Germinomas are malignant intracranial germ tumors, usually found in suprasellar regions. Less than 10% are localized in off-middle structures, and synchronous involvement of both structures has only exceptionally been published. A case of an 18-year-old male patient with progressive right-sided hemiparesis and panhypopituitarism was reviewed. Brain MRI showed a solid mass involving pituitary and hypothalamus with thickening of pituitary stalk, high intensity lesions on T2-weighted imaging in left internal capsule, caudate nucleus, globus pallidus, and mild atrophy of the left internal capsule and cerebral peduncle. Nonadenomatous lesions were considered in the differential diagnosis. Alfa-fetoprotein (AFP) levels were negative in both serum and cerebrospinal fluid (CSF), while β-human chorionic gonadotrophin (β-HCG) levels were slightly increased in CSF. A transsphenoidal biopsy identified a germinoma. Four cycles of chemotherapy with bleomicine, etoposide, and cysplatin were given, followed by radiotherapy, but patients died due to a recidiva. Conclusion. Germinoma must be considered in patients with insipidus diabetes with a sellar mass with thickening of pituitary stalk; and ectopic germinoma must be suspected in patients with slowly progressive hemiparesis with cerebral hemiatrophy. Even with a rare condition, colocalization of midline and off-midline germinoma must be suspected in the presence of these typical signs of both localizations. 1. Introduction Germ cell tumors (GCT) represent approximately 3% of neoplasms in children’s cancer registries [1]. They constitute 0.1 to 2.4% of all childhood intracranial tumors in North America and Europe, while they account for almost 2.1 to 9.5% in Japan and the Far East [2, 3]. Central nervous system germ cell tumors (CNSGCT) are rare and most of them occur in patients under 20 years of age [1, 4]. CNSGCTs have been classified in “secreting” and “nonsecreting” tumors. Secreting tumors are defined as those presenting with an elevated CSF AFP ≥ 10?ng/mL or above the local laboratory’s normal range and/or a CSF -HCG level ≥ 50?IU/l or greater than the accepted laboratory normal range. This has been shown to be related to prognosis and treatment response [1]. Brain germinomas are usually serologically negative for these markers [5]. The most common sites of involvement of intracranial germinomas are the pineal or suprasellar regions, while some patients have both localizations at the time of diagnosis [1, 4]. Off-midline germinomas arising in the basal ganglia, thalami, and internal capsule, also called ectopic
References
[1]
M. Echevarria, J. Fangusaro, and S. Goldman, “Pediatric central nervous system germ cell tumors: a review,” The Oncologist, vol. 13, pp. 690–699, 2008.
[2]
D. Keene, D. Johnston, D. Strother et al., “Epidemiological survey of central nervous system germ cell tumors in Canadian children,” Journal of Neuro-Oncology, vol. 82, no. 3, pp. 289–295, 2007.
[3]
K.-T. Cho, K.-C. Wang, S.-K. Kim, S.-H. Shin, J. G. Chi, and B.-K. Cho, “Pediatric brain tumors: statistics of SNUH, Korea (1959–2000),” Child's Nervous System, vol. 18, pp. 30–37, 2002.
[4]
S. Sartori, A. M. Laverda, M. Calderone et al., “Germinoma with synchronous involvement of midline and off-midline structures associated with progressive hemiparesis and hemiatrophy in a young adult,” Child's Nervous System, vol. 23, no. 11, pp. 1341–1345, 2007.
[5]
A. Gutenberg, J. J. Bell, I. Lupi et al., “Pituitary and systemic autoimmunity in a case of intrasellar germinoma,” Pituitary, vol. 14, no. 4, pp. 388–394, 2011.
[6]
N. A. Tritos, T. N. Byrne, C.-L. Wu, and A. Klibanski, “A patient with diabetes insipidus, anterior hypopituitarism and pituitary stalk thickening,” Nature Reviews Endocrinology, vol. 7, no. 1, pp. 54–59, 2011.
[7]
N. Karavitaki and J. A. H. Wass, “Non-adenomatous pituitary tumours,” Best Practice and Research, vol. 23, no. 5, pp. 651–665, 2009.
[8]
R. V. Ozelame, M. Shroff, B. Wood et al., “Basal ganglia germinoma in children with associated ipsilateral cerebral and brain stem hemiatrophy,” Pediatric Radiology, vol. 36, no. 4, pp. 325–330, 2006.
[9]
Y. Sadamura, K. Sugiyama, H. Uchida et al., “Intracranial germinoma presenting with hemiatrophy- follow-up results and literature review,” Neurologia Medico-Chirurgica, vol. 51, no. 2, pp. 148–152, 2011.
[10]
N. Saeki, K. Tamaki, H. Murai et al., “Long-term outcome of endocrine function in patients with neurohypophyseal germinomas,” Endocrine Journal, vol. 47, no. 1, pp. 83–89, 2000.
[11]
N. Oyama, S. Terae, S. Saitoh, A. Sudoh, Y. Sawamura, and K. Miyasaka, “Bilateral germinoma involving the basal ganglia and cerebral white matter,” American Journal of Neuroradiology, vol. 26, no. 5, pp. 1166–1169, 2005.
[12]
T.-T. Wong, Y. W. Chen, W.-Y. Guo, K.-P. Chang, D. M. Ho, and S.-H. Yen, “Germinoma involving the basal ganglia in children,” Child's Nervous System, vol. 24, no. 1, pp. 71–78, 2008.
[13]
K. Okamoto, J. Ito, K. Ishikawa et al., “Atrophy of the basal ganglia as the initial diagnostic sign of germinoma in the basal ganglia,” Neuroradiology, vol. 44, no. 5, pp. 389–394, 2002.
[14]
K. H. Yoo, S. H. Lee, J. Lee et al., “Improved outcome of central nervous system germ cell tumors: implications for the role of risk-adapted intensive chemotherapy,” Journal of Korean Medical Science, vol. 25, no. 3, pp. 458–465, 2010.
[15]
M. Kanamori, T. Kumabe, R. Saito et al., “Optimal treatment strategy for intracranial germ cell tumors: a single institution analysis—clinical article,” Journal of Neurosurgery, vol. 4, no. 6, pp. 506–514, 2009.
[16]
C. Alapetite, H. Brisse, C. Patte et al., “Pattern of relapse and outcome of nonmetastatic germinoma patients treated with chemotherapy and limited field radiation: the SFOP experience,” Neuro-Oncology, vol. 12, no. 12, pp. 1318–1325, 2010.
[17]
C. Balmaceda, G. Heller, M. Rosenblum et al., “Chemotherapy without irradiation—a novel approach for newly diagnosed CNS germ cell tumors: results of an international cooperative trial,” Journal of Clinical Oncology, vol. 14, no. 11, pp. 2908–2915, 1996.
[18]
T. Ueba, K. Yamashita, I. Fujisawa et al., “Long-term follow-up of 5 patients with intracranial germinoma initially treated by chemotherapy alone,” Acta Neurochirurgica, vol. 149, no. 9, pp. 897–902, 2007.
[19]
Y. Shibamoto, K. Sasai, M. Kokubo, and M. Hiraoka, “Salvage radiation therapy for intracranial germinoma recurring after primary chemotherapy,” Journal of Neuro-Oncology, vol. 44, no. 2, pp. 181–185, 1999.
[20]
N. S. da Silva, A. M. Cappellano, B. Diez et al., “Primary chemotherapy for intracranial germ cell tumors: results of the third International CNS germ cell tumor study,” Pediatric Blood and Cancer, vol. 54, no. 3, pp. 377–383, 2010.
[21]
Y. Sawamura, J. L. Ikeda, M. Tada, and H. Shirato, “Salvage therapy for recurrent germinomas in the central nervous system,” British Journal of Neurosurgery, vol. 13, no. 4, pp. 376–381, 1999.
[22]
H. Aoyama, H. Shirato, J. Ikeda, K. Fujieda, K. Miyasaka, and Y. Sawamura, “Induction chemotherapy followed by low-dose involved-field radiotherapy for intracranial germ cell tumors,” Journal of Clinical Oncology, vol. 20, no. 3, pp. 857–865, 2002.
[23]
P. Strojan, L. Z. Zadravec, J. An?i?, R. Korenjak, and B. Jereb, “The role of radiotherapy in the treatment of childhood intracranial germinoma: long-term survival and late effects,” Pediatric Blood and Cancer, vol. 47, no. 1, pp. 77–82, 2006.
[24]
V. Calugaru, S. Taillibert, P. Lang, J.-M. Simon, J.-Y. Delattre, and J.-J. Mazeron, “Neoadjuvant chemotherapy followed by radiotherapy adapted to the tumour response in the primary seminoma of the central nervous system: experience of the Pitié-Salpêtrière Hospital and review of literature,” Cancer/Radiotherapie, vol. 11, no. 3, pp. 122–128, 2007.
[25]
Y. Shibamoto, “Management of central nervous system germinoma: proposal for a modern strategy,” Progress in Neurological Surgery, vol. 23, pp. 119–129, 2009.
[26]
Y. Kamoshima, Y. Sawamura, J. Ikeda, H. Shirato, and H. Aoyama, “Late recurrence and salvage therapy of CNS germinomas,” Journal of Neuro-Oncology, vol. 90, no. 2, pp. 205–211, 2008.