Nadir Mesane Kanserleri ve Urakus Tümörleri
Özet
Mesane kanserleri genel olarak ürotelyal ve ürotelyal olmayan tipler olarak ikiye ayrılmakta olup, ürotelyal olmayan tümörler tüm vakaların %5'inden azını oluşturmaktadır. Bu nadir malignitelerin yaklaşık %90'ı epitel kökenli olan skuamöz hücreli karsinom, adenokarsinom ve küçük hücreli karsinomdan oluşurken; geri kalan kısmı sarkom, paraganglioma, lenfoma ve melanom gibi çok daha nadir görülen türler meydana getirmektedir. Genellikle ileri evrelerde teşhis edilen bu heterojen tümör grubu, ürotelyal kanserlere kıyasla daha agresif seyretmekte ve daha kötü bir prognoz taşımaktadır; ancak urakal tümörler istisnai olarak daha iyi bir klinik seyir gösterebilmektedir. Klasik başvuru semptomu ağrısız hematüri olan bu hastalıklarda tanı için sistoskopi altın standarttır. Lokalize hastalıklarda temel tedavi yöntemi radikal sistektomi iken, ileri evre ve metastatik vakalarda kemoterapi, radyoterapi ve multimodal protokoller uygulanmaktadır. Tümörlerin nadirliği ve randomize kontrollü çalışmaların eksikliği nedeniyle henüz standart bir tedavi algoritması bulunmamakta ve çok disiplinli, bireyselleştirilmiş yaklaşımlar önem kazanmaktadır.
Bladder cancers are broadly categorized into urothelial and non-urothelial types, with non-urothelial tumors accounting for less than 5% of all cases. Approximately 90% of these rare malignancies are of epithelial origin, including squamous cell carcinoma, adenocarcinoma, and small cell carcinoma, while the remainder comprises even rarer types such as sarcoma, paraganglioma, lymphoma, and melanoma. Usually diagnosed at advanced stages, this heterogeneous group of tumors carries a worse prognosis compared to urothelial cancers, although urachal tumors exceptionally exhibit a better clinical course. Painless hematuria is the classic presenting symptom, and cystoscopy remains the gold standard for diagnosis. While radical cystectomy is the primary treatment modality for localized disease, chemotherapy, radiotherapy, and multimodal protocols are utilized for advanced or metastatic stages. Due to the rarity of these tumors and the lack of randomized controlled trials, a standardized treatment algorithm is not yet established, necessitating aggressive and individualized therapeutic approaches.
Referanslar
UpToDate [Internet]. [cited 2022 Oct 13]. Available from: https://www.uptodate.com/contents/non-urothelial-bladdercancer?search=bladder%20squamous%20cell%20carcinoma&source=search_result&selectedTitle=1~16&usage_type=default&display_rank=1
Park S, Reuter VE, Hansel DE. Non-urothelial carcinomas of the bladder. Vol. 74, Histopathology. Blackwell Publishing Ltd; 2019. p. 97–111.
Cohen AJ, Packiam V, Nottingham C, Steinberg G, Smith ND, Patel S. Upstaging of nonurothelial histology in bladder cancer at the time of surgical treatment in the National Cancer Data Base. Urologic Oncology: Seminars and Original Investigations. 2017 Jan 1;35(1):34.e1-34.e8.
Alanee S, Alvarado-Cabrero I, Murugan P, Kumar R, Nepple KG, Paner GP, et al. Update of the International Consultation on Urological Diseases on bladder cancer 2018: non-urothelial cancers of the urinary bladder. World J Urol. 2019 Jan 23;37(1):107–14.
Ravi K, Kumar T, Bakshi H, Desai J, Sen S, Yadav V. Non Urothelial Bladder Cancers: A Case Series. Vol. 4, Indian Journal of Surgical Oncology. Springer; 2013. p. 2–8.
Dahm P, Gschwend JE. Malignant Non-Urothelial Neoplasms of the Urinary Bladder: A Review. Vol. 44, European Urology. Elsevier; 2003. p. 672–81.
Welk B, McIntyre A, Teasell R, Potter P, Loh E. Bladder cancer in individuals with spinal cord injuries. Vol. 51, Spinal Cord. 2013. p. 516–21.
Gadalla HAH, Kamel NAH, Badary FAM, Elanany FG. Expression of CD44 protein in bilharzial and non-bilharzial bladder cancers.
Swanson DA, Liles A, Zagars GK. Preoperative irradiation and radical cystectomy for stages T2 and T3 squamous cell carcinoma of the bladder. Journal of Urology. 1990;143(1):37–40.
H Rundle JS, L Hart AJ, McGEORGE A, Smith JS, Smith PM. Squamous Cell Carcinoma of Bladder. A Review of 114 Patients. Vol. 54, British Association of Urological Surgeons. 1982.
Richie JP, Waisman J, Skinner DG, Dretler SP. Squamous carcinoma of the bladder: treatment by radical cystectomy. Journal of Urology. 1976;115(6):670–2.
Baumann BC, Zaghloul MS, Sargos P, Murthy V. Adjuvant and Neoadjuvant Radiation Therapy for Locally Advanced Bladder Cancer. Clin Oncol. 2021 Jun 1;33(6):391–9.
Serretta V, Pomara G, Piazza F, Gange E. Pure Squamous Cell Carcinoma of the Bladder in Western Countries. Eur Urol. 2000;37(1):85–9.
Scosyrev E, Yao J, Messing E. Urothelial Carcinoma Versus Squamous Cell Carcinoma of Bladder: Is Survival Different With Stage Adjustment? Urology. 2009 Apr;73(4):822–7.
James ND, Hussain SA, Hall E, Jenkins P, Tremlett J, Rawlings C, et al. Radiotherapy with or without Chemotherapy in Muscle-Invasive Bladder Cancer. New England Journal of Medicine. 2012 Apr 19;366(16):1477–88.
Swanson DA, Liles A, Zagars GK. Preoperative Irradiation and Radical Cystectomy for Stages T2 and T3 Squamous Cell Carcinoma of the Bladder. Journal of Urology. 1990 Jan;143(1):37–40.
Bellmunt J, de Wit R, Vaughn DJ, Fradet Y, Lee JL, Fong L, et al. Pembrolizumab as Second-Line Therapy for Advanced Urothelial Carcinoma. New England Journal of Medicine. 2017 Mar 16;376(11):1015–26.
Hussain M, Vaishampayan U, Du W, Redman B, Smith DC. Combination Paclitaxel, Carboplatin, and Gemcitabine Is an Active Treatment for Advanced Urothelial Cancer. Journal of Clinical Oncology. 2001 May 1;19(9):2527–33.
Navon JD, Soliman H, Khonsari F, Ahlering T. Screening cystoscopy and survival of spinal cord injured patients with squamous cell cancer of the bladder. J Urol. 1997 Jun;157(6):2109–11.
Ghoneim MA, Abdel-Latif M, El-Mekresh M, Abol-Enein H, Mosbah A, Ashamallah A, et al. Radical Cystectomy for Carcinoma of the Bladder: 2,720 Consecutive Cases 5 Years Later. Journal of Urology. 2008 Jul;180(1):121–7.
el Mawla NG, el Bolkainy MN, Khaled HM. Bladder cancer in Africa: Update. Semin Oncol. 2001 Apr;28(2):asonc0280174.
Mostafa MH, Sheweita SA, O’Connor PJ. Relationship between Schistosomiasis and Bladder Cancer. Clin Microbiol Rev. 1999 Jan;12(1):97–111.
Grignon DJ, Ro JY, Ayala AG, Johnson DE, Ordóñez NG. Primary adenocarcinoma of the urinary bladder. A clinicopathologic analysis of 72 cases. Cancer. 1991 Apr 15;67(8):2165–72.
Riva G, Mian C, Luchini C, Girolami I, Ghimenton C, Cima L, et al. Urachal carcinoma: from gross specimen to morphologic, immunohistochemical, and molecular analysis. Virchows Archiv. 2019 Jan 9;474(1):13–20.
EL-Mekresh, EL-Baz, Abol-Enein, Ghoneim. Primary adenocarcinoma of the urinary bladder: BJU Int. 1998 Aug;82(2):206–12.
Bladder adenocarcinoma: 31 reported cases - PubMed [Internet]. [cited 2022 Oct 13]. Available from: https://pubmed.ncbi.nlm.nih.gov/11718635/
Yanagihara Y, Tanji N, Miura N, Shirato A, Nishimura K, Fukumoto T, et al. Modified FOLFOX6 Chemotherapy in Patients with Metastatic Urachal Cancer. Chemotherapy. 2013;59(6):402–6.
Pagliaro LC, Williams DL, Daliani D, Williams MB, Osai W, Kincaid M, et al. Neoadjuvant Paclitaxel, Ifosfamide, and Cisplatin Chemotherapy for Metastatic Penile Cancer: A Phase II Study. Journal of Clinical Oncology. 2010 Aug 20;28(24):3851–7.
Oluyadi F, Ramachandran P, Gotlieb V. A Rare Case of Advanced Urethral Diverticular Adenocarcinoma and a Review of Treatment Modalities. J Investig Med High Impact Case Rep. 2019 Jan 9;7:232470961982840.
Tsironis G, Bamias A. Treating bladder adenocarcinoma. Transl Androl Urol. 2018 Dec;7(S6):S699–701.
Vincendeau S, de Lajarte-Thirouard AS, Bensalah K, Guillé F, Lobel B, Patard JJ. [Neuroendocrine differentiation of bladder tumors]. Prog Urol. 2003 Jun;13(3):375–84.
Sehgal SS, Wein AJ, Bing Z, Malkowicz SB, Guzzo TJ. Neuroendocrine Tumor of the Bladder. Rev Urol. 2010;12(4):197–201.
Mazzucchelli R, Morichetti D, Lopez-Beltran A, Cheng L, Scarpelli M, Kirkali Z, et al. Neuroendocrine tumours of the urinary system and male genital organs: clinical significance. BJU Int. 2009 Jun;103(11):1464–70.
Mazzucchelli R, Morichetti D, Lopez-Beltran A, Cheng L, Scarpelli M, Kirkali Z, et al. Neuroendocrine tumours of the urinary system and male genital organs: clinical significance. BJU Int. 2009 Jun;103(11):1464–70.
Siefker-Radtke AO, Kamat AM, Grossman HB, Williams DL, Qiao W, Thall PF, et al. Phase II Clinical Trial of Neoadjuvant Alternating Doublet Chemotherapy With Ifosfamide/Doxorubicin and Etoposide/Cisplatin in Small-Cell Urothelial Cancer. Journal of Clinical Oncology. 2009 Jun 1;27(16):2592–7.
Kouba EJ, Cheng L. Understanding the Genetic Landscape of Small Cell Carcinoma of the Urinary Bladder and Implications for Diagnosis, Prognosis, and Treatment. JAMA Oncol. 2017 Nov 1;3(11):1570.
Akamatsu S, Kanamaru S, Ishihara M, Sano T, Soeda A, Hashimoto K. Primary large cell neuroendocrine carcinoma of the urinary bladder. International Journal of Urology. 2008 Dec;15(12):1080–3.
Peterson RO, Sesterhenn IA, Davis CJ, editors. Urologic pathology. 3rd ed. Philadelphia: Lippincott Williams & Wilkins; pp. 245–9.
Eble JN, Grignon DJ, Young RH. Tumors of the urinary bladder and urethra. In: Fletcher CDM, editor. Diagnostic histopathology of tumors. 4th ed. Philadelphia: Elsevier Saunders; 2013. pp. 610–37.
Spiess PE, Kassouf W, Steinberg JR, Tuziak T, Hernandez M, Tibbs RF, et al. Review of the M.D. Anderson experience in the treatment of bladder sarcoma. Urologic Oncology: Seminars and Original Investigations. 2007 Jan;25(1):38–45.
Russo P, Brady MS, Conlon K, Hajdu SI, Fair WR, Herr HW, et al. Adult Urological Sarcoma. Journal of Urology. 1992 Apr;147(4):1032–6.
Parekh DJ, Jung C, O’Conner J, Dutta S, Smith ER. Leiomyosarcoma in urinary bladder after cyclophosphamide therapy for retinoblastoma and review of bladder sarcomas. Urology. 2002 Jul;60(1):164.
Nawar NA, Olsen J, Jelic TM, He C. Primary Urinary Bladder Angiosarcoma with Osteoclast-Like Multinucleated Giant Cells: A Case Report and Literature Review. American Journal of Case Reports. 2016 Mar 7;17:143–9.
Daga G, Kerkar P. Sarcomatoid Carcinoma of Urinary Bladder: a Case Report. Indian J Surg Oncol. 2018 Dec 16;9(4):644–6.
Priyadarshi V, Pal D. Paraganglioma of urinary bladder. Urol Ann. 2015;7(3):402.
Leestma JE, Price EB. Paraganglioma of the urinary bladder. Cancer. 1971;28(4):1063–73.
Al-Zahrani AA. Recurrent urinary bladder paraganglioma. Adv Urol. 2010;
Bejrananda T, Sawasdee A, Boonchai S, Tanthanuch M. Primary Malignant Melanoma of the Bladder: A Rare Case Report in Asia and Review of the Literature. Res Rep Urol. 2021;13:833–9.
Karabulut YY, Erdogan S, Sayar H, Ergen A, Ertoy Baydar D. Primary malignant melanoma of the urinary bladder: clinical, morphological, and molecular analysis of five cases. Melanoma Res. 2016;26(6):616–24.
Bates AW. Malignant lymphoma of the urinary bladder: a clinicopathological study of 11 cases. J Clin Pathol. 2000 Jun 1;53(6):458–61.
Kempton CL, Kurtin PJ, Inwards DJ, Wollan P, Bostwick DG. Malignant Lymphoma of the Bladder: Evidence From 36 Cases That Low-Grade Lymphoma of the MALT-Type is the Most Common Primary Bladder Lymphoma. Am J Surg Pathol. 1997 Nov;21(11):1324–33.
Al-Maghrabi J, Kamel-Reid S, Jewett M, Gospodarowicz M, Wells W, Banerjee D. Primary Low-Grade B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue Type Arising in the Urinary Bladder. Arch Pathol Lab Med. 2001 Mar 1;125(3):332–6.
Leite KRM, Bruschini H, Camara-Lopes LH. Primary lymphoma of the bladder. International braz j urol [Internet]. 2004 [cited 2022 Oct 15];30(1):37–9. Available from: http://www.scielo.br/j/ibju/a/Gd9HBLbx6JnCCnmstZ33qKL/?lang=en
Schniederjan SD, Osunkoya AO. Lymphoid neoplasms of the urinary tract and male genital organs: a clinicopathological study of 40 cases. Modern Pathology. 2009 Aug 17;22(8):1057–65.
Mayer EK, Beckley I, Winkler MH. Lymphoepithelioma-like carcinoma of the urinary bladder—diagnostic and clinical implications. Nat Clin Pract Urol. 2007 Mar;4(3):167–71.
Porcaro AB, Gilioli E, Migliorini F, Antoniolli SZ, Iannucci A, Comunale L. Primary lymphoepithelioma-like carcinoma of the urinary bladder: report of one case with review and update of the literature after a pooled analysis of 43 patients. Int Urol Nephrol. 2003;35(1):99–106.
