A.Bocchio A.Bocchio Regional Hospital Valle d’Aosta, Italy Pilocytic juvenile astrocytoma: a difficult diagnosis?

on examination: (neurologist) presenting at emergency with Strong, pulsating headache and vomiting May,2006 Young male (14 years old) with a remote history of migraine crisis Stroke onset right arm paresis right VII nerve deficit Dysarthria

CT MR DWI (b=1000)

2006, MAY

+Gadolinium

No cardiac malformation or rhythm alterations (ecocardio). Normal hormonal profile (no hypotalamic functional disorders) No thrombophilic disorders Normal csf flow acceleration in left carotid syphon and M1.Transcranial ECD: flow acceleration in left carotid syphon and M1. Patient was subjected to stroke screening Clinical history

The patient was discharged on corticosteroid therapy, with a presumed diagnosis of inflammatory-granulomatous disease involving intracranial branches of left carotid artery,as cause of stroke (+aspirin,for stroke prophylaxis). within 15 days: Symptoms remitted At imaging : Stable left parasellar tissue Appropriate evolution of stroke Clinical history

MR follow-up, over the following 9 months, showed slow increase in the number and volume of perisylvian lesions

AT 2 MONTHS JULY 2006 ON ADMISSION May 2006

AT 5 MONTHS october 2006 AT 7 MONTHS december 2006

SURGERY Pathologic tissue in sylvian fissure and left parasellar region, infiltrating optic nerve, carotid syphon and fronto- basal gyri Subtotal resection At histology: PILOCYTIC ASTROCYTOMA

Post-surgery follow-up At the following controls, patient has been stable, without treatment, till may 2009…

May 2009, 32 months after …

May 2009, at 32 months… March, 2010, at 42 months… No spinal dissemination has been found.

Now… The patient is stable since march 2010, in good general conditions, without medical treatment, apart from antiepileptic drugs

In our case, in view of the apparently aggressive nature of the tumor, apart thinking of a pilocytic astrocitoma (PA), we also raised the possibility of it being a quite recent histologic variant, introduced in the fourth edition of the WHO classification: CONCLUSION Having a more malignant biologic behavior and shorter recurrence-free survival than PA (WHO grade II) This tumor differs both histopathologically and clinically from PA The pilomyxoid astrocytoma (PMA)

Whereas PMA predominantly affects the hypothalamic/chiasmatic region, it may be found anywhere along the central neuraxis CONCLUSION

Although it’s been suggested that an older patient with a hemorrhagic neoplasm in a location atypical for PA should be considered as having a possible PMA CONCLUSION There are no definitive pathognomonic imaging findings to distinguish PMA from PA, PMA remaining a histologic diagnosis Acta Neuropathol (2007) 114:97–109 DOI /s Pilomyxoid Astrocytoma/ Expanding the Imaging Spectrum -- Linscott et al. American Journal of Neuroradiology 29: , November-December 2008 © Identifying an astrocytoma as PMA is important for patient management because a more aggressive treatment may be justified

Thank you