Abstract
Extragonadal germ cell tumors (EGCTs) are rare tumors that predominantly affect young males. They are located in the body midline. The most common sites of EGCTs are the mediastinum (50-70%), the retroperitoneum (25-40%), the pineal gland (5%), and the sacrococcygeal area (less than 5%). 2-5% of germ cell tumors are considered of extragonadal origin. The anatomical distribution of EGCTs might be explained by the migration of primordial germ cells from the yolk sac along the hindgut and its mesentery to the genital ridges. EGCTs are considered to develop from pluripotent embryonal stem cells that because of conditions in the midline of the embryo have undergone germ cell differentiation. Presentation of EGCTs is often in an advanced local stage and with distant metastases. At diagnosis a testicular primary tumor should by all means be excluded. There is strong evidence that a considerable amount of EGCTs, especially retroperitoneal EGCTs have in fact a regressed primary testicular tumor. Treatment is based on localization, histology and extent of disease. Chemotherapy is often first line treatment. Surgery is advocated for teratomas and residual disease.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Aoyama H, Shirato H, Ikeda J, Fujieda K, Miyasaka K, Sawamura Y (2002) Induction chemotherapy followed by low-dose involved-field radiotherapy for intracranial germ cell tumors. J Clin Oncol 20(3):857–865
Billmire D, Vinocur C, Rescorla F, Colombani P, Cushing B, Hawkins E, London WB, Giller R, Lauer S (2001) Malignant mediastinal germ cell tumors: an intergroup study. J Pediatr Surg 36(1):18–24
Bokemeyer C, Droz JP, Horwich A, Gerl A, Fossa SD, Beyer J, Pont J, Schmoll HJ, Kanz L, Einhorn L, Nichols CR, Hartmann JT (2001) Extragonadal seminoma: an international multicenter analysis of prognostic factors and long term treatment outcome. Cancer 91(7):1394–1401
Bokemeyer C, Nichols CR, Droz JP, Schmoll HJ, Horwich A, Gerl A, Fossa SD, Beyer J, Pont J, Kanz L, Einhorn L, Hartmann JT (2002) Extragonadal germ cell tumors of the mediastinum and retroperitoneum: results from an international analysis. J Clin Oncol 20(7):1864–1873
Bokemeyer C, Schleucher N, Metzner B, Thomas M, Rick O, Schmoll HJ, Kollmannsberger C, Boehlke I, Kanz L, Hartmann JT (2003) First-line sequential high-dose VIP chemotherapy with autologous transplantation for patients with primary mediastinal nonseminomatous germ cell tumours: a prospective trial. Br J Cancer 89(1):29–35
Brandes AA, Pasetto LM, Monfardini S (2000) The treatment of cranial germ cell tumours. Cancer Treat Rev 26(4):233–242
Chao TY, Nieh S, Huang SH, Lee WH (1997) Cytology of fine needle aspirates of primary extragonadal germ cell tumors. Acta Cytol 41(2):497–503
Collins DH, Puch RC (1964) Classification and frequency of testicular tumours. Br J Urol 36(suppl):1–11
Comiter CV, Renshaw AA, Benson CB, Loughlin KR (1996) Burned-out primary testicular cancer: sonographic and pathological characteristics. J Urol 156(1):85–88
De Giorgi U, Demirer T, Wandt H, Taverna C, Siegert W, Bornhauser M, Kozak T, Papiani G, Ballardini M, Rosti G; Solid Tumor Working Party of the European Group for Blood and Marrow Transplantation (2005) Second-line high-dose chemotherapy in patients with mediastinal and retroperitoneal primary non-seminomatous germ cell tumors: the EBMT experience. Ann Oncol 16(1):146–151
Downie PA, Vogelzang NJ, Moldwin RL, Le Beau MM, Anastasi J, Allen RJ, Myers SE, Larson RA, Smith SD (1994) Establishment of a leukemia cell line with i(12p) from a patient with a mediastinal germ cell tumor and acute lymphoblastic leukemia. Cancer Res 54(18):4999–5004
Dueland S, Stenwig AE, Heilo A et al (1998) Treatment and outcome of patients with extragonadal germ cell tumours–the Norwegian Radium Hospital’s experience 1979–94. Br J Cancer 77(2):329–335
Fabre E, Jira H, Izard V, Ferlicot S, Hammoudi Y, Theodore C, Di Palma M, Benoit G, Droupy S (2004) “Burned-out” primary testicular cancer. BJU Int 94(1):74–78
Fizzazi K, Culine S, Droz JP, Kramar A, Théodore C, Ruffié P, Le Chevalier T (1998) Primary mediastinal nonsemi nomatous germ cell tumors: results of modern therapy in cluding cisplatin-based chemotherapy. J Clin Oncol 16(2): 725–732
Fosså SD, Aass N, Heilo A, Daugaard G, E Skakkebaek N, Stenwig AE, Nesland JM, Looijenga LH, Oosterhuis JW (2003) Testicular carcinoma in situ in patients with extragonadal germ-cell tumours: the clinical role of pretreatment biopsy. Ann Oncol 14(9):1412–1418
Goss PE, Schwertfeger L, Blackstein ME et al (1994) Extra gonadal germ cell tumors – a 14-year Toronto experience. Cancer 73:1971–1979
Hartmann JT, Nichols CR, Droz JP, Horwich A, Gerl A, Fossa SD, Beyer J, Pont J, Fizazi K, Einhorn L, Kanz L, Bokemeyer C (2000) Hematologic disorders associated with primary mediastinal nonseminomatous germ cell tumors. J Natl Cancer Inst 92(1):54–61
Hartmann JT, Fossa SD, Nichols CR, Droz JP, Horwich A, Gerl A, Beyer J, Pont J, Fizazi K, Hecker H, Kanz L, Einhorn L, Bokemeyer C (2001a) Incidence of metachronous testicular cancer in patients with extragonadal germ cell tumors. J Natl Cancer Inst 93(22):1733–1738
Hartmann JT, Einhorn L, Nichols CR, Droz JP, Horwich A, Gerl A, Fossa SD, Beyer J, Pont J, Schmoll HJ, Kanz L, Bokemeyer C (2001b) Second-line chemotherapy in patients with relapsed extragonadal nonseminomatous germ cell tumors: results of an international multicenter analysis. J Clin Oncol 19(6): 1641–1648
Hasle H, Mellemgaard A, Nielsen J et al (1995) Cancer incidence in men with Klinefelter syndrome. Br J Cancer 71(2): 416–420
International Germ Cell Cancer Collaborative Group (1997) International Germ Cell Consensus Classification: a prognostic factor-based staging system for metastatic germ cell cancers. J Clin Oncol 15(2):594–603
Kang CH, Kim YT, Jheon SH, Sung SW, Kim JH (2008) Surgical treatment of malignant mediastinal nonseminomatous germ cell tumor. Ann Thorac Surg 85(2):379–384
Keene D, Johnston D, Strother D, Fryer C, Carret AS, Crooks B, Eisenstat D, Moghrabi A, Wilson B, Brossard J, Mpofu C, Odame I, Zelcer S, Silva M, Samson Y, Hand J, Bouffet E; Canadian Pediatric Brain Tumor Consortium (2007) Epidemiological survey of central nervous system germ cell tumors in Canadian children. J Neurooncol 82(3):289–295
Kellie SJ, Boyce H, Dunkel IJ, Diez B, Rosenblum M, Brualdi L, Finlay JL (2004) Primary chemotherapy for intracranial nongerminomatous germ cell tumors: results of the second international CNS germ cell study group protocol. J Clin Oncol 22(5):846–853
Kesler KA, Rieger KM, Hammoud ZT, Kruter LE, Perkins SM, Turrentine MW, Schneider BP, Einhorn LH, Brown JW (2008) A 25-year single institution experience with surgery for primary mediastinal nonseminomatous germ cell tumors. Ann Thorac Surg 85(2):371–378
Kochi M, Itoyama Y, Shiraishi S, Kitamura I, Marubayashi T, Ushio Y (2003) Successful treatment of intracranial nongerminomatous malignant germ cell tumors by administering neoadjuvant chemotherapy and radiotherapy before excision of residual tumors. J Neurosurg 99(1):106–114
Ladanyi M, Samaniego F, Reuter VE, Motzer RJ, Jhanwar SC, Bosl GJ, Chaganti RS (1990) Cytogenetic and immunohistochemical evidence for the germ cell origin of a subset of acute leukemias associated with mediastinal germ cell tumors. J Natl Cancer Inst 82(3):221–227
Maity A, Shu HK, Janss A, Belasco JB, Rorke L, Phillips PC, Sutton LN, Goldwein JW (2004) Craniospinal radiation in the treatment of biopsy-proven intracranial germinomas: twenty-five years’ experience in a single center. Int J Radiat Oncol Biol Phys 58(4):1165–1170
Matsutani M, Sano K, Takakura K, Fujimaki T, Nakamura O, Funata N, Seto T (1997) Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 86(3):446–455
Nichols CR, Fox EP (1991) Extragonadal and pediatric germ cell tumors. Hematol Oncol Clin North Am 5(6):1189–1209
Oosterhuis JW, Looijenga LH (2005) Testicular germ-cell tumours in a broader perspective. Nat Rev Cancer 5(3): 210–222
Oosterhuis JW, Stoop H, Honecker F, Looijenga LH (2007) Why human extragonadal germ cell tumours occur in the midline of the body: old concepts, new perspectives. Int J Androl 30(4):256–263
Pectasides D, Aravantinos G, Visvikis A, Bakoyiannis C, Halikia A, Kalofonos C, Kosmidis P, Skarlos D, Fountzilas G (1999) Platinum-based chemotherapy of primary extragonadal germ cell tumours: the Hellenic Cooperative Oncology Group experience. Oncology 57(1):1–9
Rescorla FJ, Sawin RS, Coran AG, Dillon PW, Azizkhan RG (1998) Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group. J Pediatr Surg 33(2):171–176
Rogers SJ, Mosleh-Shirazi MA, Saran FH (2005) Radiotherapy of localised intracranial germinoma: time to sever historical ties? Lancet Oncol 6(7):509–519
Saltzman B, Pitts WR, Vaughan ED Jr (1986) Extragonadal retroperitoneal germ cell tumors without apparent tes ticular involvement. A search for the source. Urology 27(6):504–507
Scholz M, Zehender M, Thalmann GN, Borner M, Thöni H, Studer UE (2002) Extragonadal retroperitoneal germ cell tumor: evidence of origin in the testis. Ann Oncol 13(1): 121–124
Sebire NJ, Fowler D, Ramsay AD (2004) Sacrococcygeal tumors in infancy and childhood; a retrospective histopathological review of 85 cases. Fetal Pediatr Pathol 23(5–6): 295–303
Spermon JR, De Geus-Oei LF, Kiemeney LA, Witjes JA, Oyen WJ (2002) The role of (18)fluoro-2-deoxyglucose positron emission tomography in initial staging and re-staging after chemotherapy for testicular germ cell tumours. BJU 89(6): 549–556
Szabo PE, Mann JR (1995) Biallelic expression of imprinted genes in the mouse germ line: implications for erasure, establishment, and mechanisms of genomic imprinting. Genes Dev 9(15):1857–1868
Ustün M, Heilo A, Fosså S, Aass N, Berner A (2002) Ultrasound-guided fine needle cytology of retroperitoneal masses in patients with malignant germ cell tumours: diagnosis and therapeutic impact. Eur Urol 42(3):221–228
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2011 Springer-Verlag London Limited
About this chapter
Cite this chapter
Roeleveld, T.A., Horenblas, S. (2011). Extra-Gonadal Germ Cell Tumors. In: Laguna, M., Albers, P., Bokemeyer, C., Richie, J. (eds) Cancer of the Testis. Springer, London. https://doi.org/10.1007/978-1-84800-370-5_17
Download citation
DOI: https://doi.org/10.1007/978-1-84800-370-5_17
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-84800-369-9
Online ISBN: 978-1-84800-370-5
eBook Packages: MedicineMedicine (R0)