Datasets
This dataset has been developed for benign and malignant primary tumours of the central nervous system (CNS) and its coverings, as well as tumours from those structures of the peripheral nervous system immediately adjacent to the CNS. This dataset applies to both biopsy and resection specimens of adult and paediatric CNS tumours. Haematological lesions involving the CNS and germ cell tumours are not covered in detail as these are not the primary focus of the CNS dataset. Most sarcomas are not included and are covered by separate ICCR datasets. Secondary tumours of the CNS (for example, metastatic tumours from carcinomas, sarcomas or melanomas in other organs) are not covered in this dataset. Tumours of the pituitary gland are included as the majority of these tumours are reported by neuropathologists worldwide.
Tumours of the Central Nervous System (CNS). The dataset is split into three sections:
This dataset has been kindly sponsored by the Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands, and Stichting STOPHersentmoren, The Netherlands.
2nd edition – September 2024. Updated following the publication of the World Health Organization (WHO) Classification of Tumours of the CNS, 5th edition (CNS5), 2021. The ICCR dataset includes 5th edition Corrigenda, July 2024.
SUMMARY OF CHANGES
This updated version (2nd edition) of the ICCR Tumours of the Central Nervous System dataset includes the following changes:
Histological Assessment section
• Revised guide to harmonise with ICCR style for element titles and value list.
• Revised scope for clarity and to harmonise with ICCR style.
• Prior therapy has been relabelled and incorporated as a non-core sub-value in ‘Clinical information’, the latter containing both core and non-core sub-values.
• ‘Tumour site’ was changed to a core element (applicable for some tumours). Revised guide element to harmonise with ICCR style.
• Revised guide element ‘Tumour laterality’ to be multi-select.
• ‘Histological appearance’ was changed to a core element, applicable for when the element is an essential component of the final (integrated) diagnosis.
• ‘Histological tumour grade’ renamed ‘Tumour grade’ and moved to the Integrated diagnosis section.
• ‘Invasion’ element title was changed to ‘Invasion into surrounding tissue/structures’
• ‘Histological evidence of previous therapy’ minor revisions to wording to harmonise with ICCR style.
• Notes, references and Table 1 were revised; Table 2 added.
Molecular Information section
• Revised guide to harmonise with ICCR style for element titles and value list.
• Revised scope for clarity and to harmonise with ICCR style; Tables 1-4 added.
• New and existing elements for molecular alterations (2-38) were upgraded to core to reflect their status as essential diagnostic criteria in the WHO CNS 5th edition Tumour Classification; commentary added to allow consideration for core elements to be downgraded to non-core until resources allow.
• Revised guide to include ‘Representative blocks for ancillary studies’.
• Added new core Molecular Information elements to align with essential diagnostic criteria in the WHO CNS 5th ed Tumour Classification: ALK/ROS1/MET/NTRK family alterations, BCOR internal tandem duplication, CIC alterations, DICER1 alterations, FET alterations, FOXR2 alterations, MAPK pathway alterations, Methylome profiling, MN1 alterations, MYB/MYBL1 alterations, PDGFRA alterations, PRC2 inactivation, PRKAR1A inactivation, PRKCA inactivation, SHH pathway alterations, TTF1 expression (IHC), WNT pathway alterations, ZFTA rearrangement, and Other immunohistochemistry findings.
• The following Molecular Information elements were omitted (or can be included within other elements): PTEN, Ki-67, medulloblastoma immunohistochemistry, monosomy 6, and RELA.
Integrated Final Diagnosis section
• Revised scope for clarity and to harmonise with ICCR style.
• ‘Histological tumour grade’ was moved to the Integrated diagnosis section and renamed ‘Tumour grade’. Notes were revised.
• Revised guide element ‘Integrated final diagnosis based on’ to also include CNS WHO Tumour Classification, CNS WHO grade, and immunohistochemistry. The value list order was adjusted.
• The note for Diagnosis not elsewhere classified was incorporated into the note for ‘Integrated final diagnosis’.
• Notes, references and Table 1 were revised; Table 2 and a note for ‘Integrated diagnosis based on’ were added.
The 1st edition dataset was developed with the kind support of the Massachusetts General Hospital, Boston.
1st edition – Histological Assessment – August 2018
1st edition – Molecular Information – August 2018
1st edition – Integrated Final Diagnosis – August 2018
The 2nd edition of the dataset for Tumours of the central nervous system was developed by the following international team:
Domain experts:
Authors of the previous edition of this dataset are available here.