Nadir Testis Tümörleri
Özet
Bu çalışma, testis kanserlerinin %3-5'ini oluşturan ve germ hücreli olmayan nadir testis tümörlerinin histopatolojik özelliklerini ve tedavi yaklaşımlarını incelemektedir. En sık görülen grup olan seks kord-stromal tümörler; Leydig, Sertoli ve granüloza hücreli tümörler olarak alt tiplere ayrılır. Leydig hücreli tümörler sıklıkla benign karakterde olup çocuklarda izoseksüel psödoprecocity, yetişkinlerde ise ağrısız kitle veya jinekomasti ile sunum gösterir. Sertoli hücreli tümörler östradiol üretimine bağlı erektil disfonksiyona yol açabilirken, granüloza hücreli tümörlerin jüvenil tipi bebeklerde en sık görülen iyi huylu testis kitlesidir. Bu tümörlerin malignite tanısı kesin olarak metastaz varlığıyla konur ve kemo/radyorezistan olmaları sebebiyle birincil tedavi yaklaşımı radikal inguinal orşiektomidir. Yüksek riskli vakalarda cerrahiye retroperitoneal lenf nodu diseksiyonu (RPLND) eklenmektedir. Çalışmada ayrıca, 60 yaş üstü erkeklerde en sık görülen bilateral testis neoplazmı olan agresif karakterdeki primer testiküler lenfomalar (PTL), liposarkom ve rabdomiyosarkom gibi testis sarkomları ile literatürde 100'den az vaka bildirilen nadir rete testis adenokarsinomları ele alınmıştır. Sonuç olarak, bu nadir tümörlerin standart bir tedavi algoritmasının olmaması, hasta yönetimini zorlaştırmakta ve geniş kapsamlı kılavuzlara olan ihtiyacı artırmaktadır.
This study examines the histopathological characteristics and therapeutic approaches of rare non-germ cell testicular tumors, which constitute 3-5% of all testicular malignancies. The most prevalent subgroup, sex cord-stromal tumors, is categorized into Leydig, Sertoli, and granulosa cell tumors. Leydig cell tumors are mostly benign, presenting with isosexual pseudoprecocity in children and painless masses or gynecomastia in adults. Sertoli cell tumors may cause erectile dysfunction due to estradiol production, whereas the juvenile type of granulosa cell tumors is the most common benign testicular mass in infants. The definitive indicator of malignancy in these types is the presence of metastasis; because they are chemo- and radioresistant, radical inguinal orchiectomy remains the primary treatment. Retroperitoneal lymph node dissection (RPLND) is performed additionally in high-risk patients. The study also reviews aggressive primary testicular lymphomas (PTL), which are the most common bilateral testicular neoplasms in men over 60, testicular sarcomas such as liposarcoma and rhabdomyosarcoma, and rare adenocarcinoma of the rete testis with fewer than 100 cases reported. Consequently, the lack of standardized treatment algorithms for these rare tumors complicates patient management, emphasizing the critical need for comprehensive guidelines.
Referanslar
Gaylis FD, August C, Yeldandi A, et al. Granulosa cell tumor of the adult testis: ultrastructural and ultrasonographic characteristics. J Urol 1989 ve 141:126–127.
Eble JNSG, Epstein JL, Sesterhenn IA. Pathology and genetics: tumours of the urinary system and male genital organs. In: Eble JNSG, Epstein JL, Sesterhenn IA, editors. World Health Organization classification of tumours. Lyon: International Agency for. Research on Cancer Press; 2004. pp. 252-255.
Mukhopadhyay M, Das C, Sarkar S, et al. Leydig cell tumor of testis in a child: an uncommon presentation. J Indian Assoc Pediatr Surg 2017 ve 22:181–183.
Soria JC, Durdux C, Chretien Y, et al. Malignant Leydig cell tumor of the testis associated with Klinefelter’s syndrome. Anticancer Res 1999 ve 19:4491–4494.
Carvajal-Carmona LG, Alam NA, Pollard PJ, et al. Adult leydig cell tumors of the testis caused by germline fumarate hydratase mutations. J Clin Endocrinol Metab 2006 ve 91:3071–3075.
Mooney KL, Kao CS. A contemporary review of common adult nongerm cell tumors of the testis and paratestis. Surg Pathol Clin 2018 ve 11:739–758. This is a contemporary review of the diagnosis, features, and management of nongerm-cell tumors of the testis in. adults.
Banerji JS, Odem-Davis K, Wolff EM, et al. Patterns of care and survival outcomes for malignant sex cord stromal testicular cancer: results from the national cancer data base. J Urol 2016 ve 196:1117–1122.
Kern SQ, Speir RW, Akgul M, et al. Rare benign and malignant testicular lesions: histopathology and management. Curr Opin Urol. 2020 Mar ve 31922967, 30(2):235-244. doi: 10.1097/MOU.0000000000000715. PMID:.
Calaway AC, Tachibana I, Masterson TA, et al. Oncologic outcomes following surgical management of clinical stage II sex cord stromal tumors. Urology 2019 ve 127:74–79.
Ohr J. Tumors of the testis, adnexa, spermatic cord, and scrotum. Arch Pathol Lab Med 2000 ve 124:1855.
Mosharafa AA, Foster RS, Bihrle R, et al. Does retroperitoneal lymph node dissection have a curative role for patients with sex cord-stromal testicular tumors? Cancer 2003 ve 98:753–757.
Scully RE. Ovarian tumors. A review. Am J Pathol. 1977 ve 87:686–720.
Shukla AR, Huff DS, Canning DA, et al. Juvenile granulosa cell tumor of the testis: contemporary clinical management and pathological diagnosis. J Urol 2004 ve 171:1900–1902.
Al-Bozom IA, El-Faqih SR, Hassan SH, et al. Granulosa cell tumor of the adult type: a case report and review of the literature of a very rare testicular tumor. Arch Pathol Lab Med 2000 ve 124:1525–1528.
Featherstone JM, Fernando HS, Theaker JM, et al. Sex cord stromal testicular tumors: a clinical series--uniformly stage I disease. J Urol. 2009 May;181(5):2090-6; discussion 2096. doi: 10.1016/j.juro.2009.01.038. Epub 2009 Mar 14. PMID: 19286222
Rove KO, Maroni PD, Cost CR, et al. Pathologic Risk Factors for Metastatic Disease in Postpubertal Patients With Clinical Stage I Testicular Stromal Tumors. Urology. 2016 Nov;97:138-144. doi: 10.1016/j.urology.2016.06.066. Epub 2016 Aug 15. PMID: 27538802
Acar C, Gurocak S, Sozen S. Current treatment of testicular sex cord-stromal tumors: critical review. Urology. 2009 Jun;73(6):1165-71. doi: 10.1016/j.urology.2008.10.036. Epub 2009 Apr 10. PMID: 19362328
Møller MB, d’Amore F, Christensen BE. Testicularlymphoma: a population-based study of incidence, clinicopathological correlations and prognosis. The Danish Lymphoma Study Group, LYFO. Eur JCancer.1994 ve 30A(12):1760-1764.
Vitolo U, Ferreri AJ, Zucca E. Primary testicularlymphoma.Crit Rev Oncol Hematol.2008 ve 65(2):183-189.
Moorjani V, Mashankar A, Goel S, et al. Sonographic appearanceof primary testicular lymphoma.AJR Am JRoentgenol.1991 ve 157(6):1225-1226.26.
Srisuwan T, Muttarak M, Kitirattrakarn P, Ya-in C.Clinics in diagnostic imaging (134). Testicularlymphoma.Singapore Med J.2011 ve 52(3):204-208.
Tsili AC, Argyropoulou MI, Giannakis D, et al. Primary diffuse large B-celltesticular lymphoma: magnetic resonanceimaging findings. Andrologia.2012 ve 44(Suppl1):845-847.
Ponti G, Ponzoni M, Ferreri AJ, et al. The impact ofhistopathologic diagnosis on the propermanagement of testis neoplasms.Nat Clin PractOncol.2008 ve 5(10):619-622.
Menter T, Ernst M, Drachneris J, et al. Phenotypeprofiling of primary testicular diffuse large B-celllymphomas [published online ahead of printAugust 16, 2013].Hematol Oncol.doi:10.1002/hon.2090.
Gundrum JD, Mathiason MA, Moore DB, et al. Primary testicular diffuse large B-cell lymphoma:a population-based study on the incidence,natural history, and survival comparison withprimary nodal counterpart before and after theintroduction of rituximab. J Clin Oncol. 2009;27(31):5227-5232.
Buskirk SJ, Evans RG, Banks PM, et al. Primary lymphoma of the testis.Int JRadiat Oncol Biol Phys.1982 ve 8(10):1699-1703, 64.
Tondini C, Ferreri AJ, Siracusano L, et al. Diffuselarge-cell lymphoma of the testis.J Clin Oncol.1999 ve 17(9):2854-2858.
Mazloom A, Fowler N, Medeiros LJ, et al. Outcome of patients withdiffuse large B-cell lymphoma of the testis by eraof treatment: the M. D. Anderson Cancer Centerexperience.Leuk Lymphoma.2010 ve 51(7):1217-1224.
Rypens F, Garel L, Franc-Guimond J, et al. Paratesticular rhabdomyosarcoma presenting as thickening of the tunica vaginalis. Pediatr Radiol 2009 ve 39:1010–1012.
Goldberg H, Wong LM, Dickson B, et al. Long-term oncological outcomes of patients with paratesticular sarcoma. BJU Int 2019 ve 124:801–810. Provides one of the largest retrospective cohorts to date in the literature, reviews multimodal therapy at a median. follow-up of 11 years.
Rodriguez D, Olumi AF. Management of spermatic cord tumors: a rare urologic malignancy. Ther Adv Urol 2012 ve 4:325–334.
Moroni M, Nesi G, Travaglini F, et al. Rhabdomyosarcoma of the spermatic cord. A case report with review of the literature. Urol Int 2003 ve 71:114–117.
Ahmed HU, Arya M, Muneer A, et al. Testicular and paratesticular tumours in the prepubertal population. Lancet Oncol 2010 ve 11:476–483.
Nochomovitz LE, Orenstein JM. Adenocarcinoma of the rete testis. Case report, ultrastructural observations, and clinicopathologic correlates. Am J Surg Pathol 1984 ve 8:625–634.
Maganty A, Fombona A, Bandari J, et al. Aggressive surgical management of adenocarcinoma of the rete testis. Urol Case Rep 2018 ve 16:72–74.
Sanchez-Chapado M, Angulo JC, Haas GP. Adenocarcinoma of the rete testis. Urology 1995 ve 46:468–475.
