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.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Campagnola S, Fasoli L, Flessati P et al (1998) Congenital cystic mesoblastic nephroma. Urol Int 61:254–256
Leclair MD, El-Ghoneimi, Audry G, Ravasse P, Moscovici J, Heloury Y, French Pediatric Urology Study Group (2005) The outcome of prenatally diagnosed renal tumours. J Urol 173:186–189
Schenk JP, Schrader C, Furtwangler R et al (2005) MRI- morphology and staging of congenital mesoblastic nephroma: evaluation of a collection with 20 patients. Rofo 177:1373–1379
Moore SW, Satge D, Sasco AJ, Zimmermann A, Plaschkes J (2003) The epidemiology of neonatal tumours. Report of an international working group. Pediatr Surg Int 19:509–519
Chen WY, Lin CN, Chao CS et al (2003) Prenatal diagnosis of congenital mesoblastic nephroma in mid-second trimester by sonography and magnetic resonance imaging. Prenat Diagn 23:927–931
Bisceglia M, Carosi I, VairoM, Zaffarano L, Bisceglia M, Creti G (2000) Congenital mesoblastic nephroma: report of a case with review of the most significant literature. Pathol Res Pract 196:199–204
Daskas N, Argyropoulou M, Pavlou M, Andronikou S (2002) Congenital mesoblastic nephroma associated with polyhydramnios and hypercalcemia. Pediatr Nephrol 17:187
Garbyal RS, Aryya NC, Gangopadhya AN, Gupta DK (2002) Cystic atypical mesoblastic nephroma. Pediatr Surg Int 18:123–127
Murthi GV, Carachi R, Howatson A (2003) Congenital cystic mesoblastic nephroma. Pediatr Surg Int 19:109–111
Malone PS, Duffy PG, Ransley PG, Ridson RA, Cook T, Taylor M (1989) Congenital mesoblastic nephroma, renin production, and hypertension. J Pediatr Sur 24:599
Siemer S, Lehmann J, Reinhard H et al (2004) Prenatal diagnosis of congenital mesoblastic nephroma associated with renal hypertension in a premature child. Int J Urol 11:50–52
Glick RD, Hicks MJ, Nuchtern JG, Wesson DE, Olutoye OO, Cass DL (2004) Renal tumours in infants less than 6 months of age. J Pediatr Surg 39:522–525
Loeb DM, Hill DA, Dome JS (2002) Complete response of recurrent cellular congenital mesoblastic nephroma to chemotherapy. J Pediatr Hematol Oncol 24:478–481
Speleman F, van den Berg E, Dhooge C et al (1998) Cytogenetic and molecular analysis of cellular atypical mesoblastic nephroma. Genes Chromosomes Cancer 21:265–269
McCahon E, Sorensen PH, Davis JH, Rogers PC, Schultz KR (2003) Non-resectable congenital tumors with the ETV6-NTRK3 gene fusion are highly responsive to chemotherapy. Med Pediatr Oncol 40:288–292
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Jones, V.S., Cohen, R.C. Atypical congenital mesoblastic nephroma presenting in the perinatal period. Pediatr Surg Int 23, 205–209 (2007). https://doi.org/10.1007/s00383-006-1831-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-006-1831-4