A rare case of neuroectodermal pelvic tumor with bladder invasion
- Autores: Bazaev V.V.1, Setdikova G.R.1, Shibaev A.N.1, Podoynicyn A.A.1, Vinogradov A.V.1, Pavlova Y.V.1
-
Afiliações:
- GBUZ Moscow district “Moscow Regional Research Clinical Institute named after M.F. Vladimirsky
- Edição: Nº 3 (2024)
- Páginas: 87-92
- Seção: Clinical case
- URL: https://journals.eco-vector.com/1728-2985/article/view/634991
- DOI: https://doi.org/10.18565/urology.2024.3.87-92
- ID: 634991
Citar
Texto integral
![Acesso aberto](https://journals.eco-vector.com/lib/pkp/templates/images/icons/text_open.png)
![Acesso é fechado](https://journals.eco-vector.com/lib/pkp/templates/images/icons/text_unlock.png)
![Acesso é fechado](https://journals.eco-vector.com/lib/pkp/templates/images/icons/text_lock.png)
Resumo
Introduction. Ewing's extraosseous sarcoma of the genitourinary system is an extremely rare disease. There are sporadic publications about the genitourinary sarcomas.
We present a case of a primitive neuroectodermal pelvic tumor with bladder invasion in a 58-year-old man. Initially, he was admitted with complaints of intense lower abdominal and right lumbar pain, severe dysuria, macrohematuria, weight loss (by 15 kg in 6 months) and general weakness. Previously, a nephrostomy tube was put due the right hydronephrosis. Nephrostomy output was up to 100-150 ml per day, and glomerular filtration rate was estimated within 5 ml/min. According to MRI data, the extra-organ pelvis tumor with bladder invasion along the right posterolateral wall was diagnosed. Cystoprostatectomy, right nephroureterectomy and left ureterocutaneostomy were performed. In the postoperative period, the patient firstly manifested neurological symptoms (paresis). According to the brain CT, two lesions of the right frontal and left parietal regions were found (most likely metastases of the primary tumor). Late admission and disseminated tumor with local invasion and brain metastases, right terminal hydronephrosis, anemia due to pronounced macrohematuria and decrease of the body weight determined an unfavorable outcome.
Conclusions. Our case report allows us to remind urologists about the presence of orphan oncological diseases with an extremely aggressive course. In-depth diagnosis requires the use of immunohistochemical methods, and the treatment of such patients should be based on a multidisciplinary approach.
Texto integral
![Acesso é fechado](https://journals.eco-vector.com/lib/pkp/templates/images/icons/text_lock.png)
Sobre autores
V. Bazaev
GBUZ Moscow district “Moscow Regional Research Clinical Institute named after M.F. Vladimirsky
Email: anshibaev@rambler.ru
Ph.D., MD, professor, leading researcher at the Department of Urology
Rússia, MoscowG. Setdikova
GBUZ Moscow district “Moscow Regional Research Clinical Institute named after M.F. Vladimirsky
Email: anshibaev@rambler.ru
Ph.D., MD, Chief of the Department of Pathology of the Oncologic Division
Rússia, MoscowA. Shibaev
GBUZ Moscow district “Moscow Regional Research Clinical Institute named after M.F. Vladimirsky
Autor responsável pela correspondência
Email: anshibaev@rambler.ru
Ph.D., leading researcher at the Department of Urology
Rússia, MoscowA. Podoynicyn
GBUZ Moscow district “Moscow Regional Research Clinical Institute named after M.F. Vladimirsky
Email: anshibaev@rambler.ru
Ph.D., MD, Head of the Urologic Department
Rússia, MoscowA. Vinogradov
GBUZ Moscow district “Moscow Regional Research Clinical Institute named after M.F. Vladimirsky
Email: anshibaev@rambler.ru
urologist at the Department of Urology
Rússia, MoscowYu. Pavlova
GBUZ Moscow district “Moscow Regional Research Clinical Institute named after M.F. Vladimirsky
Email: anshibaev@rambler.ru
Ph.D., researcher at the Department of Urology
Rússia, MoscowBibliografia
- Frank G.A. Problems of morphological classification and diagnosis of soft tissue tumors. Practical oncology. 2004;5(4):231–236. Russian (Франк Г.А. Проблемы морфологической классификации и диагностики опухолей мягких тканей. Практическая онкология. 2004;5(4):231–236).
- Davydov M.I., Machaladze Z.O., Polotsky B.E., и др. Mesenchymal tumors of the mediastinum (literature review). Siberian Journal of Oncology. 2008;1(25):64–74. Russian (Давыдов М.И., Мачаладзе З.О., Полоцкий Б.Е. и др. Мезенхимальные опухоли средостения [обзор литературы]. Сибирский онкологический журнал. 2008;1(25):64–74).
- De Alava B.E., Gerald W.L. Molecular biolodgy of the Ewings sarcoma. Primitive neuroectodermal tumor family. J Clin Oncol 2000;18:204–208. doi: 10.1200/JCO.2000.18.1.204.
- Workman M.L., Soukup S.W., Neely J.E., et al. Biologic characteristics of four Ewings’s sarcomas. Cancer Genet Cytogenet. 1985;15:215–225. doi: 10.1016/0165-4608(85)90165-7.
- Захарычев В.Д., Ганул А.В., Борисюк Б.О. Орфанные опухоли: Опыт диагностики и лечения примитивной нейроэктодермальной опухоли внутригрудной локализации. Онкология. 2017;19(1):75–79.
- Sbaraglia M. et al. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives. Pathologica. 2021;113:70–84. doi: 10.32074/1591-951X-213.
- Jo V.Y., Doyle L.A. Refinements in Sarcoma Classification in the Current 2013 World Health Organization Classification of Tumours of Soft Tissue and Bone. Surg Oncol Clin N Am. 2016;25(4):621–643. doi: 10.1016/j.soc.2016.05.001. Epub 2016 Jul 30. PMID: 27591490.
- Semenova A.I. Ewing’s sarcoma and peripheral primitive neuroectodermal tumors (clinic, diagnosis, treatment). Practical oncology. 2005;6(4):234–239. Russian (Семенова А.И. Саркома Юинга и периферические примитивные нейроэктодермальные опухоли (клиника, диагностика, лечение). Практическая онкология. 2005;6(4):234–239).
- Ravi V., Patel S., Benjamin R.S. Chemotherapy for Soft-Tissue Sarcomas. Oncology (Williston Park). 2015;29(1):43–50.
- Saerens M, Brusselaers N, Rottey S, Decruyenaere A, Creytens D, Lapeire L. Immune checkpoint inhibitors in treatment soft tissue sarcoma: A systematic review and meta-analysis. Eur J Cancer. 2021;152:165–182. doi: 10.1016/j.ejca.2021.04.034.
- Aliyev M.D. Bohyan B.Yu. Burov D.A. The prognostic role of the resection margin in surgery of soft tissue sarcomas. Sarcomas of bones, soft tissues and skin tumors. 2013;(1):60–66. Russian (Алиев М.Д. Бохян Б.Ю. Буров Д.А Прогностическая роль края резекции в хирургии сарком мягких тканей. Саркомы костей, мягких тканей и опухоли кожи. 2013;(1):60–66).
- Ellinger J., Bastian P.J., Hauser S., Biermann K., Müller S.C. Primitive neuroectodermal tumor: rare, highly aggressive differential diagnosis in urologic malignancies. Urology. 2006;68(2):257–262. doi: 10.1016/j.urology.2006.02.037.
- Krüger S., Schmidt H., Kausch I., Böhle A., et al. Primitive Neuroectodermal Tumor (PNET) of the Urinary Bladder. Pathol Res Pract. 2003;199(11):751–754. doi: 10.1078/0344-0338-00492.
- Celli R., Cai G. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity. Arch Pathol Lab Med. 2016;140(3):281–285. doi: 10.5858/arpa.2014-0367-RS.
- Sivaramakrishna B., Mundada O.P., Aron M., Aron M., Vijayaraghavan M. Primary primitive neuroectodermal tumor (PNET) of the kidney with venous thrombus. Int Urol Nephrol. 2003;35(3):311–312. doi: 10.1023/b:urol.0000022921.65697.96.
- Kim M.S., Kim B., Park C.S., Song S.Y., Lee E.J., Park N.H., Kim H.S., Kim S.H. Radiologic findings of peripheral primitive neuroectodermal tumor arising in the retroperitoneum. Am J Roentgenol. 2006;186(4):1125–1132. doi: 10.2214/AJR.04.1688.
- Liu Z., Wang X., Lu Y., Chen L., Lu Y. Primary Ewing sarcoma/primitive neuroectodermal tumor of the renal pelvis: a case report. World J Surg Oncol. 2014;(12):293. doi: 10.1186/1477-7819-12-293.
- Charny C.K., Glick R.D., Genega E.M., Meyers P.A., Reuter V.E., La Quaglia M.P. Ewing’s sarcoma/primitive neuroectodermal tumor of the ureter: a case report and review of the literature. J Pediatr Surg. 2000;35(9):1356–1358. doi: 10.1053/jpsu.2000.9333.
- Liu Y., Xu B. Primary Peripheral Primitive Neuroectodermal Tumor of the Prostate on 18F-DCFPyL PET/CT. Clin Nucl Med. 2020;45(5):249–251. doi: 10.1097/RLU.0000000000002992.
Arquivos suplementares
![](/img/style/loading.gif)