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Management of Primitive Neuroectodermal Tumor of the Kidney with Inferior Vena Cava Thrombus

Management of Primitive Neuroectodermal Tumor of the Kidney with Inferior Vena Cava Thrombus Primitive neuroectodermal tumors (PNET) are an aggressive group of small round cell tumors usually arising in the nervous system and affecting children. They have a tendency for local invasion, distant spread and formation of tumor thrombi. The kidney is a rare primary location for these tumors. Outcomes are frequently poor due to late diagnosis (Wilms tumor is a more common tumor in this population) and early spread. Immunohistochemistry is invaluable in making the diagnosis of PNET. We report a case of a primary renal PNET with extensive tumor thrombus into the inferior vena cava, and lung metastasis in a pediatric patient, and its successful management. Our 14-year-old patient with renal PNET was managed with radical nephrectomy, thrombectomy and chemotherapy and remains disease free to date. The diagnosis of renal PNETs should be considered in young adult patients who present with aggressive renal masses at initial presentations. Despite its aggressive nature, good outcomes can be achieved by a multimodality therapeutic strategy. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Urology Karger

Management of Primitive Neuroectodermal Tumor of the Kidney with Inferior Vena Cava Thrombus

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Publisher
Karger
Copyright
© 2016 S. Karger AG, Basel
ISSN
1661-7649
eISSN
1661-7657
DOI
10.1159/000442851
Publisher site
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Abstract

Primitive neuroectodermal tumors (PNET) are an aggressive group of small round cell tumors usually arising in the nervous system and affecting children. They have a tendency for local invasion, distant spread and formation of tumor thrombi. The kidney is a rare primary location for these tumors. Outcomes are frequently poor due to late diagnosis (Wilms tumor is a more common tumor in this population) and early spread. Immunohistochemistry is invaluable in making the diagnosis of PNET. We report a case of a primary renal PNET with extensive tumor thrombus into the inferior vena cava, and lung metastasis in a pediatric patient, and its successful management. Our 14-year-old patient with renal PNET was managed with radical nephrectomy, thrombectomy and chemotherapy and remains disease free to date. The diagnosis of renal PNETs should be considered in young adult patients who present with aggressive renal masses at initial presentations. Despite its aggressive nature, good outcomes can be achieved by a multimodality therapeutic strategy.

Journal

Current Urology – Karger

Published: Jan 1, 2016

Keywords: Primitive neuroectodermal tumors; Kidney cancer; Immunohistochemistry; Thrombosis

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