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DOI: 10.1055/s-0028-1100418
© J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York
Intracranial Germinoma as a Very Rare Cause of Panhypopituitarism in a 23-year Old Man
Publication History
received 29.01.2008
first decision 13.10.2008
accepted 13.10.2008
Publication Date:
03 December 2008 (online)
Abstract
A 23 year-old patient was referred to the endocrine outpatient clinic with the suspicion of diabetes insipidus as he complained of nycturia and polydipsia since 2–3 months. Further he presented with nausea, vomiting, loss of appetite, rapid weight loss, diffuse body pain and fatigue. No headache, blurred vision, or fever were reported. The clinical examination showed sexual infantilism (poor beard, pubic and axillary hair growth, small testis). The patient's skin was strikingly pale as well as dry and scaly. Lymph node palpation was unremarkable. Endocrine evaluation revealed diabetes insipidus as well as complete anterior pituitary insufficiency. MR imaging demonstrated contrast-enhancing mass lesions at the pineal gland, hypothalamus, and anterior horn of lateral ventricles bilaterally. The localization pattern deemed to be highly suspicious for intracranial germinoma. As β-HCG and AFP were negative in serum and cerebrospinal fluid the diagnosis of germinoma was confirmed histologically. After radiotherapy with cranio-spinal radiation therapy with 24 Gy followed by two weeks of local tumor boost with 16 Gy, the posttherapy MRI scan indicated complete tumor removal. This case demonstrates a very rare and potentially curable tumor as the cause of panhypopituitarism in adults.
Key words
germinoma - panhypopituitarism - intracranial germ cell tumor - radiotherapy - diabetes insipidus
References
- 1 Aoyama H, Shirato H, Kakuto Y. et al . Pathologically-proven intracranial germinoma treated with radiation therapy. Radiother Oncol. 1998; 47 201-205
- 2 Bamberg M, Kortmann RD, Calaminus G. et al . Radiation therapy for intracranial germinoma: results of the German cooperative prospective trials MAKEI 83/86/89. J Clin Oncol. 1999; 17 2585-2592
- 3 Gobel U, Calaminus G, Schneider DT. et al . Management of germ cell tumors in children: approaches to cure. Onkologie. 2002; 25 14-22
- 4 Haas-Kogan DA, Missett BT, Wara WM. et al . Radiation therapy for intracranial germ cell tumors. Int J Radiat Oncol Biol Phys. 2003; 56 511-518
- 5 Hasle H, Mellemgaard A, Nielsen J. et al . Cancer incidence in men with Klinefelter syndrome. Br J Cancer. 1995; 71 416-420
- 6 Janmohamed S, Grossman AB, Metcalfe K. et al . Suprasellar germ cell tumours: specific problems and the evolution of optimal management with a combined chemoradiotherapy regimen. Clin Endocrinol (Oxf). 2002; 57 487-500
- 7 Jennings MT, Gelman R, Hochberg F. Intracranial germ-cell tumors: natural history and pathogenesis. J Neurosurg. 1985; 63 155-167
- 8 Lafay-Cousin L, Millar BA, Mabbott D. et al . Limited-field radiation for bifocal germinoma. Int J Radiat Oncol Biol Phys. 2006; 65 486-492
- 9 Lee L, Saran F, Hargrave D. et al . Germinoma with synchronous lesions in the pineal and suprasellar regions. Childs Nerv Syst. 2006; 22 1513-1518
- 10 Liang L, Korogi Y, Sugahara T. et al . MRI of intracranial germ-cell tumours. Neuroradiology. 2002; 44 382-388
- 11 Maity A, Shu HK, Janss A. et al . Craniospinal radiation in the treatment of biopsy-proven intracranial germinomas: twenty-five years’ experience in a single center. Int J Radiat Oncol Biol Phys. 2004; 58 1165-1170
- 12 Matsutani M. Clinical management of primary central nervous system germ cell tumors. Semin Oncol. 2004; 31 676-683
- 13 Nishizaki T, Kajiwara K, Adachi N. et al . Detection of craniospinal dissemination of intracranial germ cell tumours based on serum and cerebrospinal fluid levels of tumour markers. J Clin Neurosci. 2001; 8 27-30
- 14 Ogawa K, Shikama N, Toita T. et al . Long-term results of radiotherapy for intracranial germinoma: a multi-institutional retrospective review of 126 patients. Int J Radiat Oncol Biol Phys. 2004; 58 705-713
- 15 Packer RJ, Cohen BH, Cooney K. Intracranial germ cell tumors. Oncologist. 2000; 5 312-320
- 16 Surawicz TS, MacCarthy BJ, Kupelian V. et al . Descriptive epidemiology of primary brain and CNS tumors: results from the Central Brain Tumor Registry of the United States, 1990–1994. Neuro Oncol. 1999; 1 14-25
Correspondence
Dr. N. ReischMD
Medizinische Klinik Innenstadt
Klinikum der Universität München
Ziemssenstr. 1
80336 München
Germany
Phone: +49/89/5160 21 11
Fax: +49/89/5160 45 66
Email: nicole.reisch@med.uni-muenchen.de