ABSTRACT
Congenital mesoblastic nephroma (CMN) is the most common renal tumor in the first year of life. Here, we present unique findings of cellular variant CMN seen on prenatal and postnatal MRI with diffusion-weighted imaging (DWI).The mass was well-visualized on prenatal MR DWI with diffusion restriction in the solid portions. After excision of the mass, follow-up whole body MRI with DWI helped identify local tumor recurrence with suspicious liver metastasis. This hepatic lesion also showed diffusion restriction.
Subject(s)
Adult , Female , Humans , Infant, Newborn , Pregnancy , Combined Modality Therapy , Diffusion Magnetic Resonance Imaging/methods , Kidney Neoplasms/congenital , Liver Neoplasms/secondary , Nephroma, Mesoblastic/congenitalABSTRACT
INTRODUÇÃO: Nefroma Mesoblástico Con-gênito é uma rara neoplasia renal pediátrica. Apresenta dois subtipos histológicos, clássico e celular, sendo o último de pior prognóstico e responsável por aproximadamente dois terços dos casos. Esse tumor ainda é um desafio diagnóstico aos patologistas devido à similaridade com outras neoplasias pediátricas renais mais frequentes. RELATO DO CASO: Criança do gênero feminino, 2 anos e 9 meses de idade, foi encaminhada a serviço médico com referência em oncologia apresentando massa renal à esquerda. Após nefrectomia, o estudo do espécime mostrou, macroscopicamente, extensa área tumoral granular, brancoacinzentada, ocupando aproximadamente todo o rim, invadindo seio renal, cápsula e gordura perirrenal, com áreas de hemorragia e necrose. Histologicamente, caracterizava-se pela presença de células fusiformes e mitoses, sem atipias celulares. O diagnóstico foi de Nefroma Mesoblástico Congênito subtipo celular e a paciente foi submetida a quimioterapia. Durante o primeiro ano de tratamento, houve recidiva do tumor, apresentando-se irressecável e sem resposta a nova quimioterapia. A paciente foi a óbito aos 4 anos de idade. DISCUSSÃO: O subtipo celular do nefroma mesoblástico tende a ser mais agressivo, apresentando uma taxa de sobrevivência de 85 por cento, comparada com 100 por cento para a variante clássica. Geralmente, a recorrência ocorre no primeiro ano de tratamento, principalmente quando o subtipo é o celular.
INTRODUCTION: Congenital Mesoblastic Nephroma (CMN) is a rare pediatric renal tumor. It comprises two histological subtypes, namely classic and cellular, with the second accounting for two thirds of all cases and being more often associated with poor prognosis. It remains a diagnostic challenge for pathologists due to its similarity with other more frequent pediatric kidney neoplasms. CASE REPORT: We describe the case of a 2-year- old girl who presented with a left renal mass. After nephrectomy, the specimen analysis showed, on gross examination, an extensive, granular and whitish tumor lesion occupying almost the entire kidney, invading the renal sinus, capsule and perirenal fat, with areas of hemorrhage and necrosis. Histologically, it was characterized by ovoid spindle cells, mitoses and no cell atypia, which led to a diagnosis of cellular mesoblastic nephroma. Adjuvant chemotherapy was carried out, but tumor recurrence occurred in the first year, presenting as an unresectable tumor that did not respond to adjuvant chemotherapy and the patient died at 4 years of age. DISCUSSION: The cellular variant tends to be more aggressive, with a survival rate of 85 percent versus 100 percent for the classic variant. Recurrence generally occurs in the first year, particularly with the cellular variant.
Subject(s)
Humans , Female , Child, Preschool , Kidney Neoplasms/congenital , Nephrectomy , Nephroma, Mesoblastic/diagnosis , Nephroma, Mesoblastic/mortality , Nephroma, Mesoblastic/therapy , Kidney Neoplasms/diagnosis , Kidney Neoplasms/mortality , Kidney Neoplasms/pathology , Kidney Neoplasms/therapy , Nephroma, Mesoblastic/congenital , Fatal Outcome , Kidney Neoplasms/diagnosis , Kidney Neoplasms/therapy , Nephroma, Mesoblastic/diagnosis , Nephroma, Mesoblastic/therapyABSTRACT
Recent advancement in the cytopathologic features produced a number of variants of Wilms' tumor which are the primary determinant of survival of Wilms' tumor patients. This study was carried out with 47 patients of Wilms' tumor in different stages in three selected hospitals from 1991 to 1993. Among them 61.7% (29) were in Favorable histopathology and 38.3% (18) were in Unfavorable histopathology group. After managing the patients with multimodal therapy according to the protocol of National Wilms' tumor Study-III the favorable group had shown better prognosis. The difference between two groups was statistically significant (chi-square = 3.2, P < 0.05). Histopathological variations could be easily determined which might improve the overall prognosis of Wilms' tumor.
Subject(s)
Anaplasia , Bangladesh , Carcinoma, Renal Cell/pathology , Chi-Square Distribution , Child , Combined Modality Therapy , Humans , Kidney Neoplasms/congenital , Lymph Node Excision , Lymphatic Metastasis/pathology , Neoplasm Staging , Neoplasms, Germ Cell and Embryonal/pathology , Prognosis , Rhabdoid Tumor/pathology , Survival Rate , Wilms Tumor/congenitalABSTRACT
Atypical Mesoblastic Nephroma (AMN) is a rare infantile renal tumour which may behave aggressively, in contrast to conventional Mesoblastic nephroma. During a 10 year period we encountered four cases of AMN. The mean age of presentation was three months. Size varied from 9-18 cms. All were soft and fleshy with a weight varying from 290-1200 gms. Three cases revealed cystic changes. Hemorrhage and necrosis was seen in all four cases. One case had associated Nephroblastomatosis Complex. The capsule and ureter were involved in one case each. Two of the four patients died of distant metastasis. Thus a close periodic follow up by non-invasive imaging techniques is indicated for early detection of recurrence and or metastasis in cases of AMN.
Subject(s)
Child, Preschool , Female , Humans , Infant , Kidney Neoplasms/congenital , Male , Nephroma, Mesoblastic/congenitalABSTRACT
Descreve-se um caso de nefroma mesoblástico congênito em uma criança de seis anos de idade e discute-se a terapêutica e o curso da doença, como também analisa-se a literatura específica