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Pituitary, Midline & Pineal Tumours

Focus is on children's tumours.
Clinical 1

Pituitary Tumours

2 3
Imaging 4

Rare <10y but 25% of all CNS in 15-19 yo.
12% of all CNS tumours in 0-19 yo.

Can present with endocrine, visual problems or headache or obstructive hydrocephalus.
Can be slow and present with cognitive difficulties and behavioural change.
Always asses the overall midline including ? ectopic neurohypophysis.

Name Demographics Pathology Radiology Notes
Adamantinomatous Craniopharyngioma 90% of all neoplasms in kids. Derives from remnant of Rathke's pouch. 70% from intrasellar & suprasellar. Solid within cyst. Solid part enhances. Cyst often proteinaceous. Ca2++. Describing hypothalamic involvement is tricky. Rx:Resection and RT. Recurrence frequent & LT survivors often have sig Morb inc Obesity, panhypopit & cog dysfunction.Papillary Craniopharyngioma is a separate tumour in adults.
Rathke Cleft Cyst (RCC) Ectopic remnant of Rathke's pouch. Centred between anterior & posterior lobes but can extend superiorly. Cyst. But NO enhancement. NO Ca2++. Might be one end of spectrum with CPP.
Chiasmatic Glioma Often PA. Optic pathway = Mostly NF1. But, Chiastmatic glioma mostly NOT NF1. Not arise in adults No Ca2 at presentation. T2:High. Gd:Var. Can be either fusiform thickening and mostly solid OR exophytic and mixed cystic-solid.
Germ Cell Tumours 3% of all paed CNS Germinomas, Non-Germinoma GCT, Teratomas. In Suprasellar or Pineal regions Pit stalk, irregular lesions =>Large Mass with remodelling. Gd:++. ADC:Low. DD: LCH ,Sarcoid.
Pituitary Adenomas Rare in Kids qv Adults MOST endocrine active. Prolactinoma 50%, ACTH-Producing Adenoma 35% , Growth Hormone-Releasing Adenoma 10%. And are micro. Macroadenomas are rare but tend to non-secrete.
Arachnoid Cyst 16% are suprasellar but in kids this is 75%.

Pituicytoma, Granular Cell Tumour & Spindle Cell Oncocytoma are all viewed as 1 group of tumours all related. But might just be different presentations of the same tumour. 5

Pit apoplexy can be haem or non-haem and 70% have underlying macroadenoma. But can be spontaneous.Cato

Pineal Tumours

3
Imaging 6

Name Demographics Pathology Radiology Notes
Germ Cell Tumour Most common 50%. M>>>F Teen. Malignant primordial tissues . Germinomas 50% of all. 20% outside Pineal. Teratomas 2nd: Fat/Ca etc Appear:Hetero. T1:Iso/Low. T2:mild Low->High. ADC:Low.
Pineocytoma All ages. Peak =20-50yo WHO Grade 1. Histo same as normal tissue. Well-defined, High T2 lesions. Gd:Var but not ADC low. But can be mixed cyst-solid and haem.
Gd:Avid,hetero. Ca2:Central Dx:Serum & CSF aFP, bHCG, plALP Rx: Chemo & RT. 5yOS >90%.
Pineoblastoma 10% of Pineal masses. M=F WHO Grade 4. Embryonal. Infiltrative T1:Hetero. T2:Hetero. ADC:Low. Gd:0->Moderate. Ca2:Peripheral (qv GCT) 5yOS<50% [Trilateral Retinoblastoma = Bilateral retinoblastoma + pineoblastoma.

Footnotes

  1. Ramaswamy V et al, Primary Nervous System Tumours in Infants & Children, in Bradley & Daroff's Neurology in Clinical Practice, 75,1122-1144 2022, ClinicaKey. ↩

  2. Bhatia UoPhiladelphia, Supra-sellar Tumours, SPIN 2021. NotSaved. ↩

  3. Choudri A, Pediatric Neuroradiology: Clinical Practice Essentials, Thieme 2017. ↩ ↩2

  4. Seeburg D et al, Imaging of the Sella and Parasellar Region in the Pediatric Poplulation, Neuroimaging Clinics of North America, 2017-02-01, Volume 27, Issue 1, Pages 99-121, Papers+ ↩

  5. Louis D et al, The 2021 WHO Classification of Tumours of the CNS; A summary, Neuro-Oncology 23(8),1231-1251, H&P+ ↩

  6. Tamrazi B et al , Pineal Region Massed in Paediatric Patients, Neuroimag Clin N Am 27 (2017) 85–97 http://dx.doi.org/10.1016/j.nic.2016.08.002 Papers+ ↩