Pineal Region Brain Tumour Treatment Revisited: A Case Report

Pineal region tumours may affect only a relatively small subset of neurosurgical patients, but they present enormous surgical challenge to the neurosurgeon due to their inaccessibly deep locations and compounded by the complex surrounding neurovascular structures. We present a case report of a patient who had combination chemoradiation without histological diagnosis but had complete tumour regression. Cyclical combination chemotherapy with cisplatin, etoposide and bleomycin and radiotherapy followed pre- chemoradiation ventriculo-peritoneal shunt insertion for obstructive hydrocephalus. The patient’s clinical condition improved following the ventriculoperitoneal shunt insertion as walking became re-established. Post – chemoradiotherapy cranial CT scan showed complete tumour regression. Tissue diagnosis may allow for precise, targeted management of pineal region tumours. However, in the absence of facilities which enable safe neurosurgery, resorting to the traditional chemo-radiation is still a viable alternative

Pineal region tumours in thesitting position: how I do it

Background Pineal region tumours remain challenging neurosurgical pathologies. Methods Detailed anatomical knowledge of the posterior incisural space and its variations is critical. An opaque arachnoidal membrane seals the internal cerebral and basal veins, leading to thalamic, basal ganglia, mesencephalic/pontine infarctions if injured. Medium-size tumours can be removed en-bloc with all traction/manipulation applied on the tumour side, virtually without contact of ependymal surfaces of the pulvinars or third ventricle. Sacrifice of the cerebello-mesencephalic fissure vein may be required. Conclusions The sitting position offers superior anatomical orientation and remains safe with experienced teams. Meticulous microsurgical techniques and detailed anatomical knowledge are likely to secure safe outcomes.

ETMR-21. META-ANALYSIS OF PINEAL REGION TUMOURS DEMONSTRATES MOLECULAR SUBGROUPS WITH DISTINCT CLINICO-PATHOLOGICAL FEATURES: A CONSENSUS STUDY

Pineoblastomas (PB) are rare, aggressive pineal gland tumours with poor global OS of 50–70% and only 15–49% OS for patients <4 years, despite intensive treatments. Recently, three independent groups (German Cancer Research Centre, Rare Brain Tumour Consortium/SickKids, St. Jude Children’s Research Hospital) collectively analyzed large tumour cohorts and revealed molecular sub-groups of PB. To harmonize and better characterize clinico-pathologic associations of these sub-groups, we undertook a meta-analysis of molecular and clinical data of the combined cohorts. Unsupervised consensus cluster analyses of global methylation data from 227 unique cases identified five robust molecular sub-groups of pineal region tumours: PB_miRNA_1, PB_miRNA_2, PB_MYC/FOXR2, and PB_RB, mainly comprised of pediatric WHO grade 4 PBs and PNETs; and a fifth group: named PPTID, comprised of mainly pineal parenchymal tumours of intermediate differentiation, a WHO grade 2–3 tumour common in adults. PB_miRNA_1 and PB_miRNA_2 tumours, primarily arising in children (median ages 7.7, 11.4y, respectively), were characterized by alterations of miRNA biogenesis genes DICER1, DROSHA, and DGCR8. PB_MYC/FOXR2 and PB_RB groups, arising in infants/toddlers (median ages 1.4, 2.0y, respectively), were distinguished by recurrent MYC gain/amplification and RB1 loss, respectively. The PPTID group affected mainly adults (median age 33y) and exhibited limited CNAs. Higher rates of metastasis were observed with PB_miRNA_1 (42%), PB_MYC/FOXR2 (38%), and PB_RB (75%) tumours, compared to PB_miRNA_2 (20%) and PPTID (25%). Results from ongoing integrative survival analyses of this large cohort will provide critical data for design of future clinical trials.

Pineal Parenchymal Tumour of Intermediate Differentiation: a Case Report

Pineal region tumours are rare, even though it gives rise to diverse array of tumour. Among these, pineal parenchymal tumour of intermediate differentiation is a newly recognized entity. We have described a 56 year old male of pineal parenchymal tumour of intermediate differentiation. As this tumour is very rare with uncertain behavior and optimal management, this case report will add value to the existing database. DOI: http://dx.doi.org/10.3329/pulse.v6i1-2.20356 Pulse Vol.6 January-December 2013 p.60-61