Journal of Clinical Neuroscience 22 (2015) 1218
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Images in Neuroscience: Answer
Large suprasellar mass: answer Kenneth Sim a,⇑, Andrew Morokoff b, Alpha Tsui c, Frank Gaillard a a
Department of Radiology, The Royal Melbourne Hospital, Grattan Street, Parkville, VIC 3050, Australia Department of Neurosurgery, The Royal Melbourne Hospital, University of Melbourne, Parkville, VIC, Australia c Department of Pathology, The Royal Melbourne Hospital, Parkville, VIC, Australia b
1. Answer D. Intracranial teratoma
2. Discussion Teratomas are rare intracranial neoplasms that make up approximately 0.4% of all primary intracranial tumours [1]. They can be divided into extra-axial and intra-axial teratomas [2]. Extra-axial tumours usually arise in the suprasellar or pineal regions and typically present in childhood with a recent case series demonstrating a mean age of 14.5 years [3]. In contrast, intra-axial teratomas are the most common intracranial foetal tumour and present in the antenatal period with a mean gestational age at diagnosis of 27 weeks [4,5]. Intra-axial teratomas are more commonly supratentorial with a predilection for the frontal lobes [5]. Regardless of location, extragonadal teratomas are believed to develop from ectopic, totipotent primordial germ cells that have either been misplaced or undergone aberrant migration during early embryogenesis [2,6]. Because of this cellular origin, teratomas can contain diverse mixed tissues with up to 90% of childhood teratomas containing elements derived from all three embryonic layers (ectodermal, mesodermal and endodermal) [2]. This is in contrast to epidermoid and dermoid cysts which are both derived from ectodermal origin only [2]. Importantly, although dermoid cysts have sebaceous glands which produce liquid oily sebum appearing as fat density on CT scan and on MRI demonstrating T1 shortening, chemical shift artefact and signal attenuation on fat suppressed sequences, they do not contain solid adipose tissue as lipocytes are mesodermal in origin [2,7]. Similarly, dermoid cysts may contain semi-formed teeth but do not contain cartilage or bone [8]. It should be noted, however, that the absence of detectable fat or bone on imaging does not exclude the diagnosis of teratoma [9]. In this patient, the detection on CT scan of fatty tissue and bone within the mass is the key for diagnosis (Question: Fig. 1a–c). The presence of these tissues within a mixed cystic and solid enhancing mass is strongly suggestive of a pre-operative diagnosis of DOI question: http://dx.doi.org/10.1016/j.jocn.2015.01.027
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[email protected] (K. Sim). http://dx.doi.org/10.1016/j.jocn.2015.03.001 0967-5868/Ó 2015 Elsevier Ltd. All rights reserved.
*
Fig. 1. Histology (haematoxylin and eosin stain; 100 magnification) from the section of tumour adjacent to the right posterior clinoid process demonstrates a small island of mature bone (*) embedded in surrounding fat (arrow). This figure is available in colour at www.sciencedirect.com/.
teratoma, as contrast enhancement is rarely seen in dermoid cysts [10]. MRI confirmed the presence of fat adjacent, but separate to, the right posterior clinoid process identified on CT scan as an area of intrinsic high T1 signal with surrounding chemical shift artefact on the T2 sequence (Question: Fig. 1d–f). Histology confirmed the radiological diagnosis of intracranial teratoma (Fig. 1). References [1] Kuratsu J, Ushio Y. Epidemiological study of primary intracranial tumors in childhood. A population-based survey in Kumamoto Prefecture, Japan. Pediatr Neurosurg 1996;25:240–6 [discussion 247]. [2] Smirniotopoulos JG, Chiechi MV. Teratomas, dermoids, and epidermoids of the head and neck. Radiographics 1995;15:1437–55. [3] Liu Z, Lv X, Wang W, et al. Imaging characteristics of primary intracranial teratoma. Acta Radiol 2014;55:874–81. [4] Cassart M, Bosson N, Garel C, et al. Fetal intracranial tumors: a review of 27 cases. Eur Radiol 2008;18:2060–6. [5] Chien YH, Tsao PN, Lee WT, et al. Congenital intracranial teratoma. Pediatr Neurol 2000;22:72–4. [6] Lee YH, Park EK, Park YS, et al. Treatment and outcomes of primary intracranial teratoma. Childs Nerv Syst 2009;25:1581–7. [7] Leech RW, Olafson RA. Epithelial cysts of the neuraxis: presentation of three cases and a review of the origins and classification. Arch Pathol Lab Med 1977;101:196–202. [8] Skovrlj B, Mascitelli JR, Steinberger JM, et al. Progressive visual loss following rupture of an intracranial dermoid cyst. J Clin Neurosci 2014;21:159–61. [9] Buetow PC, Smirniotopoulos JG, Done S. Congenital brain tumors: a review of 45 cases. AJR Am J Roentgenol 1990;155:587–93. [10] Brown JY, Morokoff AP, Mitchell PJ, et al. Unusual imaging appearance of an intracranial dermoid cyst. AJNR Am J Neuroradiol 2001;22:1970–2.