Primary Ewing sarcoma/primitive neuroectodermal tumor of the renal pelvis: a case report

Liu, Zhihong; Wang, Xianding; Lü, You; Chen, Libo; Lu, Yiping

doi:10.1186/1477-7819-12-293

Cited by 8 publications

(5 citation statements)

References 12 publications

(9 reference statements)

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“…In 2014, Liu et al reported the world’s first renal pelvis ES/PNET ( 12 ). To our knowledge, very few cases of the renal pelvis, ES/PNET tumor are reported in the literature to date.…”

Section: Discussionmentioning

confidence: 99%

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

Hota

Kalra

Dorairajan

et al. 2020

jkcvhl

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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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“…In 2014, Liu et al reported the world’s first renal pelvis ES/PNET ( 12 ). To our knowledge, very few cases of the renal pelvis, ES/PNET tumor are reported in the literature to date.…”

Section: Discussionmentioning

confidence: 99%

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

Hota

Kalra

Dorairajan

et al. 2020

jkcvhl

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show abstract

“…Primary renal ESFT presents major diagnostic and management hurdles for providers, as these patients most often present with advanced staging with extrarenal extension and metastases. [4][5][6][7][8][9] The optimal management of ESFT continues to be debated. However, current standard of practice invokes a combination of chemotherapy, surgical resection, and/or radiotherapy.…”

Section: Discussionmentioning

confidence: 99%

“…Despite the rarity in presentation, there are many case reports and several case series that echo the aggressive clinical features, high propensity for metastasis, and overall poor outcome. 1,[4][5][6][7][8][9][10] In this paper, we present seven pediatric and young adult patients with primary renal ESFT and one patient with primary adrenal disease.…”

Section: Introductionmentioning

confidence: 99%

Primary Renal Ewing Sarcoma in Children and Young Adults

Bradford

Nobori²,

Johnson³

et al. 2020

Journal of Pediatric Hematology/Oncology

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The Ewing sarcoma family of tumors (ESFT) are high-grade small round blue cell malignancies traditionally presenting in children and adolescents. The most common site of primary disease is bone, though extraskeletal primary sites are well-recognized. 1 We present six cases of primary ESFT of the kidney and one case of the adrenal gland. Patients were 11-18 years of age at diagnosis. Metastases at diagnosis were present in most cases (n=6). All patients underwent surgery, and most received radiation (n=5). Five patients relapsed after initial remission.Comprehensive review of the primary renal ESFT literature was used to analyze various factors, including age, gender, disease metrics, metastases at diagnoses, and overall survival in a total of 362 cases. Notably, while the general ESFT population has reported rates of metastasis at diagnosis of 20-25%, 2 this rate in the renal ESFT population was 53% with a rate of 59% in adolescent and young-adult (AYA) patients (11-24 years). Nodal disease at diagnosis was present in 24% of renal ESFT cases compared with 3.2% in patients with primary skeletal ESFT. 3 While this malignant process may share histologic and molecular features with its bone and soft tissue counterparts, primary renal ESFT presentations appear to be more aggressive and have worse outcomes.

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“…For instance, an ESK/PNET patient underwent radical nephroureterectomy; the patient was disease-free (i.e., no recurrence or metastasis) at 18 months postoperatively [4]. Nevertheless, ESK/PNET tumors are typically aggressive and thereby involve the lymph nodes and metastasize to lung, bone, and liver [5], demanding for multi-modality treatment.…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing sarcoma of the kidney: a case report and treatment review

et al. 2017

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Ewing sarcomas/primitive neuroectodermal tumors (ES/PNET) of the kidney are rarely found high-grade malignant tumors, offering poor prognosis. Although established treatment guidelines for ES of kidney are scarce, a multi-modality treatment approached is typically implemented. Herein, we report a 14-year-old female patient with ES of right kidney. Post-nephrectomy disease recurrence was treated with chemotherapy (i.e., vincristine, doxorubicin and cyclophosphamide); marked reduction in tumor size (i.e., from 18.5 9 11.3 cm 2 to 3.7 9 2.2 cm 2 ; *96% reduction in size) as per computed tomography images was observed. We present our treatment experience and review from the available literature.

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Primary Ewing sarcoma/primitive neuroectodermal tumor of the renal pelvis: a case report

Cited by 8 publications

References 12 publications

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

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

Primary Renal Ewing Sarcoma in Children and Young Adults

Primary Ewing sarcoma of the kidney: a case report and treatment review

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