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.

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

Journal of Kidney Cancer and VHL ◽

10.15586/jkcvhl.2020.153 ◽

2020 ◽

Vol 7 (4) ◽

pp. 8-16

Author(s):

Sovan Hota ◽

Sidhartha Kalra ◽

Lalgudi Narayanan Dorairajan ◽

Ramanitharan Manikandan ◽

Sreerag Kodakkattil Sreenivasan

Keyword(s):

Inferior Vena Cava ◽

Renal Pelvis ◽

Inferior Vena ◽

Vena Cava ◽

Case Series ◽

Rare Presentation ◽

Friend Leukemia ◽

Renal Pnet ◽

Multimodality Approach ◽

Cluster Of Differentiation

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.

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

Current Urology ◽

10.1159/000442851 ◽

2015 ◽

Vol 9 (1) ◽

pp. 47-50 ◽

Cited By ~ 2

Author(s):

Sahil Gupta ◽

Kaustav Majumder ◽

Anurag Chahal ◽

Ashish K. Saini ◽

Arjun Gupta

Keyword(s):

Inferior Vena Cava ◽

Inferior Vena ◽

Wilms Tumor ◽

Vena Cava ◽

Renal Masses ◽

Neuroectodermal Tumors ◽

Primary Location ◽

Renal Pnet ◽

Distant Spread ◽

Round Cell Tumors

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.

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Emphysematous pyelonephritis with air bubble in the inferior vena cava

African Journal of Urology ◽

10.1186/s12301-020-00081-2 ◽

2020 ◽

Vol 26 (1) ◽

Author(s):

Tiffany A. Perkins ◽

Alberic Rogman ◽

Murali K. Ankem

Keyword(s):

Septic Shock ◽

Inferior Vena Cava ◽

Inferior Vena ◽

Vena Cava ◽

Rare Presentation ◽

Emphysematous Pyelonephritis ◽

Case Presentation ◽

Air Bubble ◽

First Case ◽

The Right

Abstract Background Emphysematous pyelonephritis (EPN) with gas in the inferior vena cava (IVC) is a rare presentation and to our knowledge, this is the first case report in the urologic literature. Case presentation A 35-Year-old obese diabetic Hispanic female presented to the emergency room with a clinical picture of septic shock. Prompt computerized tomography scan revealed EPN with gas throughout the right renal parenchyma and extending to the right renal vein, IVC, and pulmonary artery. She died before surgical intervention Conclusion This case demonstrates that patients presenting with severe EPN have a high mortality risk and providers should acknowledge that septic shock, endogenous air emboli, or a combination of both could result in cardiovascular collapse and sudden death.

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