Publicado

2015-01-01

Tumores germinales gonadales en niños: experiencia de 20 años en un centro de referencia pediátrico.

Gonadal germ cell tumors in children: experience of 20 years in a pediatric referral center

Palabras clave:

Germinales, Gonadales, Niños, Recurrencia, Marcadores biológicos tumorales, Gonadectomia (es)
Germ cells, Gonadal, Children, Recurrence, Biological tumor markers, Gonadectomy (en)

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Autores/as

  • Daniel Andres Bautista Moreno Departamento de Pediatría, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia.
  • Michael Ariza-Varon Departamento de Pediatría, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia.
  • Derly Lorena Medina-Vega Departamento de Pediatría, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia.
  • Fabio Restrepo-Angel Departamento de Pediatría, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia. Fundación Hospital de la Misericordia. Bogotá, D.C., Colombia.
  • Adriana Linares-Ballesteros Departamento de Pediatría, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia. Fundación Hospital de la Misericordia. Bogotá, D. C., Colombia.
  • Lina Eugenia Jaramillo Departamento de Patología, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia. Fundación Hospital de la Misericordia. Bogotá, D. C., Colombia.
  • Mizrahinn Mendez Departamento de Cirugía, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia. Fundación Hospital de la Misericordia. Bogotá, D. C., Colombia.
  • Ivan Dario Molina-Ramirez Departamento de Cirugía, Facultad de Medicina, Universidad Nacional de Colombia. Bogotá, D. C., Colombia. Fundación Hospital de la Misericordia. Bogotá, D. C., Colombia.
  • Isabel Cristina Sarmiento-Urbina Fundación Hospital de la Misericordia. Bogotá, D. C., Colombia.

Antecedentes. Los tumores de células germinales son un grupo heterogéneo de neoplasias que corresponden al 1-3% de los tumores en pediatría. Por lo general, se manifiestan clínicamente con masa testicular o dolor abdominal.

Objetivo. Describir las características de los pacientes con tumores germinales gonadales en un centro de referencia de cáncer pediátrico en Colombia.

Materiales y métodos. Este artículo hace una revisión retrospectiva recopilando 20 años de experiencia en el diagnóstico y manejo de estos tumores en la Fundación Hospital de la Misericordia.

Resultados. Se encontraron 79 pacientes, 35 hombres y 44 mujeres, con un promedio de edad, al diagnóstico, de 6 años. El teratoma fue la neoplasia más frecuente en el sexo femenino y el tumor del seno endodérmico en el masculino. El 70,9% de los pacientes se diagnosticaron en estadio I; se tienen datos de marcadores tumorales en 84,8%. El 98,7% se manejaron con cirugía y 58,2% recibieron además quimioterapia, especialmente protocolo BEP. 96% alcanzaron remisión de la enfermedad, 3 pacientes fallecieron.

Conclusiones. El estudio demuestra buenos resultados en el manejo de los tumores germinales gonadales con el protocolo establecido de manejo quirúrgico y quimioterapia, se encontró una baja tasa de recurrencia en el periodo evaluado y alto porcentaje de niños libres de enfermedad.

Background. Germ cell tumors are a heterogeneous group of neoplasms corresponding to 1-3% of pediatric tumors. They usually manifest clinically as testicular mass or abdominal pain.

Objective. To make a description of gonadal germ cell tumor types in pediatric referral center in Colombia.

Materials and methods. This article takes a retrospective review compiling 20 years of experience in diagnosis and management of these tumors in the Fundación Hospital de La Misericordia.

Results. We found 79 patients, 35 men and 44 women, with an average age at diagnosis of 6 years. The teratoma was the most common neoplasia in females and endodermal sinus tumor in men. 70.9% of patients were diagnosed with stage I. In 84,8% measuring tumor markers were obtained. 98.7% were managed with surgery and 58.2% also received chemotherapy, especially BEP protocol. 96% achieved disease remission, 3 patients died.

Conclusions. The study shows good results in the management of gonadal germ cell tumors with the established protocol and low recurrence rate in the evaluated period and high rates of children free of disease, were found a low rate of recurrence in the assessment period and high percentage of free of disease.

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Citas

Schneider DT, Calaminus G, Kock S, Teske C, Schmidt P, Haas RJ, et al. Epidemiologic Analysis of 1,442 children and adolescents registered in the German Germ Cell Tumor Protocols. Pediatr Blood Canc [Internet]. 2004 [cited 2014 jan 21];42(2):169-75. Available from: http://goo.gl/kB3IZg.

Al Jama FE, Al Ghami AA, Gasim T, Al Dakhiel S, Rahman J, Rahmen M. Ovarian Tumors in Children and Adolescents - A Clinical Study of 52 Patients in University Hospital. J Pediatr Adolesc Gynecol [Internet]. 2011 [cited 2015 jan 21];24(1):25-8. doi: http://doi.org/bdfztt.

Bujons A, Caffaratti J, Pascual M, Angerri O, Garat JM, Villavicencio H. [Testicular tumours in infancy and children]. Actas Urol Esp [Internet]. 2011 [cited 2015 jan 21];35(2):93-8. Spanish. doi: http://doi.org/f2jd6c.

Taskinen S, Fagerholm R, Aronniemi J, Rintala R, Taskinen M. Testicular tumors in children and adolescents [Internet]. J Pediatr Urol. 2008 [cited 2014 jan 21];4(2):134-7. doi: http://doi.org/fmqsw9.

Quero-Hernandez A, Hernandez-Arrioja J, Socorro Z, Perez A. Tumores de ovario en niñas y adolescentes, en un hospital general [Internet]. Rev Mex Pediatr. 2005 [cited 2014 jan 21];72(4):174-8. Spanish. Available from: http://goo.gl/0sThJ0.

Kumar Y, Nitin S. Extracranial malignant germ cell tumors. Indian J Pediatr [Internet]. 2012 [cited 2015 jan 21];79(7):928-35. doi: http://doi.org/zk9.

Rodríguez Y, Godoy J. Tumor de células germinales. Revista Med [Internet]. 2008 [cited 2015 jan 21];16(2):200-14. Spanish. doi: http://goo.gl/mLv6Z1.

Ulbright TM. Germ cell tumors of gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues. Mod Pathol [Internet]. 2005 [cited 2015 jan 21];18 Suppl 2:S61-79. doi: http://doi.org/b9jrgp.

Ibrahim IM, Khalil el-SA, El-Baradie TS, Shaalan MA, Shalaby LM, Nassif SL, et al. The role of surgical management in pediatric germ cell tumors (GCTs), NCI case series. J Egypt Natl Canc Inst [Internet]. 2008 [cited 2015 jan 21];20(1):70-9. Available from: http://goo.gl/33SKdB.

Cools M, Stoop H, Kersemaekers AM, Drop SL, Wolffenbuttel KP, Bourguignon JP, et al. Gonadoblastoma Arising in Undifferentiated Gonadal Tissue within Dysgenetic Gonads. J Clin Endocrinol Metab [Internet]. 2006 [cited 2015 jan 21];91(6):2404-13. doi: http://doi.org/bjkjzz.

Looijenga LH, Hersmus R, de Leeuw B, Stoop H, Cools H, Oosterhuis JW, et al. Gonadal tumours and DSD. Best Pract Res Clin Endocrinol Metab [Internet]. 2010 [cited 2015 jan 21];24(2):291-310. doi: http://doi.org/cvbbsf.

Baranzelli MC, Kramar A, Bouffet E, Quintana E, Rubie H, Edan C, et al. Prognostic factors in children with localized malignant non-seminomatous germ cell tumors. J Clin Oncol. 1999. 17(4):1212-9. PubMed PMID: 10561181.

Ablin AR, Krailo MD, Ramsay NK, Malogolowkin MH, Isaacs H, Raney RB, et al. Results of treatment of malignant germ cell tumors in 93 children: A report from the Children's Cancer Study Group. J Clin Oncol. 1991;9(10):1782-92. PubMed PMID: 1717667.

Horton Z, Schlater M, Schultz S. Pediatric germ celll tumors. Surg Oncol [Internet]. 2007 [cited 2015 jan 21];16(3):205-13. doi: http://doi.org/ck5fjp.

Lim FK, Chanrachakul B, Chong SM, Ratnam SS. Malignant Ovarian Germ Cell Tumours: Experience in the National University Hospital in Singapure. Ann Acad Med Singapure [Internet]. 1998 [cited 2015 jan 21];27(5):657-61. Available from: http://goo.gl/YdulC2.

Muñoz M, Sims MI, Latorre JJ, Beresi V, Quintana J, del Pozo H. Tumores ováricos malignos de las células germinales en la infancia y adolescencia, experiencia de 14 años. Rev Chil Pediatr [Internet]. 1986 [cited 2015 jan 21];57(3):261-6. Spanish. doi: http://doi.org/c86vps.

Nogueira FB, Petrilli AS, Macedo C, Carab EM. Testicular Tumors in Children and Adolescents. J Pediatr (Rio J) [Internet]. 2011 [cited 2015 jan 21];88(1):87-92. doi: http://doi.org/zmb.

Albers P, Albrecht W, Algaba F, Bokemeyer C, Cohn-Cedermark G, Fizazi K, et al. Guidelines on Testicular Cancer [Internet]. Europe: European Association of Urology; 2010 [cited 2015 jan 21]. Available from: http://goo.gl/Gvwajm.

Wang X, Xu S, Tang D, Li M, Wu D, Huang Y. Prepubertal testicular and paratesticular tumors in China: a single-center experience over 10 years period. J Pediatr Surg [Internet]. 2012 [cited 2015 jan 21];47(8):1576-80. doi: http://doi.org/zmc.

Murphy FL, Law H, Mushtaq I. Testicular and paratesticular pathology in infants and children: the histopatological experience of a tertiary paediatric unit over a 17 year period. Pediatr Surg Int [Internet]. 2007 [cited 2015 jan 21];23(9):867-72. doi: http://doi.org/b3d9tc.

Schneider DT, Schuster AE, Fritsch MK, Hu J, Olson T, Lauer S, et al. Multipoint imprinting analysis indicates a common precursor cell for gonadal and non-gonadal pediatric germ cell tumors. Cancer Res [Internet]. 2001 [cited 2015 jan 21];61(19):7268-76. Available from: http://goo.gl/K5NDuj.

Bussey KJ, Lawce HJ, Himoe E, Shu XO, Heerema NA, Perlman EJ, et al. SNRPN methylation patterns in germ cell tumors as a reflection of primordial germ cell development. Genes Chromosomes Cancer [Internet]. 2001 [cited 2015 jan 21];32(4):342-52. doi: 10.1002/gcc.1199.

Gonzalo E, Merino I, Fdez-Tejeiro A, Astigarraga A, Navajas A. Tumor ovárico en la infancia: a propósito de una revisión sistemática. An Esp Pediatr [Internet]. 1998 [cited 2015 jan 21];49(5):491-4. Spanish. Available from: http://goo.gl/0r2OZ1.

Kim MJ, Young NY, Lee DY, Yoon BK, Choi D. Clinical characteristics of ovarian teratoma: age-focused retrospective analysis of 580 cases. Am J Obstet Gynecol [Internet]. 2011 [cited 2015 jan 21];205(1):32.e1-4. doi: http://doi.org/fnx6kz.

Vaysse C, Delsol M, Carfagna L, Bouali O, Combelles S, Lemasson F. Ovarian germ cell tumors in children. Management, survival and ovarian prognosis. A report of 75 cases. J Pediat Surg [Internet]. 2010 [cited 2015 jan 21];45(7):1484-90. doi: http://doi.org/d9zbtt.

Yeap ST, Hsiao CC, Hsieh CS, Yu HR, Chen YC, Chuang JH, et al. Pediatric malignant ovarian tumors: 15 years of experience at a single institution [Internet]. Pediatr Neonatol. 2011 [cited 2015 jan 21];52(3):140-4. doi: http://doi.org/d3vb2f.

Ahmed HU, Arya M, Muneer A, Mushtag I, Sebire NJ. Testicular and paratesticular tumours in the prepubertal population. Lancet Oncol [Internet]. 2010 [cited 2015 jan 21];11(5):476-83. doi: http://doi.org/fk4mhs.

Martinez M, Alfonso P, Gonzalez P, Santos MS, Perera A, Orteega M, et al. Tumores Germinales Gonadales en Niños. Experiencia en el S.A. Hospital de Niños. Rev Venez Oncol [Internet]. 1998 [cited 2015 jan 21];12(2):e1-15. Spanish. Available from: http://goo.gl/glf92l.

Quero-Hernández A, Estrada-Correa R, Tenorio-Rodríguez H, Álvares RM. [Malignant germ cell ovarian tumors: clinical characteristics, treatment and outcome]. Cir Cir [Internet]. 2007;75(2):81-5. Spanish. PubMed PMID: 1751-1902.

Escudero P, Bañuelos J, Quijano F, Labastida S, López D, Álvarez M, et al. Informe clínico de 90 casos de tumores germinales de ovario. Gamo [Internet]. 2005 [cited 2015 jan 21];4(49):97-101. Spanish. Available from: http://goo.gl/KndNyr.

Von Allmen D. Malignant lesions of the ovary in childhood. Semin Pediatr Surg [Internet]. 2005 [cited 2015 jan 21];14(2):100-5. Available from: http://doi.org/bx8swk.

Jiménez-y Felipe JH. Tumores Ováricos malignos y raros en Pediatría. Experiencia en el Hospital Infantil del Estado de Sonora. Rev Mex Cir Pediatr [Internet]. 2007 [cited 2015 jan 21];14(1):6-17. Spanish. Available from: http://goo.gl/S0B2bP.

Epelman M, Chikwava K, Chauvin N, Servaes S. Imaging of pediatric ovarian neoplasms. Pediatr Radiol [Internet]. 2011 [cited 2015 jan 21];41(9):1085-99. doi: http://doi.org/cvchkr.

Zalel Y, Piura B, Elchalal U. Diagnosis and management of malignant germ cell ovarian tumors in young females. Int J Ginecol Obstet [Internet]. 1996 [cited 2015 jan 21];55(1):1-10. doi: http://doi.org/d7dc7r.

Krepart G, Smith JP, Rutledge F, Delclos L. The treatment for gysgerminoma of the ovary. Cancer [Internet]. 1978 [cited 2015 jan 21];41(3):386-90. doi: http://doi.org/bm8s5q.

Teinturier C, Gelez J, Flamant F, Habrand JL, Lemerle J. Pure disgerminoma of the ovary in childhood: treatment results anssequelae. Med Pediat Oncol [Internet]. 1994 [cited 2015 jan 21];23(1):1-7. doi: http://doi.org/cqqk9v.

Williams SD, Blessing JA, Moore DH, Homesley HD, Adcock L. Cisplatin, vinblastine and bleomycin in advencer and recurrent ovarian germ-cell tumors. A trial of the Gynecologic Oncology group [Internet]. An Intern Med. 1989 [cited 2015 jan 21];111(1):44-66. doi: http://doi.org/zmd.

Parera LA, Torán N. Tumores gonadales genéticamente determinados [Internet]. España: Sociedad Española de Endicronologia Pediática. [Date unknown] [cited 2015 jan 21]. Available from: http://goo.gl/97fjAU.

Bosl GJ, Motzer RJ. Testicular Germ-Cell Cancer. N England J Med [Internet]. 1997 [cited 2015 jan 21];337(4):242-53. doi: http://doi.org/fcwgg4.

Kusumakumary P, Mathew B, Hariharan S, Privakumari T, Rajan B. Testicular germ cell tumors in prepubertal children. Pediatr Hematol Oncol [Internet]. 2000 [cited 2015 jan 21];17(1):105-11. doi: http://doi.org/cvxmk2.

Boujelbene N, Cosinschi A, Boujelbene N, Khanfir K, Bhagwati S, Herrmann E, et al. Pure seminoma: A review and update. Radiat Oncol [Internet]. 2011 [cited 2015 jan 21];90(6):1-12. doi: http://doi.org/ddjfx6.

Ehrlich Y, Beck S, Foster RS, Bihrle R, Einhorn LH. Serum tumors markers in testicular cancer. Semin Urol Oncol [Internet]. 2013 [cited 2015 jan 21];31(1):17-23. doi: http://doi.org/btk2mw.

Cicin I, Eralp Y, Saip P, Avan I, Kebudi R. Malignant Ovarian Germ Cell Tumors A Single-Institution Experience. Am J Clinic Oncol [Internet]. 2009 [cited 2015 jan 21];32(2):191-6. doi: http://doi.org/fvhb5f.

Chow S, Yang JH, Lin YH, Chen YP, Lai JP, Chen RJ, et al. Malignant ovarian germ cell tumors. Int J Gynaecol Obstet [Internet]. 1996 [cited 2015 jan 21];53(2):151-8. doi: http://doi.org/cq9tj9.

Ray-Coquard I. Ovarian germ-cell malignant tumors. Orphanet [Internet]. 2004 [cited 2015 jan 21];1-4. Available from: http://goo.gl/OVReNu.

Ueda M, Ueki M. Ovarian tumors associated with pregnancy. Int J Gineacol Obstet [Internet]. 1996 [cited 2015 jan 21];55(1):59-65. doi: http://doi.org/fp44tb.

Smith HO, Berwick M, Vershraegen CF, Wiggins C, Lansing L, Muller CY, et al. Incidence and survival rates for female malignant germ cell tumors. Obstet Ginecol [Internet]. 2006 [cited 2015 jan 21];107(5):1075-9. Doi: http://doi.org/b3k82t.

Wheeler CA, David S, Degefu S, Thorneycroft IH, O'Quinn AG. Ovarian Choriocarcinoma: a difficult diagnosis of an unusual tumors and review of the hook effect. Obstet Ginecol [Internet]. 1990 [cited 2015 jan 21];73(2):547-9.

Krag G, Barlebo H, Olsen J, Schultz HP, Starklint H, et al. Testicular germ cell tumours in Denmark 1976-1980. Patrology of 1058 consecutive cases. Acta Radiol Oncol [Internet]. 1984 [cited 2015 jan 21];23(4):239-47. doi: http://doi.org/b7bhd2.