Zusammenfassung
Die gonadalen Tumoren bei Kindern und Jugendlichen umfassen eine klinisch äußerst heterogene Gruppe von Tumoren. Sie zeigen ein charakteristisches Muster der Alters- und Geschlechtsverteilung in Bezug auf die verschiedenen histologischen Entitäten. Diese altersabhängigen Unterschiede korrelieren mit einer unterschiedlichen Tumorbiologie und Prognose. Die Diagnostik umfasst die Messung der Tumormarker AFP und β-HCG, eine endokrinologische Status sowie die radiologische Schnittbilddiagnostik. Die multimodale Therapie der malignen Tumoren erfolgt risikostratifiziert innerhalb aktueller kinderonkologischer Therapieoptimierungsstudien und -register. In niedrigen Stadien kann nach kompletter Resektion auf die adjuvante Chemotherapie verzichtet werden, allerdings nur, wenn ein konsequentes Nachsorgeschema eingehalten wird. Bei fortgeschrittenen Stadien erreicht die platin-basierte Chemotherapie die Kontrolle einer metastatischen Erkrankung. Ebenso kann sie bei großen Tumoren in einem neoadjuvanten Setting dem Erreichen einer kompletten Tumorresektion dienen.
Literatur
Ablin AR (1981) Malignant germ cell tumors in children. Front Radiat Ther Oncol 16:141–149
Ablin AR, Krailo MD, Ramsay NK et al (1991) Results of treatment of malignant germ cell tumors in 93 children: a report from the Childrens Cancer Study Group. J Clin Oncol 9:1782–1792
Bamberg M, Kortmann RD, Calaminus G et al (1999) Radiation therapy for intracranial germinoma: results of the German cooperative prospective trials MAKEI 83/86/89. J Clin Oncol 17:2585–2592
Baranzelli MC, Kramar A, Bouffet E et al (1999) Prognostic factors in children with localized malignant nonseminomatous germ cell tumors. J Clin Oncol 17:1212–1219
Blohm ME, Vesterling-Hörner D, Calaminus G et al (1998) Alpha 1-fetoprotein (AFP) reference values in infants up to 2 years of age. Pediatr Hematol Oncol 15:135–142
Calaminus G, Bamberg M, Jürgens H et al (2004) Impact of surgery, chemotherapy and irradiation on long term outcome of intracranial malignant non-germinomatous germ cell tumors: results of the German Cooperative Trial MAKEI 89. Klin Pädiatr 216:141–149
Cheng L, Roth LM, Zhang S et al (2011) KIT gene mutation and amplification in dysgerminoma of the ovary. Cancer 117:2096–2103
Cushing B, Giller R, Cullen JW et al (2004) 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 22:2691–2700
Ernestus K, Pietsch T, Gessler M et al (2006) Structure, use, and risks of biomaterial repositories of embryonal tumors. Klin Pädiatr 218:132–138
Frappaz D, Conter C (2007) Testicular neoplasms in children: lymphatic or metastatic involvement is rare. Rev Prat 57:368–369
Fritsch MK, Schneider DT, Schuster AE et al (2006) Activation of Wnt/beta-catenin signaling in distinct histologic subtypes of human germ cell tumors. Pediatr Dev Pathol 9:115–131
Flamant F, Schwartz L, Delons E, Caillaud JM, Hartmann O, Lemerle J (1984) Nonseminomatous malignant germ cell tumors in children: multidrug therapy in stages III and IV. Cancer 54:1687–1691
Fustino N, Rakheja D, Ateek CS et al (2011) Bone morphogenetic protein signalling activity distinguishes histological subsets of paediatric germ cell tumours. Int J Androl 34:e218–e233
Giannarini G, Dieckmann KP, Albers P et al (2010) Organ-sparing surgery for adult testicular tumours: a systematic review of the literature. Eur Urol 57:780–790
Göbel U, Gutjahr P, Jürgens H et al (1986) Cooperative therapy study of nontesticular germ cell tumors MAKEI 83 of the Society of Pediatric Oncology: analysis after 3 years. Klin Pädiatr 198:237–244
Göbel U, Bamberg M, Engert J et al (1991) Treatment of non-testicular germ cell tumors in children and adolescents with BEP and VIP: initial results of the MAKEI 89 therapy study. Klin Pädiatr 203:236–245
Göbel U, Calaminus G, Engert J et al (1998) Teratomas in infancy and childhood. Med Pediatr Oncol 31:8–15
Göbel U, Schneider DT, Calaminus G et al (2000) Germ-cell tumors in childhood and adolescence. Ann Oncol 11:263–271
Göbel U, Schneider DT, Calaminus G et al (2001) Multimodal treatment of malignant sacrococcygeal germ cell tumors: a prospective analysis of 66 patients of the German cooperative protocols MAKEI 83/86 and 89. J Clin Oncol 19:1943–1950
Göbel U, Calaminus G, Schneider DT et al (2004) Klinik, Symptomatik und Therapiestrategien bei Keimzelltumoren des Ovars. Gynäkologe 37:806–814
Haas RJ, Göbel U, Harms D et al (1989) Malignant testicular tumors in children. Concept of the cooperative therapy study MAHO 88 based on MAHO 82. Klin Pädiatr 201:269–274
Haas RJ, Schmidt P, Göbel U et al (1993) Therapy of testicular germ cell tumors. Current status of the MAHO studies. Klin Padiatr 205:225–230
Harms D, Jänig U (1986) Germ cell tumours of childhood. Report of 170 cases including 59 pure and partial yolk-sac tumours. Virchows Arch A Pathol Anat Histopathol 409:223–239
Hennes E, Zahn S, Lopes LF et al (2012) Molecular genetic analysis of bilateral ovarian germ cell tumors. Klin Pädiatr 224:359–365
Hersmus R, de Leeuw BH, Stoop H et al (2009) A novel SRY missense mutation affecting nuclear import in a 46,XY female patient with bilateral gonadoblastoma. Eur J Hum Genet 17:1642–1649
Hofmann M, Schlegel PG, Hippert F et al (2013) Testicular sex cord stromal tumors: analysis of patients from the MAKEI study. Pediatr Blood Cancer 60:1651–1655
Honecker F, Kersemaekers AM, Molier M et al (2004) Involvement of E-cadherin and beta-catenin in germ cell tumours and in normal male fetal germ cell development. J Pathol 204:167–174
Isurugi K, Aso Y, Ishida H et al (1977) Prepubertal XY gonadal dysgenesis. Pediatrics 59:569–573
Kaatsch P, Kaletsch U, Michaelis J. Annual report of the German Childhood Cancer Registry 2004. http://www.kinderkrebsregister.de. Zugegriffen am 02.05.2019
Looijenga LH, Verkerk AJ, Dekker MC et al (1998) Genomic imprinting in testicular germ cell tumours. APMIS 106:187–195
Lopes LF, Macedo CR, Pontes EM et al (2009) Cisplatin and etoposide in childhood germ cell tumor: brazilian pediatric oncology society protocol GCT-91. J Clin Oncol 27:1297–1303
Mann JR, Raafat F, Robinson K et al (1998) UKCCSG’s germ cell tumour (GCT) studies: improving outcome for children with malignant extracranial non-gonadal tumours – carboplatin, etoposide, and bleomycin are effective and less toxic than previous regimens Med Pediatr Oncol 30:217–227
Mann JR, Raafat F, Robinson K et al (2000) The United Kingdom Children’s Cancer Study Group’s second germ cell tumor study: carboplatin, etoposide, and bleomycin are effective treatment for children with malignant extracranial germ cell tumors, with acceptable toxicity. J Clin Oncol 18:3809–3818
Murray MJ, Saini HK, van Dongen S et al (2010) The two most common histological subtypes of malignant germ cell tumour are distinguished by global microRNA profiles, associated with differential transcription factor expression. Mol Cancer 9:290
Murray MJ, Halsall DJ, Hook CE et al (2011) Identification of microRNAs From the miR-371~373 and miR-302 clusters as potential serum biomarkers of malignant germ cell tumors. Am J Clin Pathol 135:119–125
Neumann JC, Lillard K, Damoulis V et al (2011) Zebrafish models of germ cell tumor. Methods Cell Biol 105:3–24
Nichols CR, Heerema NA, Palmer C et al (1987) Klinefelter’s syndrome associated with mediastinal germ cell neoplasms. J Clin Oncol 5:1290–1294
Okpanyi V, Schneider DT, Zahn S et al (2011) Analysis of the adenomatous polyposis coli (APC) gene in childhood and adolescent germ cell tumors. Pediatr Blood Cancer 56:384–391
Oosterhuis JW, Looijenga LH (2005) Testicular germ-cell tumours in a broader perspective. Nat Rev Cancer 5:210–222
Palmer RD, Barbosa-Morais NL, Gooding EL et al (2008) Pediatric malignant germ cell tumors show characteristic transcriptome profiles. Cancer Res 68:4239–4247
Palmer RD, Murray MJ, Saini HK et al (2010) Malignant germ cell tumors display common microRNA profiles resulting in global changes in expression of messenger RNA targets. Cancer Res 70:2911–2923
Perlman EJ, Cushing B, Hawkins E et al (1994) Cytogenetic analysis of childhood endodermal sinus tumors: a Pediatric Oncology Group study. Pediatr Pathol 14:695–708
Pleskacova J, Hersmus R, Oosterhuis JW et al (2010) Tumor risk in disorders of sex development. Sex Dev 4(4–4):259–269
Rapley EA, Hockley S, Warren W et al (2004) Somatic mutations of KIT in familial testicular germ cell tumours. Br J Cancer 90:2397–2401
Riopel MA, Spellerberg A, Griffin CA et al (1998) Genetic analysis of ovarian germ cell tumors by comparative genomic hybridization. Cancer Res 58:3105–3110
Schmidt P, Haas RJ, Göbel U et al (2002) Results of the German studies (MAHO) for treatment of testicular germ cell tumors in children – an update. Klin Pädiatr 214:167–172
Schneider DT (2015) Sex cord stromal tumors: it is networking-or not working. Pediatr Blood Cancer 62:2065–2066
Schneider DT, Hilgenfeld E, Schwabe D et al (1999) Acute myelogenous leukemia after treatment for malignant germ cell tumors in children. J Clin Oncol 17:3226–3233
Schneider DT, Calaminus G, Reinhard H et al (2000) Primary mediastinal germ cell tumors in children and adolescents: results of the German cooperative protocols MAKEI 83/86, 89, and 96. J Clin Oncol 18:832–839
Schneider DT, Schuster AE, Fritsch MK et al (2001a) Genetic analysis of childhood germ cell tumors with comparative genomic hybridization. Klin Pädiatr 213:204–211
Schneider DT, Calaminus G, Göbel U (2001b) Diagnostic value of alpha 1-fetoprotein and beta-human chorionic gonadotropin in infancy and childhood. Pediatr Hematol Oncol 18:11–26
Schneider DT, Schuster AE, Fritsch MK et al (2001c) Multipoint imprinting analysis indicates a common precursor cell for gonadal and nongonadal pediatric germ cell tumors. Cancer Res 61:7268–7276
Schneider DT, Calaminus G, Koch S et al (2004) Epidemiologic analysis of 1,442 children and adolescents registered in the German germ cell tumor protocols. Pediatr Blood Cancer 42:169–175
Schneider DT, Calaminus G, Harms D et al (2005) Ovarian sex cord-stromal tumors in children and adolescents. J Reprod Med 50:439–446
Sievers S, Alemazkour K, Zahn S et al (2005) IGF2/H19 imprinting analysis with the methylation-sensitive single nucleotide primer extension method in human germ cell tumors reflects their origin from different stages of primordial germ cell development. Genes Chromosomes Cancer 44:256–264
Slade I, Bacchelli C, Davies H et al (2011) DICER1 syndrome: clarifying the diagnosis, clinical features and management implications of a pleiotropic tumour predisposition syndrome. J Med Genet 48:273–278
Skakkebaek NE (2004) Testicular dysgenesis syndrome: new epidemiological evidence. Int J Androl 27:189–191
Tröbs RB, Krauss M, Geyer C et al (2007) Surgery in infants and children with testicular and paratesticular tumours: a single centre experience over a 25-year-period. Klin Padiatr 219:146–151
Wessalowski R, Blohm M, Calaminus G et al (1997) Treatment results in children and adolescents with loco-regional recurrences of abdominal germ cell tumors (GCTs): a pilot-study with PEI chemotherapy and regional deep hyperthermia (RHT) in comparison to a matched cohort. Klin Pädiatr 209:250–256
Wessalowski R, Schneider DT, Mils O et al (2003) An approach to cure: PEI chemotherapy and regional deep hyperthermia in children and adolescents with unresectable malignant tumors. Klin Pädiatr 215:303–309
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Schneider, D.T. (2019). Keimzelltumoren und seltene gonadale Tumoren bei Kindern und Jugendlichen. In: Rübben, H., Hakenberg, O., Grimm, MO., Burger, M. (eds) Uroonkologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-54652-9_79-1
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