Pineal Parenchymal Tumors

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1 isclosure of elevant Financial elationships US requires that all faculty in a position to influence or control the content of disclose any relevant financial relationship WH SS which they or their spouse/partner have, or have had, within the past 12 months, which relates to the content of this educational activity and creates a conflict of interest. K H declares he has no conflicts of interest to disclose. isclosure of elevant Financial elationships US requires that all faculty in a position to influence or control the content of disclose any relevant financial relationship WH SS which they or their spouse/partner have, or have had, within the past 12 months, which relates to the content of this educational activity and creates a conflict of interest. r. arik ihan declares he/she has no conflict(s) of interest to disclose. ineal arenchymal umors arik ihan USF he pineal gland riginally described by alen as κωνοειδής (konoeidis) from the pine cone (koukonari) translated to latin as the pineal gland Secretes melatonin, and associated with circadian rhythms he function of this organ has been the subject of many debunked myths... he pine cone & the seat of the soul SS the pineal gland is suspended in air in the middle of the ventricles (But it is not) the pineal gland is full of animal spirits (air), brought to it by many small arteries which surround it (But it is not) these animal spirits inflate the ventricles just like the sails of a ship are inflated by the wind (????) 1

2 2 think therefore am! do not think therefore am! ld hilosophies ransform in the ew World S B XHB etc. etc. ollagen ype V eurofilament

3 ineal arenchymal umors ineocytoma WH rade ineal arenchymal umor of ntermediate ifferentiation WH rade or WH rade V ineocytoma well differentiated pineal parenchymal neoplasm composed of uniform cells with pineocytomatous rosettes and cells with ganglionic differentiation ypically affects adults and may cause hydrocephalus Well-demarcated, often harbors calcifications ypical presentations: headache, papilledema, ataxia, impaired vision, upward gaze palsy, dizziness, tremor an be incidental ineocytoma ineocytoma onomorphous tumor with pineocytomatous rosettes ack of nested, regular reticular network eurofilament and Synaptophysin strong and diffusely positive Variably positive with 2, au, and hromogranin ypically negative for F, but the neuropil in the adjacent parenchyma may be positive. o known positivity with tumor markers roliferation index : low 1-2% 3

4 UF SHS V : ineal arenchymal umor of ntermediate ifferentiation pineal parenchymal tumor between pineocytoma and pineoblastoma in term of cellularity and grade. ypically adults, median age in the fourth decade ften larger than pineocytomas, and often symptomatic causing tectal plate compression and upward gaze palsy adiologically may appear aggressive, lobulated and show marked contrast enhancement. ften hyperintense of 2 and F images and variable on 1- weighted images ccasionally extension into ventricles, tectum and thalamus is observed 4

5 W HS SUBUS arge ell ype n exaggerated pleomorphism in a pineocytoma-like tumor with scattered mitoses Small ell ype Hypercellular monomorphous tumor even though mitoses are rarely seen eurofilament, Synaptophysin positive but may be variable. F, S100, 2, au focally positive Some suggest 6/10 HF mitotic frequency as cut-off between grades and (not adopted by WH) UF SHS S 5

6 S S UF SHS K-67 (B1) S alignant primitive embryonal tumor with aggressive biology ypically in children and infants arge bulky tumors with SF dissemination, either at presentation or later in the disease course. adiologically solid, lobulated tumors with variable intensity on 1 and 2, and marked but heterogenous contrast enhancement. Some tumors ssociated with ermline B1 mutations (chr 13) 2 Some tumors demonstrate somatic 1 mutations (chr 14) 6

7 SHS 18 month old baby with a bulky pineal tumor aleigh et al. euro-ncology 19(1) 78-88, 2017 K-67 (B1) 1 ibonuclease, helicase, role in m splicing leading to repression of gene expression 1 SW/SF family member, helicase and ase activity, chromatin remodeling K5- histone demethylase, also involved in regulation of transcription and chromatin remodeling ractical Suggestions Beware of over-interpreting the normal pineal gland structure as tumor requires further characterization. he cut off value for mitoses has been suggested as 6/10 HF but this requires validation. he small cell and large cell subtypes of are proposed but requires validation is typically seen children/young adults; a diagnosis of pineoblastoma in older patients should be regarded with caution 7

8 mportant nformation egarding /Ss he nline /valuations/ss claim process will only be available on the US website until September 30, o claims can be processed after that date! fter September 30, 2017 you will be able to obtain any or Ss credits for attending this meeting. 8