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Case Reports
. 2017 May;11(5):ED09-ED11.
doi: 10.7860/JCDR/2017/24892.9781. Epub 2017 May 1.

Pure Primary Extragonadal Retroperitoneal Yolk Sac Tumour in a Young Child: A Case Report

Affiliations
Case Reports

Pure Primary Extragonadal Retroperitoneal Yolk Sac Tumour in a Young Child: A Case Report

Vineeth G Nair et al. J Clin Diagn Res. 2017 May.

Abstract

Germ Cell Tumours (GCTs) in children are uncommon, constituting approximately only 3% of all malignancies in children younger than 15 years of age. Primary extragonadal GCTs constitute only 1-5% of all GCTs and a retroperitoneal site is seen only in 4% of all extragonadal GCTs. Extragonadal GCTs arise from local transformation of primordial germ cells which have been misplaced during the migration of these cells through the midline dorsal mesentery in the fourth-sixth week of embryogenesis. GCTs in children show remarkable variability in age, site, presentation and histology. This is the case of a three-year-old male child who presented with a history of an abdominal swelling which was rapidly progressive in nature. Radiology showed a large retroperitoneal mass and lesions in the liver. Histopathology, immunohistochemistry and serum Alpha-fetoprotein (AFP) values confirmed a diagnosis of pure primary extragonadal yolk sac tumour.

Keywords: Alpha- fetoprotein; Germ cell tumours; Schiller–Duval bodies.

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Figures

[Table/Fig-1]:
[Table/Fig-1]:
CT: Axial section showing the large retroperitoneal mass (Arrow).
[Table/Fig-2]:
[Table/Fig-2]:
Gross picture showing the outer surface of the mass.
[Table/Fig-3]:
[Table/Fig-3]:
Gross picture showing the cut surface of the mass which was grey-white, with a few cystic spaces filled with brown mucoid fluid, and small areas of haemorrhage.
[Table/Fig-4]:
[Table/Fig-4]:
Microphotograph showing the microcystic/reticular arrangement of tumour cells (H&E; 4X).
[Table/Fig-5]:
[Table/Fig-5]:
Schiller-Duval body (Arrow) (H&E; 40X).
[Table/Fig-6]:
[Table/Fig-6]:
PAS positive hyaline globules seen (Arrows) (H&E; 40X).
[Table/Fig-7]:
[Table/Fig-7]:
Immunohistochemistry with cytokeratin AE1/3 showing diffuse cytoplasmic positivity (IHC; 10X).

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