Primitive Neuroectodermal Tumor Of Kidney Research Articles

Primitive neuroectodermal tumor of kidney in elderly -A case series with review of literature

Primitive neuroectodermal tumor of the kidney (PNET) is a very rare and highly aggressive tumor commonly seen in young adolescents. These tumors are rare in patients over 40 years. Reports of renal PNET in elderly patients over 60 years are even rarer.We report three cases of renal PNET in adult patients diagnosed over a period of three years. Among the three patients, two were above 60 years of age and the third patient was a 39-year-old male. All the three patients had extensive venous thrombosis at the time of presentation. One of the patients succumbed to death in the immediate post-operative period and the other two received chemotherapy.Renal PNET owing to its rare occurrence in middle-aged and elderly poses a diagnostic challenge to the pathologist. The clinical and radiological findings have limitations in the accurate diagnosis. Thorough morphological evaluation along with immunohistochemistry and cytogenetic analysis help in arriving at a correct diagnosis. An accurate diagnosis is crucial for the initiation of proper treatment.

PO-1437 Clinical outcomes in adult primitive neuro-ectodermal tumor of kidney: A single institute experience

PO-1437 Clinical outcomes in adult primitive neuro-ectodermal tumor of kidney: A single institute experience

Primitive neuroectodermal tumor of kidney and its rare affection for inferior vena cava

Primitive neuroectodermal tumors (PNETs) are a group of small round cell malignancies of neural crest origin. Renal PNET (rPNET) is a rare entity affecting young adults and children with a poor prognosis, and involvement of inferior vena cava (IVC) is extremely uncommon. Nonspecific clinical features often preclude preoperative diagnosis which ultimately depends on microscopy and immunohistochemistry. The preferred treatment for rPENT is surgical resection associated with chemotherapy and radiotherapy. We report a case of rPNET in a 16-year-old male which was preoperatively thought to be of renal cell carcinoma and underwent radical nephrectomy with IVC venotomy. The case on histopathology study revealed features of malignant small blue round cell neoplasm and further immunohistochemical examination diagnosed the case as rPNET.

PRIMITIVE NEUROECTODERMAL TUMOR WITH INFERIOR VENA CAVA THROMBUS: A RARE CASE REPORT

Primitive neuroectodermal tumors (PNETs) are rare type of small cell, aggressive tumors, commonly seen in the central nervous system, and PNET of the kidney with inferior vena cava thrombus is an extremely uncommon presentation of this entity. There are no established distinctive signs or radiological characteristics for diagnosis, but a majority of cases report positivity for CD99 marker. The patients usually present in the advanced stages of the disease and require progressive techniques of immunohistochemical analysis and expertise of multidisciplinary team of physicians to arrive at a correct diagnosis and have a proper management. Although rare, these tumors should be considered in the differential diagnosis of all patients with large renal masses, especially in the younger population. We present a case of a 23-years-old female, with PNET of the left kidney, treated with radical nephrectomy and adjuvant chemotherapy. Key words: Inferior vena cava thrombus, Kidney tumor, Primitive neuroectodermal tumor Key words: Inferior vena cava thrombus, Kidney tumor, Primitive neuroectodermal tumor

Unilateral Renal Primitive Neuroectodermal Tumor (PNET)

Introduction: Primitive neuroectodermal tumor (PNET) of kidney is a rare clinical condition. We report a young girl with PNET of kidney. Case Presentation: A 14-year-old female presented with chronic flank pain. Imaging studies revealed a huge renal mass with left renal vein thrombosis. Histopathological findings were consistent with PNET, which was later confirmed by immunohistochemistry and she received adjuvant chemotherapy. Conclusions: PNET is a rare clinical condition in urology and it should be considered in young patients with a renal mass, especially when it is huge. Treatment plan includes radical nephrectomy along with neoadjuvant chemotherapy.

517P Primitive neuroectodermal tumor of kidney: analysis of 20 cases

517P Primitive neuroectodermal tumor of kidney: analysis of 20 cases

517P Primitive neuroectodermal tumor of kidney: analysis of 20 cases

517P Primitive neuroectodermal tumor of kidney: analysis of 20 cases

A Rare Case of Primitive Neuroectodermal Tumour (Pnet) of Kidney with Review of Literature

We report a case of primitive neuroectodermal tumour of the kidney in a middle aged male who presented as a large left sided renal mass similar to renal cell carcinoma. A cytoreductive nephrectome was done which showed primitive neuroectodermal tumour in the left kidney. A Positron Emission Tomography CT scan was done which showed metastasis in the lung and para-aortic lymph nodes. We describe the natural history, clinical and pathological features and management strategy of this rare variety of tumour.

Primitive neuroectodermal tumors of kidney: Our experience in a tertiary care center.

Primitive neuroectodermal tumors (PNET) are rare highly aggressive neoplasms. The diagnosis is made by histopathology with the support of immunohistochemistry (IHC) and cytogenetics. The aggressive multimodality treatment is recommended for the management of these tumors. The purpose of our study is to review our experiences in the diagnoses and treatment of PNET of the kidneys. We retrospectively reviewed the data of all the patients, who were treated for the PNET of the kidneys at our institute between April and March 2011 and compared with the available literature. A total of eight patients were treated for PNET of the kidney. Out of the eight patients, four were males and four females. Nearly 50% of our patients had inferior vena caval thrombus at the time of presentation. The diagnosis was made on histopathology supported by IHC. Out of the eight patients, one patient had intraoperative death due to massive pulmonary thromboembolism and another died on the 7th post-operative day due to disseminated intravascular coagulation and multiorgan failure. Rest six patients were treated with post-operative chemotherapy or a combination of chemotherapy and radiotherapy. For these six patients, overall median survival was 45 months with a 3 year disease-free survival of 66% and 5 year survival of 44%. PNET of the kidneys are rare peripheral neuroectodermal tumors with an aggressive clinical course. These tumors carry a very poor prognosis. An aggressive treatment approach using a combination of surgery, chemotherapy and radiotherapy is recommended for a reasonable survival in these tumors.

Primitive neuroectodermal tumor of kidney with tumor thrombus extending up to right atrium

Primitive neuroectodermal tumor of kidney with tumor thrombus extending up to right atrium