Abstract
Intracranial teratomas are rare and comprise about 0.5 % of all intracranial tumours. Actually, a total of 15 cases of sellar-suprasellar teratoma have been described in the last 24 years. Although rare, hypothalamic-pituitary teratomas should be taken into account in the differential diagnosis of hypothalamic-pituitary region tumours. The current review focuses on the clinical and therapeutic management of pituitary region teratomas. Teratomas occur more frequently in children and young adults than in the older population and in males as compared to females. Symptoms at diagnosis are usually neurological defects, diabetes insipidus and hypopituitarism. Teratoma diagnosis can be suggested though neuroimaging findings. Magnetic resonance imaging remains the preferred modality for assessment of teratoma. Neuro-radiological findings of mixed-density mass, usually with mixed cystic and solid components or inclusions of teeth, fat and calcification can be suggestive. Tumour markers as beta-HCG and alpha-FP can be useful at teratoma diagnosis for distinguishing immature teratomas, mixed GCTs and mature teratomas with immature or malignant components. Optimal treatment for mature teratoma is neurosurgical excision. Radical excision is advocated as recurrence rate for a mature teratoma is extremely low in cases of complete resection and long-term outcome is excellent. During post-treatment follow-up, in cases of healing, according to tumour marker evaluation and contrasted MRI findings, hormone replacement therapy should be considered, also for secondary hypogonadism and GH deficit, with a more intense follow-up. However, as actually few evidence are available, safety data have to be confirmed also trough a surveillance study.
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References
D. Muzumdar, A. Goel, K. Desai, A. Shenoy, Mature teratoma arising from the sella. Neurol. Med. Chir. 41, 356–359 (2001)
N. Tsoukalas, M. Tolia, I.D. Kostakis, N. Pistamaltzian, D. Tryfonopoulos, G. Lypas, G. Koumakis, V. Barbounis, N. Goutas, A. Efremidis, Coexistence of intracranial germ cell tumor and craniopharyngioma in an adolescent: case report and review of the literature. Int. J. Clin. Exp. Med. 6, 211–218 (2013)
C.V. Chang, V.S. Nunes, A.C. Felicio, M.A. Zanini, M.B.C. Cunha-Neto, A.V. Barros de Castro, Mixed germ cell tumor of the pituitary-hypothalamic region presenting as craniopharyngioma: case report and review of the literature. Arq. Bras. Endocrinol. Metabol. 52(9), 1501–1504 (2008)
K. Yagi, T. Kageji, S. Nagahiro, H. Horiguchi, Growing teratoma syndrome in a patient with a non-germ germinomatous germ cell tumor in the neurohypophysis. Neurol. Med. Chir. 44, 33–37 (2004)
J.H. Shin, H.K. Lee, C.G. Choi, D.C. Suh, C.J. Kim, S.K. Hong, D.G. Na, MR imaging of central diabetes insipidus: a pictorial essay. Korean. J. Radiol. 2, 222–230 (2001)
M. Taniguchi, M. Nishihara, T. Sasayama, Y. Takahashi, E. Kohmura, A rapidly expanding immature teratoma originating from a neurohypophyseal germinoma. Neuropathol. Appl. Neurobiol. (2013). doi:10.1111/nan.12000
H. Nishioka, H. Ito, J. Haraoka, K. Akada, Immature teratoma originating from the pituitary gland: case report. Neurosurgery 44(3), 644–647 (1999)
M. Chariker, R. Ford, C. Morrison, A. Theile, K. Moeller, T. Moriarty, pituitary duplication with nasopharyngeal teratoma and cleft palate. J. Craniofac. Surg. 22(2), 755–758 (2011)
Y. Maeda, H. Suenaga, M. Sugiyama, H. Saijo, K. Hoshi, Y. Mori, T. Takato, Clinical presentation of epignathus teratoma with cleft palate; and duplication of cranial base, tongue, mandible, and pituitary gland. J. Craniofac. Surg. 24(4), 1486–1491 (2013)
T. Huismn, U. Fisher, E. Boltshauser, T. Straube, C. Gysin, Pituitary duplication and nasopharyngeal teratoma in a newborn: CT, MRI, US and correlative histopatological findings. Neuroradiology 47, 558–561 (2005)
T.A. Abele, K.L. Salzman, H.R. Harnsberger, C.M. Glastonbury, Craniopharyngeal canal and its spectrum of pathology. AJNR Am. J. Neuroradiol. 35, 772–777 (2014)
S. Nishio, T. Inamura, I. Takeshita, M. Fukui, K. Kamikaseda, Germ cell tumor in the hypothalamo-neurohypophysial region: clinical features and treatment. Neurosurg. Rev. 16(3), 221–227 (2001)
J. Ikeda, Y. Sawamura, T. Kato, H. Abe, Metachronous neurohypophyseal germinoma occurring 8 years after total resection of pineal mature teratoma. Surg. Neurol. 49, 205–209 (1998)
K. Araki, M. Koga, T. Okada, T. Kurashige, K. Naruse, M. Hiroi, A boy with normal growth in spite of growth hormone deficiency after resection of a suprasellar teratoma. Endocr. J. 47, S101–S104 (2000)
N. Phulwani, T. Pandey, J. Khatri, R.H. Ramakrishnaiah, T. Pandey, C.C. Shah, Imaging Manifestations and Techniques in Diabetes Insipidus (2011) doi: 10.5772/24765
Y.J. Lee, J.C. Lin, E.Y. Shen, D.C. Liang, T.T. Wong, F.Y. Huang, Loss of visibility of the neurohypophysis as a sign of central diabetes insipidus. Eur. J. Radiol. 21, 145–147 (1995)
Y.S. Kim, S.G. Kang, Y.O. Kim, Pituitary teratoma presenting as central diabetes insipidus with a normal MRI finding. Yonsei Med. J. 51, 293–294 (2010). doi:10.3349/ymj.2010.51.2.293
K. Sano, Pathogenesis of intracranial germ cell tumors reconsidered. J. Neurosurg. 90, 258–264 (1999)
S. Chiloiro, A. Giampietro, L. De Marinis, Safety of rhGH replacement therapy in suprasellar pituitary teratoma: a case report. Int. J. Endocr. Metab. Disord. 1(2) (2015). http://dx.doiorg/10.16966/ijemd.107
K.H. Darzy, M.S. Shalet, Hypopituitarism as a consequence of brain tumours and radiotherapy. Pituitary 8, 203–211 (2005)
M.M. Rutter, S.R. Rose, Long-Term Endocrine Sequelae of Childhood Cancer (Lippincott Williams & Wilkins, Philadelphia, 2007), pp. 1040–8703
A.R. Cohen, J.A. Wilson, A. Sadeghi-Nejad, Gonadotropin-secreting pineal teratoma causing precocious puberty. Neurosurgery 28(4), 597–602 (1991)
S. Czirjàk, E. Pasztor, F. Slowik, G. Szeifert, Third ventricle germinoma after total removal of intrasellar teratoma. J. Neurosurg. 77, 643–647 (1992)
D.Y. Cho, Y.C. Wang, W.L. Ho, Primary intrasellar mixed germ-cell tumor with precocious puberty and diabetes insipidus. Childs Nerv. Syst. 13(1), 42–46 (1997)
F.G. Greiner, D. Takhtani, Neuroradiology case of the day. malignant mixed germ cell tumor with yolk sac and teratomatous components. Radiographics 19(3), 826–829 (1999)
J.F. Vendrell, D. Hoa, G. Gahide, Mature teratoma arising from the sella. Lancet (2010). doi:10.1016/S0140-6736(09)60300-7
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Chiloiro, S., Giampietro, A., Bianchi, A. et al. Clinical management of teratoma, a rare hypothalamic-pituitary neoplasia. Endocrine 53, 636–642 (2016). https://doi.org/10.1007/s12020-015-0814-4
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DOI: https://doi.org/10.1007/s12020-015-0814-4