Primitive Neuroectodermal Tumor of the Kidney with Inferior Vena Caval Thrombus
Keywords:
Kidney, Inferior vena cava, Primitive neuroectodermal, ThrombusAbstract
Background: Primitive neuroectodermal tumor (PNET) of the kidney is extremely rare.Objective: The objective is to report a case of neuroectodermal tumor of kidney, result of the treatment
and review of the literatures.
Case report: We reported a case of young female presented with abdominal mass which computed
tomographic scan showed a large inhomogeneous enhancing solid tumor of right kidney with retrohepatic vena
caval thrombus. Radical nephrectomy with vena caval thrombectomy was performed. Histopathology
demonstrated round tumor cells with rosette formation which immunohistochemistry stained positive for CD99
(MIC-2), the characteristic of this tumor. Despite undergoing adjuvant chemotherapy, the patient developed
local recurrences and multiple distant metastases at only 8 months after operation.
Conclusion: The diagnosis of renal PNETs should be considered in young adult patients who presented
with aggressive renal masses at initial presentations. Immunohistochemistry is of invaluable help for making
a definite diagnosis. This particular type of renal tumor is highly aggressive in nature and has poor prognosis
despite any adjuvant treatments.
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