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Case 354

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2 years old male patient presented red urine 4 months ago and recurrence 2 months ago, according to his mother. Urine analysis revealed multiple red blood cells per field, with 4% dysmorphic erythrocytes and proteinuria. Abdominal ultrasound showed increased right kidney with preserved contour and morphology, and presence of nodular formation of slightly increased echogenicity on its superior third, measuring 4,7 x 3,7 cm. Upper abdomen MRI was then requested.

Considering the clinical data and the images, the most likely diagnosis is:

a) Neuroblastoma

25%

b) Renal cell carcinoma

25%

c) Wilms Tumor

25%

d) Simple renal cyst

25%
   

Image analysis

 

Image 1 - Analysis: Magnetic resonance imaging (MRI) of the upper abdomen, coronal reconstruction, T2-weighted, without contrast administration. Expansive round heterogeneous formation in the right kidney’s upper third, partially defined limits, with hypersignal areas and no dislocation. Left kidney’s parenchyma with preserved corticomedullar differentiation.

 

Image 2 - Analysis: Magnetic resonance imaging (MRI) of the upper abdomen, axial views at the kidneys level, T1-weighted, before (A and B) and after (C and D) intravenous contrast administration. Expansive round heterogeneous formation in the right kidney’s upper third (blue circle), partially defined limits, and small heterogeneous highlight from IV contrast (pink circle). Presence of peripheric hypersignal T1-wheighted areas suggestive of hemorrhage (green arrow), as well as central hyposignal area (green circle). Additional information: absence of right renal vein or inferior vena cava invasion. Absence of increased lymph nodes. Adrenal glands of preserved morphology and dimensions .

 

Highlights

-   Paediatrics haematuria approach demands: complete clinical history, cautious physical examination, urine analysis and dysmorphic erythrocytes research;

-   Some causes of macroscopic haematuria with dysmorphic erythrocytes on the infancy includes glomerulopathies (highly dysmorphic), trauma, infection, tumors and lithiasis (less dysmorphic). On Wilms tumor, haematuria may be micro or macroscopic;

-   When suspecting Wilms tumor, imaging evaluation starts with abdominal US and, when necessary, CT or MRI;

-   Wilms tumor manifestation includes: palpable abdominal mass (60%), high blood pressure (25%) and haematuria (15%);

-    Treatment consists on primary tumor resection and chemotherapy. The best moment to start chemotherapy (before or after surgery) is controversial.

 

References

- Penido MGMG, Cardoso LSB, Veloso S. Hematúrias. In: Leão E, Corrêa EJ, Mota JAC, Viana MB, Vasconcellos MC. Pediatria ambulatorial. 5 ed. Belo Horizonte: Coopmed. 2013. p. 917-28.

- Szychot et al. Wilms’ tumor: biology, diagnosis and treatment. Transl Pediatr 2014;3(1):12-24.

- Daw NC, Huff V, Anderson PM. Neoplasms of the Kidney. In: Stanton BF, St Geme, JW, Schor NF, Behrman RE. Nelson Textbook of Pediatrics. 20 ed. Philadelphia: Elsevier. 2016. p. 2464-68.

- Silva DB, Barreto JHS, Pianovski MAD. Tumores Sólidos. In: Burns DAR, Júnior DC, Silva LR, Borges WG e Blank D. Tratado de Pediatria: Sociedade Brasileira de Pediatria. Barueri: Manole. 2017. P. 1552-59.

- Guimarães MD, Chojniak R. Oncologia. 1st Edition. Rio de Janeiro: Elsevier, 2014. Colégio Brasileiro de Radiologia.

 

Author

Mateus Jorge Nardelli, 5th year medical student at Universidade Federal de Minas Gerais.

Email: mateus.nardelli[at]gmail.com

 

Supervisor

Karla Emília de Sá Rodrigues, MD, Ph.D., Associated Professor and Doctor of the Department of Pediatrics at the Medical School of Universidade Federal de Minas Gerais.

Email: kesrodrigues[at]gmail.com

 

Ana Cristina Simões e Silva, MD, Ph.D., titular professor at the Paediatrics Department of UFMG’s Medical School, CNPq’s Paediatric Nephrology Study Group’s leader.

E-mail: acs[at]medicina.ufmg.br

 

Júlio Guerra Domingues, MD, substitute professor at the Imaging and Anatomy Department of UFMG’s Medical School

Email: jgdjulio[at]gmail.com

                               

Reviewers

Vinícius Avelar, Daniela Manso, William Alves, Prof. José Nelson Mendes Vieira, Profa. Viviane Santuari Parisotto Marino.

 

Translated by

Bruno Campos Santos, 6th year medical student at Universidade Federal de Minas Gerais.

E-mail: bruno_campos[at]outlook.com

Test question

3 year-old patient, previously healthy and asymptomatic, comes to routine general paediatrics consultation which identified a mass on the right lateral portion of the abdomen. Mother revealed that the patient’s urine has recently become red. Patient was, then, referred to specialized centre for diagnostic approach.

Based on the clinical data provided, considering costs, accessibility, celerity, adverse effects and information provided, which is the most adequate approach to be requested as the first-line investigation for a neoplastic hypothesis:

a) Urine analysis

25%

b) Abdomen radiography

25%

c) Abdominal ultrasound

25%

d) Abdominal MRI

25%

e) Positron-emission tomography

25%
   

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