A prevalência de escoliose em pacientes com diagnóstico de tumor de Wilms fora de tratamento oncológico / The Prevalence of Scoliosis in Patients Diagnosed with Wilms' Tumor with Follow-up free of Disease

Introdução: O tumor de Wilms é o tumor renal maligno mais comum na infância cujo tratamento combina cirurgia, quimioterapia e, em alguns casos, radioterapia. As alterações musculoesqueléticas têm sido descritas como os efeitos tardios e sequelas mais frequentes relacionados ao tratamento de radioterapia. Objetivo: Verificar a prevalência de escoliose em pacientes diagnosticados com tumor de Wilms, tratados com quimioterapia, e com ou sem radioterapia adjuvante após ressecção tumoral, fora de tratamento há pelo menos dois anos. Método: Estudo observacional transversal com 20 pacientes. Nove realizaram somente ressecção tumoral e quimioterapia (Grupo CQ) e 11 ressecção tumoral, quimioterapia e radioterapia (Grupo CQR). Todos os indivíduos da amostra foram submetidos à avaliação física geral e à avaliação postural por fotogrametria computadorizada. Resultados: A escoliose foi observada em 75% da amostra, 67% do grupo CQ e 82% do grupo CQR. Na avaliação física, as principais alterações encontradas no total da amostra foram assimetria horizontal dos ombros e anteversão pélvica; na avaliação postural por fotogrametria, as principais foram anteversão pélvica, ângulo diferente de 0º entre acrômios e as espinhas ilíacas anterossuperiores, assimetria do alinhamento horizontal das escápulas, assimetria do alinhamento pélvico horizontal e assimetria horizontal das escápulas em relação à T3. Conclusão: Pacientes diagnosticados com Tumor de Wilms, tratados com ou sem radioterapia adjuvante, e fora de tratamento oncológico há pelo menos dois anos, apresentam grande prevalência de alterações musculoesqueléticas, principalmente em relação ao desenvolvimento da escoliose.

Recurrent pulmonary tumoral embolism and sudden death as the presenting symptom of Wilms' tumor

Only 4-6% of children with renal tumor show inferior vena caval [IVC] or right atrial infiltration at presentation. Tumor emboli are even rarer, and so far, sudden death as the presenting symptom has only been described at presentation in Wilms' tumor [WT] in eight cases. We report a unique case of Wilms' tumor that presented with small pulmonary emboli and immediately after as that was diagnosed before death by detection of small emboli and immediately after sudden death as massive pulmonary embolism. It seems that in cases of invasive vascular infiltration with a stable hemodynamic condition at diagnosis immediate surgery may be necessary

Tumor de Wilms: características clínicas e cirúrgicas / Wilms'tumor: clinical and surgical features

O tumor de Wilms (TW) é o tumor renal maligno mais comum na infância. O conhecimento de certas características clínicas e a realização de procedimentos cirúrgicos adequados podem ter impacto no prognóstico desta doença. Revisados prontuários de pacientes com diagnóstico de TW entre 1989 e 2005. Coletados dados demogrãficos, caracteríisticas clínicas e avaliação de procedimentos cirúrgicos. Durante o ato operatório, 38 pacientes foram submetidos à avaliação do rim contralateral através da palpação e em 13 relatos de cirurgia não foram encontradas descrições. Ruptura tumoral em 1 paciente; em dez prontuários havia registro de ausência de ruptura tumoral e em 41 prontuários não havia qualquer menção quanto à presença ou ausência dessa complicação. A histopatologia confirmou 45 casos de histologia favorável e os demais de histologia desfavorável. Os resultados apresentados permitem concluir que os pacientes estudados apresentam características demográficas gerais semelhantes aos da literatura.Considerando-se que em um número expressivo de pacientes observou-se falta de aderência a certas etapas do procedimento cirúrgico, incluindo ausência de biópsia de linfonodos e atrasos na realização da ressecção tumoral, os autores recomendam que o cirurgião pediátrico tenha uma participação mais efetiva na equipe multidisciplinar e na elaboração das rotinas do protocolo cirúrgico para pacientes com TW

Wilms´tumor (WT) is the most common malignant renal tumor in childhood. The knowledge of the clinical characteristics and the accomplishment of standard surgical procedures may have an impact in the prognosis of this disease. Medical records of newly diagnosed WT patients treated from 1989 to 2005 were reviewed. We collected data on demographics, clinical characteristics and whether certain recommended surgical standard procedures were carried out.The surgeon in 38 patients performed palpation of the contralateral kidney and in 13 medical records there was no report whether this procedure was carried out. Tumor spillage was reported 1 patient, reported as absent in 10 patients; we were unable to find any mention about tumor rupture on the surgeon’s report for 41 patients. There were 45 cases of favorable histology and 7 of unfavorable histology. The OS was of 69%, 71%, 79%, 50% and 40% for the stages I, II, III, IV and V, respectively. Five years OS was 73% and 65,2% for patients submitted to surgery before and after the 6th week after diagnosis, respectively. The results of the present study indicate that patients in this study show demographic characters similar to the literature. Considering that surgeons did not performed standard recommended surgical procedure such as lymph nodes biopsy and carried out late surgical resection of the primary tumor in many patients, there is a need for a more effective participation of the surgeon in the multidisciplinary team and possiblyin the designing protocols for the surgical management of patients with TW

Immunohistochemical expression of P53 protein in histologically favorable Wilms tumor and its relationship to tumor stage at presentation

Wilms tumor, as the most common renal tumor of children, has been associated with chromosomal abnormalities. Although a correlation between anaplasia and mutations of P53 tumor suppresser gene has been found in Wilms tumor, significance of these mutations in different clinical stages of favorable- Wilms tumor, remains largely unresolved. The goal of this study was to determine the frequency of P53 expression in histologically favorable Wilms tumors and its correlation to tumor-stage at presentation. In this retrospective study, 48 cases of confirmed Wilms tumor with favorable-histology were retrieved from the files of departments of pathology in three hospitals in Mashhad University of Medical Sciences between 1990 and 2004. Histological characteristics and clinicopathological staging were in accordance with National Wilms Tumor Study guidelines. P53 expression was determined by the immunohistochemical method. For each section, the proportion of neoplastic cells exhibiting nuclear positivity was broadly quantified and their intensity of staining was charted, based on visual impression by two pathologists. A total of 48 cases of histologically favorable Wilms tumor were assessed. Eleven cases [23%] showed positivity for P53 which were 3 [27.3%] with stage II, 3 [27.3%] with stage IV, 2 [18.2%] with stage I, 2 [18.2%] with stage III and 1 case [9.1%] with stage V. The P53 immunopositivity was seen in 1-25% of tumor cells in 9 cases [18.8%], in 26 to 50% of tumor cells in 1 case [2.1%] and in >75% of tumor cells in the other one case [2.1%]. The intensity of staining was moderate in 6 cases [12.5%], weak in 4 [6.3%] and strong only in one case 4.2%]. The most common component with P53 immunoreactivity was blastemal in 11 cases [100%]. In 4 cases [36.4%] there was also positivity in epithelial and in 2 cases [18.2%] in mesenchymal components. We found no correlation of P53 immunoreactivity and its intensity to tumor stage at presentation in individuals with histologyically favorable Wilms tumors [p=0.66, p=0.52 respectively]

Aniridia and Wilm's tumor.

Aniridia (absence of iris) is a congenital, bilateral, uncommon panocular disorder. Whereas the occurrence of aniridia in the general population is 1:50000, it is present in about 1 in 70 patients with Wilm's tumor. This aniridia is sporadic and Wilm's tumor in these cases presents at an unusually early age. Aniridia was present in two cases out of 60 cases of Wilm's tumor operated at PGIMS, Rohtak. Both cases presented below two years of age. The recognition of a child with sporadic aniridia should alert to the increased risk of development of Wilm's tumor.

Sarcoma de células claras de riñón metastásico a mandíbula. Informe de un caso / Clear cell sarcoma of kidney metastatic to the mandible. Report of a case

Se presenta el caso de un paciente masculino de 8 años de edad, con antecedentes de nefroblastoma (tumor de Wilms) a la edad de cinco años, con manejo terapéutico a base de nefrectomía, quimioterapia y radioterapia, quien treinta meses después presentó aumento de volumen en hemicara izquierda con lesión lítica en cuerpo y rama ascendente del lado izquierdo mandibular. La revisión de laminillas del tumor original se interpretó como sarcoma de células claras de riñón. La lesión mandibular mostró la misma imagen, con lo cual el diagnóstico fue de sarcoma de células claras de riñón, metastásico a hemimandíbula izquierda. Este caso es excepcional y en nuestro conocimiento el primero en la literatura mundial. Llamamos la atención sobre la dificultad diagnóstica, tanto en la entidad primaria como en la metástasis, en este último caso con diagnósticos diferenciaels de sarcomas primarios como metástasis

Hepatopathy-thrombocytopenia vs infection-induced hemophagocytic syndrome in Wilms' tumor: a case report.

A 3-year-old boy with Wilms' tumor, post operative left nephrectomy stage, had HTS on day 99 of the combined chemotherapy which lasted for more than 20 days. He had severe respiratory distress due to a moderate amount of ascites and marked pleural effusion. Because of high fever, thrombocytopenia and marked hemphagocytosis in the bone marrow, he received IVIG for 2 days. Normal platelet count and markedly decreased pleural fluid were attained within 3 days. He subsequently tolerated full doses of combined chemotherapeutic agents with an additional one (doxorubicin). In cases of HTS, IAHS should be suspected. The bone marrow should be done and treatment accordingly so that there is no need to decrease, the dosage of chemotherapeutic agents afterwards.

Aniridia--Wilms' tumour association--a case with 11p 13-14.1 deletion and ventricular septal defect.

A two year old female child with bilateral wilms tumor (WT) along with multiple congenital anomalies like bilateral aniridia with congenital cataracts and nystagmus, microcephaly, mental retardation and ventricular septal defect has been described. The karyotype analysis revealed 46 xx, del 11p 13-14.1. Association of ventricular septal defect with the classical features of 'Aniridia-Wilms' tumor association' is an unusual feature in this case.

Wilms tumour associated with unilateral dysplasia of the iris.

We report a case of Wilms tumour associated with unilateral partial aniridia. Although Wilms tumour is known to be associated with bilateral aniridia, there is only one previously reported case associated with dysplasia of the iris.

Wilms' tumor, male pseudohermaphroditism and glomerulonephritis: the Drash syndrome. First case report in Thailand and review the literatures.

The Drash syndrome, the first case in Thailand is reported. The patient had male pseudohermaphrodite, Wilms' tumor of the left kidney and mesangial proliferative glomerulonephritis. Metastasis of Wilms' tumor was noted in the liver, omentum, pelvic peritoneum and vertebral body. Chemotherapy (actinomycin D, vincristine and dexamethasone) and local irradiation were given. The patient developed pneumonia, diarrhea and ended up with Steven-Johnson syndrome from Cotrimoxazole hypersensitivity.

Metastasis cerebral de tumor de Wilms / Cerebral metastases of wilms' tumor

Se presenta el caso de un niño de doce años de edad, portador de una metástasis cerebral de un tumor de Wilms descubierto y extirpado mediante nefrectomía a los cinco años, de edad. Posteriormente fue intervenido en 2 ocasiones por presentar metástasis pulmonares a los seis y siete años de edad, agregándose nuevamente radioterapia y adicionándose, quimioterapia. Su enfermedad se estabilizó, entonces por espacio de cuatro años y medio al cabo de los cuales aparece signología neurológica que motiva una exploración con Tomografía Axial Computada (TAC) de cráneo demostrándose, una lesión altamente sugerente de metástasis en la región parietal superior izquierda. La posterior craneotomía y remoción confirman histopatológicamente, la presencia de una metástasis cerebral del Tumor de Wilms original