Pulmonary Function in Survivors of Wilms' Tumor

The respiratory status of 47 patients surviving childhood Wilms' tumor was studied. The group that had receivedflnnk irradiation (which impinges on the lower lung) (n - 17) had a sisnijGantly lower mean percent predicted for forced expiratory volume in one second, residual volume, and total lung capaci(v when compared to those who had received no irradiation (n - 23). Those patients who had received whole-lung irradiation (n - 3) had sisnijicantly lower transfn. foctor for carbon monoxide and gas transfer per unit lung volume whm compared to the nonirradiated group (n - 23). There was no sipiftiant dimue in the prevalence of respiratory symptoms between the three groups. Patients receiving any form of radiotherapy for Wilms' tumor may have abnormulities o f pulmonary function and should have pulmonary function tests performed as part o f their long-tmn follow-up.     

Teratoid Wilms' Tumor: Report of a Unilateral Case

A unilateral teratoid Wilms' tumor was removed 2.5 weeks after the institution of chemotherapy. Teratoid Wilms' tumor is an extremely rare renal tumor, and only four cases, all bilateral, have been reported. Because of the finding of deep cortical intralobar nephroblastomatosis, strongly associated with bilateral Wilms' tumors, the patient has been closely followed since surgery without evidence of tumor in the remaining kidney at 2 years.     

Polycythemia: A Review Article and Case Report of Erythrocytosis Secondary to Wilms' Tumor

We describe a child with Wilms' tumor whose only finding at presentation was polycythemia. A review of the mechanism of erythropoiesis regulation as well as the differential diagnosis, work-up, and management of polycythemia in children is presented. Our report emphasizes the need for a thorough evaluation of children who present with polycythemia.     

Wilms' tumor metastatic to the mediastinum

We report two children with mediastinal metastasis of Wilms' tumor. One case presented with a right hilar mass and the other with a posterior mediastinal mass. In each instance, no additional radiologic evidence of metastasis was found. Metastasis of Wilms' tumor to the mediastinum is uncommon, usually occurring with radiologic evidence of either additional metastases or direct cephalad growth of the primary tumor.Presented at the Members Miscellany, 23rd Annual Meeting, The Society for Pediatric Radiology, 18–20 April 1980, Salt Lake City, Utah, USA     

Juxtaposed Cystic Nephroma and Wilms' Tumor

We report a case of juxtaposed Wilms' tumor (WT) and cystic nephroma (CN) in a 21-month-old girl which gave rise to radiological diagnostic difficulty. Preoperative chemotherapy was given, resulting in marked tumor necrosis but the cystic nephroma remained untouched. Histological examination showed characteristic features of a triphasic WT and a CN; the two lesions were separated by a thick fibrous capsule. While everybody agrees that WT and cystic partially differentiated nephroblastoma (CPDN) are closely related, there are two opposite views about their relationship to CN. One is that CN may represent the final step in maturation of WT and CPDN. Other authors argue that there is no evidence to support this theory but believe CN might have something in common with nephrogenic rests. We suggest that the two lesions in the present case may have originated from two intralobar nephrogenic rests, which would strengthen the latter view. Received November 2, 1998; accepted February 16, 1999.     

Congestive Heart Failure Caused by Wilms' Tumor

A 2-year-old child with unilateral Wilms' tumor presented with congestive heart failure (CHF). The heart failure was secondary to severe hypertension from hyperreninemia. After surgical removal of the tumor the CHF disappeared. This case is the first one reported of a child presenting with CHF due to unilateral Wilms' tumor.     

Pilot Study of Cardiac Function After Treatment of Childhood Wilms' Tumor with Doxorubicin

To assess the cardiotoxicity of moderate doses of anthracyclines and to control for possible contributions of other drugs, cardiac function in 12 patients 1 year 11 months to 9 years 4 months off treatment for Wilms' tumor whose therapy had included vincristine, dactinomycin, and the anthracycline doxorubicin (270 to 300 mg/m² cumulative dose) was compared with that of 9 patients (controls) whose therapy had included vincristine and dactinomycin but no doxorubicin. The small numbers of patients studied suggest that at a median of 6 years off therapy only small numbers of patients will have cardiac function values below age-related normal values. To confirm these results and to determine whether abnormalities are progressive or are clinically important, large cohorts of patients such as those available to the National Wilms' Tumor Study need to be studied.     

The Effect of Abdominal Radiation on Spleen Function: A Study in Children with Wilms' Tumor

Reports of splenic dysfunction in patients with Hodgkin 's disease who received radiation therapy to the spleen raise questions concerning impairment of splenic function and the long-term risk of bacterial sepsis in children who receive abdominal radiation for other diseases. Splenic function was studied in 20 children with Wilms' tumor using a quantitative assessment of vacuolated (“pitted”) red cells as a measure of reticuloendothelial function. Fourteen children had received abdominal radiation to afield involving the spleen at a median dose of 2000 rods. Their pitted red cell counts were no different from those of 6 children who received therapy without radiation to the spleen or to those of a group of normal children and adults. We conclude that there is no demonstrable long-term impairment of spleen function with radiation doses at or below 2200 rods.     

Renin Production in Congenital Mesoblastic Nephroma in Comparison with that in Wilms' Tumor

The case of an infant with congenital mesoblastic nephroma and associated reninism is presented. The patient was a 39-day-old boy who presented with a left abdominal mass and mild hypertension (112/70 mm Hg). Both plasma renin activity (PRA) and plasma total renin concentration (PTRC) were elevated; the PTRC value of 1458 pg/mL of this case was the highest we had seen in patients with childhood renal tumors. The tumor was successfully removed and histologically confirmed as a congenital mesoblastic nephroma (cellular variant). The patient's PRA and PTRC returned to normal after nephrectomy. Indirect immunoperoxidase staining showed renin localized predominantly in the juxtaglomerular apparatuses adjacent to the glomeruli entrapped by the tumor. No positive staining was seen in the tumor cells. These findings indicate that the entrapped glomeruli may play a significant role in producing renin. More important, PTRC was extremely high compared to the moderate increase of PRA and to the mild elevation of blood pressure. The mechanism of renin production by childhood renal tumors is discussed, and the importance of studying inactive renin and total renin is emphasized.     

Epidural metastasis in chemoresistant Wilms' tumor with perilobar nephroblastomatosis

An 8-year-old boy with vertebral and epidural metastases was diagnosed with Wilms' tumor associated with perilobar nephroblastomatosis (NB) based on histologic examination. During combined chemotherapy with vincristine, actinomycin D, doxorubicin, and cyclophosphamide (NWTS-3 J protocol), a rapid increase in tumor size was observed. The treatment was replaced with etoposide and carboplatin (JET regimen). A transient response was sustained for 5 months during this chemotherapy. However, regrowth of the tumor was observed and the patient died 11 months after the initial chemotherapy.     

Growth Characteristics of Human Wilms' Tumor in Nude Mice

Ten Wilms' tumors (WT) were heierotransplated into athymic (nude) mice. Eight of the tumors (80%) grew and were serially passaged as many as 20 times. The histology of the primary heterotransplants resembled that of the surgically excised tumors (seven classical and one anaplastic). Histological examination of serial passages of the classical WT demonstrated the tendency of the stromal and tubular components to disappear. The anaplastic tumor, however, maintained histological features identical to the primary tumor through all passages examined. One WT cell line that exhibited a prominent skeletal muscle component failed to grow beyond the third passage. Spontaneous glomerular differentiation was noted in several heterotransplants. The site of transplantation (subcutaneous, peritoneal, or renal capsule) had no effect on the differentiation of the tumors, and attempts to produce intravenous metastases were unsuccessful. Unilateral nephrectomy of WT-bearing mice gave a transient increase in pulse labeling of the tumors with bromodeoxyuridine, a thymidine analogue, compared with sham-operated controls or mice bearing Ewings' sarcoma heterotransplants. The increased labeling of tumor nuclei reached a maximum at 48 h. Similar increased labeling was observed in the remaining kidney following unilateral nephrectomy. These data show that although WT is a malignant neoplasm, its cells retain the capacity to respond to physiological signals resulting from nephrectomy and that differentiation cannot be modulated by the site of heterotransplantation or serial passage in athymic mice.     

Management of Wilms' tumor and neuroblastoma

Wilms' tumor and neuroblastoma resemble each other in usual presentation and by their occurrence in young children. However they represent a dichotomy in advances in treatment. In Wilms' tumor, survival of patients has dramatically improved as a result, of multimodal therapy, whereas in neuroblastoma little advance in treatment has occurred. Development of rational chemotherapeutic regimens through better understanding of drugs and of cell cycle kinetics as well as investigation of other modalities of therapy, i.e. immunotherapy, etc., may be helpful in improving survival in the years to come.     

A single-institution Wilms' tumor and localized neuroblastoma series

Since January 1980, 120 children affected by Wilms' tumor have been treated at Bambino Gesù, mostly with multimodality treatment according to Société Internationale d'Oncologie Pediatrique (SIOP) protocols, including chemotherapy, surgery and radiotherapy in selected cases. This treatment approach emphasizes the role of preoperative (neoadjuvant) chemotherapy as opposed to the approach favored by the National Wilms' Tumor Study, which is focused on optimizing postoperative chemotherapy after primary surgery. Thus, using SIOP guidelines, staging occurs at the time of surgery, after chemotherapy administration. These differences will constitute the baseline for a comparison between the two experiences. Bilaterality, nephroblastomatosis, partial nephrectomy in unilateral Wilms' tumor and thrombosis of the vena cava are the main topics discussed. For the present study, the analysis was restricted to 98 consecutive cases diagnosed until December 1999, for whom at least 24 mo of follow-up is available. The more recent experience of treating resectable neuroblastoma in cooperative studies dates back to 1979, when the first Italian Cooperative Group Neuroblastoma protocol was introduced. This experience was continued within the frame of the first Localized Neuroblastoma European Study Group protocol (LNESG 94), and will be compared to North American Cooperative Group approaches and outcomes. Preoperative evaluation of surgical risk factors, intraoperative complications and their management, and long-term outcome will be discussed.     

Structural Chromosome Aberrations in Lymphocytes from Children Previously Treated for Wilms' Tumor or Hodgkin's Disease

Nineteen children treated for Wilms' tumor (thirteen cases) or Hodgkin's disease (six cases) with cytostatic agents and/or radiotherapy were studied cytogenetically on lymphocytes cultivated from blood samples drawn after at least 1 year of complete remission after end of therapy. A reference group of children was matched for age, sex, and residence. The frequencies of sister chromatid exchange (5.4 versus 5.6 SCE/cell), and chromosome damage type gaps (6.6 versus 7.1%) and breaks (1.9 versus 1.9%) were not different in the two groups, but exchange type aberrations were more frequent in the patients (0.9 versus 0.06%). Fifty karyotypes were analyzed in all but two cases of Hodgkin 's disease. The overall frequency of stable (3.1 versus 3.8%) and unstable (1.7 versus 1.4%) structural chromosome changes such as translocations, deletions, chromatid exchanges, and dicentrics were not different in the patient and the control groups. If the chromosome data reflect a general cancer risk, this risk cannot be considerably higher among the cancer-treated children.     

Malignant Melanotic Neuroectodermal Tumor of Infancy: A Case Report

We present a case of melanotic neuroectodermal tumor in the maxilla that followed an aggressively malignant course. In the first biopsy the tumor showed a classical histological picture, while in the last biopsy only malignant undifferentiated cells were evident. The tumor recurred twice after excision, and in spite of radiotherapy and various chemotherapeutic measures the patient died within 16 months of onset. Similar cases reported in the literature are discussed.     

Pediatric Sex Cord-Stromal Tumor with Composite Morphology: A Case Report

A 12-year-old female with developmental delay/mental retardation and a family history of gynecologic cancers presented with nonspecific abdominal complaints and was found to have a 4.5-kg, 25-− × 23-− × 15-cm pelvic mass with solid and cystic components and associated retroperitoneal and mesenteric lymphadenopathy. Laboratory studies revealed increased serum levels of CA-125 and inhibin B. Histologically, the tumor exhibited several different morphologic appearances including adult granulosa cell tumor, juvenile granulosa cell tumor (with areas of marked atypia), and Sertoli cell tumor. Immunohistochemically, the tumor was positive for calretinin, MIC-2 (CD99), S100 protein, PGP 9.5, and neuron-specific enolase. Electron microscopy of the Sertoli cell tumor-like areas showed Charcot-Bottcher filaments, a distinguishing feature of Sertoli cells. Together, these findings supported a diagnosis of mixed sex cord-stromal tumor including granulosa cell tumor of adult and juvenile types and intermediate- to high-grade Sertoli cell tumor, with large areas of markedly atypical sex cord-stromal tumor.     

畸胎瘤样肾母细胞瘤治疗体会及文献回顾

目的 探讨儿童畸胎瘤样肾母细胞瘤临床诊断和治疗方法,提高临床对该病的认识.方法 回顾我院2007年至2016年间诊治的4例儿童畸胎瘤样肾母细胞瘤,男2例,女2例,年龄2～40个月,平均年龄21个月;原发灶:右肾3例,左肾1例;根据COG分期为Ⅰ、Ⅱ、Ⅲ、Ⅳ期(肺及骨髓)各1例,分析其临床表现、诊断及治疗方法.结果 4例患儿均行肾脏肿瘤根治性切除术,其中Ⅲ、Ⅳ期患儿术前行化疗再行根治术;3例术后辅以化疗,术后复发1例(化疗不敏感);3例早中期患儿预后可(2例随访时间超9年,1例随访3年),1例Ⅳ患儿再次手术后化疗加放疗中.结论 儿童畸胎瘤样肾母细胞瘤是肾母细胞瘤的罕见病理类型,临床症状通常难与经典肾母细胞瘤鉴别,主要依靠术前影像学及病理诊断,患侧肾脏肿瘤根治性切除术为主要治疗手段,早中期TWT患儿预后良好,至于保肾肿瘤切除术在该类型肾母细胞瘤的应用,暂未见文献报道,本组病例数较少,也未涉及.对于晚期TWT患儿,可能因为其独特的病理特征,其预后可能比经典肾母细胞瘤更差.     

Somatic growth and renal function after unilateral nephrectomy for Wilms' tumor

Solitary kidneys in renal donors and patients who have undergone unilateral nephrectomy for malignant disease have been reported to undergo hyperfiltration injury. This study was undertaken to evaluate the somatic growth and development of followed-up patient after Wilms' tumor to evaluate their renal function and identify any evidence of injury in the remaining kidney. The growth and development of all the children was found to be normal, as was DTPA clearance. Microalbuminuria in 24-h urinary collections was detected in 84% of the patients, indicating evidence of hyperfiltration injury. This study highlights the need for close monitoring of the renal function of long-term follow-up patients after Wilms' tumor in addition to the routine monitoring for tumor recurrence. Accepted: 26 May 1998     

Fatal Paradoxical Embolism to the Left Carotid Artery During Partial Resection of Wilms' Tumor

An 8-year-old boy with an uncorrected ventricular septal defect, pulmonary stenosis, mental retardation, and gigantism died 24 hours after partial resection of a large right-sided Wilms' tumor. The presence of other abnormalities, including a small umbilical hernia and overgrowth of the external genitalia, raises the possibility that this case represents a variant of the Beckwith- Wiedemann syndrome. The typical facial features of Sotos' syndrome were not present. Gross examination of the surgical specimen revealed that tumor was present in the resected margin of left renal vein. Necropsy showed that death resulted from extensive cerebral infarction due to occlusion of the left internal carotid artery and its branches by tumor emboli Paradoxical embolism had occurred during or soon after partial resection of the tumor mass due to passage of tumor fragments into the systemic circulation through the ventricular septal defect.     

Pediatric Inflammatory Myofibroblastic Tumor With Late Metastasis to the Lung: Case Report and Review of the Literature

Inflammatory myofibroblastic tumors (IMTs) are challenging lesions with respect to classification, differential diagnosis, and biologic potential. In children, extrapulmonary IMTs, particularly those from the abdomen or mesentery, are generally aggressive, with frequent local recurrences. There are few documented patients with distant metastasis, and most of these had metastases at presentation or developed metastases within months to a few years. We add to the short list of metastatic extrapulmonary IMTs a pediatric patient in whom the primary lesion was widespread in the abdomen at presentation and metastatic disease to the lung was diagnosed 9 years after the primary resection. We describe the clinical and pathologic features of this patient and review the characteristics of extrapulmonary IMTs with distant metastasis reported in the literature.