OBJECTIVE AND IMPORTANCE: Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a recently recognized, rare type of neoplasm that most commonly presents as a temporal seizure focus among male patients less than 30 years of age. This case represents the only reported suprasellar presentation, with the youngest reported age at diagnosis. CLINICAL PRESENTATION: We present the case of a 12-year-old boy with new-onset diplopia who was diagnosed as having a large suprasellar mass, which was presumptively designated a craniopharyngioma on the basis of its clinical and radiological appearance. INTERVENTION: Gross total resection of a well-encapsulated tumor with adjacent cysts was achieved, without postoperative neurological deficits. Frozen-section studies suggested pilocytic astrocytoma; however, the final histological diagnosis was composite PXA-GG. CONCLUSION: Composite PXA-GG, although extremely rare, may present as a pediatric suprasellar mass. The histopathological diagnosis is contingent on the demonstration of distinct coincident PXA and GG components. The prognosis after gross subtotal resection is likely favorable; however, long-term follow-up monitoring is indicated for these rare neoplasms.
OBJECTIVE AND IMPORTANCE: Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a recently recognized, rare type of neoplasm that most commonly presents as a temporal seizure focus among male patients less than 30 years of age. This case represents the only reported suprasellar presentation, with the youngest reported age at diagnosis. CLINICAL PRESENTATION: We present the case of a 12-year-old boy with new-onset diplopia who was diagnosed as having a large suprasellar mass, which was presumptively designated a craniopharyngioma on the basis of its clinical and radiological appearance. INTERVENTION: Gross total resection of a well-encapsulated tumor with adjacent cysts was achieved, without postoperative neurological deficits. Frozen-section studies suggested pilocytic astrocytoma; however, the final histological diagnosis was composite PXA-GG. CONCLUSION: Composite PXA-GG, although extremely rare, may present as a pediatric suprasellar mass. The histopathological diagnosis is contingent on the demonstration of distinct coincident PXA and GG components. The prognosis after gross subtotal resection is likely favorable; however, long-term follow-up monitoring is indicated for these rare neoplasms.
Authors: Cristiane M Ida; Fausto J Rodriguez; Peter C Burger; Alissa A Caron; Sarah M Jenkins; Grant M Spears; Dawn L Aranguren; Daniel H Lachance; Caterina Giannini Journal: Brain Pathol Date: 2014-12-05 Impact factor: 6.508
Authors: Calixto-Hope G Lucas; Christian J Davidson; Mouied Alashari; Angelica R Putnam; Nicholas S Whipple; Carol S Bruggers; Joe S Mendez; Samuel H Cheshier; Jeffrey B Walker; Biswarathan Ramani; Cathryn R Cadwell; Daniel V Sullivan; Rufei Lu; Kanish Mirchia; Jessica Van Ziffle; Patrick Devine; Ezequiel Goldschmidt; Shawn L Hervey-Jumper; Nalin Gupta; Nancy Ann Oberheim Bush; David R Raleigh; Andrew Bollen; Tarik Tihan; Melike Pekmezci; David A Solomon; Joanna J Phillips; Arie Perry Journal: J Neuropathol Exp Neurol Date: 2022-07-19 Impact factor: 3.148
Authors: Ramón Martínez; F Javier Carmona; Miguel Vizoso; Veit Rohde; Matthias Kirsch; Gabriele Schackert; Santiago Ropero; Werner Paulus; Alonso Barrantes; Antonio Gomez; Manel Esteller Journal: BMC Cancer Date: 2014-03-20 Impact factor: 4.430
Authors: Edvin Telemi; Nikolay L Martirosyan; Mauricio J Avila; Ashley L Lukefahr; Christopher Le; G Michael Lemole Journal: Surg Neurol Int Date: 2019-04-24