Pineal anlage tumor: clinical and diagnostic features, and rationales for treatment
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Pineal anlage tumor: clinical and diagnostic features, and rationales for treatment. / Obrecht-Sturm, Denise; Pfaff, Elke; Mynarek, Martin; Bison, Brigitte; Rodehüser, Martina; Becker, Martina; Kietz, Silke; Pfister, Stefan M; Jones, David T; Sturm, Dominik; von Deimling, Andreas; Sahm, Felix; Kortmann, Rolf-Dieter; Schwarz, Rudolf; Pietsch, Torsten; Fleischhack, Gudrun; Rutkowski, Stefan.
in: J NEURO-ONCOL, Jahrgang 166, Nr. 2, 22.01.2024, S. 359-368.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › Case Report › Forschung › Begutachtung
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TY - JOUR
T1 - Pineal anlage tumor: clinical and diagnostic features, and rationales for treatment
AU - Obrecht-Sturm, Denise
AU - Pfaff, Elke
AU - Mynarek, Martin
AU - Bison, Brigitte
AU - Rodehüser, Martina
AU - Becker, Martina
AU - Kietz, Silke
AU - Pfister, Stefan M
AU - Jones, David T
AU - Sturm, Dominik
AU - von Deimling, Andreas
AU - Sahm, Felix
AU - Kortmann, Rolf-Dieter
AU - Schwarz, Rudolf
AU - Pietsch, Torsten
AU - Fleischhack, Gudrun
AU - Rutkowski, Stefan
N1 - © 2024. The Author(s).
PY - 2024/1/22
Y1 - 2024/1/22
N2 - PURPOSE: To provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT).METHODS: Prospectively collected data of three patients with pineal anlage tumor from Germany was combined with clinical details and treatment information from 17 published cases.RESULTS: 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.CONCLUSION: 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.
AB - PURPOSE: To provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT).METHODS: Prospectively collected data of three patients with pineal anlage tumor from Germany was combined with clinical details and treatment information from 17 published cases.RESULTS: 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.CONCLUSION: 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.
KW - Adolescent
KW - Adult
KW - Child
KW - Child, Preschool
KW - Humans
KW - Infant
KW - Young Adult
KW - Brain Neoplasms/diagnosis
KW - Neoplasm Recurrence, Local/pathology
KW - Pineal Gland/surgery
KW - Pinealoma/diagnosis
KW - Recurrence
KW - Supratentorial Neoplasms/pathology
KW - Treatment Outcome
U2 - 10.1007/s11060-023-04547-5
DO - 10.1007/s11060-023-04547-5
M3 - Case report
C2 - 38253790
VL - 166
SP - 359
EP - 368
JO - J NEURO-ONCOL
JF - J NEURO-ONCOL
SN - 0167-594X
IS - 2
ER -