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@ARTICLE{ObrechtSturm:267496,
author = {Obrecht-Sturm, Denise and Pfaff, Elke and Mynarek, Martin
and Bison, Brigitte and Rodehüser, Martina and Becker,
Martina and Kietz, Silke and Pfister, Stefan M and Jones,
David T and Sturm, Dominik and von Deimling, Andreas and
Sahm, Felix and Kortmann, Rolf-Dieter and Schwarz, Rudolf
and Pietsch, Torsten and Fleischhack, Gudrun and Rutkowski,
Stefan},
title = {{P}ineal anlage tumor: clinical and diagnostic features,
and rationales for treatment.},
journal = {Journal of neuro-oncology},
volume = {166},
number = {2},
issn = {0167-594X},
address = {Dordrecht [u.a.]},
publisher = {Springer Science + Business Media B.V},
reportid = {DZNE-2024-00142},
pages = {359 - 368},
year = {2024},
abstract = {To provide a treatment-focused review and develop basic
treatment guidelines for patients diagnosed with pineal
anlage tumor (PAT).Prospectively collected data of three
patients with pineal anlage tumor from Germany was combined
with clinical details and treatment information from 17
published cases.Overall, 20 cases of PAT were identified (3
not previously reported German cases, 17 cases from
published reports). Age at diagnosis ranged from 0.3 to 35.0
(median: 3.2 ± 7.8) years. All but three cases were
diagnosed before the age of three years. For three cases,
metastatic disease at initial staging was described. All
patients underwent tumor surgery (gross-total resection: 9,
subtotal resection/biopsy: 9, extent of resection unknown:
2). 15/20 patients were alive at last follow-up. Median
follow-up for 10/15 surviving patients with available
follow-up and treatment data was 2.4 years (0.3-6.5).
Relapse was reported for 3 patients within 0.8 years after
diagnosis. Five patients died, 3 after relapse and 2 from
early postoperative complications.
Two-year-progression-free- and -overall survival were 65.2
± $12.7\%$ and 49.2 ± $18.2\%,$ respectively. All 4
patients who received intensive chemotherapy including
high-dose chemotherapy combined with radiotherapy (2 focal,
2 craniospinal [CSI]) had no recurrence. Focal radiotherapy-
and CSI-free survival rates in 13 evaluable patients were
$46.2\%$ (6/13) and $61.5\%$ (8/13), respectively.PAT is an
aggressive disease mostly affecting young children.
Therefore, adjuvant therapy using intensive chemotherapy and
considering radiotherapy appears to comprise an appropriate
treatment strategy. Reporting further cases is crucial to
evaluate distinct treatment strategies.},
subtyp = {Review Article},
keywords = {Child / Humans / Child, Preschool / Infant / Adolescent /
Young Adult / Adult / Treatment Outcome / Pineal Gland:
surgery / Pineal Gland: pathology / Neoplasm Recurrence,
Local: pathology / Pinealoma: diagnosis / Pinealoma: surgery
/ Supratentorial Neoplasms: pathology / Brain Neoplasms:
diagnosis / Brain Neoplasms: surgery / Recurrence / PINEAL
anlage tumor (Other) / Pineal gland (Other) / Pineoblastoma
(Other)},
cin = {Brainbank Unit Bonn},
ddc = {610},
cid = {I:(DE-2719)1011009},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:38253790},
pmc = {pmc:PMC10834633},
doi = {10.1007/s11060-023-04547-5},
url = {https://pub.dzne.de/record/267496},
}