Obrecht-Sturm, Denise and Pfaff, Elke and Mynarek, Martin and Bison, Brigitte and Rodehueser, 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 (2024) Pineal anlage tumor: clinical and diagnostic features, and rationales for treatment. JOURNAL OF NEURO-ONCOLOGY, 166 (2). pp. 359-368. ISSN 0167-594X, 1573-7373
Full text not available from this repository. (Request a copy)Abstract
PurposeTo provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT).MethodsProspectively collected data of three patients with pineal anlage tumor from Germany was combined with clinical details and treatment information from 17 published cases.ResultsOverall, 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.ConclusionPAT 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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | CENTRAL-NERVOUS-SYSTEM; PINEALOBLASTOMA; CLASSIFICATION; DIFFERENTIATION; CHILDREN; PINEAL anlage tumor; Pineoblastoma; Pineal gland |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Abteilung für Pädiatrische Hämatologie, Onkologie und Stammzelltransplantation |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 16 Jan 2026 11:57 |
| Last Modified: | 16 Jan 2026 11:57 |
| URI: | https://pred.uni-regensburg.de/id/eprint/65458 |
Actions (login required)
 |
View Item |
