Primitive neuroectodermal tumour of the kidney with vena caval and atrial tumour thrombus: a case reportReport as inadecuate

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Journal of Medical Case Reports

, 2:265

First Online: 11 August 2008Received: 19 December 2007Accepted: 11 August 2008DOI: 10.1186-1752-1947-2-265

Cite this article as: Ong, P.H., Manikandan, R., Philip, J. et al. J Med Case Reports 2008 2: 265. doi:10.1186-1752-1947-2-265


IntroductionRenal primitive neuroectodermal tumour is an extremely rare malignancy.

Case presentationA 21-year-old woman presented with microscopic haematuria, a palpable right loin mass, dyspnoea, dizziness and fatigue. Initial ultrasound scan of the kidneys revealed an 11 cm right renal mass with venous extension into the inferior vena cava. Computed tomography of the thorax and abdomen revealed an extension of the large renal mass into the right renal vein, inferior vena cava and up to the right atrium. A small paracaval lymph node was noted and three small metastatic nodules were identified within the lung parenchyma. The patient underwent a radical nephrectomy and inferior vena caval tumour level IV thrombectomy with cardiopulmonary bypass and deep hypothermic circulatory arrest. Immunohistochemical staining of the specimen showed a highly specific cluster of differentiation CD 99, thus confirming the diagnosis of a primitive neuroectodermal tumour.

ConclusionIt is important that a renal primitive neuroectodermal tumour be considered, particularly in young patients with a renal mass and extensive thrombus.

AbbreviationsCDCluster of differentiation

CTComputed tomography

DNADeoxyribose nucleic acid

IVCInferior vena cava

MRIMagnetic resonance imaging

PNETPrimitive neuroectodermal tumour

USSUltrasound scan.

Electronic supplementary materialThe online version of this article doi:10.1186-1752-1947-2-265 contains supplementary material, which is available to authorized users.

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Author: Poh Ho Ong - Ramaswamy Manikandan - Joe Philip - Kirsten Hope - Michael Williamson


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