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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

Histology of the tumour. Cohesive sheets of small, uniform, primitive, blastema-like malignant cells are separated by fibrous bands. Perivascular rosetting was seen but there was no architectural arrangement (haematoxylin and eosin, magnification ×20).
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Figure 3: Histology of the tumour. Cohesive sheets of small, uniform, primitive, blastema-like malignant cells are separated by fibrous bands. Perivascular rosetting was seen but there was no architectural arrangement (haematoxylin and eosin, magnification ×20).

Mentions: Gross examination revealed a friable, greyish white, lobulated mass (125 mm × 90 mm), which replaced most of the kidney with only a small amount of uninvolved parenchyma at the lower pole. Haematoxylin and eosin staining showed the tumour to comprise cohesive sheets of small, uniform, primitive, blastema-like malignant cells separated by fibrous bands. Perivascular rosetting was noted, but there was no architectural arrangement. The malignant cells had only a small amount of cytoplasm, and there was brisk mitotic activity (Figure 3). The tumour also infiltrated the IVC.


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)

Histology of the tumour. Cohesive sheets of small, uniform, primitive, blastema-like malignant cells are separated by fibrous bands. Perivascular rosetting was seen but there was no architectural arrangement (haematoxylin and eosin, magnification ×20).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Histology of the tumour. Cohesive sheets of small, uniform, primitive, blastema-like malignant cells are separated by fibrous bands. Perivascular rosetting was seen but there was no architectural arrangement (haematoxylin and eosin, magnification ×20).
Mentions: Gross examination revealed a friable, greyish white, lobulated mass (125 mm × 90 mm), which replaced most of the kidney with only a small amount of uninvolved parenchyma at the lower pole. Haematoxylin and eosin staining showed the tumour to comprise cohesive sheets of small, uniform, primitive, blastema-like malignant cells separated by fibrous bands. Perivascular rosetting was noted, but there was no architectural arrangement. The malignant cells had only a small amount of cytoplasm, and there was brisk mitotic activity (Figure 3). The tumour also infiltrated the IVC.

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