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.     

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.     

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.     

Distinct features of teratoid Wilms tumor

Teratoid Wilms tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. Typically, histopathologic examination shows a mixture of mature squamous and mucus-producing columnar epithelium and a prominent component of mature adipose tissue. The treatment of this tumor has not been established because of its rarity with some reports doubting the value of chemotherapy. We report 3 new cases and review the 23 previously reported cases that we were able to identify. The first case was a 2-year-old boy with a huge tumor that occupied most of the abdominal cavity. The second case was a 5-year-old girl with a metastatic tumor in association with horseshoe kidney and is the first case of teratoid Wilms tumor with brain metastasis. The third case was an 11-month-old girl with bilateral involvement. Imaging studies of our patients were similar to those reported in the literature with areas of fat attenuation and calcification. Failure to respond to preoperative chemotherapy was seen in our patients and in most previously reported cases that were treated with preoperative chemotherapy. We recommend treating this entity with protocols designed for Wilms tumor and to conduct further basic research to understand its biologic nature.     

Cystic nephroma of childhood: A case report and review of the literature

Cystic nephroma of childhood is a rare renal tumor. It is generally considered as a benign end of the spectrum with cystic partially differentiated nephroblastoma and nephroblastoma (Wilms tumor). Most of the cases are unilateral and sporadic. However, bilateral and familial cases have also been reported. The clinical presentation is usually as an asymptomatic abdominal mass. It is almost impossible to differentiate cystic nephroma from cystic partially differentiated nephroblastoma or other cystic renal tumor on radiological studies. The final diagnosis depends on the histopathologic examination. We present a case of an 8-month-old female baby with a left renal tumor. The patient accepted left total nephrectomy after physical examinations and radiological studies. Microscopically, the tumor was composed of variable-sized cysts separated by fibrous septa without blastemal elements. A diagnosis of cystic nephroma was made.     

Wilms' tumor in adults

A rare case of metastatic nephroblastoma on the right side in a 28-year-old female patient is presented. Complete remission was achieved by inductive chemotherapy with vincristine, Adriamycin, actinomycin D and cyclophosphamide and subsequent radical nephrectomy. There was no evidence of disease 24 months after the initial diagnosis had been made. The problems specific to the diagnosis and treatment of adult Wilms tumor are discussed. A chemotherapy regimen known to be successful in childhood Wilms tumor was shown to be equally effective in our adult patient.     

A case of malignant fibrous histiocytoma occurring in the retroperitoneum with giant pyonephrosis

A case of malignant fibrous histiocytoma (MFH) occurring in th retroperitoneum with giant pyonephrosis is reported. The patient was a 45-year-old male and his chief complaint was an abdominal mass. The abdominal fullness progressed so rapidly that he was admitted to our hospital. After examination, this case was diagnosed as a malignant tumor with left hydronephrosis, and an operation was performed on August 5, 1982. At operation, the left kidney contained about 11,000 ml of a pus-like fluid and in the retroperitoneum was found a hen-egg-sized solid tumor which was invading into the left kidney and the feeding vessels of the descending colon. So the tumor, left kidney and a part of the descending colon were resected en bloc. Pathological diagnosis was malignant fibrous histiocytoma. Chemotherapy (PPM regimen) and immunotherapy (OK-432) were administered after the operation, but multiple metastases appeared in the liver and bilateral lungs within 3 months. Then, the CY-VA-DIC regimen was followed. But, local recurrence was found in about 5 months, and the patient died on the 174 th day after the operation. Local recurrence and metastases in the liver, bilateral lungs, pleura and bones were confirmed at autopsy. Besides our case, a review of case reports of retroperitoneal MFH in Japan and comments are presented.     

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。     

Wilms tumor and the VATER association

We describe an infant with the VATER association in whom a Wilms tumor was noted when she was 5 months old. The lower pole tumor arose near the pelvis and grew into an adjacent calix producing a cystic and solid mass with numerous papillary projections resembling sarcoma botryoides. Histopathologically, the tumor was a classical Wilms tumor but it was unusual in that the papillary projections of the tumor were covered by intestinal-like epithelium, which was interpreted as metaplastic urothelium. The margins were free of tumor and the patient is being treated according to the National Wilms Tumor Study Protocol for stage I lesions. She is currently without evidence of recurrence.     

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.     

Multimodal treatment of advanced adult Wilms tumor

We report 2 cases of advanced adult Wilms tumor that were treated with surgery, radiation and chemotherapy. The first patient had relapse of a Wilms tumor in the liver 2 years after nephrectomy. Combination chemotherapy, consisting of actinomycin D and vincristine, radiation therapy and final resection of the liver metastasis were successful and the patient has been free of disease for 4 years. The second patient had undergone transcatheter embolization of the renal artery elsewhere with the tentative diagnosis of an inoperable renal cell carcinoma metastatic to both lungs. A left renal tumor, weighing 4,500 gm., and a tumor thrombus in the vena cava extending to the right atrium were removed, and histologically diagnosed as a Wilms tumor. Subsequent chemotherapy and radiotherapy resulted in complete disappearance of the lung metastases. We conclude that multimodal treatment, namely a well timed combination of surgery, chemotherapy and radiotherapy, could potentially eradicate the disease even at an advanced stage.     

Preoperative chemotherapy for Wilms tumor

Preoperative chemotherapy was administered to 19 children with Wilms tumor judged clinically to be unresectable at M. D. Anderson Hospital between January 1, 1962, and September 1, 1980. After 2 to 4 doses of vincristine, marked reduction in tumor size occurred in 16 patients. After chemotherapy 16 tumors could be resected completely, another required irradiation to reduce the tumor, and only 2 tumors could not be excised. Pathologically the most dramatic changes occurred in the undifferentiated interstitial stroma, followed next by changes in the nodular blastema. Differentiated elements were apparently not affected. No serious complications were attributed to the preoperative drug treatment. This experience suggests that in selected instances preoperative chemotherapy can affectively facilitate the therapy of Wilms tumor.     

Chromophobe cell renal carcinoma: a case report

Chromophobe cell renal carcinoma is an uncommon subtype of renal cell carcinoma and the number of cases studied is still limited in Japan. We here report a case of chromophobe cell renal carcinoma in a 41-year-old Mexican male. He visited our branch hospital with the symptom of upper abdominal pain. Ultrasound examination showed a left renal mass. He was admitted to our hospital for treatment of a left renal mass. Radiological examinations revealed a hypervascular tumor in the left kidney. Under the clinical diagnosis of possible renal cell carcinoma, left radical nephrectomy was performed. This tumor was diagnosed as chromophobe cell renal carcinoma with a microscopic examination of H & E stained specimens, histochemical staining using Hale's colloidal iron and an ultrastructural study.     

Wilms tumor presenting with abdominal pain: A special subgroup of patients

Background: Although significant progress has been made in the management of children with Wilms tumor, two major controversies still exist: the extent of radiographic evaluation necessary before surgery and the role of preoperative chemotherapy. This study sought to determine whether patients with Wilms tumor who presented with abdominal pain defined a special subset of patients who might require a more extensive preoperative work-up and neoadjuvant chemotherapy. Methods: From 1970 to 1995, 250 children were treated for Wilms tumor at a single pediatric institution. A retrospective chart review determined presenting signs and symptoms for each patient. Results: Thirty-four (14%) patients (mean age 5.5 years) sought medical attention with a chief complaint of abdominal pain. The stage distribution for these patients tended to be higher and was significantly different (P<.001, x²-analysis) from those presenting without pain. Four (14%) of 29 patients with tumor available for analysis were found to have anaplastic histology. Conclusion: These data suggest that patients with Wilms tumor who present with abdominal pain represent a special subgroup that tends to be older and has an increased incidence of tumor rupture, anaplasia, and higher stage. These patients may benefit from a more extensive preoperative evaluation and consideration of neoadjuvant chemotherapy.Presented at the 50th Annual Cancer Symposium of The Society of Surgical Oncology, Chicago, Illinois, March 20–23, 1997.     

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.     

Intussusception following resection of Wilms tumor

Postoperative intussusception is a documented complication of pediatric surgical and pediatric urologic abdominal operations. In contrast to "primary" intussusception's triad of crampy abdominal pain, palpable abdominal mass, and "currant jelly" stools, postoperative intussusception is generally characterized by abdominal pain and vomiting. An abdominal mass is not usually palpable, and few children have bloody stools. Proper diagnosis and treatment may be delayed because of similar abdominal symptoms in children who may be receiving radiation and chemotherapy, or with prolonged ileus. Two children operated on for Wilms tumor demonstrate the need for awareness of this potential problem in the postoperative patient.     

Spontaneous renal rupture resulting from ureteral tumor left untreated for 6 years: a case report]

A 57-year-old man, 6 years after discovery of a left ureteral tumor was admitted to our hospital complaining of severe left abdominal pain. With the diagnosis of acute abdomen, the patient was examined by computed tomography, which showed severe left hydronephrosis with renal rupture. Retrograde and antegrade pyelography showed the hydroureteronephrosis due to the tumor in the lower ureter, so left nephroureterectomy was performed. The ureteral tumor was diagnosed as transitional cell carcinoma, grade 3 and pT2. The present case is the 9th case of spontaneous renal rupture caused by renal pelvic and ureteral tumors in Japan.     

Management of Wilms tumor: current standard of care

Wilms tumor is the most common renal malignancy in children. In the 1930s, overall survival for children with Wilms tumor was approximately 30%. Use of multidisciplinary therapy, guided by results from multi-institutional, randomized trials, has substantially improved overall survival to about 90%. Management of Wilms tumor differs substantially between Europe and the US. In Europe, the International Society of Pediatric Oncology protocols call for management of patients with presumptive Wilms tumor with neoadjuvant chemotherapy followed by nephrectomy and further chemotherapy. In the US, protocols developed by the National Wilms Tumor Study Group advise primary nephrectomy followed by a chemotherapy regimen tailored to the pathologic tumor stage. Despite these disparate strategies, overall survival is similar in patients managed according to European and US protocols. Patients with Wilms tumor now have excellent survival. Therefore, current goals aim to reduce the morbidity associated with therapy. Important complications of treatment for Wilms tumor include cardiomyopathy, renal failure, and increased risk of a secondary malignancy. Currently, the role of laparoscopic surgery in management of Wilms tumor remains extremely limited.     

Surgery-related factors and local recurrence of Wilms tumor in National Wilms Tumor Study 4

OBJECTIVE: To assess the prognostic factors for local recurrence in Wilms tumor. SUMMARY BACKGROUND DATA: Current therapy for Wilms tumor has evolved through four studies of the National Wilms Tumor Study Group. As adverse prognostic factors were identified, treatment of children with Wilms tumor has been tailored based on these factors. Two-year relapse-free survival of children in the fourth study (NWTS-4) exceeded 91%. Factors once of prognostic import for local recurrence may lose their significance as more effective therapeutic regimens are devised. METHODS: Children evaluated were drawn from the records of NWTS-4. A total of 2482 randomized or followed patients were identified. Local recurrence, defined as recurrence in the original tumor bed, retroperitoneum, or within the abdominal cavity or pelvis, occurred in 100 children. Using a nested case-control study design, 182 matched controls were selected. Factors were analyzed for their association with local failure. Relative risks and 95% confidence intervals were calculated, taking into account the matching. RESULTS: The largest relative risks for local recurrence were observed in patients with stage III disease, those with unfavorable histology (especially diffuse anaplasia), and those reported to have tumor spillage during surgery. Multiple regression analysis adjusting for the combined effects of histology, lymph node involvement, and age revealed that tumor spillage remained significant. The relative risk of local recurrence from spill was largest in children with stage II disease. The absence of lymph node biopsy was also associated with an increased relative risk of recurrence, which was largest in children with stage I disease. The survival of children after local recurrence is poor, with an average survival rate at 2 years after relapse of 43%. Survival was dependent on initial stage: those who received more therapy before relapse had a worse prognosis. CONCLUSIONS: This study has demonstrated that surgical rupture of the tumor must be prevented by the surgeon, because spills produce an increased risk of local relapse. Both local and diffuse spills produce this risk. Stage II children with local spill appear to require more aggressive therapy than that used in NWTS-4. The continued critical importance of lymph node sampling in conjunction with nephrectomy for Wilms tumor is also established. Absence of lymph node biopsy may result in understaging and inadequate treatment of the child and may produce an increased risk of local recurrence.     

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.