title: Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases.
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.
authors.affiliation: INSERM, U433, Fac Med RTH Laennec, Lyon, France. montange@lyon.inserm.fr
authors.forename: Michelle
authors.initials: M
authors.lastname: Fèvre-Montange
authors.forename: Martin
authors.initials: M
authors.lastname: Hasselblatt
authors.forename: Dominique
authors.initials: D
authors.lastname: Figarella-Branger
authors.forename: Laurent
authors.initials: L
authors.lastname: Chauveinc
authors.forename: Jacques
authors.initials: J
authors.lastname: Champier
authors.forename: Ghislaine
authors.initials: G
authors.lastname: Saint-Pierre
authors.forename: Luc
authors.initials: L
authors.lastname: Taillandier
authors.forename: Alix
authors.initials: A
authors.lastname: Coulon
authors.forename: Werner
authors.initials: W
authors.lastname: Paulus
authors.forename: François
authors.initials: F
authors.lastname: Fauchon
authors.forename: Anne
authors.initials: A
authors.lastname: Jouvet
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002291:Carcinoma, Papillary / Q000378:metabolism / Q000401:mortality* / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology / Q000473:pathology; D019635:Neurosurgical Procedures; D010871:Pinealoma / Q000378:metabolism / Q000401:mortality* / Q000473:pathology*; D011379:Prognosis; D011878:Radiotherapy; D012189:Retrospective Studies; D015996:Survival Rate