Pilomyxoid astrocytoma (PMA) is a recently described brain tumor. PMA shares similar features with pilocytic astrocytoma (PA), the most common central nervous system (CNS) tumor in the pediatric population, yet displays subtle histologic differences. We describe a case of PMA in a six-year-old male involving sellar and suprasellar region presenting with failure to thrive and delayed developmental milestones. The histological findings revealed a tumor composed of a monotonous population of loosely arranged cells with delicate piloid like processes, within a prominent myxoid background. The tumor lacked biphasic appearance, Rosenthal fibers, eosinophilic granular bodies and calcification that are commonly observed in classical PA. Previous studies have shown PMA to behave more aggressively than PA, with shorter progression-free survival as well as a higher rate of recurrence and CNS dissemination. Thus, recognition of PMA and its distinction from classical PA is very important.
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http://dx.doi.org/10.4103/0377-4929.64283 | DOI Listing |
Neurosurg Rev
December 2024
Department of Neurosurgery and Neuro-Oncology, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, China.
Pilocytic astrocytoma (PA) is a WHO grade I neoplasm with a favorable prognosis. It is the most common pediatric benign tumor. Recently, PA has attracted more and more attention and discussion from scholars.
View Article and Find Full Text PDFPediatr Blood Cancer
February 2025
Department of Neurological Surgery, Oregon Health & Science University, Portland, Oregon, USA.
Heliyon
September 2024
Department of Neurosurgery, Lanzhou University Second Hospital, Lanzhou, Gansu, 730030, China.
There are few reports about primary intracranial granulomas without an identifiable infectious history. A 25-year-old male with intracranial granuloma. The patient presented with a history of tinnitus with intermittent headache for 1 week.
View Article and Find Full Text PDFArch Argent Pediatr
October 2024
Clínica Universitaria Reina Fabiola, Córdoba, Argentina.
Russell's diencephalic syndrome is a set of signs and symptoms characterized by extreme weight loss, with no impairment of height or head circumference, without changes in intake or appetite. It is due to hypothalamic dysfunction associated with space-occupying lesions in this region. It is a rare cause of malnutrition in pediatrics, so its diagnosis is often delayed.
View Article and Find Full Text PDFCureus
August 2024
Neurosurgery, Dr. D. Y. Patil Medical College, Hospital & Research Centre, Dr. D. Y. Patil Vidyapeeth (Deemed to be University), Pune, IND.
Pilomyxoid astrocytoma (PMA) is a subtype of pilocytic astrocytoma (PA). PMA tends to exhibit a more aggressive course compared to PA. We present a case of a two-year-old male with a PMA in the suprasellar region who presented with developmental regression, loss of previously attained milestones such as the ability to hold his neck, walk, and talk, along with hypotonia in all four limbs.
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