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Primitive neuroectodermal tumour (PNET) of the kidney: a rare renal tumour in adolescents with seemingly characteristic radiological features

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Abstract

Background

Primitive neuroectodermal tumours (PNETs) constitute a family of neoplasms of presumed neuroectodermal origin that predominantly present as bone or soft-tissue masses in adolescents and young adults. PNET arising in the kidney is rare.

Objective

To describe the radiological features in three patients with primary renal PNET.

Materials and methods

The radiological features of primary renal PNET in three adolescent patients (age 10, 14 and 16 years) are described.

Results

Tumour thrombus extending into the renal vein and inferior vena cava was noted in all three patients. In addition, further tumour extension into the atrium was seen in two patients with extension into a pulmonary artery in one patient. Neural foraminal and intraspinal extension close to the origin of the tumour was identified in two patients. Liver, bone and lung metastases were identified.

Conclusion

While rare, one should consider the diagnosis of PNET when encountering a renal mass with aggressive features such as inferior vena cava tumour thrombus, direct intraspinal invasion and distant metastasis.

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Acknowledgements

The work was supported in part by a GE-AUR Research Award, a Society of Thoracic Radiology Research Grant, and a Society of Pediatric Radiology Research Fellow Grant (E.Y.L).

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Authors and Affiliations

  1. Department of Diagnostic Radiology and Organ Imaging, Faculty of Medicine, Prince of Wales Hospital, The Chinese University of Hong Kong, Ngan Shing Street, Shatin, New Territories, Hong Kong

    Winnie C. Chu

  2. Department of Radiology, Lahey Clinic Medical Center, Burlington, MA, USA

    Boris Reznikov

  3. Department of Radiology, Children’s Hospital, Boston and Harvard Medical School, Boston, MA, USA

    Edward Y. Lee

  4. Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Ronald M. Grant

  5. Department of Paediatrics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, New Territories, Hong Kong

    Frankie W. T. Cheng

  6. Department of Diagnostic Imaging, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada

    Paul Babyn

Authors
  1. Winnie C. Chu
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  2. Boris Reznikov
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  3. Edward Y. Lee
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  4. Ronald M. Grant
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  5. Frankie W. T. Cheng
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  6. Paul Babyn
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Corresponding author

Correspondence to Winnie C. Chu.

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Chu, W.C., Reznikov, B., Lee, E.Y. et al. Primitive neuroectodermal tumour (PNET) of the kidney: a rare renal tumour in adolescents with seemingly characteristic radiological features. Pediatr Radiol 38, 1089–1094 (2008). https://doi.org/10.1007/s00247-008-0971-1

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  • DOI: https://doi.org/10.1007/s00247-008-0971-1

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