Journals / Eurasian Journal of Medical Investigation / 2020 / Cilt: 4 - Sayı: 2

Clinical and Pathological Features of Ewing Sacoma Family Tumors in Uro-oncology: A Single-Institute Experience

Pages
247–252
Publication Date
2022-10-12
DOI
—
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AI Summary & Key Takeaways

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⚡ TL;DR(Too Long; Didn't Read)

We aimed todemonstrate the clinical features and prognosis of 6 genitourinary ESFT patients from a single institute from Turkey.Methods: A total of 6 cases of genitourinary ESFT (3 renal, 2 bladder and 1 adrenal) treated at our department werereviewed retrospectively and data considered with the literature.Results: Half of the patients in our cohort consisted of women and the median age of the cohort was 39 years at presentation.Median follow-up time was 46.3 (range; 3.6-103.2) months and median overall survival was 25 months.

📌 3 Key Takeaways

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Objective & Hypothesis:Objectives: Genitourinary Ewing Sarcoma Family Tumors (ESFT) are rare and very aggressive tumors.
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Key Findings & Data:We aimed todemonstrate the clinical features and prognosis of 6 genitourinary ESFT patients from a single institute from Turkey.Methods: A total of 6 cases of genitourinary ESFT (3 renal, 2 bladder and 1 adrenal) treated at our department werereviewed retrospectively and data considered with the literature.Results: Half of the patients in our cohort consisted of women and the median age of the cohort was 39 years at presentation.Median follow-up time was 46.3 (range; 3.6-103.2) months and median overall survival was 25 months.
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Conclusion & Impact:We suggest that ESFT should be kept in mind as a rare differentialdiagnosis of all genitourinary tumors in advanced stage disease of young patients and large primary tumor especiallyif renal origin is involved.
🔬Design:Review & Systematic Survey
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Objectives: Genitourinary Ewing Sarcoma Family Tumors (ESFT) are rare and very aggressive tumors. We aimed todemonstrate the clinical features and prognosis of 6 genitourinary ESFT patients from a single institute from Turkey.Methods: A total of 6 cases of genitourinary ESFT (3 renal, 2 bladder and 1 adrenal) treated at our department werereviewed retrospectively and data considered with the literature.Results: Half of the patients in our cohort consisted of women and the median age of the cohort was 39 years at presentation.Median follow-up time was 46.3 (range; 3.6-103.2) months and median overall survival was 25 months. Halfof patients were diagnosed at metastatic stage. Four cases were undergone radical surgery, while the remaining caseswere performed diagnostic biopsy due to metastatic disease. Five cases received adjuvant or palliative chemotherapy,while one case (patient 5) rejected to receive chemotherapy.Conclusion: ESFT is rare among urologic malignancies, it is often overlooked for differential diagnosis preoperativelyand mortality is high despite multimodal treatment. We suggest that ESFT should be kept in mind as a rare differentialdiagnosis of all genitourinary tumors in advanced stage disease of young patients and large primary tumor especiallyif renal origin is involved.

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Cite This Article

BAŞARAN, M., EKENEL, M., SARI, M., ÖZLÜK, M. Y. (2022). Clinical and Pathological Features of Ewing Sacoma Family Tumors in Uro-oncology: A Single-Institute Experience. Eurasian Journal of Medical Investigation, Cilt: 4 - Sayı: 2, 247–252. https://ajindex.com/articles/508637

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References (3)

  1. Carpentieri DF, Qualman SJ, Bowen J, Krausz T, Marchevsky A, Dickman PS. Protocol for the examination of specimens from pediatric and adult patients with osseous and extraosseous ewing sarcoma family of tumors, including peripheral primitive neuroectodermal tumor and ewing sarcoma. Archives of pathology & laboratory medicine 2005;129:866–73
  2. Rodriguez-Galindo C, Marina NM, Fletcher BD, Parham DM, Bodner SM, Meyer WH. Is primitive neuroectodermal tumor of the kidney a distinct entity? Cancer 1997;79:2243–50. [CrossRef ] 3. Pomara G, Cappello F, Cuttano MG, Rappa F, Morelli G, Mancini P, et al. Primitive Neuroectodermal Tumor (PNET) of the kidney: a case report. BMC cancer 2004;4:3. [CrossRef ] 4. Jurgens H, Bier V, Harms D, Beck J, Brandeis W, Etspuler G, et al. Malignant peripheral neuroectodermal tumors. A retrospective analysis of 42 patients. Cancer 1988;61:349–57. [CrossRef ] 5. Marina NM, Etcubanas E, Parham DM, Bowman LC, Green A. Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management. Cancer 1989;64:1952–60. [CrossRef ] 6. Ellinger J, Bastian PJ, Hauser S, Biermann K, Muller SC. Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. Urology 2006;68:257– 62. [CrossRef ] 7. Jimenez RE, Folpe AL, Lapham RL, Ro JY, O'Shea PA, Weiss SW, et al. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases. The American journal of surgical pathology 2002;26:320–7. [CrossRef ] 8. Parham DM, Roloson GJ, Feely M, Green DM, Bridge JA, Beckwith JB. Primary malignant neuroepithelial tumors of the kidney: a clinicopathologic analysis of 146 adult and pediatric cases from the National Wilms' Tumor Study Group Pathology Center. The American journal of surgical pathology 2001;25:133–46. [CrossRef ] 9. Thyavihally YB, Tongaonkar HB, Gupta S, Kurkure PA, Amare P, Muckaden MA, et al. Primitive neuroectodermal tumor of the kidney: a single institute series of 16 patients. Urology 2008;71:292–6. [CrossRef ] 10. Seemayer TA, Thelmo WL, Bolande RP, Wiglesworth FW. Peripheral neuroectodermal tumors. Perspectives in pediatric pathology 1975;2:151–72
  3. Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal