Ewing’s Sarcoma / Primitive Neuroectodermal Tumor of the Kidney

Funahashi, Yasuhito; Hattori, Ryohei; Yamamoto, Takumi; Mizutani, Kosuke; Yumura, Yasushi; Matsukawa, Yukihiro; Sassa, Naoto; Okumura, Koji; Gotoh, Michihiro

doi:10.1055/s-0028-1098827

Cited by 14 publications

(13 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In addition to these entities, the differential diagnosis for renal PNET also includes Wilms tumor, neuroblastoma, small cell neuroendocrine tumor, desmoplastic small cell round tumor, RCC, and synovial sarcoma. 2,3,10 In order to make a diagnosis of renal PNET, one must consider the tumor's morphology, immunostaining profile, and, in some instances, genetic mutations. Perhaps the most important identifying histologic feature to diagnose renal PNET is the presence of pseudorosettes.…”

Section: Differential Diagnosis and Diagnostic Pitfallsmentioning

confidence: 99%

“…2012;136:686-690; doi: 10.5858/ arpa.2011-0104-RS) P rimitive neuroectodermal tumors (PNETs), along with Askin tumor and Ewing sarcoma (ES), exist as members of the ES/PNET family of tumors, 1 which comprises 1% of all sarcomas. 2 As their name would suggest, PNETs arise from the cells of the primitive neuroectoderm. 3 Primitive neuroectodermal tumors may occur both within and outside of the central nervous system.…”

mentioning

confidence: 99%

“…12 It is also notable that extensive areas of necrosis or hemorrhage may be seen in renal PNETs, which may also alter their gross appearance. [1][2][3] Renal PNETs are often reported to have a lobular shape, 2,10 and may potentially contain a fibrous capsule or cystic components. 3,6 Many cases have been documented to feature some sort of angiolymphatic invasion, 10 with involvement of the inferior vena cava, renal vein, and tumor thrombus frequently reported.…”

mentioning

confidence: 99%

See 2 more Smart Citations

Renal Primitive Neuroectodermal Tumors

Bartholow

Parwani²

2012

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

Primitive neuroectodermal tumors exist as a part of the Ewing sarcoma/primitive neuroectodermal tumor family. These tumors most commonly arise in the chest wall and paraspinal regions; cases with a renal origin are rare entities, but have become increasingly reported in recent years. Although such cases occur across a wide age distribution, the average age for a patient with a renal primitive neuroectodermal tumor is the mid- to late 20s, with both males and females susceptible. Histologically, these tumors are characterized by pseudorosettes. Immunohistochemically, CD99 is an important diagnostic marker. Clinically, these are aggressive tumors, with an average 5-year disease-free survival rate of only 45% to 55%. Given that renal primitive neuroectodermal tumor bears many similarities to other renal tumors, it is important to review the histologic features, immunostaining profile, and genetic abnormalities that can be used for its correct diagnosis.

show abstract

Section: Differential Diagnosis and Diagnostic Pitfallsmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 1 more Smart Citation

Renal Primitive Neuroectodermal Tumors

Bartholow

Parwani²

2012

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

show abstract

“…It was first described in 1975 by Seemayer and colleagues [1]. Only very few cases of primary EWS have been reported in the literature till date [2][3][4][5][6][7][8][9]. Sources of renal EWS include neural cells that invaginate into the kidney during development.…”

Section: Introductionmentioning

confidence: 99%

Ewings Sarcoma of the Kidney: A Rare Entity

Choubey

Pipara

Kumar

2017

World J Nephrol Urol

View full text Add to dashboard Cite

Primary Ewings sarcoma (EWS) of the kidney is a very rare tumor and very few cases have been reported till date. We present a case of a young girl presenting to us with flank pain. On evaluation, she was found to have a renal mass. She was explored and a nephrectomy was done. Histopathology was reported as EWS. She was then planned for adjuvant chemotherapy. Primary EWS of the kidney is characterized by an aggressive course and poor prognosis. Hence, while dealing with renal masses in young patients, this entity should always be kept in mind.

show abstract

“…Peripheral primitive neuroectodermal tumor/Ewing's sarcoma (PNET/ES) belongs to "Ewing family of tumors" and comprises 1% of all sarcomas [1] . They have a predilection for the adolescent age group.…”

Section: Discussionmentioning

confidence: 99%

Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Chinnaa

Das

Sharma

et al. 2014

WJR

View full text Add to dashboard Cite

Peripheral primitive neuroectodermal tumor (PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava (IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up. Core tip: Traditionally, an aggressive renal mass with pulmonary tumor embolism is an entity well described in renal cell carcinomas. However, renal primitive neuroectodermal tumor (PNET), a rare and aggressive tumor, can have a similar presentation. The severity of the clinical manifestations of pulmonary tumor embolism is highly variable and less predictable. Bland thrombus often mimics tumor emboli in imaging and histopathology is often confirmatory. Tumoral extension into the pulmonary artery is not necessarily associated with pulmonary metastasis. Surgical removal of the thrombus along with the primary tumor followed by adjuvant chemotherapy may prolong the survival in these patients.Chinnaa S, Das CJ, Sharma S, Singh P, Seth A, Purkait S, Mathur SR. Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report. World

show abstract

Ewing’s Sarcoma / Primitive Neuroectodermal Tumor of the Kidney

Cited by 14 publications

References 6 publications

Renal Primitive Neuroectodermal Tumors

Renal Primitive Neuroectodermal Tumors

Ewings Sarcoma of the Kidney: A Rare Entity

Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

Contact Info

Product

Resources

About