Prognosis and Histopathologic Features in Papillary Tumors of the Pineal Region

Abstract

Papillary tumor of the pineal region (PTPR) is a recently described tumor entity thought to arise from the specialized ependyma of the subcommissural organ. Whereas histologic features of PTPR are well defined, data on the prognostic value of PTPR remain scarce. We therefore investigated clinicopathologic features, including data on progression-free survival and overall survival, in a retrospective series of 31 PTPR. The age of the 14 males and 17 females ranged from 5 to 66 years (median age, 29 years). Histologically, all tumors were characterized by an epithelial-like growth pattern in which the vessels were covered by layers of columnar or cuboidal tumor cells forming perivascular pseudorosettes. Most of the tumor cells showed strong expression of neuron-specific enolase, cytokeratins (particularly CK18), S-100 protein, and vimentin. Most PTPRs examined also expressed microtubule-associated protein-2. Expression of synaptophysin, epithelial membrane antigen, transthyretin, neural cell adhesion molecule, and nestin was encountered in some tumors. Gross total resection could be achieved in 21 of 31 cases; 15 patients received radiotherapy on resection of the primary tumor. Nevertheless, the majority of patients experienced recurrences; 5-year estimates for overall survival and progression-free survival were 73% and 27%, respectively. To conclude, the clinical course of PTPR is characterized by frequent local recurrence, and the value of radiotherapy on disease progression will need to be investigated in future prospective trials.

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].…”

“…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]. Fèvre-Montange et al performed univariate analysis to test the prognostic significance of clinical variables such as age, gender, tumor size, gross total tumor resection and adjuvant radiotherapy [6]. The only clinical factor that tended to be associated with overall survival and recurrence was the extent of resection [6].…”

“…Fèvre-Montange et al performed univariate analysis to test the prognostic significance of clinical variables such as age, gender, tumor size, gross total tumor resection and adjuvant radiotherapy [6]. The only clinical factor that tended to be associated with overall survival and recurrence was the extent of resection [6].…”

“…Among 70 PTPR reported to date, examples are recognized in both children and adults with ages ranging from 5-67 years (mean, 34 years) [5]. PTPR has a distinctive epithelial character and shows both solid and papillary architecture [3,5,6]. Immunohistochemical analysis is crucial to distinguish PTPR from morphological similar pineal tumors, mainly papillary ependymoma and choroid plexus papilloma [7].…”