Papillary tumor of the pineal region in a 15-month-old boy

Abstract

<jats:p>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.</jats:p>

Pediatric papillary tumors of the pineal region: to observe or to treat following gross total resection?

“…[5,11]. Among these patients, 10 were children under 16 years of age (Table 1) [5][6][7]12,13]. Clinical manifestations include headache, diplopia, dizziness and vomiting [5,[12][13][14].…”

“…A key feature of PTPR is their high risk for local recurrence. Four out of 6 pediatric patients suffered from local tumor recurrence, the others were lost to follow-up [6,12,13]. Poulgrin et al investigated the 5-year and 10-year progression free survival of all reported cases to date, which has been estimated at 34.5% and 8.6%, respectively [5].…”

Papillary Tumor of the Pineal Region: Clinical Presentations, Prognostic Factors, Treatments, and Outcomes

“…The age distribution of PTPR patients was remarkable. The youngest patient was an infant (a 15 -month -old boy), while the oldest patient was 70 years old (1,8). Three patients were younger than ten years (1.25, 3, and 4 years).…”

“…Proton RT produced complete tumor responses in a four -year -old child (1). Another RT technique is brachytherapy.…”

“…In pediatric patients, pineal tumors constitute 3-4% of CNS tumors. Pineal parenchymal tumors, meningioma, germ cell tumor, glioma metastasis, and arachnoid cyst are the main differential diagnoses in the pineal region (1).…”