Rosette-Forming Glioneuronal Tumor in the Pineal Region: A Series of 6 Cases and Literature Review.
Chun-Chieh LinMahesh M MansukhaniJeffrey N BrucePeter D CanollGeorge ZanazziPublished in: Journal of neuropathology and experimental neurology (2021)
Resected lesions from the pineal region are rare specimens encountered by surgical pathologists, and their heterogeneity can pose significant diagnostic challenges. Here, we reviewed 221 pineal region lesions resected at New York-Presbyterian Hospital/Columbia University Irving Medical Center from 1994 to 2019 and found the most common entities to be pineal parenchymal tumors (25.3%), glial neoplasms (18.6%), and germ cell tumors (17.6%) in this predominantly adult cohort of patients. Six cases of a rare midline entity usually found exclusively in the fourth ventricle, the rosette-forming glioneuronal tumor, were identified. These tumors exhibit biphasic morphology, with a component resembling pilocytic astrocytoma admixed with variable numbers of small cells forming compact rosettes and perivascular pseudorosettes. Targeted sequencing revealed a 100% co-occurrence of novel and previously described genetic alterations in the mitogen-activated protein kinase (MAPK) and phosphoinositide 3-kinase (PI3K) signaling pathways, suggesting a synergistic role in tumor formation. The most common recurrent mutation, PIK3CA H1047R, was identified in tumor cells forming rosettes and perivascular pseudorosettes. A review of the literature revealed 16 additional cases of rosette-forming glioneuronal tumors in the pineal region. Although rare, this distinctive low-grade tumor warrants consideration in the differential diagnosis of pineal region lesions.
Keyphrases
- low grade
- single cell
- signaling pathway
- end stage renal disease
- induced apoptosis
- prognostic factors
- germ cell
- ejection fraction
- oxidative stress
- high grade
- healthcare
- pulmonary hypertension
- heart failure
- chronic kidney disease
- cancer therapy
- peritoneal dialysis
- dna methylation
- gene expression
- drug delivery
- cell death
- spinal cord
- pulmonary artery
- left ventricular
- congenital heart disease
- low cost