Primitive Neuroectodermal Tumor of the Kidney: A Single Institute Series of 16 Patients

Thyavihally, Y.; Tongaonkar, Hemant; Gupta, Sudeep; Kurkure, Purna; Amare, Pratibha; Muckaden, Mary Ann; Desai, Sangita B.

doi:10.1016/j.urology.2007.09.051

Cited by 101 publications

(133 citation statements)

References 24 publications

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“…The advanced disease rate was 57.6% and the most frequent metastases were lymph node (25%), lung (20%), and liver (14%). In another study, Yuvaraja et al [2] reported that the most common symptoms are abdominal pain (68%), abdominal mass (37%), and hematuria (31%) in their case series of 16 patients. In this case series, 10 patients (63%) had localized disease, 5 (31%) had metastasis (2 lungs, 1 lung and lymph nodes, 1 lymph nodes, and 1 liver), and 1 (6%) had locally advanced disease.…”

Section: Discussionmentioning

confidence: 95%

“…Because renal PNET includes Blastemal Wilm's tumor and other small blue round cell tumors such as neuroblastoma, rhabdomyosarcoma, small cell carcinoma, poorly differentiated synovial sarcoma, desmoplastic small round cell tumor, nephroblastoma, and lymphoma, diagnosis of renal PNET is therefore based on histopathology, immunohistochemistry, and cytogenetic studies. [2] On microscopic examination, renal PNET typically shows small round cells that may form characteristic Homer-Wright rosettes. CD99 expression is not pathognomonic for PNET, although overexpression of the surface membrane protein CD99 is almost always observed in these tumors.…”

Section: Discussionmentioning

confidence: 99%

“…The patients are mostly young adults, and the clinical course is very aggresive. [2] We aimed to present a case of renal PNET admitted to our clinic with metastatic disease.…”

Section: Introductionmentioning

confidence: 99%

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A large and metastatic primitive neuroectodermal tumor of the kidney

Hamidi

Esen

Kıvrak

et al. 2015

Turkish Journal of Urology

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Primitive neuroectodermal tumor (PNET) of the kidney is rare. Mostly patients with renal PNET are young adults and the survival rates are poor. Although radiological and pathological investigations, differential diagnosis from other kidney tumours is very difficult. The treatment is often delayed because of difficulties with diagnosis. In most cases of renal PNET, as in this case, prognosis is poor. Particularly, in young adults with large renal masses, it must be diagnosed and treatment should be started immediately.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

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A large and metastatic primitive neuroectodermal tumor of the kidney

Hamidi

Esen

Kıvrak

et al. 2015

Turkish Journal of Urology

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“…ESK/PNET has been speculated as relatively sensitive to chemotherapy. In particular, initially, vincristine, actinomycin, and cyclophosphamide were found effective in treating ESK/PNET [6]. Later, doxorubicin, ifosfamide, and etoposide also demonstrated activity towards these tumors [7].…”

Section: Discussionmentioning

confidence: 99%

“…In particular, radiation to the tumor bed to eradicate local residual disease has demonstrated promising results. Specifically, adjuvant radiotherapy (dose 50-60 Gy) to renal bed and positive regional lymph nodes was implemented in nine patients of ESK/PNET; complete response in terms of reducing residual local disease was achieved [6]. Moreover, Miser et al claimed that radiotherapy (recommended dose of 50.4-60 Gy) can produce good local control in ESK/PNET patients, particularly when total tumor resection is not possible [12].…”

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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Adolescent–adult nonmetastatic Ewing sarcoma—Experience from a large developing country

Bajpai

Panda

Chandrasekharan

et al. 2021

Pediatric Blood & Cancer

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Background: Outcome and toxicity data in adolescent-adult Ewing sarcoma (AA-ES) patients are sparse and merits exploration. Methods: Histopathologically confirmed, nonmetastatic AA-ES patients, who received standard institutional combination chemotherapy regimen (Ewing's family of tumors-2001 [EFT-2001]) comprising of ifosfamide plus etoposide and vincristine, doxorubicin plus cyclophosphamide, lasting a total of 12 months between 2013 and 2018, were analyzed for treatment-related toxicities, event-free survival (EFS), and overall survival (OS). Results: There were 235 patients (primary safety cohort [PSC]) with median age of 23 (15-61) years; 159 (67.7%) were males, 155 (65.9%) had skeletal primary and 114(48.5%) had extremity tumors. One hundred ninety-six (83.4%) were treatment naïve (primary efficacy cohort [PEC]) and of these 119 (60.7%) had surgery. In PEC, at a median follow-up of 36.4 (interquartile range [IQR] 20-55) months, estimated 3-year EFS and OS were 67.3% (95% CI 60.3-75.1%) and 91.1% (95% CI 86.7-95.7%), respectively. Of these, 158 (80.6%) complying with intended treatment, at a median followup of 39 (IQR 26-57) months had an estimated 3-year EFS of 68.2% (95% CI 60.3-76.1%). In multivariable analysis, good prognostic factors included longer symptom(s) duration (HR 0.93, 95% CI 0.86-0.994), ≥99% necrosis (HR 0.30, 95% CI 0.11-0.77), and treatment completion (HR 0.32, 95% CI 0.14-0.74). Among PSC, grade 3-4 toxicities were febrile neutropenia (119, 50.6%), anemia (130, 55.3%), peripheral neuropathy (37, 15.7%), with three (1.3%) chemo-toxic deaths. Conclusions:The outcomes of AA nonmetastatic ES patients treated with EFT-2001 regimen were comparable to those reported by others, with acceptable toxicity. This regimen can be considered a standard of care in AA-ES.

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Primitive Neuroectodermal Tumor of the Kidney: A Single Institute Series of 16 Patients

Cited by 101 publications

References 24 publications

A large and metastatic primitive neuroectodermal tumor of the kidney

A large and metastatic primitive neuroectodermal tumor of the kidney

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

Adolescent–adult nonmetastatic Ewing sarcoma—Experience from a large developing country

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