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Case 184

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MCT, female, 28 years old, gestational age of 32 weeks, was referred to the fetal medicine service due to the suspicion of fetal hydronephrosis in the previous ultrasonography (USG). A new USG showed a solid abdominal mass, with varying echogenicity, measuring 5,7x6,6 cm with intense flow on the Doppler velocimetry. For better assessment of the mass, a MRI was requested, which revealed a complex and well-defined image.

Based on clinical history and imaging tests, which the most likely diagnosis?

a) Congenital adrenal neuroblastoma

25%

b) Congenital mesoblastic nephroma

25%

c) Wilms Tumor

25%

d) Congenital malignant rhabdoid tumor

25%
   

Image Analysis

 

Image 1: obstetric ultrasound, in which one can notice a solid, unilateral, encapsulated mass, of heterogeneous echogenicity, measuring 5,7x6,6cm (in green).

 

Image 2: Intense flow on the Doppler velocimetry, which makes  the tumor’s vasculature clear (red).

 

Images 3 and 4: Magnetic resonance imaging in the sagittal and axial cuts, respectively, showing more clearly the fetal anatomy and the contrast between the tissues, showing unilateral involvement of the kidney, restricted to the capsule without invasion of adjacent structures, as marked in blue.

Highlights

- Mesoblastic Congenital Nephroma (MCN) is the most common renal tumor in infants under three months of age;

- There are two main subtypes of MCN: the classic, most common and benign in nature; and the cellular, the rarest and most aggressive type;

- Prenatal diagnosis is made by ultrasound, which reveals unilateral solid, encapsulated mass of heterogeneous echogenicity;

- The MRI is a valuable complementary assessment method since it permits better view of  the anatomy, as well as the relation between tumor and the adjacent structures and the limited involvement of the renal capsule;

- Despite the good prognosis, in most cases recurrence can occur after surgical resection, making long-term follow-up necessary.

References

- Glick RD, Hicks MJ, Nuchtern JG, et al. Renal tumors in infants less than 6 months of age. J PediatrSurg 2004;39:522–525.

- Isaacs H Jr. Fetal and neonatal renal tumors. J PediatrSurg2008;43:1587–95.

- Sebire NJ, Vujanic GM. Paediatric renal tumours: re­cent developments, new entities and pathological featu­res. Histopathology 2009; 54:516-28.

- Ahmed HU, Arya M, Levitt G, Duffy PG, Mushtaq I, Sebire NJ. Part I: Primary malignant non-Wilms’ renal tumours in children. Lancet Oncol 2007; 8:730-7.

-Kotani TSumigama SHayakawa HMano YTsuda H, Sugiyama C, Kawachi FHironaka M, Kato T, Kaneko K, Hayakawa M, Kikkawa F. Elevated levels of aldosterona in the amniotic fluid in two cases of congenital mesoblásticonephroma. UltrasoundObstetGynecol. 2010 Aug;36(2):256-8.

- Gruver AM, Hansel DE, Luthringer DJ, MacLennan GT. Congenital mesoblásticonephroma. J Urol. 2010 Mar;183(3):1188-9.

- Montaruli EFouquet V. Prenataldiagnosisof congenital mesoblasticnephroma. Fetal DiagnTher. 2013;33(1):79-80

Authors

- André L. Pimenta, fourth year medical student at UFMG. E-mail: andre.19.lp[at]hotmail.com

- Ana Luiza F. Campos, fourth year medical student at UFMG. E-mail: aninhaalfc8[at]hotmail.com

- Ana Luiza M. Tavares, sixth year medical student at UFMG. E-mail: analuizamt[at]gmail.com

Supervisor

Prof. Dr. Júlio Cesar Faria Couto. Master's degree in Obstetrics and Gynecology by UFMG. Specialist in Fetal Medicine by FEBRASGO. Professor of Obstetrics in Faculdade de Medicina do Vale do Aço.

Reviewers

André Guimarães, Carla Faraco, Luanna Monteiro, Bárbara Queiroz, Daniela Braga, Débora Faria and prof. Viviane Parisotto.

Translated by

Bárbara de Queiroz e Bragaglia, 5th year medical student at UFMG. E-mail: barbara.bragaglia[at]gmail.com

 

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