Suprahepatic Cava Thrombus Due to Wilms Tumor: Technique Without Cardiopulmonary Bypass.

Wilms tumor is a frequent malignant neoplasia in pediatric population. Extension to the inferior vena cava is a complication that occurs in approximately 4%-15% of cases. Surgical techniques derived from the field of adult transplant surgery allow the resection of the tumor with its thrombus extension. In the case of a 6-year-old male patient with a stage III Wilms tumor that originated from the left renal vein, thrombectomy and left radical nephroureterectomy were accomplished without extracorporeal circulation. Surgical technique applied in adult transplant surgery for removal of advanced renal tumors, could be a safe and feasible technique in pediatric population.

Unusual cases: melanomatosis and nephroblastomatosis treated with hyperthermic intraperitoneal chemotherapy.

Hyperthermic intraperitoneal chemotherapy (HIPEC) is a well-established therapy for carcinomas, mesotheliomas, and some sarcomas. However, HIPEC has not been reported in melanomatosis or nephroblastomatosis. Here we present 2 unique cases of cytoreductive surgery and HIPEC. Cytoreductive surgery followed by HIPEC was performed in a 4-year old with melanomatosis and a 12-year old with nephroblastomatosis. A 4-year-old girl presented with leptomeningeal melanoma that metastasized from a congenital nevus. She had a ventricular peritoneal shunt placed as an infant. Melanomatosis involving the meninges and peritoneal surfaces was identified by positron emission tomographic scan imaging. Extensive plaques of melanoma were removed at the time of surgery, followed by HIPEC. She had no significant postoperative complications. Her abdomen remained free of disease 7 months after treatment. A 13-year-old girl presented with a Wilms' tumor at age 10 years. She then presented to us at the second local recurrence with multiple intraabdominal tumor implants (ie, nephroblastomatosis). We performed cytoreductive surgery followed by HIPEC. She had no postoperative complications and, at 1 year post-HIPEC, had no abdominal recurrence. We conclude that HIPEC can be performed safely for rare diseases such as melanomatosis and nephroblastomatosis. Although abdominal disease control can be realized, further systemic treatment options are necessary. A review of indications for HIPEC is included.

A retrospective study of the preoperative treatment of advanced Wilms tumor in children with chemotherapy versus transcatheter arterial chemoembolization alone or combined with short-term systemic chemotherapy.

PURPOSE: To evaluate the therapeutic effect of preoperative transcatheter arterial chemoembolization (TACE) combined with short-term systematic chemotherapy in the treatment of advanced Wilms tumor. MATERIALS AND METHODS: This was a retrospective study on 66 patients with unilateral advanced Wilms tumor, age 5 months to 11 years (median, 2.9 years; 30 boys and 36 girls), treated at our institution between 1995 and 2007. Characteristics of the patient population were maximal tumor diameter > 10 cm, or involvement of periaortic lymph nodes, or inferior vena cava invasion, or distal metastasis, or tumor with anaplastic histology. Patients were divided into three groups. Twenty patients were treated with conventional preoperative chemotherapy (PC group) using vindesine, actinomycin D, and pirarubicin for 4 weeks; 21 patients were treated in the TACE group with preoperative renal arterial chemoembolization using Lipiodol-pirarubicin-vindesine emulsion; and 25 patients were treated with preoperative chemoembolization combined with short-term systematic chemotherapy (T+S) for 2 weeks. RESULTS: No drug-induced cardiotoxicity, nephrotoxicity, or hepatic dysfunction was observed. Complete surgical removal of the tumor was achieved in 12 (65.0%), 17 (80.9%), and 22 (88.0%) patients in the PC, TACE, and T+S groups, respectively (T+S group vs PC group, P = .030). The 2-year relapse-free survival rates were 65.0%, 80.9%, and 100.0% in the PC, TACE, and T+S groups, respectively (T+S vs PC, P = .001). CONCLUSIONS: From our experience, preoperative chemoembolization combined with short-term systematic chemotherapy is able to achieve higher rates of complete tumor resection and relapse-free survival in the treatment of advanced Wilms tumor.

Acute malnutrition is common in Malawian patients with a Wilms tumour: A role for peanut butter.

BACKGROUND: Children with cancer in resource limited countries are often malnourished at diagnosis. Acute malnutrition is associated with more infectious complications and an increased risk of morbidity and mortality in major surgery. METHODS: All new patients with the clinical diagnosis of a Wilms tumour admitted in the Queen Elizabeth Central Hospital, Blantyre, Malawi from January 2007 until June 2008 were included. We documented anthropometric parameters, tumour size and serum levels of micronutrients at diagnosis. Corrected weight (body weight - tumour weight) was repeated after 4 weeks of preoperative chemotherapy. During therapy oral feeds were encouraged and a locally made ready to use therapeutic peanut butter-based food (chiponde) supplied. RESULTS: A high rate of acute malnutrition was found in patients with Wilms tumour at diagnosis (45-55%), much higher than in community controls (11%). Patients (40%) and community controls (37%) had a similar, high rate of stunting (low height for age), a sign of chronic malnutrition. Tumour size at diagnosis and the degree of acute malnutrition at diagnosis was correlated; patients with a larger tumour had more severe acute malnutrition (r = -0.88, P < 0.01). With a supply of chiponde, 7 of 18 patients had a >5% increase in corrected weight during preoperative chemotherapy. Patients with a more positive nutritional course had a better tumour response to chemotherapy (r = 0.52, P < 0.05). Surprisingly, few micronutrient deficiencies were found, except for low serum levels of vitamin A (44% of patients). CONCLUSION: Acute malnutrition, superimposed on chronic malnutrition, is common in patients with Wilms tumour in Malawi. Earlier presentation needs to be encouraged. Chiponde, a peanut butter based ready-to-use-therapeutic-food, is an attractive means of nutritional support which needs further study.

Chylous ascites following surgical treatment for wilms tumor.

PURPOSE: Postoperative chylous ascites is a rare complication of retroperitoneal surgery that has considerable morbidity. We review the pathogenesis and management of chylous ascites following surgical treatment of Wilms tumor. MATERIALS AND METHODS: We identified 9 children with chylous ascites after surgical treatment of Wilms tumor. Of these cases 3 were treated at a single institution during the last 20 years and 6 were identified during retrospective chart reviews of patients enrolled in National Wilms Tumor Studies 3 and 4 to identify surgical complications. Chylous ascites presented as increased abdominal girth and poor feeding. Paracentesis or laparotomy was diagnostic. RESULTS: Patient age at presentation with Wilms tumor ranged from 6 to 95 months (median 15). Left nephrectomy was performed in 5 cases, right nephrectomy in 3, and left nephrectomy and partial right nephrectomy in 1 with bilateral disease. Lymphadenectomy including the hilar and periaortic lymph nodes was performed in 5 patients, 4 of whom also underwent some form of suprahilar lymph node dissection. Three patients underwent lymph node sampling of the hilar, periaortic and some suprahilar lymph nodes. All children received adjuvant chemotherapy and 4 were treated with adjuvant irradiation to the surgical bed before the diagnosis of chylous ascites. The interval between surgery and diagnosis of ascites ranged from 12 to 49 days (median 21). Of the patients 7 were successfully treated with conservative measures, total parenteral nutrition and/or a diet containing primarily medium chain triglycerides, and 2 required invasive procedures, including exploratory laparotomy and ligation of disrupted lymphatic vessels or placement of a peritoneovenous shunt. CONCLUSIONS: Extensive lymph node dissection, particularly above the level of the renal hilum, appears to be associated with the development of postoperative chylous ascites. The National Wilms Tumor Study guidelines do not require formal lymph node dissection for staging and only lymph node sampling is recommended. Elimination of formal lymphadenectomy along with meticulous ligation of lymphatics should decrease the incidence of this complication. Fortunately, conservative treatment with total parenteral nutrition and/or medium chain triglycerides will remedy the problem in the majority of children.

[Antioxidant defense status of children in the early post-operative period in Wilms' tumor] . / Aktivnost' antioksidantnoi zashchity v rannem posleoperatsionnom periode u detei, bol'nykh nefroblastomoi.

Lipid peroxidation (LP) was studied in 85 patients, aged 1-14, with Wilms tumor (stage II-IV). In addition to standard infusions, patients of group I received ascorbic acid (AA) 15 mg/body weight postoperatively while, in group II, AA 25-35 mg/body was supplemented with alpha-tocopherol 1.4-1.9 mg/body. LP was evaluated by assaying malonic dialdehyde (MDA) and--such enzymes A3 superoxide dismutase--SOD; catalase--CA and nonenzymatic components (AA and blood-alpha-tocopherol) for status of antioxidant defense. Medium mass molecules (MMM) were used as markers of endogenous intoxication. Said procedures were carried out before surgery and on days 1, 3 and 5 after operation. A significant decrease in endogenous AA and alpha-tocopherol was established, as compared with healthy children, while a significant increase was registered in MDA, MMM, SOD and CA levels. The LP investigation confirmed the systemic impact of tumor. Surgical stress contributed to the rise of LP thus compromising antioxidant defense. Postoperative administration of high doses of AA in combination with alpha-tocopherol reduced the rates of LP processes.

Uterine mullerian adenosarcoma following adenomyoma in a woman on tamoxifen therapy.

The clinical and pathologic findings in a 53-year-old woman who developed a uterine adenosarcoma following an adenomyoma are described. During the interval between the diagnosis of adenomyoma and the subsequent diagnosis of adenosarcoma, the patient developed breast carcinoma and received adjuvant chemotherapy that included tamoxifen. The possible stimulatory effects of this drug upon the patient's pre-existing adenomyoma are discussed in view of reports of tamoxifen-associated endometrial carcinoma and uterine sarcomas developing in the setting of estrogen excess.

Autotransfusion

En este trabajo se reporta un caso en el cual se usó el método de autotransfusión en lugar de la transfusión de sangre homóloga durante la extirpación de un tumor abdominal gigante (Wilms), llevando un estricto control de los parámetros sanguíneos con énfasis en el nivel de hemoglobina y hematocrito antes, durante y después de la operación. Se hace una evaluación de los peligros de una transfusión de sangre homóloga, sobre todo ahora con la amenaza del S.I.D.A., en comparación con las ventajas de los diferentes métodos de autotransfusión como una alternativa en las intervenciones quirúrgicas