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

2015 ◽  
Vol 9 (1) ◽  
pp. 47-50 ◽  
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.

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

2020 ◽  
Vol 7 (4) ◽  
pp. 8-16
Author(s):  
Sovan Hota ◽  
Sidhartha Kalra ◽  
Dorairajan Lalgudi Narayanan ◽  
Ramanitharan Manikandan ◽  
Sreerag Kodakkattil Sreenivasan
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Vena Cava ◽  
Rare Occurrence ◽  
Rare Presentation ◽  
Friend Leukemia ◽  
Neuroectodermal Tumors ◽  
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 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.

scholarly journals Sonographic Evaluation of Inferior Vena Cava Tumor Thrombus in Renal Cell Carcinoma: A Case Study of a Rare Condition

2018 ◽  
Vol 34 (5) ◽  
pp. 375-382
Author(s):  
Viyana Hamblen
Keyword(s):  
Renal Cell Carcinoma ◽  
Inferior Vena Cava ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Tumor Thrombus ◽  
Inferior Vena ◽  
Wilms Tumor ◽  
Circulatory Arrest ◽  
Vena Cava ◽  
Ivc Tumor

Inferior vena cava (IVC) tumor thrombus in renal cell carcinoma is a rare entity that suggests heightened biologic behavior and a surgical challenge during the course of treatment. Tumor thrombus can extend from the renal vein to the right atrium. This cephalad extension is classified by four different levels. These levels determine which surgical approach is used, whether a thoracoabdominal incision is needed, and whether a patient needs to be placed in circulatory arrest. Complete surgical resection of the tumor is potentially the only curative treatment, although it supposes a challenge because of operative difficulty and the potential for massive bleeding or tumor pulmonary thromboembolism. IVC tumor thrombus presents with a few differentials that need to be assessed, including bland thrombus, primary IVC leiomyosarcoma, hepatocellular carcinoma, adrenal cortical carcinoma, primary lung carcinoma, and Wilms tumor. The importance of diagnosing IVC tumor thrombus secondary to renal cell carcinoma is demonstrated as well as a sonographic protocol for assessing IVC tumor thrombus.

Case profile: Wilms tumor completely obstructing inferior vena cava without invasion

Urology ◽  
1980 ◽  
Vol 16 (2) ◽  
pp. 224 ◽  
Author(s):  
Stanford M. Goldman ◽  
Eva S. Zinreich ◽  
Joel M. Cherry
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Wilms Tumor ◽  
Vena Cava

Inferior vena cava involvement in children with Wilms tumor

2017 ◽  
Vol 33 (5) ◽  
pp. 569-573 ◽  
Author(s):  
Abeer Al Diab ◽  
Nader Hirmas ◽  
Abdellatif Almousa ◽  
Ramiz Abu-hijlih ◽  
Fatinah Aljlouni ◽  
...  
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Wilms Tumor ◽  
Vena Cava

scholarly journals A case of Wilms tumor with right ventricular extension of tumor thrombus

2017 ◽  
Vol 4 (1) ◽  
pp. 60
Author(s):  
Mustafa Çakan ◽  
Ayşe Gülnur Tokuç ◽  
Kıvılcım Karadeniz Cerit ◽  
Koray Ak ◽  
Rabia Ergelen
Keyword(s):  
Inferior Vena Cava ◽  
Right Ventricle ◽  
Right Atrium ◽  
Tumor Thrombus ◽  
Inferior Vena ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Vena Cava ◽  
Renal Tumors ◽  
Tumor Extension

Primary renal tumors comprise 6% of all childhood cancers. Wilms tumor is the most common primary renal tumor in pediatric age group and the peak age of diagnosis is 3-4 years. In 10% of cases tumor extension into hepatic vein and inferior vena cava can be seen. But tumor extension into whole inferior vena cava, right atrium and right ventricle is only seen in less than 1% of patients. A 2-year-old girl was admitted to the hospital because of abdominal distension that was noticed by the parents two weeks ago. Imaging studies revealed that she had a mass at the right renal lodge which was favoring to Wilms tumor and on thorax tomography tumor thrombus was seen in the whole inferior vena cava, right atrium and right ventricle. Neoadjuvant chemotherapy was given for 7 weeks. On the 8th week of diagnosis, under cardiopulmonary bypass, surgical operation by pediatric and cardiovascular surgery teams for primary renal tumor and for cavo-atrial tumor thrombus was performed. Pathological examination of the mass was reported as stage 3 diffuse anaplastic Wilms tumor. The patient completed 24 weeks of chemotherapy protocol and she is being followed for 15 months without any morbidity. We present our case to emphasize the importance of multidisciplinary approach in Wilms tumor with cardiac extension.

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