Cancer UrologyCancer UrologyОнкоурология1726-97761996-1812Publishing House ABV Press173210.17650/1726-9776-2024-20-1-116-133CLINICAL NOTESКЛИНИЧЕСКИЕ СЛУЧАИUndifferentiated pleomorphic prostate sarcoma: a rare clinical case and systematic literature reviewНедифференцированная плеоморфная саркома предстательной железы: редкий клинический случай и систематический обзор данных литературыhttps://orcid.org/0000-0001-9466-7567SorokinN. I.СорокинН. И.
Russian Federation

1 Leninskie Gory, Moscow 119991; Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234

119991 Москва, Ленинские горы, 1; 119234 Москва, Ломоносовский пр-кт, 27, корп. 10

https://orcid.org/0000-0001-7558-589XDzitievV. K.ДзитиевВ. К.
Russian Federation

Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234

119234 Москва, Ломоносовский пр-кт, 27, корп. 10

https://orcid.org/0000-0002-6375-8164KadrevA. V.КадревА. В.
Russian Federation

Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234; Build. 1, 2/1 Barrikadnaya St., Moscow 125993

119234 Москва, Ломоносовский пр-кт, 27, корп. 10; 125993 Москва, ул. Баррикадная, 2/1, стр. 1

https://orcid.org/0000-0001-7848-6707DanilovaN. V.ДаниловаН. В.
Russian Federation

1 Leninskie Gory, Moscow 119991; Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234

119991 Москва, Ленинские горы, 1; 119234 Москва, Ломоносовский пр-кт, 27, корп. 10

https://orcid.org/0000-0003-3355-4547NesterovaO. Yu.НестероваО. Ю.
Russian Federation

Ol’ga Yu. Nesterova.

1 Leninskie Gory, Moscow 119991; Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234

Нестерова Ольга Юрьевна.

119991 Москва, Ленинские горы, 1; 119234 Москва, Ломоносовский пр-кт, 27, корп. 10

oy.nesterova@gmail.comhttps://orcid.org/0000-0003-4518-634XStrigunovA. A.СтригуновА. А.
Russian Federation

1 Leninskie Gory, Moscow 119991; Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234

119991 Москва, Ленинские горы, 1; 119234 Москва, Ломоносовский пр-кт, 27, корп. 10

https://orcid.org/0000-0002-0512-3152TsiguraD. A.ЦигураД. А.
Russian Federation

1 Leninskie Gory, Moscow 119991

119991 Москва, Ленинские горы, 1

https://orcid.org/0000-0002-0161-6072Afanas’evskayaE. V.АфанасьевскаяЕ. В.
Russian Federation

1 Leninskie Gory, Moscow 119991; Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234; 83 Uritskogo St., Istra, Moscow Region 143500

119991 Москва, Ленинские горы, 1; 119234 Москва, Ломоносовский пр-кт, 27, корп. 10; 143500 Московская область, Истра, ул. Урицкого, 83

https://orcid.org/0000-0002-6359-0998GallyamovE. A.ГаллямовЭ. А.
Russian Federation

Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234

119234 Москва, Ломоносовский пр-кт, 27, корп. 10

https://orcid.org/0000-0003-4251-7545KamalovA. A.КамаловА. А.
Russian Federation

1 Leninskie Gory, Moscow 119991; Build. 10, 27 Lomonosovskiy Prospekt, Moscow 119234

119991 Москва, Ленинские горы, 1; 119234 Москва, Ломоносовский пр-кт, 27, корп. 10

Lomonosov Moscow State UniversityФГБОУ ВО «Московский государственный университет им. М.В. Ломоносова»Medical Scientific and Educational Center, Lomonosov Moscow State UniversityМедицинский научно-образовательный центр ФГБОУ ВО «Московский государственный университет им. М.В. Ломоносова»Russian Medical Academy of Continuing Professional Education, Ministry of Health of RussiaФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава РоссииIstra Clinical HospitalГБУЗ МО «Истринская клиническая больница»180520242011161332510202306022024https://oncourology.abvpress.ru/oncur/article/view/1732

Undifferentiated pleomorphic prostate sarcoma is an extremely rare neoplasm. In the available literature, less than 50 clinical cases were described. Usually, the clinical manifestation of the disease is nonspecific and is primarily associated with bladder outlet obstruction. Additionally, most patients have normal levels of prostate-specific antigen, while ultrasound and MRI can visualize irregularly shaped lesions, often extending beyond the prostate gland. Diagnosis requires exclusion of other signs of specific differentiation, apart from fibroblastic and myofibroblastic. There is no established treatment plan for patients with pleomorphic prostate sarcoma due to extreme rarity of the disease. For localized forms of the disease, radical laparoscopic prostatectomy is often used, but more aggressive treatment options are also being considered, including total pelvic exenteration, chemotherapy and radiation therapy. However, in most cases metastasis is revealed after primary treatment. Regional metastases are often localized in the rectum, bladder, in the area of previous surgery, and regional lymph nodes, while distant metastases are found in the lungs, liver, bones and distant lymph nodes, thereby indicating aggressive progression and poor prognosis of the disease.

In this article, we present our own clinical observation of a patient with confirmed undifferentiated pleomorphic prostate sarcoma.

Недифференцированная плеоморфная саркома предстательной железы является крайне редким новообразованием, насчитывающим менее 50 случаев. Клиническая манифестация заболевания неспецифическая и связана с инфравезикальной обструкцией. Для подавляющего большинства пациентов характерен нормальный уровень простатического специфического антигена, в то время как при ультразвуковом исследовании и магнитно-резонансной томографии удается визуализировать очаговые образования неправильной формы, часто распространяющиеся за пределы предстательной железы. Данная опухоль является гистологическим диагнозом исключения. Постановка диагноза требует исключить другие признаки специфической дифференцировки, кроме фибробластической и миофибробластической. Алгоритм ведения пациентов с плеоморфной саркомой предстательной железы ввиду чрезвычайной редкости заболевания отсутствует. Чаще всего при локализованных формах прибегают к выполнению радикальной лапароскопической простатэктомии, однако рассматриваются и варианты более агрессивного лечения, включая тотальную экзентерацию малого таза, химио- и лучевую терапию. После первичного лечения в большинстве случаев отмечается метастазирование. Метастазы чаще всего локализуются в прямой кишке, мочевом пузыре, зоне предыдущей операции, регионарных лимфатических узлах, легких, печени, костях и отдаленных лимфатических узлах, указывая тем самым на агрессивное течение и неблагоприятный прогноз.

В настоящей статье представлено собственное клиническое наблюдение пациента с подтвержденным диагнозом недифференцированной плеоморфной саркомы предстательной железы.

prostate sarcomaundifferentiated pleomorphic prostate sarcomaradical prostatectomyсаркома предстательной железынедифференцированная плеоморфная саркома предстательной железырадикальная простатэктомияCancer Today. Available at: https://gco.iarc.fr/today/home.Ball M.W., Sundi D., Reese A.C. et al. Multimodal therapy in the treatment of prostate sarcoma: the Johns Hopkins experience. Clin Genitourin Cancer 2015;13(5):435—40. DOI: 10.1016/j.clgc.2015.04.011Yang B., Yuan J., Yu S. et al. Case report of primary mesenchymal sarcoma of the prostate and thoughts on treatment. Asian J Surg 2023;46(11):4934-5. DOI: 10.1016/j.asjsur.2023.06.008Urinary and Male Genital Tumours. WHO Classification of Tumours. 5th edn. Vol. 8.Wang L., Wang L., Hou D. et al. Benefits from standalone durvalu-mab treatment in an elderly patient with advanced prostatic sarcoma: a case report. Translat Androl Urol 2021;10(11):4298-303. DOI: 10.21037/тау-21-125Lee K., Song J.S., Kim J.E. et al. The clinical outcomes of undifferentiated pleomorphic sarcoma (UPS): a single-centre experience of two decades with the assessment of PD-L1 expressions. Eur J Surg Oncol 2020;46(7):1287-93. DOI: 10.1016/j.ejso.2020.02.029Wang Z., Xiao Y., Zhang B. et al. Ultrasonographic diagnosis of primary hepatic undifferentiated pleomorphic sarcoma. J Clin Ultrasound 2023;51(1):169-76. DOI: 10.1002/jcu.23293Kilitci A., Yilmaz F., Kilicgun H. et al. A case of primary undifferentiated pleomorphic sarcoma of the lung. J Coll Physicians Surg Pak 2021;31(11):1380-1. DOI: 10.29271/jcpsp.2021.11.1380Yoshida M., Seo M., Watanabe T. et al. A case of cardiac undifferentiated pleomorphic sarcoma with right ventricular outflow tract obstruction. Int Heart J 2023;64(4):779-82. DOI: 10.1536/ihj.23-037Saneesh P.S., Verma P., Bano S., Hemanth G.R.K. CT of primary undifferentiated pleomorphic sarcoma of small intestine with distant metastases presenting with intussusception. Radiol Case Rep 2020; 15(1):44-8. DOI: 10.1016/j.radcr.2019.10.015Sexton W.J., Lance R.E., Reyes A.O. et al. Adult prostate sarcoma: the M. D. Anderson Cancer Center Experience. J Urol 2001;166(2):521-5. DOI: 10.1016/s0022-5347(05)65974-5Ding B., Zhang Y., Hu W. et al. Adult primary prostate sarcoma: a multi-center cohort study and comparison between Chinese and American cases. Asian J Surg 2021;44(1):247-53. DOI: 10.1016/j.asjsur.2020.06.004Abbas S.M., Hill A.G. Prostatic sarcoma after treatment of rectal cancer. World J Surg Oncol 2007;5:82. DOI: 10.1186/1477-7819-5-82Ueda S., Okada K., Kanzawa M. et al. A case of prostate stromal sarcoma involving the rectum. J Surg Case Rep 2020;2020(6): rjaa165. DOI: 10.1093/jscr/rjaa165.Wickramasinghe S., Beenen E., He M. Is it really an abscess? An unusual case of metastatic stromal cell sarcoma of the prostate. Int J Surg Case Rep 2015;17:82-4. DOI: 10.1016/j.ijscr.2015.10.043Cavaliere E., Alaggio R., Castagnetti M. et al. Prostatic stromal sarcoma in an adolescent: the role of chemotherapy. Rare Tumors 2014;6(4):5607. DOI: 10.4081/rt.2014.5607Addesso M., Caputo A., Zeppa P. et al. Prostatic stromal sarcoma: report of a rare case in a young male and review of the literature. Int J Surg Case Rep 2023;106:108193. DOI: 10.1016/j.ijscr.2023.108193Beddouch A., Ameur A., al Bouzidi A. et al. Undifferentiated sarcoma of the prostate: report of a case. Ann Urol 2000;34(1):49-52.De Bari B., Stish B., Ball M.W. et al. Adult prostatic sarcoma: a contemporary multicenter Rare Cancer Network study. Prostate 2017;77(10):1160-6. DOI: 10.1002/pros.23375Mukouyama H., Sugaya K., Ogawa Y. et al. Poorly differentiated sarcoma of the prostate causing obstructive acute renal failure: a case report. Int J Urol 1999;6(12):615-9. DOI: 10.1046/j.1442-2042.1999.00123.xJaouani L., Zaimi A., Al Jarroudi O. et al. Undifferentiated sarcoma: a rare tumor of the prostate. Cureus 2023;15(6):e41056. DOI: 10.7759/cureus.41056Ehat N.T., Chuong L., Dickman J. et al. Prostate sarcoma. IJU Case Rep 2023;6(3):165-8. DOI: 10.1002/iju5.12578Iwahashi Y., Matsumura N., Kusumoto H. et al. Undifferentiated pleomorphic sarcoma of the prostate in a young man. IJU Case Rep 2020;3(4):145-8. DOI: 10.1002/iju5.12174Mathew B., Agrawal A., Purandare N.C. et al. Incidental detection of pleomorphic sarcoma on 68Ga-PSMA PET/CT in a patient with prostate cancer. Clin Nucl Med 2020;45(2):e120-1. DOI: 10.1097/RLU.0000000000002760Shen M.Q., Pan H., Wang S. et al. Prostate sarcoma: a retrospective analysis of 26 cases. Zhonghua Nan Ke Xue 2018;24(11):983-6.Wang X., Liu L., Tang H. et al. Twenty-five cases of adult prostate sarcoma treated at a high-volume institution from 1989 to 2009. Urology 2013;82(1):160-5. DOI: 10.1016/j.urology.2013.01.034Chen J., Quan C., Chang J. et al. Undifferentiated prostate sarcoma with cartilage metaplasia: a case report and review of the literature. Zhonghua Nan Ke Xue 2011;17(10):918-22.Janet N.L., May A.W., Akins R.S. Sarcoma of the prostate: a single institutional review. Am J Clin Oncol 2009;32(1):27-9. DOI: 10.1097/COC.0b013e31817b6061Stilgenbauer R., Benedict M., Bamshad R. et al. Sarcoma of the prostate: sonographic findings and pathologic correlation. J Ultrasound Med 2007;26(12):1789-93. DOI: 10.7863/jum.2007.26.12.1789Wakabayashi K., Konishi K., Komatsu T. et al. Radiation-induced sarcoma after radiation therapy for prostate adenocarcinoma. IJU Case Rep 2019;2(2): 98-101. DOI: 10.1002/iju5.12052Prevost J.B., Bossi A., Sciot R. et al. Post-irradiation sarcoma after external beam radiation therapy for localized adenocarcinoma of the prostate. Tumori 2004;90(6):618-21. DOI: 10.1177/030089160409000615Canfield S.E., Gans T.H., Unger P. et al. Postradiation prostatic sarcoma: de novo carcinogenesis or dedifferentiation of prostatic adenocarcinoma? Tech Urol 2001;7(4):294-5.McKenzie M., MacLennan I., Kostashuk E. et al. Postirradiation sarcoma after external beam radiation therapy for localized adenocarcinoma of the prostate: report of three cases. Urology 1999;53(6):1228. DOI: 10.1016/s0090-4295(98)00477-4Joerger M., Oehlschlegel C., Cerny T. et al. Postradiation highgrade myofibroblastic sarcoma of the prostate - a rare entity of prostatic tumors - responding to liposomal Doxorubicin. Onkologie 2002;25(6):558-61. DOI: 10.1159/000068628Thota V., Joseph K., Konduru S. et al. Synchronous development of prostate sarcoma and squamous cell carcinoma following radiotherapy. Proc (Bayl Univ Med Cent) 2022;35(2):243–4. DOI: 10.1080/08998280.2021.1994909Nghiem H.V., Sommer F.G., Moretto J.C. MRI of radiation-induced prostate sarcoma. Clin Imaging 1995;19(1):54–6. DOI: 10.1016/0899-7071(94)00017-7Atherton P.J., Stockdale A.D., Rennie C.D. Sarcoma of the prostate treated with radiotherapy. Clin Oncol 1994;6(4):269–70. DOI: 10.1016/s0936-6555(05)80299-7Froehner M., Bartholdt E., Meye A. et al. Adult prostate sarcoma diagnosed from tissue spontaneously excreted through the urethra. Urol Oncol 2004;22(2):119–20. DOI: 10.1016/S1078-1439(03)00120-0Xu W., Dong H., Ru G. et al. ARID1A deficient undifferentiated spindle cell and rhabdoid sarcoma of the prostate: report of a unique case with emphasis on diagnostic implications. Diagn Pathol 2022;17(1):22. DOI: 10.1186/s13000-022-01198-4Rojas-Jiménez A., Otero-Garcia M., Mateos-Martin A. Stromal prostatic sarcoma: a rare tumor with rare clinical and imaging presentation. J Radiol Case Rep 2013;7(7):24–31. DOI: 10.3941/jrcr.v7i7.1177Yang W., Liu A., Wu J. et al. Prostatic stromal sarcoma: a case report and literature review. Medicine 2018;97(18):e0495. DOI: 10.1097/MD.0000000000010495Wang K.F., Wu B., Zhang Y. Adult prostate sarcoma: a report of 6 cases with clinical analysis. Zhonghua Nan Ke Xue 2007;13(7):617–9.Ren F., Lu J., Wang J. et al. Adult prostate sarcoma: radiological-clinical correlation. Clin Radiol 2009;64(2):171–7. DOI: 10.1016/j.crad.2008.07.013Ohashi M., Shiraishi T., Fujihara A. et al. Detection of relatively poor but definitive blood supply in prostate stromal sarcoma using transrectal ultrasonography with superb microvascular imaging. Int Cancer conf J 2022;11(3):215–8. DOI: 10.1007/s13691-022-00552-zWu G., Sun R., Hong H. et al. Contrast-enhanced ultrasound diagnosis of prostatic sarcoma: two case reports. Medicine 2021;100(2):e24038. DOI: 10.1097/MD.0000000000024038Guo L.H., Xu H.X., Fu H.J. et al. Contrast-enhanced ultrasound aids in the detection of prostate rhabdomyosarcoma: a case report and literature review. Oncol Lett 2015;10(3):1541–4. DOI: 10.3892/ol.2015.3449Sidhu P.S., Cantisani V., Dietrich C.F. et al. The EFSUMB guidelines and recommendations for the clinical practice of Contrast-Enhanced Ultrasound (CEUS) in non-hepatic applications: update 2017 (long version). Ultraschall Med 2018;39(2):e2–44. DOI: 10.1055/a-0586-1107Kadrev A.V., Mitkova M.D., Kamalov A.A. et al. Multiparametric ultrasound diagnosis of prostate cancer. Ultrazvukovaya i funktsionalnaya diagnostika = Ultrasound and Functional Diagnostics 2021;(3): 37–70. (In Russ.). DOI: 10.24835/1607-0771-2021-3-37-70Barr R.G., Cosgrove D., Brock M. et al. WFUMB guidelines and recommendations on the clinical use of ultrasound elastography: part 5. Prostate. Ultrasound Med Biol 2017;43(1):27–48. DOI: 10.1016/j.ultrasmedbio.2016.06.020Anbarasan T., Wei C., Bamber J.C. et al. Characterisation of prostate lesions using transrectal Shear Wave Elastography (SWE) ultrasound imaging: a systematic review. Cancers 2021;13(1):122. DOI: 10.3390/cancers13010122Kamalov A.A., Kadrev A.V., Mitkova M.D. et al. The use of targeted biopsy under shear wave elastography control in the diagnosis of prostate cancer. Urologia = Urology 2020;(6):106–13. (In Russ.). DOI: 10.18565/urology.2020.6.106-113Kadrev A.V., Mitkova M.D., Kamalov D.M. et al. Targeted elastometry (shear wave elastography) in the diagnosis of prostate cancer (preliminary results). Ultrazvukovaya i funktsionalnaya diagnostika = Ultrasound and Functional Diagnostics 2019;(1):17–29. (In Russ.). DOI: 10.24835/1607-0771-2019-1-17-29Bhargava R., Shia J., Hummer A.J. et al. Distinction of endometrial stromal sarcomas from “hemangiopericytomatous” tumors using a panel of immunohistochemical stains. Modern Pathol 2005;18(1):40–7. DOI: 10.1038/modpathol.3800248Henry M., Britton C., Coco C. et al. Stromal sarcoma of the prostate. Can J Urol 2019;26(1):9683–5.Busto Martín L., Carral Freire M., Hermida T. et al. Prostate sarcoma: report of 2 cases and bibliographic review. Arch Esp Urol 2014;67(8):699–704.Lai R., Ding C. 18F-FDG PET/CT imaging in an adolescent patient with primary prostatic stromal sarcoma. Clin Nucl Med 2019;44(1):45–7. DOI: 10.1097/RLU.0000000000002372