Pediatrik Olgularda Testis Tümörlerine Yaklaşım
Özet
Çocukluk çağında nadir görülen testis tümörleri, yetişkinlere kıyasla daha iyi huylu ve az agresif bir seyir izler; 2 yaş civarı ve puberte olmak üzere iki pik dönemi mevcuttur. Saptanan kitlelerin büyük kısmı iyi huylu olup, primer tümörler çoğunlukla germ hücre kaynaklıdır. Germ hücreli tümörler arasında en sık Endodermal Sinüs Tümörü görülürken, stromal kaynaklı olanlar puberte prekoks bulgularıyla ortaya çıkabilir. Etiyolojide inmemiş testis, aile öyküsü, perinatal faktörler ve genetik anomaliler suçlanmaktadır. Tanıda yüksek duyarlılığa sahip ultrasonografi ile AFP, β-hCG ve LDH gibi tümör belirteçlerinden yararlanılır; evreleme için Bilgisayarlı Tomografi tercih edilir. Tedavi algoritmasında, pubertal dönemde ve malignensi durumlarında altın standart yüksek inguinal radikal orşiektomi iken, prepubertal çocuklarda fertilitenin korunması amacıyla konservatif yaklaşım ve testis koruyucu cerrahi her zaman ön planda tutulmalıdır. Operasyon sırasında şüpheli durumlarda frozen biyopsi yol göstericidir. Tedavi sonrası özellikle ilk yıllarda nükslerin takibi için klinik, radyolojik ve biyokimyasal izlem büyük önem taşır.
Testicular tumors, which are rare in childhood, follow a more benign and less aggressive course compared to adults, with two peak periods around the age of 2 and puberty. Most detected masses are benign, and primary tumors are mostly of germ cell origin. While Endodermal Sinus Tumor is the most common among germ cell tumors, stromal tumors may present with symptoms of precocious puberty. Undescended testis, family history, perinatal factors, and genetic abnormalities are implicated in the etiology. Diagnosis utilizes highly sensitive ultrasonography and tumor markers such as AFP, β-hCG, and LDH; Computed Tomography is preferred for staging. In the treatment algorithm, while high inguinal radical orchiectomy is the gold standard during the pubertal period and in cases of malignancy, conservative approaches and testis-sparing surgery should always be prioritized in prepubertal children to preserve fertility. Frozen biopsy during operation guides management in suspicious cases. Post-treatment clinical, radiological, and biochemical follow-up is of great importance for monitoring recurrences, especially in the first years.
Referanslar
Horner MJ, Ries LAG, Krapcho M, et al. SEER Cancer Statistics Review, 1975–2005. U.S. National Institutes of Health, National Cancer Institute 2008 http://seer.cancer.gov/csr/1975_2005
Balcı Ö, Karaman A, Şahin G, et al. Çocuklarda testis tümörleri: Tek merkez deneyimi. Türkiye Çocuk Hast Derg 2015;3:167-70.
3.Tal R, Holland R, Belenky A, et al. Incidental Testicular Tumors in Infertile Men. 2004; 82(2) 469–71.
Hannah J, Nicholas GC, Amanda FS. Testicular tumors in the pediatric patient Seminars in Pediatric SurgeryVolume 30, Issue 4, August 2021, 151079
Walsh TJ, Davies BJ, Croughan MS, et al. Racial differences among boys with testicular germ cell tumors in the United States. J Urol 2008; 179: pp. 1961-1965.
Ghazarian AA, McGlynn KA. Increasing incidence of testicular germ cell tumors among racial/ethnic minorities in the United States. Cancer Epidemiol Biomark Prev. 2020 Published online May 8. doi:10.1158/1055-9965.EPI-20-0107
Başaklar AC. (2006 ). Skrotal Kitleler. A.Can Başaklar, (Ed.), Bebek ve Çocukları Cerrahi ve Ürolojik Hastalıkları. 1. baskı. (s:1765-86). Ankara: Palme Yayıncılık).
Dieckmann KP, Pichlmeier U. Clinical epidemiology of testicular germ cell tumours. World J Urol 2004;22:2-14
Çiftçi AO, Koloğlu MB, Şenocak ME, et al. Testicular Tumors in Children. Journal of Pediatric Surgery. 2001; 36(12): 1796-1801.
McGlynn KA, Trabert B. Adolescent and adult risk factors for testicular cancer. Nat Rev Urol 2012;9(6):339-49.
Manecksha RP, Fitzpatrick JM. Epidemiology of testicular cancer. BJU Int 2009;104:1329-33.
Walsh TJ, Dall’Era MA, Croughan MS, et al. Prepubertal orchiopexy for cryptorchidism may be associated with lower risk of testicular cancer. J Urol 2007;178:1440-6
Pettersson A, Richiardi L, Nordenskjold A, et al. Age at surgery for undescended testis and risk of testicular cancer. N Engl J Med 2007;356:1835-41.
Richiardi L, Akre O, Bellocco R, et al. Perinatal determinants of germ-cell testicular cancer in relation to histological subtypes. Br J Cancer 2002; 87: pp. 545-550.
McGlynn KA, Cook MB. Etiologic factors in testicular germ cell tumors. Future Oncol 2009;5(9): 1389-402.
Ritchey ML, Shamberger RC. (2011). Pediatric urologic oncology. In: Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA, editors.Campbell-Walsh urology. 10th (Ed). Philadelphia: Saunders;.p. 3696-730.
Zahn S, Sievers S, Alemazkour K, et al. Imbalances of chromosome arm 1p in pediatric and adult germ cell tumors are caused by true allelic loss: a combined comparative genomic hybridization and micro- satellite analysis. Genes Chromosomes Cancer 2006;45:995-1006.
Bussey KJ, Lawce HJ, Olson SB, et al. Chromosome abnormalities of eighty-one pediatric germ cell tumors: sex-, age-, site-, and histopathology-related differences-a Children’s Cancer Group study. Genes Chromoso- mes Cancer 1999;25:134-146.
Cortes D, Thorup J, Petersen BL. Testicular neoplasia in undescended testes of cryptorchid boys-does surgical strategy have an impact on the risk of invasive testicular neoplasia? Turk J Pediatr 2004;46 Suppl:35-42.
Luker GD, Siegel MJ. Pediatric testicular tumors: Evaluation with gray-scale and color Doppler US. Radiology 1994;191:561-4.
Agarwal PK, Palmer JS. Testicular and paratesticular neoplasms in prepubertal males. J Urol 2006; 1176: pp. 875-881.
Sangüesa C, Veiga D, Llavador M, et al. Testicular tumours in children: an approach to diagnosis and management with pathologic correlation. Insights Imaging. 2020;11(1):74. doi:10.1186/s13244-020-00867-6.
Langer JE, Ramchandani P, Siegelman ES, et al. Epidermoid cysts of the testicle: sonographic and MR imaging features. AJR Am J Roentgenol 1999; 173: pp. 1295-1299.
Blohm MEG, Vesterling-Horner D, Calaminus G, et al. Alpha1-fetoprotein (AFP) reference values in infants up to 2 years of age. Pediatr Hematol Oncol. 1998;15:135–142.
Grantham EC, Caldwell BT, Cost NG. Current urologic care for testicular germ cell tumors in pediatric and adolescent patients. Urol Oncol. 2016;34(2):65–75. doi:10.1016/j.urolonc.2015.06.008.
Ross JH, Rybicki L, Kay R. Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the prepubertal testis tumor registry. J Urol. 2002;168(2):1675–1678. doi:10. 1097/01.ju.0000030749.27823.f5.
Wiener ES, Lawrence W, Hays D, et al. Retroperitoneal node biopsy in paratesticular rhabdomyosarcoma. J Pediatr Surg 1994;29:171-177.
Cecchetto G. Gonadal germ cell tumors in children and adolescents. J Indian Assos Pediatr Surg 2014;19:189-94.
Saltzman AF, Cost NG. Adolescent and young adult testicular germ cell tumors: special considerations. Adv Urol. 2018;2018. doi:10.1155/2018/2375176.
Stokes W, Amini A, Maroni PD, et al. Patterns of care and survival outcomes for adolescent and young adult patients with testicular seminoma in the United States: a national cancer database analysis. J Pediatr Urol. 2017;13(4):386 e1- 386.e7. doi:10.1016/j.jpurol.2016.12.009
Zampila I, Koyle MA. Pediatric testicular tumors. Fundamentals of pediatric surgery. Springer New York.2011. pp 749-753.
Albers P, Albrecht W, Algaba F, et al. EAU guidelines on testicular cancer: 2011 update. Eur Urol 2011;60:304-319.
Zuniga A, Lawrentschuk N, Jewett MA. Organ sparing approaches for testicular masses. Nat Rev Urol 2010;7:454-464.
Chung JM, Lee SD. Overview of Pediatric Testicular Tumors in Korea. Korean J Urol 2014;55:789-796.
Rogers PC, Olson TA, Cullen JW, et al. Treatment of children and adolescents with stage II testicular and stages I and II ovarian malignant germ cell tumors: A Pediatric Intergroup Study--Pediatric Oncology Group 9048 and Children's Cancer Group 8891. J Clin Oncol 2004;22:3563-9.
Cushing B, Giller R, Cullen JW, et al. Randomized comparison of combination chemotherapy with etoposide, bleomycin, and either high-dose or standard-dose cisplatin in children and adolescents with high-risk malignant germ cell tumors: a pediatric intergroup study-Pediatric Oncology Group 9049 and Children's Cancer Group 8882. J Clin Oncol 2004;22:2691-700
Mann JR, Pearson D, Barrett A, et al. Results of the United Kingdom Children’s Cancer Study Group’s malignant germ cell tumor studies. Cancer (1989) 63(9):1657–67.
Metcalfe PD, Farivar-Mohseni H, Farhat W, et al. Pediatric testicular tumors: contemporary incidence and efficacy of testicular preserving surgery. J Urol 2003;170(6 Pt 1):2412-5.
Shukla AR, Woodard C, Carr MC, et al. Experience with testis sparing surgery for testicular teratoma. J Urol 2004;171:161-3.
De Backer A, Madern GC, Pieters R, et al. Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors. Eur J Pediatr Surg 2008;18:1-6.
Hasegawa T, Maeda K, Kamata N, et al. A case of immature teratoma originating in intra-abdominal undescended testis in a 3-month-old infant. Pediatr Surg Int 2006;22:570-2
Ross JH. Testicular tumors. In: Wilcox D, Godbole P, Cooper C, editors. Pediatric urology book [monograph on the Internet]. [cited 2014 Jul 3]. Available from: http://www.pediatricurologybook.com/tumors.html
J.S. Valla for the Group D'Etude en Urologie Pédiatrique. Testis-sparing surgery for benign testicular tumors in children. J Urol 2001;165(6 Pt 2):2280-3
Pohl HG, Shukla AR, Metcalf PD, et al. Prepubertal testis tumors: actual prevalence rate of histological types. J Urol 2004;172(6 Pt 1):2370-2.
Langer JE, Ramchandani P, Siegelman ES, et al. Epidermoid cysts of the testicle: sonographic and MR imaging features. AJR Am J Roentgenol 1999;173(5):1295–9. 4.
Metcalfe PD, Farivar-Mohseni H, Farhat W, et al. Pediatric testicular tumors: contemporary incidence and efficacy of testicular preserving surgery. J Urol 2003;170(6 Pt 1):2412–5.
Shukla AR, Huff DS, Canning DA, et al. Juvenile granulosa cell tumor of the testis: contemporary clinical management and pathological diagnosis. J Urol 2004;171:1900-2.
Urban MD, Lee PA, Plotnick LP, et al. The diagnosis of leydig cell tumors in childhood. Am J Dis Child 1978;132:494-497.
Mengel W, Knorr D. Leydig cell tumours in childhood. Prog Pediatr Surg 1983;16:133-138.
Wegner HE, Dieckmann KP, Herbst H, et al. Leydig cell tumor: comparison of results of radical and testis-sparing surgery in a single center. Urol Int 1997;59:170-3.
Brosman SA. Testicular tumors in prepubertal children. Urology 1979;13:581-8.
Schultz KAP, Schneider DT, Pashankar F, et al. Management of ovarian and testicular sex cord-stromal tumors in children and adolescents. J Pediatr Hematol Oncol. 2012;34. https://journals.lww.com/jphoonline/Fulltext/2012/ 05001/Management_of_Ovarian_and_Testicular_Sex.7.aspx
Romo Muñoz MI, Núñez Cerezo V, Dore Reyes M, et al. Testicular tumours in children: Indications for testis-sparing surgery. An Pediatr Engl Ed. 2018;88(5):253–258. doi:10.1016/j.anpede.2017.05.009
Dudani R, Giordano L, Sultania P, et al. Juvenile granulosa cell tumor of testis: case report and review of literature. Am J Perinatol 2008;25:229-31
Manuel M, Katayama PK, Jones HW Jr. The age of occurrence of gonadal tumors in intersex patients with a Y chromosome. Am J Obstet Gynecol 1976;124:293-300.
Cools M, Drop SL, Wolffenbuttel KP, et al. Germ cell tumors in the intersex gonad: old paths, new directions, moving frontiers. Endocr Rev 2006;27:468-84.
Olsen MM, Caldamone AA, Jackson CL, et al. Gonadoblastoma in infancy: indications for early gonadectomy in 46XY gonadal dysgenesis. J Pediatr Surg 1988;23:270-1.
Askin FB, Land VJ, Sullivan MP, et al. Occult testicular leukemia: testicular biopsy at three years continuous complete remission of childhood leukemia: a Southwest Oncology Group Study. Cancer 1981; 47: pp. 470-475.
Trigg ME, Steinherz PG, Chappell R, et al. Early testicular biopsy in males with acute lymphoblastic leukemia: lack of impact on subsequent event-free survival. J Pediatr Hematol Oncol 2000; 22: pp. 27-33.
Wong KY, Ballard ET, Strayer FH et al. Clinical and occult testiscular leukemia in long-term survivors of acute lymphoblastic leukemia. J Pediatr 96: 569,1980.
Noh PH, Cooper CS, Snyder HM. Conservative management of cystic dysplasia of the testis. J Urol 1999;162:2145.
Eberli D, Gretener H, Dommann-Scherrer C, et al. Cystic dysplasia of the testis: a very rare paediatric tumor of the testis. Urol Int 2002;69:1-6.
Goede J, Hack WW, van der Voort-Doedens LM, et al. Prevalence of testicular microlithiasis in asymptomatic males 0 to 19 years old. J Urol 2009; 182: pp. 1516-1520.
Leenen AS, Riebel TW. Testicular microlithiasis in children: sonographic features and clinical implications. Pediatr Radiol 2002;32:575-9
Volokhina YV, Oyoyo UE, Miller JH: Ultrasound demonstration of testicular microlithiasis in pediatric patients: is there an association with testicular germ cell tumors?. Pediatr Radiol 2014; 44: pp. 50-55.
Trout AT, Chow J, McNamara ER, et al. Association between testicular microlithiasis and testicular neoplasia: large multicenter study in a pediatric population. Radiology 2017; 285: pp. 576-583.