Renal Ewing’s sarcoma/primitive neuroectodermal tumor (PNET): a case series of 7 patients and literature review

Liang, Lianchun; Song, Haifeng; Ma, Binglei; Zhang, Zhenan; Zhu, Kun; Li, Qinhan; Zhou, Chaohui; Li, Aolin; Li, Jun; Zhang, Quan; Zhu, Shiyu; Zhang, Qian

doi:10.21037/tau-20-1122

Cited by 7 publications

(9 citation statements)

References 19 publications

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“…In addition, survivors of childhood cancers are more likely develop second cancers due to genetic factors and side effects of treatment. According to the ESFT summary and treatment policy in past reports, 1,3,4,6,7 the most common chemo regimen is known as VD(or A)C/IE (vincristine, doxorubicin (or Adriamycin), and cyclophosphamide). It alternates between 2 combinations of drugs, given every 2 to 3 weeks at least 9 weeks before surgery or radiation, and then will get more chemo afterward as well.…”

Section: Discussionmentioning

confidence: 99%

“…The ESFT are primary tumors that affects bones or soft tissue, and they are highly cellular‐malignant small round‐cell tumors. Although it has been thought that ESFTs rarely occur in the retroperitoneum, there are a few reports of this 1 . In clinical practice, <1% of retroperitoneum tumors are observed as a renal mass 2 .…”

Section: Introductionmentioning

confidence: 99%

“…Although it has been thought that ESFTs rarely occur in the retroperitoneum, there are a few reports of this. 1 In clinical practice, <1% of retroperitoneum tumors are observed as a renal mass. 2 ESFT of the kidney has aggressive clinical behavior and a poor prognosis, and its preoperative discrimination from RCC is very difficult.…”

Section: Introductionmentioning

confidence: 99%

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Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney and its clinical features

et al. 2022

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Introduction Ewing sarcoma family tumor is a malignant tumor that is primarily of bone origin; it rarely occurs in the kidney. Case presentation A 22‐year‐old woman presented with hematuria. Computed tomography revealed a 6 × 6‐cm mass in the lower pole of the right kidney with invasion into the right renal vein. A right laparoscopic radical nephrectomy was performed. The tumor was completely encapsulated. Based on the small‐round‐cell histology, diffusely CD99‐positive tumor cells, and EWS (ex7)–FLi1 (ex6) fusion gene break point transcript, we diagnosed Ewing sarcoma/primitive neuroectodermal tumor of the kidney. After surgery, eight cycles of adjuvant chemotherapy including vincristine, doxorubicin (Adriamycin®), cyclophosphamide, ifosfamide, and etoposide were given. No evidence of recurrence has been observed 13 months from diagnosis. Conclusion This was a rare Ewing sarcoma family tumor in the kidney of a young female with no remarkable family medical history.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney and its clinical features

et al. 2022

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“…The imaging findings of EWS/pPNETs are nonspecific. We used contrast-enhanced ultrasound to make a diagnosis of EWS/pPNETs in our patient, which had never been reported previously to our knowledge.EWS/pPNETs of the kidney are more common in male adolescents, with an average age of 29 years, and the male:female sex incidence ratio ranges from 2:1 to 3:1 [ 14 ]. The clinical symptoms and imaging findings are nonspecific.…”

Section: Discussionmentioning

confidence: 99%

Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour of the kidney: a case report and literature review

Nie

2022

BMC Urol

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Background Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumours(EWS/pPNETs) of the kidney are rare. Signs and symptoms are atypical in EWS patients. Presenting symptoms include haematuria, abdominal pain, or a palpable mass. A comprehensive review of the literature shows that it is difficult to make an accurate diagnosis based on physical examination alone. The imaging findings of EWS/pPNETs are nonspecific. We used contrast-enhanced ultrasound (CEUS) to diagnose an EWS/pPNET in our patient, which had never been reported previously to our knowledge. Case presentation This article reports the case of a 20-year-old female with an abdominal mass and gross haematuria for 1 month. The ultrasound revealed a hypoechoic mass with a clear margin at the lower pole in the left kidney. CEUS demonstrated signs of annular enhancement and heterogeneous enhancement of the tumour, and simultaneous wash-in was predominant. Computed tomography images showed an elliptical low-density tumour. The patient underwent a left kidney resection, and the pathological diagnosis was an EWS/pPNET. Twenty-one days after the kidney operation, the patient underwent 8 cycles of a CAV (vinorelbine, ifosfamide, epirubicin) + IE (isocyclophosphamide, etoposide) chemotherapy regimen. Subsequently, radiotherapy (dose: 45 Gy, radiation field:the tumour bed following surgical resection) was administered for nearly 30 days. The patient had no signs of local recurrence or metastasis within a follow-up of 4 years. Conclusions As a radiation-free, inexpensive, convenient, and repeatable examination method, ultrasound was the primary choice for kidney examination. Early CEUS was helpful to make an accurate diagnosis. Surgery and adjuvant radiation or chemotherapy administered in a timely manner can prevent further deterioration.

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“…Aunque suele producirse en los tejidos blandos de niños y adultos jóvenes, se ha notificado casos primarios en sitios viscerales como riñón, ovario, vagina, testículo, útero, cuello uterino, vejiga urinaria, glándula parótida, corazón, pulmón, recto, páncreas y vesícula biliar. Aquellos que se producen en el ovario son relativamente raros y se asocian con alta mortalidad (3) . Se presenta un caso de tumor neuroectodérmico primitivo de ovario.…”

Section: Introductionunclassified

Tumor neuroectodérmico primitivo primario de ovario

Torres-Cepeda¹,

Rondón-Tapía²

2022

Rev peru ginecol obstet.

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El tumor neuroectodérmico primitivo del ovario es un sarcoma de tejido blando de células redondas pequeñas, raro y agresivo, de origen neural que generalmente se asocia con una alta morbilidad y mortalidad. La inmunohistoquímica es un complemento útil en el diagnóstico diferencial. Se describe un caso de tumor neuroectodérmico primitivo del ovario en paciente nulípara de 21 años que refería dolor y aumento de la circunferencia abdominal. La ecografía mostró tumoración de contenido heterogéneo sólido-quística que aparentemente se originaba del anexo izquierdo. La resonancia magnética confirmó la presencia de tumoración que se extendía hacia la fosa iliaca izquierda sin afectación de órganos locales ni metástasis regionales o a distancia. Los marcadores tumorales estaban todos dentro del rango normal. Durante la laparotomía se observó tumoración de ovario izquierdo con ovario derecho normal. Se realizó salpingoforectomía izquierda debido al tamaño del tumor, resección en cuña de ovario derecho, linfadenectomía pélvica y omentectomía. El examen histopatológico reveló tumor compuesto por láminas de células redondas. Las células tumorales fueron positivas para cromogranina A, sinatrofisina, vimentina y enolasa específica de neuronas, lo que confirmó el diagnóstico de tumor neuroendocrino primitivo de ovario izquierdo, que se originaba de teratoma quístico inmaduro. La paciente rechazó la quimioterapia postoperatoria.

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Renal Ewing’s sarcoma/primitive neuroectodermal tumor (PNET): a case series of 7 patients and literature review

Cited by 7 publications

References 19 publications

Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney and its clinical features

Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney and its clinical features

Extraosseous Ewing's sarcoma/peripheral primitive neuroectodermal tumour of the kidney: a case report and literature review

Tumor neuroectodérmico primitivo primario de ovario

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