alexa Primary Ewing Sarcoma of the Kidney with Inferior Vena Cava Invasion | OMICS International
ISSN: 2168-9857
Medical & Surgical Urology

Like us on:

Make the best use of Scientific Research and information from our 700+ peer reviewed, Open Access Journals that operates with the help of 50,000+ Editorial Board Members and esteemed reviewers and 1000+ Scientific associations in Medical, Clinical, Pharmaceutical, Engineering, Technology and Management Fields.
Meet Inspiring Speakers and Experts at our 3000+ Global Conferenceseries Events with over 600+ Conferences, 1200+ Symposiums and 1200+ Workshops on
Medical, Pharma, Engineering, Science, Technology and Business

Primary Ewing Sarcoma of the Kidney with Inferior Vena Cava Invasion

Jong Kil Nam*

Department of Urology, Pusan National University, School of Medicine, Yangsan Hospital, Beomo-ri, Mulgeum-eup, Yangsan, Gyeongnam 626-770, Korea

*Corresponding Author:
Jong Kil Nam
Department of Urology
Pusan National University
School of Medicine
Yangsan Hospital
Beomo-ri, Mulgeumeup
Yangsan, Gyeongnam 626-770, Korea
Tel: 82-55-360-2134
E-mail:[email protected]

Received date: March 24, 2014; Accepted date: December 30, 2014; Published date: January 03, 2015

Citation: Nam JK (2015) Primary Ewing Sarcoma of the Kidney with Inferior Vena Cava Invasion. Med Surg Urol 4:146. doi: 10.4172/2168-9857.1000146

Copyright: © 2015 Nam JK. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Visit for more related articles at Medical & Surgical Urology

Abstract

Ewing sarcoma is typically a skeletal based tumor of presumed neuroectodermal origin. Primary Ewing sarcoma of the kidney is exceedingly rare and is usually found in young adults. We report a case of primary Ewing sarcoma with inferior vena cava (IVC) invasion that was managed successfully with radical nephrectomy and partial replacement of IVC using bovine pericardial patch.

Keywords

Ewing sarcoma; Kidney; Inferior Vena Cava

Introduction

Ewing sarcoma is high-grade malignant tumors typically manifest in children and young adults and most common in bone [1,2]. However, extraskeletal Ewing sarcoma such as skin, soft tissue, viscera is less common in and can affect the skin, soft tissue or viscera. Few case of Ewing sarcoma arising from kidney has been previously described in the literature and is potential clinical course toward metastatic disease and death [3,4]. Most patients are young adults with a median age of 28 years (range 4 to 69) and a slight male predominance (1.5:1) [2]. We believe that surgery is the mainstay of treatment [4]. In patient with tumor thrombus or IVC invasion, aggressive surgery to remove the thrombus and invasion in the IVC is reported to be effective in prolonging survival in patients with renal cell carcinoma [5]. However, its value in treating Ewing sarcoma of the kidney is unclear because of limited data and the aggressive nature of the disease. In case of IVC invasion a complete resection is necessary and then IVC resection is main technical challenge.

We report a case of radical nephrectomy and partial replacement of IVC using bovine pericardial patch for primary Ewing sarcoma of the kidney with IVC invasion.

Case Report

A 30-year-old man complained of a one-month history of intermittent palpitation and left flank dull pain. He denied having another symptoms including gross hematuria, fever or general symptoms and looked healthy. He had no remarkable past medical history or familial history. The physical examination showed mild costovertebral angle tenderness. Urine analysis, urine culture, and routine blood chemistry results were within normal limits. Abdominal computed tomography showed that most of the left kidney had been occupied by a huge irregular tumor about 30×20 cm size (Figure 1). The tumor had heterogenous enhancemant and some areas of necrosis. The left peri-renal space had strand-like infiltrations and the renal fascia had thickened. The tumor had invaded into the left renal vein and the IVC. Under the impression of renal cell carcinoma, we underwent left radical nephrectomy with IVC partial replacement of IVC using bovine pericardial patch.

medical-surgical-urology-Abdominal-computed

Figure 1: Abdominal computed tomography showing that most of the left kidney had been occupied by a huge irregular tumor.

The tumor was mobilized en bloc with the left kidney, adrenal gland, ureter, and regional lymph node. The IVC was clamped proximally, contralaterally and distally, and resected for infiltrated tissue of 2 cm in length. The tumor was then removed en bloc with the infiltrated IVC, the left kidney, adrenal gland, ureter. The vascular defect was therefore repaired with the bovine pericardial patch (Figure 2).

medical-surgical-urology-pericardial-patch

Figure 2: Intraoperative findings after caval reconstruction with a bovine pericardial patch.

Grossly, a huge brown-white color tumor measuring 29×18 cm in dimension was seen, involving the whole kidney. The tumor had invaded the perinephric fat and the renal vein and IVC. Microscopically, the tumor cells were composed predominantly of monomorphic, small round, hyperchromatic nuclei with scanty eosinophilic cystoplasm, and show rossetoid arrangement (Figure 3). All nodes were negative. In immunohistochemical analysis the tumor showed diffuse strong staining of CD99, a focal staining of synaptophysin and Ki-67 (Figure 4). The tumor cells were negative for the vimentin, CD56, desmin, myogenin, panCK and LCA markers. The final pathological diagnosis was a primary Ewing’s sarcoma of the kidney with IVC invasion.

medical-surgical-urology-resected-inferior

Figure 3: (A) The resected inferior vena cava showed invasive sarcoma (H&E, x40). (B) The tumor cells have round and hyperchromatic nuclei and show rossetoid arrangement (H&E, x400).

medical-surgical-urology-tumor-cells

Figure 4: The tumor cells showing strong membranous immunoreactivity for CD99 (x200).

The patient was discharged on the 8th day after surgery. 1 month later, the patient initiated chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with isosfamide and etoposide. The patient has completed 12 cycles of chemotherapy and has experienced no adverse event. There is no sign of recurrence in this patient two year after the operation.

Discussion

Ewing’s sarcoma is the second most common skeletal tumor in children and young adults [1]. However, primary Ewing’s sarcoma of the kidney is rare, with few cases reported in the literature and no available treatment strategies [2-4]. Also, much less frequently reported is venous involvement by Ewing’s sarcoma of the kidney, and it is exceedingly rare to invade the wall of the IVC [4].

The imaging appearance of Ewing’s sarcoma of the kidney is nonspecific, and these tumors should be in the differential diagnosis when a large retroperitoneal mass with aggressive features is encountered [6]. The major differential consideration for a perinephric soft tissue mass includes Wilms tumor, neuroblastoma, lymphoma, rhabdomyosarcoma, renal cell carcinoma, transitional cell carcinoma and metastatic tumors [6].

In some literature, long-term disease free survival can be achieved with chemotherapy alone [7]. The role of cytoreductive nephrectomy is not defined in this patient population [7]. However, we think that surgical resection followed by chemotherapy and local radiation therapy offers the best chance for long-term survival, similary to renal cell carcinoma [5]. Parham et al. reported 79 cases of primary Ewing’s sarcoma of the kidney. However, it is uncertain whether all cases were indeed primary Ewing’s sarcoma of the kidney, and only small cases provided follow-up results [8]. Thyavihally et al. reported a single institution experience of 16 patients with primary Ewing’s sarcoma of the kidney, which were treated with radical nephrectomy in operable cases and chemotherapy in all patients [9]. Overall median survival was 40 months with a 3-year survival of 60% and 5-year survival of 42%. A 5-year disease free survival rate of 36% was observed in patients with no lymph mode or distant metastasis [9].

Ewing’s sarcomas are sensitive to chemotherapy [7,10]. The use of chemotherapy has greatly improved survival for patients with localized tumor, from 10% to 70-80% [10]. However, it has less effective for patients with metastases [10]. A combination of neoadjuvant chemotherapy with local control, followed by adjuvant chemotherapy, is implemented. Local control is achieved with surgical resection and/or radiation therapy. In our cases, under the impression of renal cell carcinoma, we underwent left radical nephrectomy with IVC reconstruction. In this case, that tumor invaded the caval wall, medial venotomy carries a higher risk of narrowing after caval wall excision and renal vein repair, and then we underwent caval wall reconstruction using a bovine pericardial patch.

Despite attempts at multimodal therapies, prognosis of metastatic cases remains poor. However, we think that aggressive surgical treatment followed by chemotherapy and local radiation therapy offers the best chance for long-term survival. Long-term follow up will be necessary to verify prognosis of aggressive therapies. In this report the feasibility and safety of the aggressive surgery is documented.

References

Select your language of interest to view the total content in your interested language
Post your comment

Share This Article

Relevant Topics

Recommended Conferences

  • World Summit on Trauma and Reconstructive Surgery Sep 10-11, 2018 Singapore Theme: Expanding new horizons in Trauma and Surgery
    Sep 10-11, 2018 Singapore City, Singapore
  • International Conference on Reproduction and Fertility October 18-19, 2018 Abu Dhabi, UAE
    October 18-19, 2018 Abu Dhabi, UAE
  • 11th International Conference on Clinical and Medical Case Reports October 22-23, 2018 Turkey
    October 22-23, 2018 Istanbul, Turkey

Article Usage

  • Total views: 12423
  • [From(publication date):
    March-2015 - Jul 20, 2018]
  • Breakdown by view type
  • HTML page views : 8603
  • PDF downloads : 3820
 

Post your comment

captcha   Reload  Can't read the image? click here to refresh

Peer Reviewed Journals
 
Make the best use of Scientific Research and information from our 700 + peer reviewed, Open Access Journals
International Conferences 2018-19
 
Meet Inspiring Speakers and Experts at our 3000+ Global Annual Meetings

Contact Us

Agri & Aquaculture Journals

Dr. Krish

[email protected]

+1-702-714-7001Extn: 9040

Biochemistry Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Business & Management Journals

Ronald

[email protected]

1-702-714-7001Extn: 9042

Chemistry Journals

Gabriel Shaw

[email protected]

1-702-714-7001Extn: 9040

Clinical Journals

Datta A

[email protected]

1-702-714-7001Extn: 9037

Engineering Journals

James Franklin

[email protected]

1-702-714-7001Extn: 9042

Food & Nutrition Journals

Katie Wilson

[email protected]

1-702-714-7001Extn: 9042

General Science

Andrea Jason

[email protected]

1-702-714-7001Extn: 9043

Genetics & Molecular Biology Journals

Anna Melissa

[email protected]

1-702-714-7001Extn: 9006

Immunology & Microbiology Journals

David Gorantl

[email protected]

1-702-714-7001Extn: 9014

Materials Science Journals

Rachle Green

[email protected]

1-702-714-7001Extn: 9039

Nursing & Health Care Journals

Stephanie Skinner

[email protected]

1-702-714-7001Extn: 9039

Medical Journals

Nimmi Anna

[email protected]

1-702-714-7001Extn: 9038

Neuroscience & Psychology Journals

Nathan T

[email protected]

1-702-714-7001Extn: 9041

Pharmaceutical Sciences Journals

Ann Jose

[email protected]

1-702-714-7001Extn: 9007

Social & Political Science Journals

Steve Harry

[email protected]

1-702-714-7001Extn: 9042

 
© 2008- 2018 OMICS International - Open Access Publisher. Best viewed in Mozilla Firefox | Google Chrome | Above IE 7.0 version
Leave Your Message 24x7