Zusammenfassung
Wir berichten über den Fall eines 19-jährigen Patienten, der trotz normaler Testosteronspiegel klinische Zeichen eines Hypogonadismus aufwies. In der weiterführenden Diagnostik zeigte sich das Bild eines Panhypopituitarismus infolge eines β-HCG-produzierenden, intrazerebral metastasierten suprasellären Germinoms. Aufgrund der paraneoplastischen Produktion von β-HCG bestand trotz gonadotroper Insuffizienz eine ausreichende Stimulation der Leydigschen Zwischenzellen mit normalen Testosteronspiegeln, was zu einer teilweisen Maskierung der gonadotropen Insuffizienz geführt hatte. Der Patient wurde mittels einer kombinierten Radiochemotherapie behandelt und ist seit über 7 Jahren rezidivfrei.
Abstract
We report on a 19 year old patient with clinical signs of hypogonadism (small testicles, missing pollutions and diminished beard growth) despite of normal testosterone levels. Further diagnostic procedures revealed panhypopituitarism with insufficiency of the gonadotrope, somatotrope and corticotrope axis due to a β-HCG-producing suprasellar germinoma with intracerebral metastases. Paraneoplastic production of β-HCG resulted in sufficient stimulation of Leydig cells with normal production of testosterone, which had partly masked clinical symptoms of gonatrope insufficiency. The patient was treated by combined radiochemotherapy and is in remission since 7 years.
Literatur
Aoyama H, Shirato H, Ikeda J et al. (2002) Induction chemotherapy followed by low-dose involved-field radiotherapy for intracranial germ cell tumors. J Clin Oncol 20: 857–865
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
Buchfelder M, Fahlbusch R, Walther M et al. (1989) Endocrine disturbances in suprasellar germinomas. Acta Endocrinol 120: 337–342
Calaminus G, Andreussi L, Garre ML et al. (1997) Secreting germ cell tumors of the central nervous system (CNS). First results of the cooperative German/Italian pilot study (CNS sGCT). Klin Pädiatr 209: 222–227
Fuller BG, Kapp DS, Cox R (1993) Radiation therapy of pineal region tumors: 25 new cases and a review of 208 previously reported cases. Int J Radiat Oncol Biol Phys 28: 2229–2245
Gobel U, Calaminus G, Schneider DT et al. (2002) MAKEI and MAHO Study Groups of the German Society of Pediatric Oncology and Hematology, and the SIOP CNS GCT Study Group. Management of germ cell tumors in children: approaches to cure. Onkologie 25: 14–22
Gutjahr P (2003) Supratentorielle Tumoren der Mittellinie. In: Gutjahr P (Hrsg) Krebs bei Kindern und Jugendlichen. Deutscher Ärzteverlag, Köln, S 308–314
Hammer F, Arlt W (2004) Hypophysenvorderlappeninsuffizienz. Internist 45: 795–814
Horowitz MB, Hall WA (1991) Central nervous system germinomas. A review. Arch Neurol 48: 652–657
Imura H, Kato Y, Nakai Y (1987) Endocrine aspects of tumors arising from suprasellar, third ventricular regions. Prog Exp Tumor Res 30: 313–324
Jennings MT, Gelman R, Hochberg F (1985) Intracranial germ-cell tumors: natural history and pathogenesis. J Neurosurg 63: 155–167
Kretschmar CS (1997) Germ-cell tumors of the brain in children: A review of current literature and new advances in therapy. Cancer Invest 15: 187–98
Motzer RJ, Mazumdar M, Sheinfeld J et al. (2000) Sequential dose-intensive Paclitaxel, Ifosfamide, Carboplatin and Etoposide salvage therapy for germ cell tumor patients. J Clin Oncol 18: 1173–1180
Sawamura Y, Ikeda J, Shirato H et al. (1998) Germ cell tumours of the central nervous system: treatment consideration based on 111 cases and their long-term clinical outcomes. Eur J Cancer 34: 104–110
Shinoda J, Miwa Y, Sakai N et al. (1985) Immunohistochemical study of placental alkaline phosphatase in primary intracranial germ-cell tumors. J Neurosurg 63: 733–739
Sklar CA, Grumbach MM, Kaplan SL et al. (1981) Hormonal and metabolic abnormalities associated with central nervous system germinomas in children and adolescents and the effect of therapy: report of 10 patients. Clin Endocrinol Metabol 52: 9–16
Tscherry G, Jacky E, Jost LM et al. (2000) Risk-adapted Chemotherapy of Germ Cell Tumors with Carboplatin, Etoposide and Bleomycin for low-risk and Cisplatin, Etoposide and Ifosfamide for high-risk patients. Oncology 59: 110–117
Interessenkonflikt:
Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Bauditz, J., Ventz, M., van Landeghem, F. et al. Hypogonadismus bei einem jungen Mann mit normalen Testosteronspiegeln. Internist 46, 334–340 (2005). https://doi.org/10.1007/s00108-004-1326-0
Issue Date:
DOI: https://doi.org/10.1007/s00108-004-1326-0