Papillary tumor of the pineal region – a recently described entity: A report of three cases and review of the literature

Papillary tumour of the pineal region

“…The most distinctive feature of its immunophenotype is the strong reactivity for a broad spectrum of CK (including Cam5.2, AE1.AE3, CK18 and KL1). 1,2,[4][5][6][9][10][11][13][14][15][16][17][18]20,21,[23][24][25][26][27][28][29]31 Intense CK18 staining is a consistent finding and this marker is also expressed in the specialised ependymocytes arising from the SCO, which lends further support to the proposed histogenesis of PTPR. 2,10,17,23,24,28,30 CK7, CK20 and CK5/6 are predominantly negative in the minority of cases examined.…”

“…In 60% of the 52 cases examined, EMA has shown variable staining intensity, in a membranous, cell surface, small dot or ring-like distribution, similar to ependymomas. 1,2,[4][5][6][7][12][13][14][15][16][17][18][19][20]24,[26][27][28][29][30][31] Focal immunoexpression of transthyretin may be seen, which has been confirmed on microarray analysis and reverse transcriptase-polymerase chain reaction. 3 Transthyretin is known to be expressed in bovine SCO.…”

“…2 MAP2 is positive in most patients. 2,12,17,28,29 Immunoreactivity for nestin and CD99 has been reported. 2,17 Only an occasional patient demonstrates NeuN and weak focal NFP staining.…”

“…Strong staining of S100, NCAM, NSE and vimentin is frequently observed. 1,2,4,5,9,10,[12][13][14][15][16][17][19][20][21]24,26,27,29,30 The tumour may exhibit focal or low immunoreactivity for GFAP and neuronal/neuroendocrine antigens, synaptophysin and chromogranin. In 60% of the 52 cases examined, EMA has shown variable staining intensity, in a membranous, cell surface, small dot or ring-like distribution, similar to ependymomas.…”

Complete regression of papillary tumor of the pineal region after radiation therapy: case report and review of the literature

“…Due to the location and papillary features of PTPR, it must be differentiated from other tumor types that are commonly located in the pineal region, such as papillary ependymomas, papillary meningiomas, choroid plexus tumors, metastatic carcinomas, pineal parenchymal tumors, intraventricular neurocysticercosis and germ cell tumors [1,[12][13][14]. Papillary ependymomas tend to be similar in morphology to PTPR, however, have a distinct immunohistochemical profile that is strongly positive for GFAP in addition to broad spectrum CK (AE1/AE3) [1,15]. Choroid plexus tumors, on the other hand, are similar with respect to immunoreactivity to PTPR, but have more papillary features than that observed with PTPR [1,16,17].…”