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4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Pineocytoma and pineal parenchymal tumour of intermediate differentiation are rare brain tumours of the pineal gland in adults, sitting between the benign end and the aggressive pineoblastoma. Pineocytoma is cured by surgery; the intermediate tumour recurs and spreads through the spinal fluid more often, so radiotherapy is usually added after surgery.
The WHO classification of central nervous system tumours grades the pineal parenchymal tumours from pineocytoma (grade 1) through pineal parenchymal tumour of intermediate differentiation (grade 2 or 3) to pineoblastoma (grade 4); the intermediate tumour is defined molecularly by recurrent small in-frame insertions in KBTBD4 and the absence of the DROSHA deletions or DICER1 mutations of pineoblastoma (Acta Neuropathologica 2019). In the pooled analysis of 127 patients with the intermediate tumour, median age was 33 (range 4.5 to 75), the MIB-1 labelling index a median of 7 percent (range 1 to 30), and adjuvant radiotherapy was widely used with surgery (Radiotherapy and Oncology 2016).
How it differs from its parent: the brain tumours page groups all central nervous system tumours; these are adult tumours of one small midline gland, presenting with hydrocephalus and eye movement disorders, staged with spinal imaging and cerebrospinal fluid because of the risk of seeding, and diagnosed now by methylation profiling and KBTBD4 testing.
| Setting | Approach | Guideline |
|---|---|---|
| Pineocytoma | Complete resection alone. | not mapped |
| Intermediate differentiation | Resection followed by radiotherapy, focal or craniospinal by grade and staging; chemotherapy for recurrence. | not mapped |