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Case 342

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Woman, 13 years old, looked for medical assistance because of abdominal increasing volume, starting about 6 months ago. Her family suspect of pregnancy. At physical examination: skinny, weight: 30kg (zscore – 2.4), height: 1,54 m (zscore -0.6), anicteric; restrictive respiratory pattern (RR: 22bpm) and abolished breath sound at the base of the right hemithorax. Big abdominal mass without defined limits, petreous consistence without pain on palpation, associated to collateral circulation since upper abdominal level to the neck. Laboratorial exams: diminished levels of albumin and hemoglobin; high levels of gamma glutamyl transferase, alkaline phosphatase and lactate dehydrogenase; normal levels of transaminases, bilirubin, sorologys, alpha fetoprotein and beta hCG. An abdominal ultrasonography confirmed the suspect of a liver mass.

Based at the clinical history and the presented images, what is the most probable diagnosis?

a) Hepatoblastoma

25%

b) Hepatic hemangioma

25%

c) Rhabdomyosarcoma of the biliary tree

25%

d) Undifferentiated embryomal sarcoma

25%
   

Image analysis

Image 1: Chest simple radiography, anteroposterior view (AP). Elevation of the right dome of the diaphragm (blue line), due to big liver mass, that provoques dimensional reduction of ipsilateral lung (red dotted line).

 

Image 2: Chest and abdominal computed tomography (CT), before and after intravenous injection of iodinated contrast medium, axial slices. Non-contrast-enhanced phase (A and B). Post-contrast phase: portal (C) and balance (D). Voluminous liver mass, mostly solid, without defined limits, showing heterogeneous enhancement by contrast, with peripheral areas with more evident enhancement (green arrow). Presence of some internal dense formations, probably corresponding to septa.

 

Image 3: Chest and abdominal magnetic resonance (MR), before and after intravenous injection of gadolinium. Non-contrast-enhanced phase, T2 weighted image, axial plane (E). Portal phase, T1 weighted image, axial plane (F) and coronal plane (G). Voluminuous hypotense liver mass, heterogeneous (yellow circles), with multiple septa (blue arrows).

Highlights

- The UESL is the third most common primary liver neoplasia in childhood;
- The differential diagnosis is made with: hepatoblastoma, mesenchymal hamartoma and the rhabdomyoscaroma of the biliary tree.
- It has few symptoms and few alterations on liver function tests, even when it gets a big volume.
- The image test of choice to define the diagnosis is the CT, while the MR is important to define vascular invasion on surgical planning. 
- The diagnosis confirmation requires anatomopathological examination using immunofluorescence technique.
- Treatment is surgical, combined to adjuvant chemotheraphy to reduce recurrence and increase survival.

References

- Silva Isabela C, Rocha Antonio José da, Giuseppe Dippolito, Caldana Rogério. Série Gastrointestinal: Colégio Brasileiro de Radiologia e Diagnóstico por Imagem. 1. Ed. Elsevier; 2011.
- Putra J, Ornvold K. Undifferentiated Embryonal Sarcoma of the Liver: A Concise Review. Archives of Pathology & Laboratory Medicine: February 2015, Vol. 139, No. 2, pp. 269-273.
- Lenze F, Birkfellner T, Lenz P, et al. Undifferentiated embryonal sarcoma of the liver in adults. Cancer. 2008;112(10):2274–2282.
- Nishio J, Iwasaki H, Sakashita N, et al. Undifferentiated (embryonal) sarcoma of the liver in middle-aged adults: smooth muscle differentiation determined by immunohistochemistry and electron microscopy. Hum Pathol. 2003;34(3):246–252.
- Bisogno G, Pilz T, Perilongo G, et al. Undifferentiated sarcoma of the liver in childhood: a curable disease. Cancer. 2002;94(1):252–257.
- Wei ZG, Tang LF, Chen ZM, Tang HF, Li MJ. Childhood undifferentiated embryonal liver sarcoma: clinical features and immunohistochemistry analysis. J Pediatr Surg. 2008;43(10):1912–1919

Authors

Daniela Tereza Gonçalves Manso, 5th year medical school at Universidade Federal de Minas Gerais.
Email: danimanso.dm[at]gmail.com

Rafael Valério Gonçalves, 5th year medical school at Universidade Federal de Minas Gerais.
Email: rafaelvg[at]gmail.com

Supervisors

Karine Corrêa Fonseca, pediatric oncologist at Hospital das Clínicas of Universidade Federal de Minas Gerais.
Email: karine.fonseca[at]yahoo.com.br

Karla Emília de Sá Rodrigues, pediatric oncologist, Adjunct Professor of the Department of Pediatrics at Medical School of Universidade Federal de Minas Gerais.
Email: kesrodrigues[at]gmail.com

Túlio Cezar de Souza Bernardino, radiologist, Professor of the Department of Anatomy and Image at Medical School of Universidade Federal de Minas Gerais.
Email: itulio[at]gmail.com

Reviewers

Bruno Campos, Eduardo Paolinelli, Guilherme Carvalho, professor José Nelson Mendes and professor Viviane Santuari Parisotto Marino.

Translated by

Leandra Prates Diniz, 4th year medical student at Universidade Federal de Minas Gerais.
E-mail: leandraprates[at]hotmail.com

Test question

(SESRJ - GENERAL SURGERY - 2013) The most prevalent malign hepatic tumor on childhood is:

a) Teratoma

25%

b) Angiosarcoma

25%

c) Choriosarcoma

25%

d) Hepatoblastoma

25%

e)

25%
   

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