Atypical congenital mesoblastic nephroma presenting in the perinatal period.

Department of Paediatric surgery, The Children's Hospital at Westmead, Corner Hawkesbury Road and Hainsworth Street, Locked Bag 4001, Westmead, Sydney, 2145 NSW, Australia.
Pediatric Surgery International (Impact Factor: 1.06). 04/2007; 23(3):205-9.
Source: PubMed

ABSTRACT Congenital mesoblastic nephroma (CMN) is a rare tumour of infancy having an overall good prognosis. The less common, atypical CMNs have cellular elements in them and tend to have an unpredictable course. Occurrence in the perinatal period may further change the outcome. By reporting three patients presenting in the perinatal period with atypical CMN, an attempt is made in this paper to characterize the clinical behaviour of these variant tumours. Though one of our patients had an uneventful course, the other two had several complications including polyhydramnios, prematurity, hypertension, haemodynamic instability and tumour spillage. The course was complicated by recurrence in the latter two and refractoriness to chemotherapy and death in one. That the atypical subset of CMNs occurring in the perinatal period can have a stormy course is well illustrated by this report. Possible prognostic factors are evaluated and the sparse reports of similar cases in the literature are reviewed and compared.

  • [Show abstract] [Hide abstract]
    ABSTRACT: The prenatal diagnosis of abdominal mass poses the problem of its origin. Renal tumors are rarer than neuroblastoma but they are most often congenital mesoblastic nephroma. The congenital mesoblastic nephroma has a good forecast in spite of a sonographic impressive aspect. MRI can help to locate tumor but cannot tell difference between the different kinds of renal tumor. Prenatal forecast is especially linked with hydramnios and hydrops fetalis. Histolological study of the tumor is important for the prognosis. Two morphological subtypes are currently distinguished: the classic type with a good forecast and the atypical or cellular type. Distant metastases have been related only to the cellular form but especially in infants aged more than 3 months and never in the newborns. The diagnosis of the tumor does not change the mode of delivery except in case of an important volume. Complications are searched during the firsts days of life: hypertension, hypercalcemia, vomiting, hyperreninemia. Radical nephrectomy is performed after the end of the first week. In case of a classic form, the healing is always obtained. In case of cellular form, distant metastases are searched. In any rate, the follow-up is recommended until the end of the growth.
    Journal de Gynécologie Obstétrique et Biologie de la Reproduction 06/2009; 38(4):277-285. · 0.62 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Congenital mesoblastic nephroma is a rare renal tumor of early infancy with a favorable outcome after complete surgical removal. It consists of a heterogeneous group of spindle cell tumors. Early and accurate prenatal diagnosis of the renal tumor may improve the outcome of affected pregnancies by implementing the best strategy for prenatal management and delivery. But detection of congenital mesoblastic nephroma in a fetus is rare. To the authors' knowledge, there are fewer than 30 reports of a prenatal diagnosis of a mesoblastic nephroma in the literature. This case describes the prenatal sonographic diagnosis of cellular congenital mesoblastic nephroma.
    Journal of Diagnostic Medical Sonography 03/2009; 25(2):112-115.
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Congenital mesoblastic nephroma (CMN) is a mesenchymal renal tumor. The aim of the present study was to review the clinical characteristics and outcome of CMN in infants. A retrospective file review was conducted of eight cases of CMN treated at the Children's Hospital of Fudan University between 2004 and 2012. Ultrasound and computerized tomography scans had been performed on all eight patients. Two cases presented with a solid tumor and exhibited pathological features consistent with those of classic CMN, five cases exhibited cystic, hemorrhagic and necrotic characteristics, with calcification and pathology consistent with the cellular variant of CMN and one case presented with a solid tumor, which exhibited pathological features consistent with ceullular CMN. Histology confirmed classic CMN in two patients and cellular CMN in six patients. For surgical intervention, four cases had radical nephrectomy, one case had a half nephrectomy and three cases had tumor enucleation performed. Two cases had received pre-operative chemotherapy, but exhibited no response, and three cases received post-operative chemotherapy. Two patients were lost to follow-up, but the remaining six patients survived to the end of follow-up without further complications. The mean follow-up time was 24.6 months. In conclusion, the differential diagnosis between CMN and Wilms' tumor is critical. Imaging characteristics are partially correlated with pathological characteristics. Surgery is the main treatment for CMN, but pre-operative chemotherapy is not particularly effective. The efficacy of post-operative chemotherapy requires further investigation, but the prognosis is positive.
    Oncology letters 11/2014; 8(5):2007-2011. · 0.99 Impact Factor