Pineal Parenchymal Tumour of Intermediate Differentiation

Case Contribution: Dr Radhiana Hassan


  • A 43 year old male with no known medical illness.
  • Referred from district hospital with initial CT brain shows pineal region mass with hydrocephalus.
  • Presented with progressively worsening headache for 2 months associated with worsening vision and multiple fall due to giddiness.
  • On examination, GCS E4V5M6, pupil was reactive and symmetrical. He had no opthalmoplegia. No motor deficit. Cerebellar sign was negative.
  • Right burr hole, endoscopic examination with VP shunt was performed.
  • He was extubated post VP shunt and re-admitted for MRI Brain.

MRI findings:

  • A well-circumscribed lesion at the pineal region, mainly hypointense on T1, hyperintense on T2 and not enhanced post contrast suggestive of cystic lesion.
  • An oval lesion periphery to this cystic lesion is seen which is hypointense on all sequence and shows blooming artifact on GRE sequence suggestive of a calcification.
  • A small area suggestive of soft tissue component with enhancement is seen below the area of calcification.
  • Compression to the tectal plate and thalamus on the right side
  • Minimal perilesional oedema

Progress of patient:

  • Underwent sub occipital craniotomy and tumor excision.
  • He was extubated post op day 1 and discharged well after 4 days with review HPE in subsequent clinic follow up.
  • HPE: Pineal parenchymal tumor of intermediate differentiation (PPTID).
  • Post op MRI Brain and Spine were done after oncology consultation;
  • MRI Brain post operative: residual cystic component of pineal lesion with similar size of solid component and calcification.
  • MRI Spine: No MR evidence of drop metastases
  • Patient was planned for gamma knife radiosurgery.

Diagnosis: Pineal parenchymal tumor of intermediate differentiation (PPTID).


  • Mass at pineal region can cause a defect in up-gaxe (Parinaud syndrome) due to compression of tectal plate.
  • It can also cause obstructive hydrocephalus due to compression of cerebral aqueduct.
  • Pineal parenchymal tumours are seen in about 30% of primary pineal region tumours.
  • Pineal parenchymal tumours of intermediate differentiation (PPTID) are tumours that fall between pineocytoma (WHO Grade 1) and pineoblastoma (WHO grade 4). It is considered as WHO grade 2 or 3 tumours
  • The radiographic appearances are also intermediate.
  • PPTID is commonly seen in middle-aged adult aged 20-70 years with slight female predilection (similar to pineocytomas)
  • Can extend into adjacent structures (ventricles, tectum, thalamus). CSF dissemination is rare
  • CT scan seen as hyperdense mass centered in pineal region which engulfs pineal gland calcification. Gross hemorrhage and cysts are rare. Hydrocephalus is common. Post contrast: strong uniform enhancement
  • MRI shows a lesion with T1WI-mixed iso and hypointense, T2WI-isointense to gray matter, small cystic appearing foci is common, FLAIR-hyperintense, GRE-foci of blooming, calcification is common, hemorrhage is rare, post contrast shows strong heterogenous enhancement.
  • Differential diagnosis: germinoma, pineocytoma, pineoblastoma, papillary tumour of pineal region
  • It may invade adjacent structures ad also spread along CSF and therefore imaging of the entire craniospinal axis is required
  • Treatment and prognosis fall between pineocytomas and pineoblastomas
Author: radhianahassan