Ewing sarcoma/primitive neuroectodermal tumor of the kidney

Abolhasani, Maryam; Salarinejad, Sareh; Moslemi, Mohammad Kazem

doi:10.1016/j.ijscr.2016.10.014

Cited by 9 publications

(10 citation statements)

References 7 publications

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“…4 Other studies also showed mean age of 27 for the diagnosis of these cases. 1,2,4 In our study, the age of two patients was 25 years and the third one was 35 years.…”

Section: Discussionmentioning

confidence: 65%

“…Renal PNET/ESis a member of Ewing sarcoma family. [1][2][3] They are rare tumors originating from the neural crest and are more aggressive than PNET at any other site. 5 Rafael et al in study of 11 cases observed that most common age of diagnosis was between 18 to 45 years.…”

Section: Discussionmentioning

confidence: 99%

“…These tumors are mostly seen in young adults in second and third decade of life. 2 They are commonly confused with other small round cell tumors.Also clinical findings are very similar to other renal malignancies, hence histopathology and immunohistochemistry remains the gold standard for confirming the diagnosis. 3,4 We report three cases of PNET / Ewing sarcoma .Two patients were males 25 and 35 years and third patient was25 year old female.…”

Section: Introductionmentioning

confidence: 99%

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Primitive neuroectodermal tumor (PNET)/Ewing sarcoma of kidney: A rare case report of three cases

Padmanabhan¹,

Kanhe²,

Gadgil³

2020

JDPO

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Primitive neuroectodermal tumor (PNET) / Ewing sarcoma of kidney are rare aggressive tumor with poor prognosis. It occurs in young adults generally in 2nd to 3rd decade of life We report three cases of PNET/Ewing sarcoma of kidney in patients of age 25and 35 years with complain of hematuria and abdominal pain .CT scan revealed heterogeneously enhancing lesion in kidney and we received radical nephrectomy specimen with variegated appearance on gross. Microscopic examination showed tumor with malignant cells in sheets and pseudorosettes .The diagnosis was given as PNET/ES and was confirmed with immunohistochemistry. Renal PNET/ES is a member of Ewing sarcoma family. They are rare tumor originating from the neural crest and are more aggressive than PNET at any other site. The presence of Homer Wright rosettes is less common in extra-osseous Ewing sarcoma and their presence is a sure diagnosis of PNET/ES. The differential diagnosis for these small round cell tumor of kidney other than PNET include malignant lymphoma, renal neuroblastoma,,embryonalrhanbdomyosarcoma, wilms tumor. The immunohistochemical marker CD99 positivity is diagnostic. Other markers positive are S-100, vimentin and NSE (neuron specific enolase). Histopathology and immunohistochemistry remains gold standard for confirming the diagnosis.

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“…4 Other studies also showed mean age of 27 for the diagnosis of these cases. 1,2,4 In our study, the age of two patients was 25 years and the third one was 35 years.…”

Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Primitive neuroectodermal tumor (PNET)/Ewing sarcoma of kidney: A rare case report of three cases

Padmanabhan¹,

Kanhe²,

Gadgil³

2020

JDPO

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“…Both types have characteristics of a high degree of malignancy, a common distant metastasis, and local recurrence. The incidence ratio of male to female is around 3:1 (5,6). PNET tends to recur locally and to metastasize early to regional lymph nodes, lungs, liver, bone, and bone marrow within 2 to 3 years after surgery, with a resulting of very poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

Liu

Zhao

Shi

et al. 2019

Quant. Imaging Med. Surg

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Background: Primitive neuroectodermal tumor (PNET) is extremely rare and highly aggressive with poor prognosis. Few studies concerning PNET's the imaging features have been published.Methods: Six cases of PNET, all confirmed by pathology and immunohistochemical (IHC) examinations, were treated during January 2012 to December 2016. These cases' clinical course and imaging findings were retrospectively analyzed.Results: Among six PNET cases, one located in left superior abdomen, one case at posterior abdominal wall, one case in right orbit, one case in left frontal temporal lobe, one case in pelvic cavity, and one case located in left supraclavicular fossa. The tumor's density was uniform for small tumor and heterogeneous for large tumors on CT images, while the size of tumors differed during presentation depending on the location of the tumor. Marked enhancement was visualized after injection of contrast media. The demarcation between the lesion and adjacent tissues or organs tended to be unclear. On magnetic resonance imaging (MRI) images, the mass mainly showed heterogeneously long T1 and long T2 signal intensity, mixed high signal intensity on fluid-attenuated inversion recovery (FLAIR) image. In two cases maximum intensity projection image reconstruction demonstrates tortuous blood vessels within the tumor on enhanced CT images.Five cases were treated by surgical resection with two cases received adjuvant radiotherapy and three cases received adjuvant chemotherapy. Six patients were followed up with a mean period of 34.5 months (ranging from 6 to 55 months). Five patients survived and one died. Among the five patients undergoing surgeries, one patient presented pelvic and abdominal recurrence/metastasis 2 months after abdominal PNET resection. One patient had a recurrent lesion in the right orbit involving the right ethmoid sinus 6 months after right orbital PNET resection. One patient's pelvic tumor recurred 7 months after PNET operation, and this patient died after 1 year and 10 months of follow-up. During the follow-up period, the remaining three cases did not show obvious recurrence and/or metastasis.Conclusions: Overall, the imaging appearances of PNET lack characteristics. PNETs generally do not have clear boundary, or partially so, with its adjacent organs or tissues suggesting their invasive nature. Upon further validation, maximum intensity projection image reconstruction demonstrates tortuous blood vessels within the tumor on enhanced CT images may be valuable information for the diagnosis of PNET.

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“…This case has been approached and reported according to the SCARE criteria [3] . Presentation of such tumors reported in the literature is non-specific and frequently involves localized pain which usually involves flank, subcostal or abdominal area with or without hematuria [4] , [5] , [6] , [7] . The presence of a vascular thrombus has been reported as a distinctive feature of renal ESFT/PNET [7] , [8] , [9] , [10] , [11] .…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing’s sarcoma of the kidney: A case report

Alasmari

Albadawe

Sultan

et al. 2017

International Journal of Surgery Case Reports

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This is a 15-year-old female who presented with sudden onset left flank pain associated with nausea and vomiting and a history of weight loss. Radiological investigation revealed a large non-obstructive tumor involving the lower pole of the left kidney which was primarily thought to be a renal cell carcinoma. She underwent left open radical nephrectomy with adrenalectomy. Histopathology of the resected specimen showed features of Ewing’s sarcoma of the kidney which was confirmed by cytogenetic analysis. This is a rare disease especially in the pediatric group and in reporting such a rare case we hope it helps in identifying a potential course of the disease and its response to the involved treatment.

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Ewing sarcoma/primitive neuroectodermal tumor of the kidney

Cited by 9 publications

References 7 publications

Primitive neuroectodermal tumor (PNET)/Ewing sarcoma of kidney: A rare case report of three cases

Primitive neuroectodermal tumor (PNET)/Ewing sarcoma of kidney: A rare case report of three cases

Primitive neuroectodermal tumors: a clinical and radiological analysis of six cases

Primary Ewing’s sarcoma of the kidney: A case report

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