Primitive neuroectodermal tumor of the kidney: a rare case

Background Primitive neuroectodermal tumor commonly occurs in bones and is equivalent to Ewing’s sarcoma. Very few cases have been reported in the literature and they had a very different presentation and very aggressive behavior. Case presentation We present a case of a young 23-year-old male who presented with complaints of pain in left lumbar region of abdomen since 8 months and hematuria off and on. CT scan of the abdomen was done which revealed a large heterogeneously enhancing mass lesion in the left lumbar region arising from the superior and mid-pole of left kidney showing multiple non-enhancing necrotic areas. A diagnosis of left renal cell carcinoma was kept. Through the transperitoneal approach, the left kidney was approached and a left radical nephrectomy was done. The histopathology report revealed the tumor cells to be positive for CD99 and focally positive for Vimentin and negative for cytokeratin thereby making a diagnosis of primary neuroectodermal tumor (PNET). Conclusions Renal PNET is a rare renal malignancy that should be kept in the differential diagnosis of a renal SOL especially when it is a presenting feature in adolescent and young adult. It has a very aggressive course and multimodal therapy has to be considered in its treatment

Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement? A Case Series and Review of the Literature

The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon histopathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing’s sarcoma (ES)/PNET of the renal pelvis. The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor.

Do Primitive Neuroectodermal Tumors of the Kidney Have a Predilection for Inferior Vena Cava Involvement?

The primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare neoplasm, the diagnosis of which mainly depends upon his-topathology, immunohistochemistry (IHC), and cytogenetics. A handful of cases reported in the literature mention about aggressive features of this neoplasm. The purpose of our study was to review our experience in not only the diagnosis and management of the patients with renal PNET but also to highlight its propensity to involve inferior vena cava (IVC) and also present a rare occurrence of Ewing’s sarcoma (ES)/PNET of the renal pelvis.The clinical, operative, and histopathology records of four patients of renal PNET treated between January 2017 and December 2019 were reviewed and data analyzed concerning the available literature. Out of the four patients treated, two had level III and IV IVC thrombus, and one had dense desmoplastic adhesions with the IVC wall. One of the cases had a rare presentation of ES/PNET of the renal pelvis. All patients were managed surgically, while only one patient received adjuvant chemotherapy and following up with remission for the last 2 years and 4 months. On IHC, cluster of differentiation-99 (CD-99) was positive in all patients, and three were positive for Friend leukemia integration-1. PNET of the kidney is primarily an immunohistopathological diagnosis. This neoplasm has an increased propensity for the local invasion of surrounding structures. A multimodality approach with surgery, chemotherapy, and radiotherapy could offer better outcomes, although the prognosis of these tumors remains poor.

Second New Primary PNET of Kidney in a Treated Case of Carcinoma Breast: A Case Report

Primitive Neuroectodermal Tumour (PNET) is a member of small round cell “Blue tumour” family. The overall incidence of PNET is <1%. PNET of kidney is uncommon and was first reported in 1975. Since the first report around 150 cases have been published in medical literature. It exhibits highly aggressive behaviour. It usually affects young adults and has a male predominance of 3:1. Only six cases of PNET of kidney have been reported in older patients and PNET of kidney as a second new primary has rarely been reported, only three cases of renal PNET with history of an earlier or synchronous primary cancer were reported in literature from the USA, Germany and China. Here, the author reported a case of renal PNET in a 48-year-old female who has been treated for right breast cancer by surgery, adjuvant chemotherapy and radiotherapy three years (July 2015) prior to diagnosis of PNET of left kidney. Upon diagnosis, patient underwent left nephrectomy. Postoperatively, patient was started on Ewing’s Family of Tumours (EFT) 2001 protocol. Patient completed induction therapy followed by radiation to tumour bed with concurrent weekly single agent vincristine and was planned for maintenance chemotherapy. After one cycle of maintenance chemotherapy, patient developed severe febrile pancytopenia and was admitted in the ICU, even after aggressive medical line of management, patient could not be revived. Although, the patient tolerated the treatment protocol for breast malignancy well and the primary was under control but after three years, with the diagnosis of a very rare second new primary PNET of left kidney which has a very aggressive nature and poor prognosis, the patient could not tolerate the multimodality treatment which was required.

Management of Primitive Neuroectodermal Tumor of the Kidney with Inferior Vena Cava Thrombus

Primitive neuroectodermal tumors (PNET) are an aggressive group of small round cell tumors usually arising in the nervous system and affecting children. They have a tendency for local invasion, distant spread and formation of tumor thrombi. The kidney is a rare primary location for these tumors. Outcomes are frequently poor due to late diagnosis (Wilms tumor is a more common tumor in this population) and early spread. Immunohistochemistry is invaluable in making the diagnosis of PNET. We report a case of a primary renal PNET with extensive tumor thrombus into the inferior vena cava, and lung metastasis in a pediatric patient, and its successful management. Our 14-year-old patient with renal PNET was managed with radical nephrectomy, thrombectomy and chemotherapy and remains disease free to date. The diagnosis of renal PNETs should be considered in young adult patients who present with aggressive renal masses at initial presentations. Despite its aggressive nature, good outcomes can be achieved by a multimodality therapeutic strategy.

Ewing Sarcoma of the Kidney: A Rare Entity

Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumors typically found in children and adolescents. These tumors belong to the family of small round cell tumors and are of neuroectodermal origin. Primary Ewing sarcoma of the kidney is rare and because of that is an infrequent differential diagnosis in urologic malignancies. Renal PNET mostly presents with nonspecific symptoms such as hematuria and abdominal pain. The imaging findings are uncharacteristic. The diagnosis is based on the histology, immunohistochemistry, and molecular pathologic findings. Once PNET has been diagnosed, multimodal treatment is indicated. Despite all treatment options, the prognosis of those with metastatic disease is poor.

Renal primitive neuroectodermal tumour in childhood: Case report and review of literature

Primitive neuroectodermal tumour (PNET) is presumed to be ofneural crest origin, mostly presenting as bone or soft tissue masses.It usually occurs in the trunk or axial skeleton; while renal PNET isconsidered an extremely rare tumour. We report a case of 11-yearoldmale who presented with right flank pain and gross hematuriaafter suffering blunt trauma. During investigations, he was found tohave a large renal mass on computed tomography. He underwenta right radical nephrectomy where the pathology report showedPNET of the kidney. The patient received chemotherapy afterwards.Despite the chemotherapy, he had a local tumour recurrence 3months after and continued to deteriorate and developed distantmetastasis. Primitive neuroectodermal tumour of the kidney is adistinct and rare entity. It is very aggressive, with a poor survivaldespite combined modality treatment.

Renal Peripheral Neuroectodermal Tumor Presenting at Age 78: Case Report

Primitive neuroectodermal tumor (PNET) of the kidney is a rare and aggressive tumor, often presenting in advanced stages and progressing rapidly. Renal PNET (rPNET) may affect a wide age spectrum, but the majority of cases are in young adults. We present a case of advanced rPNET in a 78-year-old woman. To our knowledge, this is the most advanced age of presentation of this neoplasm to date. We report on her presentation and management, and discuss the current clinical management of this tumor.