patients, treated according to the consecutive SIOP protocols 1, 2, 5, 6, 9, and 93-01. All children with WT from 1971 until 2000 were enrolled in the study (3,040 eligible patients). Specimens at diagnosis and if possible at relapse were centrally reviewed. Patients with renal neoplasms other than WT were excluded. RESULTS: CNS relapse was documented in 14 patients (0.5%). Median time to CNS relapse was 16 months (3-69). The occurrence of relapse was not associated with specific histological subtypes. In seven patients intracranial metastases occurred at first relapse, of which two were isolated relapses. In five patients no treatment was started because of the poor condition of the patient, the other nine cases were treated with (a combination of) chemotherapy (n = 6), surgery (n = 4), and radiotherapy (n = 6). CONCLUSIONS: CNS relapse in WNT is rare. In contrast to reports of other Wilms tumor study groups, although four patients reached (local) CR, the SIOP registry showed that eventually none of the documented WT patients survived.     

���ζ�ͯ����������ĸϸ����COG-RTC�ٴ��о��������

??Childhood kidney tumors account for about 7% of all childhood cancers. Most childhood kidney tumors are Wilms tumor??but in the 15- to 19-year age group??most tumors are renal cell carcinoma. In medically developed countries??clinical trials in Wilms tumor??WT?? have resulted in overall survival rates of greater than 90%. Children’s Oncology Group Renal Tumor Committee??COG-RTC?? is one of the clinical study groups internationally known for its clinical research in childhood kidney tumor. Its standard treatment for children with Wilms tumor consists of initial nephrectomy??when feasible?? followed by chemotherapy and??in some patients??radiation therapy. This summary reviewed peer-reviewed??evidence-based reports about the treatment for Wilms tumor published recently and intended to be a resource to assist clinicians who care for children with Wilms tumor.     

Survivin Caspase-3 mRNA在肾母细胞瘤中的表达及意义

目的：在大多数肿瘤组织中都发现了Survivin异常高表达现象,它直接抑制Caspase-3,这说明Survivin在肿瘤发生中具有重要作用。本文的目的在于探讨Survivin、Caspase-3表达与肾母细胞瘤发生发展的关系。方法：应用RT-PCR技术检测Survivin、Caspase-3 mRNA在48例肾母细胞瘤、24例癌旁组织标本中的表达。结果：48例肾母细胞瘤组织中Survivin阳性表达率为 72.9%(35/48),在24例肾母细胞瘤癌旁组织中未检测到Survivin的表达,肾母细胞瘤组织与癌旁组织之间Survivin表达阳性率差异有非常显著性(P<0.01);48例肾母细胞瘤组织中Caspase-3阳性表达率为 8.3%(4/48),在24例肾母细胞瘤癌旁组织中Caspase-3阳性表达率为 45.8%(11/24),肾母细胞瘤组织与癌旁组织之间Caspase-3表达阳性率差异有显著性(P<0.05)。结论：Survivin在肾母细胞瘤组织中高度表达和Caspase-3在肾母细胞瘤中低表达可能与肾母细胞瘤的发生发展有关,与预后的关系有待进一步的探讨。［中国当代儿科杂志,2004, 6(5): 381-384]     

Wilms tumor with extension to the right atrium

A seven-year-old boy had cardiac symptoms for five months before the diagnosis of Wilms tumor was made. The tumor extended into the inferior vena cava through the renal vein and protruded into the right atrium. All demonstrable tumor masses could be removed at a one-step surgical procedure without using cardiopulmonary bypass. Intensive postoperative chemotherapy has been given, and 15 months after surgery he was without signs of relapse.     

肾母细胞瘤瘤栓诊疗进展

肾母细胞瘤(Wilms' tumor,WT)是小儿时期最常见的肾脏肿瘤,也是第二大腹腔内肿瘤.WT的特征之一就是易于侵入血管形成瘤栓,发生率大约在6.1％～17.3％.WT瘤栓的存在要求医生对需要接受手术切除或术后施行化疗的患儿采取不同的处理方式.而无论采取何种方式,WT瘤栓都会增加手术风险.     

Paratesticular metastasis from Wilms tumor associated with a hydrocele

Metastatic sites other than the lungs, lymph nodes, and liver are unusual for Wilms tumor (WT). Intra-scrotal metastasis is very rare. We report a 3-year-old boy with stage IIA WT, who experienced paratesticular metastasis 2 months after surgery for an abdominal recurrence. He had right scrotal hydrocele at initial diagnosis. The patient underwent right radical orchiectomy, and pathological examination revealed paratesticular WT metastasis. Intra-abdominal and peritoneal disseminated metastases followed. We considered that tumor cells spread through the patent processus vaginalis and grew at paratesticular space in hydrocele. One month after the end of 12 months of salvage chemotherapy and abdominal radiotherapy, the patient has no evidence of disease.     

Impact of nephrectomy on long-term renal function in non-syndromic children treated for unifocal Wilms tumor

ObjectiveThe present study is designed to assess the long-term renal function of children who underwent radical nephrectomy for unifocal Wilms tumor.MethodsA single institution retrospective cohort study of non-syndromic children treated with radical nephrectomy for unifocal Wilms tumor between 1995 and 2011 was performed to identify risk factors for decreased glomerular filtration rate (GFR). The primary endpoint was decrease in age-adjusted GFR below normal published ranges. The secondary endpoint was progression to chronic renal insufficiency (CRI).ResultsA total of 55 patients were identified in the cohort. Eight (15%) patients exhibited decreased age-adjusted GFR during the follow-up period, with 2 (4%) progressing to CRI. Increasing time between surgery and the last known GFR follow-up was associated with decreased GFR, with the normal GFR group having median follow-up of 7.32 years versus 11.47 years (p = 0.019) in the decreased GFR group.ConclusionsA trend toward decline in GFR was detected with longer follow-up. Longer follow-up may reveal that clinically significant decline in renal function occurs years following nephrectomy among a subset of Wilms tumor survivors, even among those who do not progress to end stage renal disease.     

Nephroblastoma (Wilms tumor): Tubule density and prognosis

Relative differences in tubule density of the primary tumors have been suggested to be prognostically useful in nephroblastoma (Wilms tumor). Forty cases from our institution were retrospectively graded according to tubule density. There were significant differences in disease-free survivals between histologic grades. When compared to other clinical and pathologic staging criteria, tubule density was not more useful prognostically than the staging systems tested. However, when used in conjunction with clinical or pathologic stage, tumor grade improved prognostic sensitivity. Regardless of grade or stage, patients less than 24-months-old at diagnosis had better disease-free survival.     

Treatment of Wilms tumor according to SIOP 9 protocol in Casablanca, Morocco

BACKGROUND: Childhood Wilms tumor represents one of the challenge for pediatric oncologists in developing countries. We report the characteristics and treatment results of patients with Wilms tumor according to SIOP 9 protocol in Morocco. PROCEDURE: From January 1989 to December 2000, 86 children with Wilms tumor were admitted. The diagnosis was based on physical exam and abdominal ultrasound. The metastatic work-up was based on abdominal ultrasound and chest X-ray. RESULTS: The mean age was 36 months (3-120 months). The sex-ratio was 1. Abdominal mass was the main symptom at presentation (84 cases). There were 13 metastatic cases. Treatment applied was according to SIOP 9 Protocol without randomization. Local deases was present in 75 patients with stage I in 38 cases (50%), IIN0 in 4 cases (6%), IIN1 in 9 cases, and III in 24 cases (44%). The distribution of pathologic groups was: favorable in 4 cases, standard in 69 cases, and unfavorable anaplastic type in 2 cases. Sixty-nine patients were evaluable for therapeutic evaluation. Other patients were lost to follow-up. Three patients died of treatment related toxicity and 13 patients relapsed. With a median follow-up of 70 months, the 5-year EFS and 5 years overall survival for evaluable patients are 77.4% and 79%, respectively while the 5-year EFS for all patients was 56%. CONCLUSION: These results are encouraging for a developing country but special efforts should be done to reduce the rate of abandonment.