Giovanni Cochetti , Alessio Paladini , Jacopo Adolfo Rossi de Vermandois , Sonia Fatigoni , Magda Zanelli , Stefano Ascani and Ettore Mearini

Metastatic renal Ewing’s sarcoma in adult woman: Case report and review of the literature

De Gruyter | Published online: March 9, 2021

Abstract

Primary renal extra-skeletal Ewing sarcoma is a rare neoplasm, often metastatic at diagnosis, and with a poor outcome. A multimodal approach is often the treatment of choice in this aggressive neoplasm. We present a case of primary renal extra-skeletal sarcoma in a 45-year-old woman who underwent tumor resection without clear margins. After no response to the first cycle of chemotherapy, we documented an early onset of local recurrence. The patient refused any other treatment and died four months after surgery.

1 Introduction

The Ewing Sarcoma (ES) family represents a group of high-grade malignancy tumors including ES of bone, Extra-skeletal Ewing sarcoma (EES), peripheral neuroectodermal tumor (PNET), and Askin tumor (a thoracopulmonary PNET). The ES family is a group of poorly differentiated tumors made up of small round blue cells and it recognizes the rearrangement of the ESWR1 gene as a pathognomonic sign [1]. The ES breakpoint region (ESWR1) maps on 22q12 and is one of the most involved genes in sarcoma, firstly identified in the ES family, but also present in other neoplasms.

ES is the second most common pediatric bone tumor, with a peak of incidence between the first and second decade and 80% of cases arise from the skeleton [2]. In adults, the most frequent primary presentation is an EES [3], which accounts for about 5% of all soft tissue sarcomas [1]. EES has no pathognomonic symptoms or signs and the clinical features depend mainly on the primary site. Potentially, EES could affect everywhere as single or multiple lesions [4]. The most common primary sites include the paravertebral spaces, lower extremities, head, neck, and pelvis. Sites rarely involved are the retroperitoneum, omentum, orbit, skin, and chest wall. The imaging features of EES are non-pathognomonic as well as clinical manifestations. It often presents as a well-defined, heterogeneous mass with areas of hemorrhage or necrosis in absence of calcification and nodal metastases [3]. EES can rarely arise in the kidney [4], resembling a renal cancer often with involvement of renal vein, inferior vena cava, or adjacent organs [3]. Most common metastatic sites in EES are lung, bone, and brain [3]. Therapeutic strategy of EES is debated. About 40–50% of the patients already present metastasis at diagnosis, so the role of the different therapies is yet to be defined. Multimodal treatment (surgery, radiotherapy, chemotherapy) determines an improvement in prognosis for single primary lesion [2], beside a poor prognosis for metastatic disease.

Herein, we report a case of an adult woman affected by an EES treated with surgery and chemotherapy. Secondary aim was to perform a review of the literature about EES and its therapeutic management.

2 Material and methods – case report

A 45-year-old female with persistent chest pain underwent chest-abdomen CT scan for suspicion of pulmonary thromboembolism. The imaging revealed a heterogeneous retroperitoneal mass, sized as 17 cm in diameter, extending from the superior left renal pole and infiltrating posterior abdominal wall and partially the diaphragm (Figure 1). The tumor was associated with neoplastic thrombosis of the renal vein and liver metastasis; moreover, bulky mass caused compression of the other abdominal organs and displaced them from the physiological position. The pulmonary thromboembolism was excluded, although mild left basal pleural effusion with dysventilation of the left lower lobe was evident and related to bulky mass effect. The patient was on follow-up for breast cancer treated 3 years before by surgery followed by four cycles of epirubicin and cyclophosphamide-based adjuvant chemotherapy, paclitaxel for 12 weeks, radiotherapy, and tamoxifen-based hormonal therapy, with a residual ejection fraction of 35% probably as a consequence of anthracycline therapy. Previously, she underwent also total thyroidectomy for multinodular goiter. The selective arteriography, performed 24 h before surgery, showed a mass blood supply from the first three lumbar arteries and splenic artery short branches that were embolized by hemostatic agents. A cava filter was also placed. A total body bone scan was performed for staging and was negative for bone metastasis (Figure 2).

Figure 1 CT-scan, the imaging revealed a heterogeneous retroperitoneal mass of 17 cm in diameter containing necrotic and hemorrhagic areas, at the level of the superior left renal pole infiltrating partially the diaphragm (arrows).

Figure 1

CT-scan, the imaging revealed a heterogeneous retroperitoneal mass of 17 cm in diameter containing necrotic and hemorrhagic areas, at the level of the superior left renal pole infiltrating partially the diaphragm (arrows).

Figure 2 Total body bone scan performed for staging revealed no bone metastasis.

Figure 2

Total body bone scan performed for staging revealed no bone metastasis.

The patient underwent a laparotomic left radical nephrectomy with adrenalectomy and partial resection of psoas muscle and diaphragm, which were macroscopically involved by tumor, in order to achieve a radical surgery. Hilar and para-aortic lymphadenectomy were also carried out. Operative time was 200 min. Estimated blood loss was 550 cc. There were not any perioperative complications. No blood transfusion was needed. The patient was dismissed on post-operatory day 13.

Macroscopic examination showed a 200 × 150 × 90 mm grey-yellow, friable mass with capsular interruption and containing necrotic and hemorrhagic areas. Surgical margins were unclear at level of psoas and diaphragm resection. Histologically, the tumor was made up of nodules separated by sclero-hyaline septa and it was composed of small to medium-sized cells with oval nuclei, small nucleoli, granular chromatin, and scanty cytoplasm. The mitotic activity was high. On immunohistochemistry, the neoplastic cell resulted to be diffusely positive for CD99, focally positive for CD56, and focally and weakly positive for synaptophysin. The neoplastic cells were negative for cytokeratin (CKAE1/AE3), epithelial membrane antigen (EMA), S-100, leucocyte common antigen (LCA), CD2, CD20, CD79 alpha, CD117, LAT, CD10, MPO, TdT, CD68PGM1, NPM1, CD38, PAX5, CD31, CD34, and desmin. The proliferative index (KI67/MIB-1) was about 70%. The genetic analysis revealed the presence of EWSR1 rearrangement. The immuno-morphological analysis highlighted a malignant aggressive neoplasm with EWSR1 rearrangement, in keeping with EES. Lymph nodes were free of tumor. Four weeks after surgery, the patient underwent ESFT 2001 chemotherapy protocol, including 5 cycles of injectable vincristine (1.6 mg), ifosfamide (2.2 g), mesna (440 mg), and etoposide (110 mg). The protocol, the most suitable in this case, comprehensive of adriamycin in addition to vincristine et ifosfamide, was not administered due to the poor heart ejection fraction.

After the first cycle of chemotherapy, the patient required emergency care for dyspnea. An X-ray exam showed left pleural effusion and a chest drainage was placed. A CT scan disclosed a huge recurrence in the surgical site and a peritoneal carcinomatosis as shown in Figures 3 and 4. Patient refused a second cycle of chemotherapy and died four months after surgery.

Figure 3 CT-scan, the imaging revealed a huge recurrence in the surgical site (arrows) with involvement of thoracic wall.

Figure 3

CT-scan, the imaging revealed a huge recurrence in the surgical site (arrows) with involvement of thoracic wall.

Figure 4 CT-scan, the imaging revealed a peritoneal carcinomatosis (arrow).

Figure 4

CT-scan, the imaging revealed a peritoneal carcinomatosis (arrow).

    Informed consent: Written informed consent was obtained from the patient for publication of relevant medical information and all of accompanying images within the manuscript.

3 Discussion

ES was first described by Stout in 1918 and later on in 1921 by James Ewing who characterized this tumor describing it in the diaphysis of long bones. ES in children and adolescents is defined as a bone tumor, which may occur at any site within the skeleton, but preferentially affects the trunk and the diaphysis of long bones [5]. However, in 15% of cases it may occur in extra-skeletal soft tissue. On the opposite, when diagnosed in adults, ES often affects soft tissues [6,7].

About 85–90% of EES cases present a somatic reciprocal t (11; 22)(q24; q12) chromosomal translocation, which fuses EWSR1 to the FLI1 ETS family gene to generate EWSR1-FLI1 fusion transcripts which induce mitotic defects leading to genomic instability and subsequent malignant transformation [3,8,9].

A first challenge for this kind of lesion is the diagnosis. Clinical symptoms are not characteristic when present.

As in other pathologic conditions, radiological findings are usually not typical and present a wide spectrum of imaging features and metastatic patterns. The imaging features of primary EES on contrast-enhanced CT or magnetic resonance imaging (MRI) are bulky heterogeneous masses with frequent local invasion or with compression of adjacent organs. Retroperitoneal masses are usually large at diagnosis with 50% of lesions bigger than 20 cm [10]. Most cases show heterogeneous enhancement with large necrotic foci. Only rare cases present relatively well-defined margins, while local invasion to adjacent organs is commonly observed. The invasion of adjacent organs and nearby muscles is commonly noted in tumors of the abdomen, pelvis, and thorax as it was in our case [1,11,12,13,14]. The definitive diagnosis is histopathologic with immunohistochemical analysis. At the histological level, EES appears as poorly differentiated, small round blue cells tumor positive for the transmembrane glycoprotein CD99, vimentin, FLI1, CKAE1/AE3, and CD 117 staining and negative TLE and WT-1 [15,16,17,18] (Figure 5). The tumor cells have a pale-to-clear scanty cytoplasm and glycogen could be highlighted on PAS staining [17].

Figure 5 Histologic findings (a–e) revealed EES appearing as a poorly differentiated, small round blue cells tumor positive for the transmembrane glycoprotein CD99, vimentin, and CD 117 staining. Hematoxylin and eosin staining (a–c) show the lesion composed by small to medium-sized cells arranged in nodules separated by sclero-hyaline septa. Synaptophysin marker (d and e).

Figure 5

Histologic findings (a–e) revealed EES appearing as a poorly differentiated, small round blue cells tumor positive for the transmembrane glycoprotein CD99, vimentin, and CD 117 staining. Hematoxylin and eosin staining (a–c) show the lesion composed by small to medium-sized cells arranged in nodules separated by sclero-hyaline septa. Synaptophysin marker (d and e).

In recent literature, more and more cases of extra-skeletal sites in adults are reported. Probably, this increase is related to the availability of molecular diagnostic techniques and not to a true increase in its incidence [19].

ES of the kidney is a rare tumor in adults and it was described for the first time in 1975 by Seemayer and colleagues. Some authors suggested that the origin of ES in the kidney could be neural cells invaginated into the kidney during their development or embryonic neural crest cells migrating into the kidney and undergoing tumorigenesis [20]. The occurrence of EES in the kidney is uncommon and represents about 1% of all renal tumors, with less than 150 cases described in literature [21,22]. Male sex is more involved with a ratio M:F of 2:1.

At diagnosis, the most common symptom is pain (in 63%), followed by hematuria in 41% and a palpable mass in 25% [22]. In our case, the patient underwent chest-abdomen CT scan for persistent chest pain and suspicion of pulmonary thromboembolism.

Regarding renal ES (Table 1), 57% of patients had metastatic disease at the time of diagnosis [23]. Almost all of the patients (89%) who underwent biopsy had a metastatic disease at time of presentation, whereas about 1/3 of the patients (35%) who did not undergo biopsy had metastatic disease at the time of diagnosis.

Table 1

Renal Ewing Sarcoma/primitive neuroectodermal tumor (PNET)

Reference Year No of cases Mean age (year) Sex Side Symptoms at diagnosis Mean size of tumor (cm) Metastasis at diagnosis Biopsy Therapy Median FU (months) Outcome
Soni and Wei [38] 2017 1 28 F Left AbP, FP 14 IVC and left renal vein No RN + adjuvant chemotherapy ND ND
Teegavarapu et al. [39] 2017 13 33 11 M; 2 F 8 left; 5 right 6 FP, 2 AbP, 1 BP, 2 PM, 4 Hmt; 1 fv; 1 wt loss; 1 testicular swelling; 12 8 lung; 7 Rp; 1 liver; 1 LNs; 1 bone; 1 brain, 1 eyes, 1 adrenal, 1 pulmonary vasculature, 11 pts with metastasis yes 9 RN + adjuvant chemotherapy; 4 chemotherapy 36.5 2 CFS; 1 OS
Abolhasani et al. [40] 2016 1 21 M Right FP, wt loss 18 × 8 × 5 No No RN ND ND
Abolhasani et al. [40] 2016 1 31 M Right Hmt 2 × 2 × 2 No Yes Neoadjuvant chemotherapy + RN ND ND
Abolhasani et al. [40] 2016 1 34 F Left Abd swelling, left lower chest pain, PM ND No No RN ND ND
Yamamoto et al. [41] 2015 1 35 M Right AbP, Hmt 15.5 × 13.5 × 10 IVC, Lung Yes RN + metastasectomy 27 OS
Chakrabarti et al. [42] 2015 1 24 M Right AbP, PM 6 × 5 No No RN + adjuvant chemotherapy 15 OS
Almeida et al. [43] 2014 1 19 M Right FP, fv, vomit ND Lung Yes chemotherapy ND ND
Liu et al. [44] 2014 1 37 M Left FP, Hmt 4 × 2.3 × 1.5 No No Open RNU + adjuvant chemotherapy 18 CFS
Hakky et al. [20] 2013 1 33 M Left nausea, vomit, FP 5.1 × 4.8 × 3.3 No No Robotic PN 12 CFS
Richey et al. [45] 2012 1 50 M Right FP, Hmt, PM 15.9 × 10.6 intrathoracic and Abd LNs, renal fat and vessel, lung, liver Yes chemotherapy ND ND
Tariq et al. [46] 2012 1 57 F ND ND ND Lung ND Multimodal 96 CFS
Alonso et al. [47] 2011 1 20 M Left Hmt 7 Lung, retroperitoneal carcinomatosis No Open RN + adjuvant chemotherapy 24 CFS
Alonso et al. [47] 2011 1 43 M Right Pneumonic process ND Lung, IVC No Open RN + thrombectomy + adjuvant chemotherapy 9 CFS
Pathak et al. [48] 2011 1 44 F Right PM 17.4 × 11.5 × 9.3 Renal vein, IVC, atrium No RN + thrombectomy ND ND
Mohsin et al. [49] 2011 1 26 M Right FP, AbP, Hmt, wt loss, PM 20 × 13 × 10 Lung, lymphadenopathy, renal vein No RN + adjuvant chemotherapy 10 days OS
Mohsin et al. [49] 2011 1 25 M Left Hmt, FP, PM 15 × 11 × 8 Renal vein, lung, liver No RN 2 OS
Mohsin et al. [49] 2011 1 30 F Right Abd swelling, PM 18 × 12 Vertebral bodies Yes Chemotherapy 1 ND
Mohsin et al. [49] 2011 1 34 M Right Hmt, PM 17 × 10 × 9 Liver, lung No RN 14 days OS
Wedde et al. [51] 2011 1 73 M Right Hydrocele ND No Yes RN + adjuvant chemotherapy 7 CFS
Badar et al. [50] 2010 1 13 F Right AbP, Hmt 6 × 9 Lung, liver Yes RN + adjuvant chemotherapy + RT 26 OS
Asiri and Al-Sayyad [52] 2010 1 11 M Right FP, Hmt, PM 10 × 9 × 11 No No RN + lymph node dissection + adjuvant chemotherapy 3 OS
Angel et al. [53] 2010 1 31 M Left FP, Hmt 7.9 No No RN 12 CFS
Ohgaki et al. [54] 2010 1 21 M Right AbP, hemorrhage ND No No RN 6 OS
Fergany et al. [55] 2009 1 31 M Right Hmt 16 Renal vein, IVC, lung No RN + lymph node dissection + IVC thrombectomy 24 CFS
Businger et al. [56] 2009 1 46 F Right AbP 5 × 12 × 16 Renal vein No Open retroperitonealectomy with RN + adjuvant chemotherapy ND ND
Ishii et al. [57] 2009 1 21 M Right ND ND No No RN 6 OS
Zhang et al. [58] 2009 1 41 M ND ND ND IVC No RN + adjuvant chemotherapy + RT 9 CFS
Ong et al. [59] 2008 1 21 F Right PM, pain, Hmt 12.5 × 9 Renal vein, IVC, atrium, lung No RN + thrombectomy 10 CFS
Koski et al. [60] 2008 1 78 F Left Syncope, respiratory failure, Hmt 9 × 9 × 8.7 Pulmonary embolus, renal vein, IVU, lymphadenopathy No RN + lymph node dissection + thrombectomy 2 weeks OS
Chu et al. [61] 2008 1 14 F Left FP ND Renal vein, IVC, psoas, spinal canal, liver Yes Chemotherapy 22 OS
Chu et al. [61] 2008 1 16 F Left BP, decrease of sensation, PM ND Renal vein, IVC, spinal canal, L1, lung Yes Neoadjuvant chemotherapy + RN + thrombectomy + RT 4.5 CFS
Chu et al. [61] 2008 1 10 F Right Hmt ND Renal vein, IVC, atrium, lung Yes Neoadjuvant chemotherapy + RN + RT 24 CFS
Kang et al. [62] 2007 1 34 M Left Hmt, pain 8.6 × 6 Bone Yes RN ND ND
Moustafellos et al. [63] 2007 1 32 M Right AbP, FP 3.5 × 4.3 × 4 No No RN + adjuvant chemotherapy 36 CFS
Parada et al. [64] 2007 1 19 M Left FP, fv 7.5 ND No RN + adjuvant chemotherapy ND ND
Ellinger et al. [23] 2006 1 39 M Left Hmt, testicular pain, varicocele 12 Yes No RN + thrombectomy + splenectomy + adjuvant chemotherapy 6 CFS
Ellinger et al. [23] 2006 1 28 M Right FP, leg pain 9 × 5 × 5 Lung, renal vein, IVC, lymph nodes RN + vena cava resection + adjuvant chemotherapy + RT 15 CFS
Saxena et al. [65] 2006 1 26 F Left Dispnea, nausea 18 × 14 × 13 Lung Yes RN + adjuvant chemotherapy 6 OS
Maeda et al. [66] 2006 1 6 F Right AbP, PM 5 × 4.5 × 4.5 No No RN + lymph node dissection + adjuvant chemotherapy 90 CFS
Erkiliç et al. [67] 2006 1 45 M Left FP, Hmt ND No No RN 12 CFS
Pomara et al. [68] 2004 1 27 F Left FP, Hmt 11 × 8 × 6 Renal vein No Surgery + adjuvant chemotherapy + RT 24 CFS
Sivaramakrishna et al. [69] 2003 1 27 M Left FP, PM 16 × 11 Renal vein No RN + adjuvant chemotherapy 9 CFS
Murphy et al. [70] 2003 1 26 M Right Bilateral FP 6 Renal vein, IVC No RN + thrombectomy + adjuvant chemotherapy ND ND
Wada et al. [71] 2003 1 23 F Right Fatigue, fv, FP, Hmt 13 × 10 Lung, renal vein IVC No RN + thrombectomy 12 CFS
Vicha et al. [72] 2002 1 9 F Right PM 15 × 14 × 11 No No RN + adjuvant chemotherapy 5 OS
Karnes et al. [73] 2000 1 28 M Right Hmt, BP 13 × 13 Renal vein + IVC No RN + adjuvant chemotherapy 12 CFS
Kuroda et al. [74] 2000 1 28 M Left AbP 7.4 × 5.7 × 7.6 No No RN ND ND
Herman et al. [75] 1997 1 17 ND Right Abd swelling, Hmt ND ND No RN ND ND
Fontaine et al. [76] 1997 1 42 M ND ND ND ND ND Surgery + RT + adjuvant chemotherapy 60 CFS
Mor et al. [77] 1994 1 61 ND ND ND ND ND No Surgery + adjuvant chemotherapy + RT 6 OS

FP, flank pain; AbP, abdominal pain; BP, back pain; PM, palpable mass; Hmt, hematuria; fv, fever; RN, radical nephrectomy; PN, partial nephrectomy; RNU, radical nephroureterectomy; RT, radiotherapy; IVC, inferior vena cava; LNs, lymph nodes; Rp, retroperitoneal; Abd, abdominal; wt, weight; CSF, cancer free survival; OS, overall survival.

In our case, the huge mass showed imaging features of malignant tumor, and for this reason, we performed a surgical treatment without biopsy. Due to bulky size and wide blood supply of the tumor, a preoperative renal angio-embolization was carried out in order to reduce the risk of intraoperative bleeding and to facilitate surgical procedure, thus decreasing operative time and perioperative morbidity. Other important benefits of preoperative embolization included the potential role of an early ligation of the renal vein before the renal artery has been fully controlled, according to the indications given by Robson. In literature, the real usefulness of preoperative renal embolization is still debated. However, a recent prospective, randomized study showed preoperative renal embolization to be a safe and well-tolerated procedure that allowed to reduce median blood loss in patients with huge kidney cancer who underwent embolization before nephrectomy compared to patients who did not undergo preoperative embolization [24].

Prognosis is poor in metastatic disease [25,26,27,28,29], whereas in cases of nonmetastatic lesions a survival benefit is reported from 18 to 51 months which is best related with negative surgical margins [30,31,32,33]. Also, age (≥40 years) and dimensions of tumor (larger diameter of at least 8 cm) are considered prognostic factors associated with poor cancer-specific survival rate [34]. Our case seemed to be one of them with poor prognosis related to its huge local extension in addition to metastasis.

Multimodal approach including surgery associated to the adjuvant chemotherapy is the standard therapeutic approach, with radiotherapy (RT) playing an optional role in localized and nonsurgical tumors. However, 32.5% of patients received only surgical treatment, while 13% received only chemotherapy. In the 87% of the patients who received surgical treatment, a nephrectomy was performed. About 5.5% of surgical patients received neoadjuvant chemotherapy, 47% adjuvant chemotherapy, and 15% adjuvant chemotherapy and radiotherapy. The mean overall survival (OS) in the group of patients treated by multimodal approach was 20.8 months, whereas that in the patients who received only surgery was 10.3 months [25,26,27,28,29,30,31,32].

The key point in the management of these tumors is to obtain a complete surgical debridement with clear negative margins, but this is affected by the disease stage at diagnosis [35,36]. In our case, the multimodal approach did not improve patient prognosis with a rapid onset of local recurrence and cancer-specific survival was 4 months. This may be due to unclear surgical margins at level of psoas and diaphragm. A reasonable therapeutic approach in case of doubt to obtain clear surgical margins could be the choice of neoadjuvant chemotherapy. An eventual response to it could be the permission to perform a surgical operation. However, early relapse, within 2 years of first diagnosis, is reported in 70% of cases. In 66% of relapsing disease, it occurs at distant sites in metastatic disease at diagnosis, while isolated local recurrence is described in 20% of cases and is more frequent in ES localized at diagnosis [37].

Table 2 shows the review of the literature concerning nonrenal extraosseous ES/PNET. In this type of tumor, 26% of patients had metastatic disease at the time of diagnosis. The ratio M:F is lower than that in renal ES and it is around 1.25:1. Thirty percent of the patients underwent surgery, 50% of whom received neoadjuvant chemotherapy, 25% adjuvant one. Radiotherapy was carried out in 56% of all patients. Differences between these two groups concern metastatic disease which is more common in the renal ES one at diagnosis. In this group, the percentage of patient treated by nephrectomy was significantly higher, while neoadjuvant chemotherapy associated with radiotherapy was used in a multimodal treatment in a low percentage of cases. On the other hand, patients with nonrenal extraosseous ES received often a multimodal treatment with neoadjuvant chemotherapy associated with radiotherapy. In this group, surgical approach is not the main treatment of choice.

Table 2

Nonrenal extraosseous ES/PNET

Reference Year No of cases Mean age (year) Sex Site Symptoms at diagnosis Mean size of tumor (cm) Metastasis at diagnosis Biopsy Therapy Median FU (months) Outcome
Singla et al. [78] 2016 1 26 M Lumbar epidural space BP ND No No Laminectomy + adjuvant chemotherapy + RT 12 CFS
Lu et al. [79] 2015 1 40 F Rp in the right hepatorenal recess FP 10 ND No Tumor resection 12 OS
Mohsin et al. [49] 2011 1 29 M Prostate Burning micturation, PM ND Bladder, lymphadenopathy, lung, pleural Yes Chemotherapy 4 ND
Mohsin et al. [49] 2011 1 20 F Right adrenal FP, anorexia, wt loss, PM ND Lung, ascite Yes Chemotherapy ND ND
Yip et al. [80] 2009 1 28 F Near the vaginal introitus ND 2 ND No RT 12 OS
García-Morena Nisa et al. [81] 2007 1 21 F Rp and retrodiaphragmatic FP, AbP, PM 21 × 17 × 12 No Yes Neoadjuvant chemotherapy + mass excision + adjuvant chemotherapy 16 CFS
Venkitaraman et al. [82] 2007 19 21 12 M; 7 F Thorax (4), upper extremity (3), Rp (2), paraspinal (3), Pelvis (3), lower extremity (4) ND 10.5 4 Local 15 chemotherapy + 3 surgery 9 RT Metastatic RT + chemotherapy 12 7 CFS; 4 OS
Ellinger et al. [23] 2006 1 72 M Bladder Hmt, oliguria ND Prostate, Abd wall, peritoneum No TURB then surgery 2 OS
Thebert et al. [83] 1993 1 22 F Rp BP, AbP, PM 18 × 15 × 22 ND No Tumor resection + RN + LNs dissection ND ND

FP, flank pain; AbP, abdominal pain; BP, back pain; PM, palpable mass; Hmt, hematuria; LNs, lymph nodes; Rp, retroperitoneal; wt, weight; RT, radiotherapy; CSF, cancer free survival; OS, overall survival.

4 Conclusion

Reporting this case, we would point out that in presence of a renal mass, especially if huge, it has to be borne in mind that EES can occur primarily in the kidney. The local invasion should be well-evaluated before the surgery because, in case of EES, only a complete surgical ablation of the tumor could improve cancer-specific survival.

In adult, advanced ES is a dramatic condition with inauspicious outcome. Often, successful rate of surgical treatment may be affected by complexity to obtain negative surgical margins due to disease extension. Due to this, surgery in advanced disease may be considered as an important step of multimodal treatment.

Acknowledgments

We are thankful to the patient for her cooperation and allowing us to use her medical records in our case report.

    Funding: This study was not supported by any external sources of funding.

    Author contributions: J. A. R. V. and A. P. independently performed online bibliographic searches in order to identify titles and abstracts of interest and G. C. select full-text to be included. E. M. was responsible for conception and design. J. A. R. V., A. P. and S. F. acquired the clinical data. M. Z., S. A., G. C., S. F. and J. A. R. V. took part in either drafting the article and revising it critically for important intellectual content. All authors gave final approval of the version to be published, agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors have read and approved the final manuscript.

    Conflict of interest: Giovanni Cochetti, and Ettore Mearini serve as Section Editors in Open Medicine, but it hasnt affected the peer-review process, authors do not state any other conflict of interest.

    Availability of data and materials: Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study. The authors presented, in the manuscript, all the necessary information about their case report.

Reference

[1] Gupta AA , Pappo A , Saunders N , Hopyan S , Ferguson P , Wunder J , et al. Clinical outcome of children and adults with localized Ewing sarcoma. Cancer [Internet]. 2010 Apr 13;116(13):3189–94. Available from: http://doi.wiley.com/10.1002/cncr.25144 Search in Google Scholar

[2] Esiashvili N , Goodman M , Marcus RB . Changes in incidence and survival of ewing sarcoma patients over the past 3 decades. J Pediatr Hematol Oncol [Internet]. 2008 Jun;30(6):425–30. Available from: https://insights.ovid.com/crossref?an=00043426-200806000-00003 Search in Google Scholar

[3] May WA , Gishizky ML , Lessnick SL , Lunsford LB , Lewis BC , Delattre O , et al. Ewing sarcoma 11; 22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLI1 for transformation. Proc Natl Acad Sci [Internet]. 1993 Jun 15;90(12):5752–6. Available from: http://www.pnas.org/cgi/doi/10.1073/pnas.90.12.5752 Search in Google Scholar

[4] Alghamdi MHA , Alawad SA , Alharbi MG , Alabdulsalam AK , Almodhen F , Alasker A . A rare case of Ewing’s sarcoma of the kidney. Urol Case Rep [Internet]. 2020 Mar;29:101094. Available from: https://linkinghub.elsevier.com/retrieve/pii/S2214442019304401 Search in Google Scholar

[5] Riggi N , Stamenkovic I . The biology of Ewing sarcoma. Cancer Lett [Internet]. 2007 Aug;254(1):1–10. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0304383506006811 Search in Google Scholar

[6] Lynch AD , Gani F , Meyer CF , Morris CD , Ahuja N , Johnston FM . Extraskeletal versus skeletal Ewing sarcoma in the adult population: controversies in care. Surg Oncol [Internet]. 2018 Sep;27(3):373–9. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0960740418301105 Search in Google Scholar

[7] Lau YS , Adamopoulos IE , Sabokbar A , Giele H , Gibbons CLMH , Athanasou NA . Cellular and humoral mechanisms of osteoclast formation in Ewing’s sarcoma. Br J Cancer [Internet]. 2007 Jun 29;96(11):1716–22. Available from: http://www.nature.com/articles/6603774 Search in Google Scholar

[8] Delattre O , Zucman J , Plougastel B , Desmaze C , Melot T , Peter M , et al. Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours. Nature [Internet]. 1992 Sep;359(6391):162–5. Available from: http://www.nature.com/articles/359162a0 Search in Google Scholar

[9] Embree LJ , Azuma M , Hickstein DD . Ewing sarcoma fusion protein EWSR1/FLI1 interacts with EWSR1 leading to mitotic defects in zebrafish embryos and human cell lines. Cancer Res [Internet]. 2009 May 15;69(10):4363–71. Available from: http://cancerres.aacrjournals.org/cgi/doi/10.1158/0008-5472.CAN-08-3229 Search in Google Scholar

[10] Gemici K , Buldu İ , Acar T , Alptekin H , Kaynar M , Tekinarslan E , et al. Management of patients with retroperitoneal tumors and a review of the literature. World J Surg Oncol [Internet]. 2015 Dec 9;13(1):143. Available from: http://www.wjso.com/content/13/1/143 Search in Google Scholar

[11] Baldini EH , Demetri GD , Fletcher CDM , Foran J , Marcus KC , Singer S . Adults with Ewing’s sarcoma/primitive neuroectodermal tumor. Ann Surg [Internet]. 1999 Jul;230(1):79. Available from: https://insights.ovid.com/crossref?an=00000658-199907000-00012 Search in Google Scholar

[12] Cotterill SJ , Ahrens S , Paulussen M , Jürgens HF , Voûte PA , Gadner H , et al. Prognostic factors in Ewing’s tumor of bone: analysis of 975 patients from the European intergroup cooperative Ewing’s sarcoma study group. J Clin Oncol [Internet]. 2000 Sep 17;18(17):3108–14. Available from: http://ascopubs.org/doi/10.1200/JCO.2000.18.17.3108 Search in Google Scholar

[13] Lee J , Hoang BH , Ziogas A , Zell JA . Analysis of prognostic factors in Ewing sarcoma using a population-based cancer registry. Cancer [Internet]. 2010 Apr 15;116(8):1964–73. Available from: http://doi.wiley.com/10.1002/cncr.24937 Search in Google Scholar

[14] Gagliotti C , Morsillo F , Moro ML , Masiero L , Procaccio F , Vespasiano F , et al. Infections in liver and lung transplant recipients: a national prospective cohort. Eur J Clin Microbiol Infect Dis [Internet]. 2018 Mar 29;37(3):399–407. Available from: http://link.springer.com/10.1007/s10096-018-3183-0 Search in Google Scholar

[15] Kovar H , Dworzak M , Strehl S , Schnell E , Ambros I , Ambros P , et al. Overexpression of the pseudoautosomal gene MIC2 in Ewing’s sarcoma and peripheral primitive neuroectodermal tumor. Oncogene. 1990;5(7):1067–70. Search in Google Scholar

[16] Bilgetekin I , Karaca M , Gönül I , Üner A , Şahinli H , Demir H , et al. Ewing′s sarcoma of kidney in a 60-year-old patient with local recurrence: A rare occurrence. J Cancer Res Ther [Internet]. 2018;14(6):1422–4. Available from: http://www.cancerjournal.net/preprintarticle.asp?id=191062 Search in Google Scholar

[17] Sadiq M , Ahmad I , Shuja J , Ahmad K . Primary Ewing sarcoma of the kidney: a case report and treatment review. CEN Case Rep [Internet]. 2017 Nov 21;6(2):132–5. Available from: http://link.springer.com/10.1007/s13730-017-0259-0 Search in Google Scholar

[18] Errico G , Gagliotti C , Monaco M , Masiero L , Gaibani P , Ambretti S , et al. Colonization and infection due to carbapenemase-producing Enterobacteriaceae in liver and lung transplant recipients and donor-derived transmission: a prospective cohort study conducted in Italy. Clin Microbiol Infect [Internet]. 2019 Feb;25(2):203–9. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1198743X18304099 Search in Google Scholar

[19] Karski EE , Matthay KK , Neuhaus JM , Goldsby RE , DuBois SG . Characteristics and outcomes of patients with Ewing sarcoma over 40 years of age at diagnosis. Cancer Epidemiol [Internet]. 2013 Feb;37(1):29–33. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1877782112001154 Search in Google Scholar

[20] Hakky TS , Gonzalvo AA , Lockhart JL , Rodriguez AR . Primary Ewing sarcoma of the kidney: a symptomatic presentation and review of the literature. Ther Adv Urol [Internet]. 2013 Jun 20;5(3):153–9, 1756287212471095. Available from: http://journals.sagepub.com/doi/10.1177/ Search in Google Scholar

[21] Bontoux C , Khaddour S , Pérot G , Vaessen C , Boostandoost H , Augustin J , et al. Case report of an ewing’s sarcoma/primitive neuroectodermal tumor of the kidney. Int Urol Nephrol [Internet]. 2018 Aug 26;50(8):1449–51. Available from: http://link.springer.com/10.1007/s11255-018-1922-x Search in Google Scholar

[22] Suzuki I , Kubota M , Murata S , Makita N , Tohi Y , Kawakita M . A case of Ewing sarcoma family tumor of the kidney treated with robotic-assisted partial nephrectomy. Urol Case Rep [Internet]. 2019 Jul;25:100900. Available from: https://linkinghub.elsevier.com/retrieve/pii/S2214442019300439 Search in Google Scholar

[23] Ellinger J , Bastian PJ , Hauser S , Biermann K , Müller SC . Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. Urology [Internet]. 2006 Aug;68(2):257–62. Available from: https://linkinghub.elsevier.com/retrieve/pii/S009042950600272X Search in Google Scholar

[24] Cochetti G , del Zingaro M , Boni A , Allegritti M , Rossi de Vermandois JA , Paladini A , et al. Renal artery embolization before radical nephrectomy for complex renal tumour: which are the true advantages? Open Med [Internet]. 2019 Nov 7;14(1):797–804. Available from: http://www.degruyter.com/view/j/med.2019.14.issue-1/med-2019-0095/med-2019-0095.xml Search in Google Scholar

[25] McCluggage WG , Sumathi VP , Nucci MR , Hirsch M , Cin PD , Wells M , et al. Ewing family of tumours involving the vulva and vagina: report of a series of four cases. J Clin Pathol [Internet]. 2007 Jun 1;60(6):674–80. Available from: http://jcp.bmj.com/cgi/doi/10.1136/jcp.2006.040931 Search in Google Scholar

[26] Halil S , Kucuk M , Arvas M , Aydin O , Calay Z . Peripheral primitive neuroectodermal tumor (PNET) of the vulva: a case report. Eur J Gynaecol Oncol. 2011;32:117–8. Search in Google Scholar

[27] Kelling K , Noack F , Altgassen C , Kujath P , Bohlmann MK , Hoellen F . Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature. Arch Gynecol Obstet [Internet]. 2012 Mar 30;285(3):785–9. Available from: http://link.springer.com/10.1007/s00404-011-2011-x Search in Google Scholar

[28] Che S-M , Cao P-L , Chen H-W , Liu Z , Meng D . Primary Ewing’s sarcoma of vulva: a case report and a review of the literature. J Obstet Gynaecol Res [Internet]. 2013 Mar;39(3):746–9. Available from: http://doi.wiley.com/10.1111/j.1447-0756.2012.02019.x Search in Google Scholar

[29] Xiao C , Zhao J , Guo P , Wang D , Zhao D , Ren T , et al. Clinical analysis of primary primitive neuroectodermal tumors in the female genital tract. Int J Gynecol Cancer [Internet]. 2014 Mar 1;24(3):404–9. Available from: http://ijgc.bmj.com/lookup/doi/10.1097/IGC.0000000000000082 Search in Google Scholar

[30] Vang R , Taubenberger JK , Mannion CM , Bijwaard K , Malpica A , Ordonez NG , et al. Primary vulvar and vaginal extraosseous ewing’s sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction. Int J Gynecol Pathol [Internet]. 2000 Apr;19(2):103–9. Available from: https://insights.ovid.com/crossref?an=00004347-200004000-00002 Search in Google Scholar

[31] Çetiner H , Kr G , Gelmann EP , Ozdemirli M . Primary vulvar Ewing sarcoma/primitive neuroectodermal tumor: a report of 2 cases and review of the literature. Int J Gynecol Cancer [Internet]. 2009 Jul 1;19(6):1131–6. Available from: http://ijgc.bmj.com/lookup/doi/10.1111/IGC.0b013e3181acae33 Search in Google Scholar

[32] Boldorini R , Riboni F , Cristina S , Allegrini S , Valentini S , Muscarà M , et al. Primary vulvar Ewing’s sarcoma/primitive neuroectodermal tumor in a post-menopausal woman: a case report. Pathol Res Pract [Internet]. 2010 Jul;206(7):476–9. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0344033809002015 Search in Google Scholar

[33] Baldassarri M , Fallerini C , Cetta F , Ghisalberti M , Bellan C , Furini S , et al. Omic approach in non-smoker female with lung squamous cell carcinoma pinpoints to germline susceptibility and personalized medicine. Cancer Res Treat [Internet]. 2018 Apr 15;50(2):356–65. Available from: http://www.e-crt.org/journal/view.php?doi=10.4143/crt.2017.125 Search in Google Scholar

[34] Grier HE , Krailo MD , Tarbell NJ , Link MP , Fryer CJH , Pritchard DJ , et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med [Internet]. 2003 Feb 20;348(8):694–701. Available from: http://www.nejm.org/doi/abs/10.1056/NEJMoa020890 Search in Google Scholar

[35] Voltolini L , Rapicetta C , Luzzi L , Paladini P , Ghiribelli C , Scolletta S , et al. Lung resection for non-small cell lung cancer after prophylactic coronary angioplasty and stenting: short- and long-term results. Minerva Chir. 2012;67(1):77–85. Search in Google Scholar

[36] Marulli G , Breda C , Fontana P , Ratto GB , Leoncini G , Alloisio M , et al. Pleurectomy–decortication in malignant pleural mesothelioma: are different surgical techniques associated with different outcomes? Results from a multicentre study. Eur J Cardiothorac Surg [Internet]. 2017 Jul;52(1):63–9. Available from: https://academic.oup .com/ejcts/article-lookup/doi/10.1093/ejcts/ezx079 Search in Google Scholar

[37] van Mater D , Wagner L . Management of recurrent Ewing sarcoma: challenges and approaches. OncoTargets Ther [Internet]. 2019 Mar;12:2279–88. Available from: https://www.dovepress.com/management-of-recurrent-ewing-sarcoma-challenges-and-approaches-peer-reviewed-article-OTT Search in Google Scholar

[38] Soni A , Wei S . Primary renal Ewing sarcoma in an adult. Urology [Internet]. 2017 Jan;99:e11–3. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0090429516306525 Search in Google Scholar

[39] Teegavarapu PS , Rao P , Matrana MR , Cauley DH , Wood CG , Patel S , et al. Outcomes of adults with Ewing sarcoma family of tumors (ESFT) of the kidney. Am J Clin Oncol [Internet]. 2017 Apr;40(2):189–93. Available from: http://Insights.ovid.com/crossref?an=00000421-201704000-00015 Search in Google Scholar

[40] Abolhasani M , Salarinejad S , Moslemi MK . Ewing sarcoma/primitive neuroectodermal tumor of the kidney. Int J Surg Case Rep [Internet]. 2016;28:330–4. Available from: https://linkinghub.elsevier.com/retrieve/pii/S2210261216304114 Search in Google Scholar

[41] Yamazaki K , Ishida Y , Yamamoto Y . Upregulation of NKX2.2, a target of EWSR1/FLI1 fusion transcript, in primary renal Ewing sarcoma. J Cytol [Internet]. 2015;32(1):30. Available from: http://www.jcytol.org/text.asp?2015/32/1/30/155229 Search in Google Scholar

[42] Chakrabarti N , Dewasi N , Das S , Bandyopadhyay A , Bhaduri N . Primary Ewing’s sarcoma/primitive neuroectodermal tumor of of kidney – a diagnostic dilemma. Iran J Cancer Prev. 2015;8(2):129–33. Search in Google Scholar

[43] Almeida MFA , Patnana M , Korivi BR , Kalhor N , Marcal L . Ewing sarcoma of the kidney: a rare entity. Case Rep Radiol [Internet]. 2014;1–5. Available from: http://www.hindawi.com/journals/crira/2014/283902/ Search in Google Scholar

[44] Liu Z , Wang X , Lu Y , Chen L , Lu Y . Primary Ewing sarcoma/primitive neuroectodermal tumor of the renal pelvis: a case report. World J Surg Oncol [Internet]. 2014;12(1):293. Available from: http://wjso.biomedcentral.com/articles/10.1186/1477-7819-12-293 Search in Google Scholar

[45] Richey SL , Rao P , Wood CG , Patel S , Tannir NM . Metastatic extraosseous Ewing’s sarcoma (EES)/primitive neuroectodermal tumor (PNET) of the kidney: 8-year durable response after induction and maintenance chemotherapy. Clin Genitourin Cancer [Internet]. 2012 Sep;10(3):210–2. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1558767312000559 Search in Google Scholar

[46] Tariq Z , Ghose A , Sofi A , Mohamed I , Harmon D . Metastatic Renal Extraskeletal Ewing Sarcoma in Complete Remission for the Last 8 Years. Am J Ther [Internet]. 2012 May;19(3):120–1. Available from: https://insights.ovid.com/crossref?an=00045391-201205000-00001 Search in Google Scholar

[47] Alonso AH , Gárate MM , Amo FH , Iribarren IM , Escudero RM , Sánchez JP , et al. Primary renal Ewing’s sarcoma. Arch Esp Urol. 2011;64(7):636–9. Search in Google Scholar

[48] Pathak G , Neve R , Ashturkar A , Deshmukh S . Primitive neuroectodermal tumor of kidney with tumor thrombus extending up to right atrium. Indian J Cancer [Internet]. 2011;48(2):274. Available from: http://www.indianjcancer.com/text.asp?2011/48/2/274/82906 Search in Google Scholar

[49] Mohsin R , Hashmi A , Mubarak M , Sultan G , Shehzad A , Qayum A , et al. Primitive neuroectodermal tumor/Ewing′s sarcoma in adult uro-oncology: A case series from a developing country. Urol Ann [Internet]. 2011;3(2):103. Available from: http://www.urologyannals.com/text.asp?2011/3/2/103/82180 Search in Google Scholar

[50] Badar Q , Ali N , Abbasi N , Ashraf S , Karsan F , Hashmi R . Ewing’s sarcoma/PNET of kidney in 13-year-old girl. J Pak Med Assoc. 2010;60(4):314–5. PMID: 20419980. Search in Google Scholar

[51] Wedde TB , Lobmaier IVK , Brennhovd B , Lohne F , Hall KS . Primary Ewing’s sarcoma of the kidney in a 73-year-old man. Sarcoma [Internet]. 2011;2011:1–4. Available from: http://www.hindawi.com/journals/sarcoma/2011/978319/ Search in Google Scholar

[52] Asiri M , Al-Sayyad A . Renal primitive neuroectodermal tumour in childhood: case report and review of literature. Can Urol Assoc J [Internet]. 2010 Apr 22;4(6):158. Available from: http://www.cuaj.ca/index.php/journal/article/view/969 Search in Google Scholar

[53] Angel JR , Alfred A , Sakhuja A , Sells RE , Zechlinski JJ . Ewing’s sarcoma of the kidney. Int J Clin Oncol [Internet]. 2010 Jun 4;15(3):314–8. Available from: http://link.springer.com/10.1007/s10147-010-0042-0 Search in Google Scholar

[54] Ohgaki K , Horiuchi K , Mizutani S , Sato M , Kondo Y . Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the kidney that responded to low-dose chemotherapy with ifosfamide, etoposide, and doxorubicin. Int J Clin Oncol [Internet]. 2010 Apr 26;15(2):210–4. Available from: http://link.springer.com/10.1007/s10147-010-0031-3 Search in Google Scholar

[55] Fergany AF , Dhar N , Budd GT , Skacel M , Garcia JA . Primary extraosseous Ewing sarcoma of the kidney with level III inferior vena cava thrombus. Clin Genitourin Cancer [Internet]. 2009 Oct;7(3):E95–7. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1558767311700398 Search in Google Scholar

[56] Businger A , Zettl A , Sonnet S , Ruszat R , von Flüe M . Primitive neuroectodermal tumor of the kidney in an adult: a case report. Cases J [Internet]. 2009;2(1):6791. Available from: http://www.casesjournal.com/content/2/1/6791 Search in Google Scholar

[57] Ishii H , Ogaki K . Primitive neuroectodermal tumor of the kidney. Med Mol Morphol [Internet]. 2009 Sep 26;42(3):175–9. Available from: http://link.springer.com/10.1007/s00795-009-0453-z Search in Google Scholar

[58] Zhang L , Wang T , Zheng L , Wu G . Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus. J Natl Med Assoc [Internet]. 2009 Dec;101(12):1291–4. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0027968415311421 Search in Google Scholar

[59] Ong PH , Manikandan R , Philip J , Hope K , Williamson M . Primitive neuroectodermal tumour of the kidney with vena caval and atrial tumour thrombus: a case report. J Med Case Rep [Internet]. 2008 Dec 11;2(1):265. Available from: http://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-2-265 Search in Google Scholar

[60] Koski ME , Tedesco JM , Clark PE . Renal peripheral neuroectodermal tumor presenting at age 78: case report. Sci World J [Internet]. 2008;8:830–4. Available from: http://www.hindawi.com/journals/tswj/2008/459082/abs/ Search in Google Scholar

[61] Chu WC , Reznikov B , Lee EY , Grant RM , Cheng FWT , Babyn P . Primitive neuroectodermal tumour (PNET) of the kidney: a rare renal tumour in adolescents with seemingly characteristic radiological features. Pediatr Radiol [Internet]. 2008 Oct 19;38(10):1089–94. Available from: http://link.springer .com/10.1007/s00247-008-0971-1 Search in Google Scholar

[62] Kang SH , Perle MA , Nonaka D , Zhu H , Chan W , Yang GCH . Primary Ewing sarcoma/PNET of the kidney: fine-needle aspiration, histology, and dual color break apart FISH Assay. Diagn Cytopathol [Internet]. 2007 Jun;35(6):353–7. Available from: http://doi.wiley.com/10.1002/dc.20642 Search in Google Scholar

[63] Moustafellos P , Gourgiotis S , Athanasopoulos G , Karagianni E , Hadjiyannakis E . A spontaneously ruptured primitive neuroectodermal tumor/extraosseous Ewing’s sarcoma of the kidney with renal vein tumor thrombus. Int Urol Nephrol [Internet]. 2007 Jun 14;39(2):393–5. Available from: http://link.springer.com/10.1007/s11255-006-9073-x Search in Google Scholar

[64] Parada D , Godoy A , Liuzzi F , Peña KB , Romero A , Parada AM . Primary Ewing´s sarcoma/primitive neuroectodermal tumor of the kidney: an infrequent finding. Arch Esp Urol (Ed impresa) [Internet]. 2007 Apr;60:3. Available from: http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0004-06142007000300020&lng=en&nrm=iso&tlng=en Search in Google Scholar

[65] Saxena R , Sait S , Mhawech-Fauceglia P . Ewing sarcoma/primitive neuroectodermal tumor of the kidney: a case report. Diagn Immunohistochem Mol Anal Ann Diagn Pathol [Internet]. 2006 Dec;10(6):363–6. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1092913405001851 Search in Google Scholar

[66] Maeda M , Tsuda A , Yamanishi S , Uchikoba Y , Fukunaga Y , Okita H , et al. Ewing sarcoma/primitive neuroectodermal tumor of the kidney in a child. Pediatr Blood Cancer [Internet]. 2008 Jan;50(1):180–3. Available from: http://doi.wiley.com/10.1002/pbc.20831 Search in Google Scholar

[67] Erkılıç S , Özsaraç C , Koçer NE , Erbağcı A . Primary primitive neuroectodermal tumor of the kidney: A case report. Int Urol Nephrol [Internet]. 2006 Jun;38(2):199–202. Available from: http://link.springer.com/10.1007/s11255-006-6675-2 Search in Google Scholar

[68] Pomara G , Cappello F , Cuttano MG , Rappa F , Morelli G , Mancini P , et al. Primitive Neuroectodermal Tumor (PNET) of the kidney: a case report. BMC Cancer [Internet]. 2004 Dec 26;4(1):3. Available from: http://bmccancer.biomedcentral.com/articles/10.1186/1471-2407-4-3 Search in Google Scholar

[69] Sivaramakrishna B , Mundada OP , Aron M , Aron M , Vijayaraghavan M . Primary primitive neuroectodermal tumor (PNET) of the kidney with venous thrombus. Int Urol Nephrol [Internet]. 2003;35(3):311–2. Available from: http://link.springer .com/10.1023/B:UROL.0000022921.65697.96 Search in Google Scholar

[70] Murphy SM , Browne RFJ , Finn S , Myers E , Crotty P , Grainger R . Non-metastatic primitive peripheral neuroectodermal tumour of the kidney (extraskeletal Ewing’s sarcoma) with vena caval tumour thrombus. BJU Int [Internet]. 2007 Dec 7;92:e44–e44. Available from: http://doi.wiley .com/10.1111/j.1464-410X.2003.04059.x Search in Google Scholar

[71] Wada Y , Yamaguchi T , Kuwahara T , Sugiyama Y , Kikukawa H , Ueda S . Primitive neuroectodermal tumour of the kidney with spontaneous regression of pulmonary metastases after nephrectomy. BJU Int [Internet]. 2003 Jan;91(1):121–2. Available from: http://doi.wiley.com/10.1046/j.1464-410X.2003.04019.x Search in Google Scholar

[72] Vicha A , Stejskalova E , Sumerauer D , Kodet R , Malis J , Kucerova H , et al. Malignant peripheral primitive neuroectodermal tumor of the kidney. Cancer Genet Cytogen [Internet]. 2002 Nov;139(1):67–70. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0165460802006027 Search in Google Scholar

[73] Karnes RJ , Gettman MT , Anderson PM , Lager DJ , Blute ML . Primitive neuroectodermal tumor (extraskeletal Ewing’s sarcoma) of the kidney with vena caval tumor thrombus. J Urol [Internet]. 2000 Sep;164(3 Pt 1):772. Available from: http://gateway.ovid.com/ovidweb.cgi?T=JS&PAGE=crossref&AN=00005392-200009010-00036 Search in Google Scholar

[74] Kuroda M , Urano M , Abe M , Mizoguchi Y , Horibe Y , Murakami M , et al. Primary primitive neuroectodermal tumor of the kidney. Pathol Int [Internet]. 2000 Dec 30;50(12):967–72, Availablefrom:https://onlinelibrary.wiley.com/doi/abs/10.1046/j.1440-1827.2000.01147.x Search in Google Scholar

[75] Herman TE , McAlister WH , Dehner LP . Peripheral primitive neuroectodermal tumor: report of a case arising in the kidney. Pediatr Radiol [Internet]. 1997 Jul 14;27(7):620–1. Available from: http://link.springer.com/10.1007/s002470050200 Search in Google Scholar

[76] Fontaine C , Schots R , Braeckman J , Goossens A , Soete G , de Grève G . Long-term survival in an adult metastatic renal peripheral primitive neuroectodermal tumor (PPNET) with multimodality treatment including high-dose chemotherapy. Ann Oncol [Internet]. 1997 Jul;8(7):691–4. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0923753419483300 Search in Google Scholar

[77] Mor Y , Nass D , Raviv G , Neumann Y , Nativ O , Goldwasser B . Malignant peripheral primitive neuroectodermal tumor (PNET) of the kidney. Med Pediatr Oncol [Internet]. 1994;23(5):437–40. Available from: http://doi.wiley .com/10.1002/mpo.2950230508 Search in Google Scholar

[78] Singla N , Kapoor A , Radotra BD , Chatterjee D . Extraosseous Ewing sarcoma in the lumbar epidural space: an uncommon malignant tumor mimicking a spinal schwannoma. Spine J [Internet]. 2016 Oct;16(10):e675–7. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1529943016004484 Search in Google Scholar

[79] Lu J , Xiong X-Z , Li L , Cheng N-S . An unusual retroperitoneal tumour mimicking adrenal pheochromocytoma. Digest Liver Dis [Internet]. 2015 Oct;47(10):e18. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1590865815003771 Search in Google Scholar

[80] Yip C-M , Hsu S-S , Chang N-J , Wang J-S , Liao W-C , Chen J-Y , et al. Primary vaginal extraosseous Ewing sarcoma/primitive neuroectodermal tumor with cranial metastasis. J Chin Med Assoc [Internet]. 2009 Jun;72(6):332–5. Available from: https://linkinghub.elsevier.com/retrieve/pii/S1726490109703818 Search in Google Scholar

[81] García-Moreno Nisa F , López Quindos P , García Teruel D , Beni Pérez R . Extraosseous retroperitoneal Ewing’s sarcoma. Clin Transl Oncol [Internet]. 2007 Jun 6;9(6):404–5. Available from: http://link.springer.com/10.1007/s12094-007-0074-8 Search in Google Scholar

[82] Venkitaraman R , George MK , Ramanan SG , Sagar T . A single institution experience of combined modality management of extra skeletal Ewings sarcoma. World J Surg Oncol [Internet]. 2007;5(1):3. Available from: http://wjso.biomedcentral.com/articles/10.1186/1477-7819-5-3 Search in Google Scholar

[83] Thebert A , Francis IR , Bowerman RA . Retroperitoneal extraosseous ewing’s sarcoma with renal involvement: US and MRI findings. Clin Imaging [Internet]. 1993 Apr;17(2):149–52. Available from: https://linkinghub.elsevier.com/retrieve/pii/089970719390057T Search in Google Scholar

Received: 2020-02-17
Revised: 2020-11-22
Accepted: 2020-12-09
Published Online: 2021-03-09

© 2021 Giovanni Cochetti et al., published by De Gruyter

This work is licensed under the Creative Commons Attribution 4.0 International License.