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Primitive neuroectodermal tumour of the kidney with vena caval and atrial tumour thrombus: a case report.

Ong PH, Manikandan R, Philip J, Hope K, Williamson M - J Med Case Rep (2008)

Bottom Line: A small paracaval lymph node was noted and three small metastatic nodules were identified within the lung parenchyma.Immunohistochemical staining of the specimen showed a highly specific cluster of differentiation (CD) 99, thus confirming the diagnosis of a primitive neuroectodermal tumour.It is important that a renal primitive neuroectodermal tumour be considered, particularly in young patients with a renal mass and extensive thrombus.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Urology, University Hospital Aintree, Liverpool, UK. catphong@doctors.org.uk

ABSTRACT

Introduction: Renal primitive neuroectodermal tumour is an extremely rare malignancy.

Case presentation: A 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.

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

No MeSH data available.


Related in: MedlinePlus

Computed tomography scan of the chest showing the tumour thrombus (TH) in the right atrium.
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Figure 2: Computed tomography scan of the chest showing the tumour thrombus (TH) in the right atrium.

Mentions: Ultrasound scan (USS) revealed a large 11 cm mass arising from the lower aspect of the right kidney, which extended along the right renal vein and into the inferior vena cava (IVC) and up to the diaphragm. Further radiological studies included magnetic resonance imaging (MRI) and computed tomography (CT) of the thorax and the abdomen. MRI of the kidney revealed a large 13 cm, right encapsulated, lower-aspect renal mass with tumour thrombus extending into the right renal vein, IVC and into the right atrium and occupying a significant proportion of the right atrial volume. The right atrium appeared largely distended with thrombus with a faint trickle of contrast just getting past its wall (Figures 1 and 2). There was a 12 mm paracaval lymph node and increased vascularity in the adjacent perinephric bed. Three nodules of less than 5 mm were identified within the lung parenchyma.


Primitive neuroectodermal tumour of the kidney with vena caval and atrial tumour thrombus: a case report.

Ong PH, Manikandan R, Philip J, Hope K, Williamson M - J Med Case Rep (2008)

Computed tomography scan of the chest showing the tumour thrombus (TH) in the right atrium.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2527024&req=5

Figure 2: Computed tomography scan of the chest showing the tumour thrombus (TH) in the right atrium.
Mentions: Ultrasound scan (USS) revealed a large 11 cm mass arising from the lower aspect of the right kidney, which extended along the right renal vein and into the inferior vena cava (IVC) and up to the diaphragm. Further radiological studies included magnetic resonance imaging (MRI) and computed tomography (CT) of the thorax and the abdomen. MRI of the kidney revealed a large 13 cm, right encapsulated, lower-aspect renal mass with tumour thrombus extending into the right renal vein, IVC and into the right atrium and occupying a significant proportion of the right atrial volume. The right atrium appeared largely distended with thrombus with a faint trickle of contrast just getting past its wall (Figures 1 and 2). There was a 12 mm paracaval lymph node and increased vascularity in the adjacent perinephric bed. Three nodules of less than 5 mm were identified within the lung parenchyma.

Bottom Line: A small paracaval lymph node was noted and three small metastatic nodules were identified within the lung parenchyma.Immunohistochemical staining of the specimen showed a highly specific cluster of differentiation (CD) 99, thus confirming the diagnosis of a primitive neuroectodermal tumour.It is important that a renal primitive neuroectodermal tumour be considered, particularly in young patients with a renal mass and extensive thrombus.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Urology, University Hospital Aintree, Liverpool, UK. catphong@doctors.org.uk

ABSTRACT

Introduction: Renal primitive neuroectodermal tumour is an extremely rare malignancy.

Case presentation: A 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.

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

No MeSH data available.


Related in: MedlinePlus