• Amjad Ali Khan
  • Ahmed Kamal El-Borai


We report the case of a child who presented with diencephalic syndrome. During diagnostic work-up, he was found to have a supra-sellar hypothalamic tumour. Histopathological examination of the tumour revealed it to be pilomyxoid astrocytoma, which is a WHO grade-II tumour, previously considered to be part of the spectrum of WHO grade-I pilocytic astrocytomas. However, because of its characteristic histopathology and behaviour, it was later segregated from pilocytic astrocytomas. In this case report, we discuss the cytological and histopathological features of this tumor with the aim of increasing awareness of this tumour amongst general histopathologists, to highlight the importance of its differentiation from pilocytic astrocytomas in view of its different behaviour, treatment and prognosis and that it should be included in the differential diagnosis of diencephalic syndrome.Keywords: Diencephalic syndrome, Pilocytic astrocytomas, supra-sellar hypothalamic tumour, pilocytic astrocytoma


Linscott LL, Osborn AG, Blaser S, Castillo M, Hewlett RH, Wieselthaler N, et al. Pilomyxoid Astrocytoma: expanding the imaging spectrum. AJNR Am J Neuroradiol 2008; 29:1861–6.

Tihan T, Fisher PG, Kepner JL, Godfraind C, McComb RD, Goldthwaite PT, et al. Pediatric astrocytomas with monomorphous pilomyxoid features and a less favorable outcome. J Neuropathol Exp Neurol 1999;58:1061–8.

Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. World Health Organization Classification of Tumours of the Central Nervous System. Acta Neuropathol 2007;114(2):97–109.

Petito CK. Suprasellar monomorphous pilomyxoid gliomas. AJNR Am J Neuradiol 2003,24:1931–2.

Gottfried ON, Fults DW, Townsend JJ, Couldwell WT. Spontaneous hemorrhage associated with a pilomyxoid astrocytoma. Case report. J Neurosurg 2003;99(2):416–20.

Lin A, Rodriguez FJ, Karajannis MA, Williams SC, Legault G, Zagzag D, et al. BRAF alterations in primary glial and glioneuronal neoplasms of the central nervous system with identification of 2 novel KIAA1549: BRAF fusion variants. J Neuropathol Exp Neurol 2012;71(1):66-72.

Poussaint TY, Barnes PD, Nichols K, Anthony DC, Cohen L, Tarbell NJ, et al. Diencephalic Syndrome: clinical features and imaging findings. AJNR Am J Neuroradiol;18:1499–505.

Komotar RJ, Burger PC, Carson BS, Brem H, Olivi A, Goldthwaite PT, et al. Pilocytic and pilomyxoid hypothalamic/chiasmatic astrocytomas. Neurosurgery 2004;54:72–9.

Komotar RJ, Mocco J, Jones JE, Zacharia BE, Tihan T, Feldstein NA, et al. Pilomyxoid astrocytoma: diagnosis, prognosis, and management. Neurosurg Focus 2005;18(6a):E7.




Most read articles by the same author(s)