Adult Wilms tumor: a case of prolonged survival achieved by multimodal treatment

Wilms tumor is one of the most common malignant neoplasms in children. In the last 2 decades the survival rate of children with this disease has dramatically increased. However, in adults, the guideline for its management is less clear. We herein report the prolonged survival of a patient with advanced adult Wilms tumor after treatment with chemotherapy, irradiation and operations. Adult Wilms tumor should be treated aggressively with a well-timed combination of surgery, chemotherapy and radiotherapy (i.e., multimodal treatment), even if the stage of the disease was advanced. Thereby, we suspect that the prognosis of adult Wilms tumor would be markedly improved.     

The role of renal salvage procedures for bilateral Wilms tumor: a 15-year review

PURPOSE: We reviewed our experience with renal salvage procedures in patients with bilateral Wilms tumor to determine the clinical outcome. MATERIALS AND METHODS: From 1982 to 1997, 23 children with bilateral Wilms tumor were treated with partial nephrectomy at our institution, including 7 who were also treated with brachytherapy. Medical history, use and response to chemotherapy and brachytherapy, operative records, renal function, pathological results, survival, and techniques for partial and repeat nephrectomy and brachytherapy were reviewed. RESULTS: We treated 8 boys and 15 girls, of whom 21 who presented with synchronous bilateral Wilms tumor underwent primary chemotherapy followed by secondary partial nephrectomy. A total of 44 partial nephrectomies were performed and brachytherapy was done in 7 patients. Ten children have normal renal function and no disease, 10 are dead and 2 have metastatic disease. Anaplasia was the most significant factor associated with an unfavorable outcome (p = 0.003). Of the patients who were cured 60% had a positive response to initial chemotherapy compared with only 25% who had an unfavorable outcome (p = 0.09). No significant differences were noted with respect to gender, age at presentation, highest local tumor stage at presentation or initial nephrectomy. No patient treated with brachytherapy had local recurrence. CONCLUSIONS: Preoperative chemotherapy followed by nephron sparing surgery is indicated in patients with bilateral Wilms tumor, while in those with diffuse anaplasia nephron sparing surgery is contraindicated. Brachytherapy should be considered for treating local disease involving chemoresistant tumors.     

Triple therapy for adult Wilms tumor

Adult Wilms tumor is still considered a rarity. Approximately 170 adult Wilms tumors have been reported. The final diagnosis is usually established with surgery. There is no specific radiologic diagnosis of adult Wilms tumor. We report on a case of Wilms tumor in a twenty-two-year-old black man. The tumor was removed with radical nephrectomy and classified as Stage II. Radiation of the renal fossa (4,000 rad) and chemotherapy with actinomycin D and vincristine were administered after surgery. Six years after surgery the patient is disease free. A review of published cases of adult Wilms tumor is presented, and a plea is made for triple therapy.     

Metachronous bilateral Wilms tumor. Report of longest-known survivor and guidelines for conservative management

In a one-year-old black male patient a classic Wilms tumor of the left kidney was treated with left nephrectomy, chemotherapy, and irradiation to the entire abdomen. Two years later a mature Wilms tumor, consisting predominantly of rhabdomyomatous elements, was discovered in the right kidney. Although a second course of chemotherapy was given and 2,000 rad were delivered to the right flank, the mass continued to enlarge with progressive deterioration of renal function. A nephron-sparing procedure was performed, in which a 3,400-g tumor measuring 19 cm X 16 cm X 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms tumor with a malignant anaplastic sarcoma arising in the central portion. Currently, he is disease-free with normal renal function more than twenty years after diagnosis of the metachronous bilateral Wilms tumor. We believe he is the longest known surviving metachronous Wilms tumor patient and emphasizes the importance of conservative, meticulous surgery in the management of both unilateral and bilateral Wilms tumors. The role of multimodal therapy (chemotherapy, irradiation, and surgery) as well as the current guidelines for the management of bilateral Wilms tumors as proposed by the National Wilms Tumor Study are reviewed.     

The current management of bilateral Wilms tumor

The good prognosis of synchronous bilateral Wilms tumor seems inappropriate for the magnitude of the disease process. Our experience with 11 cases demonstrates the unusual tumor response to chemotherapy and limited preservative surgery. Although 2 patients died 2 with metastatic disease have responded to chemotherapy and are alive at 5 and 8 years after treatment. In addition, 1 patient has survived with biopsy only and no definitive surgery. Although all surgical options have been used, ranging from biopsy alone to bench surgery with autotransplantation to bilateral nephrectomy, our experience demonstrates the effectiveness of a conservative approach with initial biopsy, chemotherapy and subsequent partial nephrectomy if needed. Our survival data and the histological examination of the tumors after chemotherapy suggest a strong relationship of bilateral Wilms tumor to the nodular renal blastema-nephroblastomatosis complex, and a mechanism to explain the excellent tumor response to chemotherapy.     

Management of intravascular thrombus in cases of bilateral Wilms tumor or horseshoe kidney

PurposeTo describe the oncologic and surgical management of bilateral Wilms tumor or Wilms tumor arising in a horseshoe kidney with intravenous tumor thrombus to help pediatric surgeons negotiate this rare and difficult anatomic circumstance.MethodsA single-institution, retrospective medical record review identified 4 cases of bilateral WT and one case of WT arising in a horseshoe kidney with intravenous tumor thrombus between 2009 and 2021. The presentation, imaging, chemotherapy regimen, intraoperative approach, and surgical and oncologic outcomes were reviewed for each of these patients.ResultsAll patients received a total of 12 weeks of neoadjuvant chemotherapy. In two patients, a staged approach to the bilateral tumors was undertaken with the first side being operated on after six weeks of therapy and the other side undergoing surgery after an additional six weeks of therapy. Of five patients, four underwent nephron-sparing surgery of all tumors and one underwent unilateral radical nephroureterectomy with contralateral nephron-sparing surgery. Tumor thrombectomy was performed in four of five cases; one patient demonstrated a complete response of the intravenous tumor thrombus to neoadjuvant chemotherapy and did not require thrombectomy. Three patients received adjuvant flank radiotherapy. Three patients developed medically managed stage II or III chronic kidney disease and no patient required renal replacement therapy or kidney transplant to date.ConclusionNephron-sparing surgery is feasible and safe to perform in selected cases of bilateral Wilms tumor with intravascular thrombus by utilizing three-drug neoadjuvant chemotherapy, staged approaches to each kidney when appropriate, and detailed preoperative and/or intraoperative mapping of renal venous anatomy. Successful nephron-sparing surgery with tumor thrombectomy is dependent on a branched renal venous system or the presence of accessory renal veins.Level of evidenceLevel 4.     

Pediatric clear cell sarcoma of the kidney with cavoatrial thrombus

Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor. Only 4 cases of CCSK with vascular thrombus have been reported, and 2 of these were pediatric cases. One of the children had an intraatrial thrombus as well. We describe a 3-year-old boy who was diagnosed as having a Wilms tumor but did not respond to preresection chemotherapy. He underwent complete resection of the tumor under cardiopulmonary bypass. Histologic examination indicated that the tumor was a CCSK. The patient was then managed with appropriate chemotherapy and radiation therapy and is well 16 months after diagnosis.     

Repeat nephron-sparing surgery for children with bilateral Wilms tumor

Background

Renal insufficiency is a significant complication of Wilms tumor treatment in the 5% with bilateral disease. Nephron-sparing surgery (NSS) is recommended after neoadjuvant chemotherapy initially. However, the role of NSS in recurrent disease is unknown. We reviewed our experience to assess the feasibility and oncologic and functional outcomes of repeat NSS for children with recurrent disease.

Methods

A retrospective review was performed of all children treated at our institution for bilateral, favorable histology (FH) Wilms tumor. Patients undergoing repeat NSS for locally recurrent disease were identified. The outcomes evaluated included tumor recurrence, renal function, and patient survival.

Results

Since 2001, 36 children with bilateral FH Wilms tumor have been treated at our institution. Eight patients (22%) underwent repeat NSS for locally recurrent disease. Two patients had a second local recurrence and underwent a third NSS. Six patients are alive without disease (75%) with an average follow-up of 4.5 years. Two patients have died, each with blastemal-predominant histology at repeat NSS. The surviving patients have normal renal function, although two patients require medical management of hypertension.

Conclusions

Our experience suggests that repeat NSS for local recurrence of FH bilateral Wilms tumor is feasible and affords acceptable oncologic outcome with preservation of renal function. However, more aggressive therapy may be required for patients whose recurrence has blastemal-predominant histology, given the poor outcome for these patients in our series.     

成人肾母细胞瘤的诊疗及预后分析（附10例报告）

目的：探讨成人肾母细胞瘤的诊疗方法和预后。方法：回顾性分析10例成人肾母细胞瘤患者的临床资料。男8例,女2例,年龄16～62岁,平均29岁。就诊症状为单纯肉眼血尿3例、腰腹部肿块或腰腹痛4例（伴血尿2例）、无症状查体发现3例。患者均行B超、IVU、尿脱落细胞学检查,8例行CT平扫＋强化检查。影像学检查提示肾占位6例、肾盂占位3例、输尿管占位1例。CT提示肾门或腹腔淋巴结肿大4例。10例均行手术治疗,根治性肾切除6例,患肾、输尿管全段切除＋膀胱输尿管口袖口状切除4例,2例根治性肾切除患者因肿物与周围粘连严重切除部分腹膜。行腹膜后淋巴结清扫5例。结果：所有病例均于术后病理确诊为肾母细胞瘤,预后良好组织型（FH）4例,预后不良组织型（UH）6例,病理证实淋巴结转移3例。按美国国家肾母细胞瘤研究组（NWTS）分期标准,分别为Ⅰ期4例、Ⅱ期3例、Ⅲ期3例。7例患者接受术后辅助放化疗。随访9例患者1～13年,5例无瘤生存,生存时间为1．5～13年,平均4．4年。1例术后1年死于肿瘤肺转移,1例术后1年死于肿瘤脑转移,2例术后2年内局部复发,再次接受手术。结论：成人肾母细胞瘤为罕见的恶性肿瘤,预后较差,确诊主要依靠病理诊断,目前尚无公认的最佳治疗手段。手术治疗并辅以合理、及时的放疗、化疗是改善其预后的有效措施。     

Renal cell carcinoma in Japanese children

Renal cell carcinoma in children is extremely rare compared to Wilms tumor. We report on a 7-year-old Japanese boy with left renal cell carcinoma. Since the disease was clinical stage I, nephrectomy with tumor extirpation was performed after a short course of initial chemotherapy had been administered. The patient is free of disease 3 years postoperatively. In a review of the Japanese literature we found 71 reports of renal cell carcinoma in children less than 15 years old. Mean patient age at occurrence was 8.1 years, there were no differences in regard to the sex or the affected side, and the most frequent symptom was the presence of a mass. We stress the early establishment of diagnosis in children with a persistent abdominal mass, hematuria and flank pain, since surgical treatment leads to a favorable prognosis only in the early stage of renal cell carcinoma.     

Renal findings on radiological followup of patients with Beckwith-Wiedemann syndrome

PURPOSE: The Beckwith-Wiedemann syndrome is most commonly characterized by macroglossia and abdominal wall defect(s), and it carries a predisposition to embryonal tumors, including Wilms tumor. We report our experience with the character and incidence of renal disease in patients with the Beckwith-Wiedemann syndrome, and discuss the role of radiological followup. MATERIALS AND METHODS: We reviewed the medical records of all patients diagnosed with the Beckwith-Wiedemann syndrome who were treated at our institution between March 1979 and February 1998. Radiological followup consisted of renal ultrasound at approximately 3 to 6-month intervals with the addition of computerized tomography or magnetic resonance imaging (MRI) in patients with an indeterminate lesion(s) or nephrogenic rest(s). RESULTS: A total of 29 patients were identified. Of these cases renal ultrasound showed normal kidneys bilaterally in 19 (70%), simple cysts in 5 (19%), indeterminate lesion(s) in 2 (7%) and nephrocalcinosis in 1 (4%). Nephrogenic rests were followed with MRI in 1 patient, and 1 in whom a 2 cm. mass was revealed by followup MRI underwent partial nephrectomy and chemotherapy for stage I Wilms tumor. CONCLUSIONS: The 3.7% incidence of Wilms tumor in our patients with the Beckwith-Wiedemann syndrome is similar to that in previously published reports. Aggressive follow-up by a sensitive radiological technique is warranted in cases of the Beckwith-Wiedemann syndrome, and associated hemihypertrophy and/or nephromegaly with or without evidence of a Wilms tumor precursor. The detection of suspected malignant disease at an early stage may permit curative nephron sparing surgery.     

Renal angiosarcoma

Renal angiosarcoma, an uncommon vascular sarcoma, is primarily treated surgically. We present a patient who, at nephrectomy, had tumor adherent to the diaphragm and renal artery. Postoperative radiation was given to the renal fossa. Despite the subsequent development of bone and liver metastases, disease in the renal fossa was apparently controlled. Symptomatic bone lesions were effectively palliated with radiotherapy. This report confirms those of others suggesting that angiosarcoma does respond to radiation.     

Management of unresectable Wilms tumor

Wilms tumor has become a model of highly curable cancer. With the addition and refinement of chemotherapy protocols, survival rates have risen from 20 per cent three decades ago to 80 per cent or greater in the past decade. Surgery remains an integral part of the management of Wilms tumor both as a diagnostic tool and for removal of tumor bulk. Few primary tumors cannot be surgically removed during the initial phase of therapy. However, bilateral Wilms tumor and large unilateral tumors may occasionally be initially unresectable without prohibitive loss of renal or nonrenal organ function. The National Wilms Tumor Study group has no protocol for management of these tumors but does offer guidelines unsupported by large numbers of patients. Two cases are presented in which the tumors were initially deemed surgically unresectable. Initial management with chemotherapy (both cases) and radiotherapy (1 case) followed by surgical removal of tumor left both patients free of gross tumor, with relapse-free survivals of two and one-half years and six years thus far. Based on our experience and that found in the literature, we believe that preoperative chemotherapy is indicated for children suspected of having Wilms tumors judged surgically unresectable.     

成人肾母细胞瘤的诊断与治疗（附12例报告）

目的探讨成人肾母细胞瘤的诊断和治疗方法。方法回顾性分析12例成人肾母细胞瘤患者的临床资料。结果本组患者年龄16-63岁,表现为肉眼血尿2例,腰腹痛6例,4例为体检发现;术前诊断为肾肿瘤9例,肾盂肿瘤3例。12例均行手术治疗,术后病理均诊断为肾母细胞瘤。按照美国国家肾母细胞瘤研究组（NWTS）分期标准,本组分别为Ⅰ期5例、Ⅱ期3例、Ⅲ期3例、Ⅳ期1例。术后辅以放疗和化疗,平均随访41个月,3年生存率为48%。结论成人肾母细胞瘤是一种比较少见的恶性肿瘤,术前确诊困难,手术治疗并辅以放疗、化疗是其主要的治疗方法。     

Adult Wilms tumor. Treatment with surgery, radiotherapy, and chemotherapy

A twenty-nine-year-old patient with a Wilms tumor on the right side was treated with surgery, radiotherapy, and chemotherapy. Since no study of treatment of adult Wilms tumor is yet available, the combined therapy was instituted in accordance with the one existing for children. The results of the 5 adult cases previously reported which were treated with triple therapy, and the relevant literature, are reviewed.     

Nephroblastoma in adults

Nephroblastoma, or Wilms tumor, is the most common renal neoplasm in children and accounts for approximately a fifth of all malignant growths in this age group. However, the incidence of Wilms tumor in adults is much less common, with less than 200 cases having been reported in the literature. In addition, since there are at least 53 synonyms for this tumor an adequate computer search for articles relating to this subject is difficult. Because of the scarcity of this disease in adults definite treatment modalities have not been accepted thoroughly until recently. The rationale for the present treatment modalities is based largely on the National Wilms Tumor Study groups 1 to 3. We present radiologic and histologic findings of this tumor in 2 additional cases. The current treatment modalities, consisting of chemotherapy with or without adjunctive radiotherapy, are discussed.     

p53 immunopositivity in histologically favorable Wilms tumor is not related to stage at presentation or to biological aggression

PURPOSE: Previous studies have suggested that increased p53 expression is associated with advanced stage and biologically aggressive (chemotherapy resistant) Wilms tumors. We decided to test the hypothesis that increased immunopositivity of p53 is associated with biological aggressiveness in patients with histologically favorable Wilms tumors. MATERIALS AND METHODS: We reviewed the charts of all patients with unilateral Wilms tumor treated at our institution between 1976 and 2001. Histological characteristics, tumor stage, clinical course and p53 expression as determined by immunohistochemical analysis were determined. All immunohistological evaluations were performed on tissue obtained before administration of chemotherapy. RESULTS: A total of 63 cases of unilateral histologically favorable Wilms tumor were assessed. Five cases (8%) were p53 positive. No significant relationship to p53 expression or stage at presentation was noted in 1 of 21 (5%) stage 1, 3 of 21 (14%) stage 2, 1 of 11 (9%) stage 3 and 0 of 10 stage 4 tumors positive for up-regulation of p53. Of the 5 patients with up-regulated p53 expression 1 (20%) had documented disease progression or relapse while on standard National Wilms Tumor Study chemotherapy. Of the 58 patients who were p53 negative 10 (17%) had disease progression or relapse while on standard National Wilms Tumor Study chemotherapy (p >0.3). CONCLUSION: In contrast to previously published studies, we found no correlation of p53 expression to either tumor stage at presentation (p >0.3) or prognosis (p >0.3) in individuals with histologically favorable Wilms tumor assessed for immunopositivity before administration of chemotherapy.     

Management of undifferentiated sarcoma of the liver including living donor liver transplantation as a backup procedure

We present the cases of 3 children with huge undifferentiated sarcoma of the liver who were treated with surgical excision including liver transplantation as an option and adjuvant chemotherapy. All 3 patients were males aged 10, 13, and 15 years old. The size of the tumor was 10, 15, and 20 cm in diameter, respectively. The youngest patient is disease free and doing well 43 months after resection. The 13-year-old patient presented with tumor rupture and underwent operation. The primary tumor and the ruptured tissue fragments were removed and he was given postoperative chemotherapy. The patient is disease free and doing well 52 months after surgery. The oldest patient had an unresectable tumor in the hilar region. Preoperative chemotherapy was given but later discontinued owing to severe side effects. He underwent living donor liver transplantation followed by postoperative chemotherapy. The patient had recurrent tumor 24 months after transplantation that was excised at reoperation. He is doing well and is disease free 18 months after the second procedure. Complete removal of the tumor including total hepatectomy and transplantation when indicated and suitable pre- and/or postoperative chemotherapy is an effective treatment for children with undifferentiated sarcoma of the liver.     

新辅助治疗低位局部进展期直肠癌35例结果分析

目的 探讨新辅助治疗对低位局部进展期直肠癌的临床治疗价值。方法 对35例低位局部进展期直肠癌患者，采用新辅助治疗方案。常规分割放疗，放疗总剂量DT：46Gy，每次2Gy，每周5次。全身化疗2个疗程，每次予以奥沙利铂130mg／m^2，第1天静脉点滴；甲酰四氢叶酸钙(CF)200mg／m^3，第1～3天静脉点滴；氟尿嘧啶(5-FU)500mg／m^2，第1～3天静脉点滴。治疗结束后4～6周进行手术。结果 经新辅助治疗后，病理完全缓解7例，肿瘤平均缩小34．4％，65．7％的病例T分期下降，淋巴结阴转率为55．6％。根治切除34例，其中腹会阴联合切除18例，保肛手术16例，保肛率为45．7％。姑息性Hartmann术1例。随访至今，肝转移2例，根治切除术后无1例局部复发。保肛患者肛门功能良好。结论 对低位局部进展期直肠癌患者采用新辅助治疗，可使肿瘤分期降低，提高手术切除率和保肛率。