title: Papillary tumor of the pineal region in a 15-month-old boy.
abstract: The papillary tumor of the pineal region (PTPR) is a distinct entity that is particularly rare in the pediatric population. The authors document the youngest reported patient with this clinicopathological entity to date. A case of PTPR in a 15-month-old boy is described. Initially thought to be a tectal glioma, the tumor was later identified as a pineal region tumor after demonstrating growth on routine imaging. Diagnosis of PTPR was established by histopathological evaluation of biopsy samples, which revealed papillary, cystic, and solid tumor components. The patient's postoperative course was complicated by tumor growth despite several debulking procedures and chemotherapy, as well as persistent hydrocephalus requiring 2 endoscopic third ventriculostomies and eventual ventriculoperitoneal shunt placement. After a 15-month follow-up period, the patient has received proton-beam therapy and has a stable tumor size. The PTPR is a recently described tumor of the CNS that must be included in the differential diagnosis of pineal region masses. The biological behavior, prognosis, and appropriate treatment of PTPR have yet to be fully defined.
authors.affiliation: Division of Pediatric Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
authors.forename: Jessica
authors.initials: J
authors.lastname: Li
authors.forename: Pablo F
authors.initials: PF
authors.lastname: Recinos
authors.forename: Brent A
authors.initials: BA
authors.lastname: Orr
authors.forename: Peter C
authors.initials: PC
authors.lastname: Burger
authors.forename: George I
authors.initials: GI
authors.lastname: Jallo
authors.forename: Violette Renard
authors.initials: VR
authors.lastname: Recinos
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D005500:Follow-Up Studies; D006801:Humans; D006849:Hydrocephalus / Q000601:surgery; D007091:Image Processing, Computer-Assisted; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D018365:Neoplasm, Residual / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D010870:Pineal Gland / Q000473:pathology / Q000601:surgery; D010871:Pinealoma / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D011183:Postoperative Complications / Q000601:surgery; D018714:Radiotherapy, Adjuvant; D014057:Tomography, X-Ray Computed; D017287:Ventriculoperitoneal Shunt