Suprasellar pilocytic astrocytoma: one national centre’s experience
- 396 Downloads
Pilocytic astrocytomas in the supratentorial compartment make up 20 % of all brain tumours in children with only 5 % of these arising in the suprasellar region. Optic pathway gliomas or suprasellar gliomas are often seen in neurofibromatosis type 1 (NF1) patients. Given their location, suprasellar pilocytic astrocytomas are challenging to manage surgically with high morbidity rates from surgical resection. We assess our cohort of patients with suprasellar pilocytic astrocytoma and document our experience.
A retrospective review of patients diagnosed with suprasellar glioma between 2000–October 2012. We included patients diagnosed with optic pathway glioma based on radiological features (with or without biopsy) and those who had a biopsy confirming pilocytic astrocytoma.
Fifty-three patients included (sporadic tumours 24 and NF1 related 29). Fifteen sporadic and four NF1 patients were biopsied. Twelve sporadic and 13 NF1 patients were initially treated with chemotherapy while only 1 patient had radiotherapy initially. Progression was noted in 58 % of the sporadic group and 24 % of the NF1 group. The only significant factor for progression was NF1 status (p = 0.026).
Management should be guided by individual patient circumstance. In our cohort, chemotherapy did not significantly improve progression free survival; however, NF1 status significantly correlated with the decreased progression.
KeywordsPilocytic astrocytoma Suprasellar Optic pathway glioma Neurofibromatosis type 1
The first author is in receipt of an Educational grant from DePuy Synthes.
- 5.Grill J, Laithier V, Rodriguez D, Raquin MA, Pierre-Kahn A, Kalifa C (2000) When do children with optic pathway tumours need treatment? An oncological perspective in 106 patients treated in a single centre. European Journal of Pediatrics 159(9):692–696. doi: 10.1007/s004310000531 PubMedCrossRefGoogle Scholar
- 10.Massimino M, Spreafico F, Cefalo G, Riccardi R, Tesoro-Tess J, Gandola L, Riva D, Ruggiero A, Valentini L, Mazza E, Genitori L, Di Rocco C, Navarria P, Casanova M, Ferrari A, Luksch R, Terenzani M, Balestrini M, Colosimo C, Fossati-Bellani F (2002) High response rate to cisplatin/etoposide regimen in childhood low-grade glioma. Journal of Clinical Oncology 20(20):4209–4216PubMedCrossRefGoogle Scholar
- 11.McKinney P (2004) Brain tumours: incidence, survival and aetiology. Journal of Neurology Neurosurgery Psychiatry 75(suppl 2):ii12–ii17Google Scholar
- 14.Parsa C, Hoyt C, Lesser R, Weinstein J, Strother C, Muci-Mendoza R, Ramella M, Manor R, Fletcher W, Repka M, Garrity J, Ebner R, Monteiro M, McFadzean R, Rubtsova I, Hoyt W (2001) Spontaneuos regression of optic gliomas: thirteen cases documented by serial neuroimaging. Archives of Ophthalmology 119(4):516–529PubMedCrossRefGoogle Scholar
- 15.Piccirilli M, Lenzi J, Delfinis C, Trasimeni G, Salvati M, Raco A (2006) Spontaneous regression of optic pathways gliomas in three patients with neurofibromatosis type I and critical review of the literature. Childs Nervous System 22(10):1332–1337. doi: 10.1007/s00381-006-0061-3 CrossRefGoogle Scholar
- 18.Sharif S, Ferner R, Birch JM, Gillespie JE, Gattamaneni HR, Baser ME, Evans DGR (2006) Second primary tumors in neurofibromatosis I patients treated for optic glioma: substantial risks after radiotherapy. Journal of Clinical Oncology 24(16):2570–2575. doi: 10.1200/jco.2005.03.8349 PubMedCrossRefGoogle Scholar
- 20.British Neuro-oncology Society/National cancer Action team (2011) Guidelines on the diagnosis and management of Optic Pathway Glioma (OPG): Rare Brain and CNS Tumour Guidelines. http://www.bnos.org.uk/documents/rare_tumours_guidelines/Optic%20pathway%20glioma%20guidelines.pdf. Accessed 19 Feb 2013
- 24.von Hornstein S, Kortmann R, Pietsch T, Emser A, Warmuth-Metz M, Soerensen N, Straeter R, Graf N, Thieme B, Gnekow A (2011) Impact of chemotherapy on disseminated low-grade glioma in children and adolescents: report from the HIT-LGG 1996 Trial. Pediatric Blood & Cancer 56(7):1046–1054CrossRefGoogle Scholar