third eye blind? pearls for differentiating pineal lesion asnr 2015 eede-68; sn: 956 ammar chaudhry...
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Third Eye Blind? Pearls for Differentiating Pineal Lesion
ASNR 2015
eEdE-68; SN: 956
Ammar Chaudhry MD; Robert Shroyer MD; Alexander Filatov MD; Robert Peyster MD; Lev Bangiyev DO
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Disclosures
• None
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What is the Pineal Gland?
• Functions:1. Neuroendocrine: melatonin synthesis
- Regulated by sympathetic input
2. Biological rhythms (e.g. circadian, puberty)• Histologically: pineocytes (95%) and astrocytes
(5%) separated by fibrovascular stroma• No blood brain barrier enhances
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Why is the Pineal the “Third Eye”?
• Similar Embryologic Development– Evaginates from caudal aspect of third ventricular
roof during 7th week of gestation– Similar to the optic vesicles
• Retains photosensitivity in lower vertebrates– No longer photosensitive in mammals
• Humans: accessory optic pathway– Retinohypothalamic tract reticulo-activating
system autonomic function
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Quadreminal Cistern Anatomy
http://mystatdx.com
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Pineal Region Anatomy on T2 MRI
http://mystatdx.com
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Pineal Region Mass DDX• Germ Cell Tumors (60%)
– Germinoma (40%)– Teratoma (15%)– Malignant GCT NOS
• Pineal Parenchymal Tumors (15%)– Pineocytoma (7%) – WHO Grade I– Pineal Parenchymal Tumor of
Intermediate Differentiation (PPTID) (3%)– Pineoblastoma (6%) – WHO Grade IV– Trilateral Retinoblastoma
• Neoplasms of Adjacent Tissues– Astrocytoma (e.g. Tectal Plate Glioma)– Meningioma– Lymphoma
• Papillary Tumor of the Pineal Region
• Non-neoplastic– Pineal Cyst– Lipoma– Arachnoid Cyst – Dermoid /Epidermoid– Vein of Galen
Malformation– Neurocysticercosis– Neurosarcoid– Medial Atrial
Diverticulum of Lateral Ventricle
– Cavum Velum Interpositum
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Case #1: 11 y/o girl with migraine HA
T1WI T2WI T1 C+
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Pineal Cyst
Case #1: Incidental finding
• Typically clinically silent– Rarely symptomatic
when large of hemorrhage (5%)
• Incidence among women between 21-30 years higher than any other group; F:M = 3:1
• Histology: Non-neoplastic glial-lined protienaceous cyst
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Pineal Cyst Pearls• CT:
– Isodense to slightly hyperdense c/w CSF– Mural calcification (25%), – Multicystic/septated (20-25%)
• MRI: Incidental finding in 1-5% of MRIs– T1: Isointense (40%) to slightly hyperintense
(60%) c/w CSF– T2: Isointense to slightly hyperintense c/w CSF– FLAIR: Does not fully suppress– DWI: Typically no restriction– T1 C+: 90% enhance
• Usually thin rim; nodular/irregular less common• May demonstrate progressive fill-in
• DDx: Pineocytoma, Epidermoid cyst, Arachnoid cyst
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ADC
CT C-
DWISag T1WI C+
Sag T2WI
Case #2: 12 y/o boy with emesis
• Symptomatic at diagnosis: Parinaud syndrome, headache, hydrocephalus– Elevated placental alkaline
phosphatase (PLAP)– ± Elevated β-HCG
• Incidence Highest in young male Asian patients– 90% < 20 y/o (peak: 10-12)– M:F = 10:1 in pineal region
• Histology: Similar to ovarian dysgerminoma and testicular seminoma
Germinoma
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Germinoma Pearls• 80-90% in midline by 3rd ventricle
– Pineal region (50-65%)– Suprasellar (25-35%)– Basal ganglia/thalami (10%)
• CT:– “Engulfs” pineal calcification – High cellularity hyperdense c/w grey matter– When large become cystic, necrotic, and/or
hemorrhagic• MR:
– High cellularity T2 hypointense c/w gray matter– DWI: restricted diffusion– T1 C+: Avid homogeneous enhancement– Look for leptomeningeal seeding and brain
invasion– Image entire spine before surgery
• DDx: Non-germinomatous GCTs, pineoblastoma
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9 y/o boy with four-months of HA and Pineal Germinoma
NCCT T2 T1 C+
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Case #3: 10 y/o boy with headache
Ax T1WI Post
Ax CT C-
Ax T2WI Sag T1WI Pre
Sag T1WI Post
• Teratoma• Congenital midline mass
– Large mass → Macrocephaly
– Pineal origin → Perinaud’s
– Increased serum carcinoembryonic antigen (CEA)
– May rupture and cause chemical meningitis
• Most common in male Asians
• Histology: contains elements of 3 germ layers (ectoderm, mesoderm, endoderm)
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Teratoma Pearls• Suprasellar or pineal in origin
– Origin indeterminate in ~50%• CT:
– Heterogeneous mixture of calcification, soft tissue, multilocular cysts, and fat
• MRI:– High cellularity T2 hypointense c/w gray
matter– DWI: Restricted diffusion in solid components – T1 C+: Solid component enhances
• WHO: mature (cystic), immature, malignant
• DDx: craniopharyngioma, dermoid, germinomatous GCT, pineoblastoma
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20 year old male p/w HA, double vision, and N/V x 2 months; slightly elevated HCG and AFP
• Choriocarcinoma: β-hCG hemorrhage (T1 can mimic fat)• Endodermal sinus tumor: α-fetoprotein (AFP)• Embryonal cell carcinoma: β-hCG and AFP• Difficult to differentiate from other CNS GCTs on imaging• Visual/endocrine symptoms, Parinaud syndrome• Signs of hypothalamic/pituitary dysfunction• +/- hydrocephalus• Female predominance in suprasellar cases• Locally invasive with metastatic potential• Malignant GCTs often histologically mixed
– May exist with both germinomatous, other nongerminomatous GCTs
– Prognosis correlated with most malignant component• Prognosis
– Median survival < 2 years– 5-year survival rate < 25%
• Surgical resection → chemotherapy → neuraxis radiation• Combination of pre- and post-irradiation chemotherapy:
Improved survival
Malignant Germ Cell Tumor (GCT)
Ax CT C-
Ax T1WI Post Ax T2WI FS
Sag T1 Pre
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Malignant GCT• Heterogeneous midline lesions composed of undifferentiated cells
(serum/CSF markers)– Soft tissue ± hemorrhage, cysts, fat– Pineal and suprasellar most common
• Seen in late adolescents (around puberty): peak = 10-15 y/o– M:F = 14:1 for purely pineal lesions– More common in Asians
• Includes: embryonal carcinoma, yolk sac tumor, choriocarcinoma, teratoma with malignant transformation, and mixed GCT– Can have variable leptomeningeal spread– Heterogeneous enhancement with reduced diffusion in solid
components (cellularity)• DDx: dermoid, other GCT, pineoblastoma
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Sag FLAIR
Ax FLAIR Ax T2WI Ax T1WI C+
Sag T1WIC+
Ax CT C-
Case #4: 21 y/o male with worsening headaches x 1 year and new nausea, blurry vision
Pineocytoma• Stable or slow growing
– Symptoms: HA, Parinaud’s, increased ICP, ataxia, hydrocephalus, mental status changes
• Mean age at diagnosis: 35 years– Peak incidence: 10-20
years– No gender predilection– Germ cell markers
negative• Histology: Composed of
small, uniform, mature cells that resemble pineocytes
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Pineocytoma Pearls• WHO grade I
– 100% 5-year survival• CT
– Well circumscribed, typically <3 cm– Isodense to hypodense– Peripheral "exploded" pineal calcifications– Can be cystic with occasional hemorrhage
• MRI– T1W: Isointense to hypointense c/w gray
matter– T2W/FLAIR: Hyperintense c/w gray matter– T1C+: solid, rim, nodular
• DDx: Pineal cyst, PPTID, pineoblastoma, GCT
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DWI
Sag T1WI C+Sag T1WI
Ax T2WI ADCAx CT C-
Case #5: 32 y/o male with severe headache and gait instability
PPTID• Intermediate in
malignancy between pineocytoma and pineoblastoma
• Primary adult parenchymal neoplasm
• Histology: Dysplastic, atypical pineocytes with variable number of mitotic figures
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Pineal Parenchymal Tumor of Intermediate Differentiation
• WHO grade II or III, depending on number of mitotic figures– Infiltrate adjacent structures (e.g., ventricles, tectum, thalamus)– Local recurrence common– Leptomeningeal dissemination rare
• CT– Hyperdense due to increased cellularity– “Engulfs” pineal calcification
• MR– T1: Mixed isointense and hypointense c/w gray matter– T2/FLAIR: Isointense c/w gray matter; small cystic foci– DWI: Not diffusion restricting– T1C+: Strong, heterogeneous enhancement
• DDx: GCT, pineocytoma, pineoblastoma
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Case #6: 3 y/o boy with lethargy and emesis for 1 ½ months
DWI
Sag T1WI Pre
Ax T1WI PostAx T2WI
ADC
• Aggressive symptomatic mass– Hydrocephalus– Headache, nausea, vomiting,
lethargy– Papilledema, abducens nerve
palsy– Parinaud syndrome, ataxia
• Mean age at diagnosis: 3 years– F:M = 2:1– Germ cell markers negative
• Histology: Highly malignant primitive neuroectodermal tumor (PNET) derived from embryonic precursors of pinealocytes
Pineoblastoma
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Pineoblastoma• WHO grade IV
– Median survival 16-25 months – Frequent brain invasion
• Corpus callosum, thalamus, midbrain, vermis
• CT– Large, heterogeneous mass with poorly defined margins– solid component hyperdense– peripheral “exploded”calcifications
• MRI (heterogeneous with necrosis/hemorrhage)– T1W: isointense to hypointense c/w gray matter– T2W: isointense to hypointense c/w gray matter– DWI: Restricted– T1W+: variable heterogeneous enhancement
• Frequent (15-45%) leptomeningeal seeding– Image entire spine preoperatively
• DDx: GCT, pineocytoma, astrocytoma
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Case #7: 4 y/o girl with congenital bilateral retinoblastomas p/w severe HA and vomiting x 4d
Ax T1WI PostAx T2WI Sag T1WI Post
Ax CT C- Ax CT C+
• Combination of bilateral retinoblastoma and midline intracranial neuroblastic mass– 80% pineal, 20% suprasellar– Represents 5-15% of familial lesions;
rarely sporadic cases– Quadrilateral (tetralateral) = bilateral
Rb plus pineal AND suprasellar masses
• 90-95% diagnosed by age 5 years– Sporadic (nongermline): 60%– Inherited (germline): 40%– Prognosis <24 months
• Histology: Primitive neuroectodermal tumor (PNET)
“Trilateral” Retinoblastoma
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• Circumscribed (pilocytic) or diffusely infiltrating
• MRI:– T1: isointense– T2/FLAIR:
hyperintense – T1C+: variable
enhancement• Histology: Typically
low grade
Case #8: 28 y/o male with long standing stable pineal mass
Sag T1WI Post Ax FLAIR Ax CT C-
Tectal Astrocytoma
Sag T1WI Post
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Case #9: 51 y/o woman presented after an episode of severe headache and memory loss
Papillary Tumor of the Pineal Region• Primary tumor of adults• MRI:
– T1W: can be heterogeneously hyperintense– T2W/FLAIR: heterogeneously isointense to hyperintense; can have cystic regions– T1W+: moderate heterogeneous enhancement
• Histology: specialized ependymocytes of the subcommissural organ or ependymal cells of pineal recess
Sag T1WI Post AX T1WI Pre Ax FLAIR Ax T2WI
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Case #10: 43 y/o female with vertigo
• Typically asymptomatic • Congenital malformation of meninx primitive
– Associated anomaly of corpus callosum, cephalocele, or spinal dysraphism in 1/3 cases• Lobulated midline extra-axial mass with fat attenuation/intensity across modalities/sequences
– No enhancement– Variable calcification– May encase vessels and cranial nerves resection difficult
• Histology: Mature non-neoplastic adipose tissue
Pineal Lipoma Sag T1WI FS Post AX T1WI Pre Ax T2WI Sag T1WI Pre
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12 year old girl p/w HA, blurry vision, diplopia, N/V x 4 days
Arachnoid Cyst• Intra-arachnoid CSF-filled cyst• Exerts mass effect• No communication with ventricular
system• Any age; M:F = 3-5:1• Incidental finding on 2% of scans
CT: isodense c/w CSF (unless hemorrhage)MR
T1W/T2W/FLAIR: isointense c/w CSF (unless hemorrhage)T1W+: no enhancementDWI: no reduction
DDx: Epidermoid cyst
Ax FLAIR Sag T2WI Ax CT C- ADC
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19 year old male with progressive HA and ataxia x 1month
• Benign squamous epithelial cyst with dermal elements• Midline fat-containing unilocular cystic lesion• Suprasellar most common location• Rupture can cause chemical meningitis– Subarachnoid lipid droplets– Fat-fluid level in ventricles• CT
– hypodensity (lipid)– fat-fluid level in cyst • MRI
– T1W: hyperintense (lipid)– T2W: heterogeneous– signal drop with fat suppression
• DDx: epidermoid cyst, craniopharyngioma, teratoma, lipoma
Dermoid Cyst Ax T2WI Ax CT C- Sag T1WI Pre
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40 year old male presents following seizure
• Ectodermal inclusions • Intradural (90%), primarily in basal cisterns• Extradural (10%): skull and spine• Lobulated cauliflower-like mass with “fronds”
– Insinuates cisterns and encases neurovascular structures
• Chemical meningitis and CSF seeding with rupture• CT
– > 95% hypodense (CSF-like); rare "dense" variant
• MRI– T1W: typically slightly hyperintense c/w CSF (75%)– T2W: isointense (65%) to slightly hyperintense (35%) c/w
CSF– FLAIR: usually incomplete nulling– T1W+: minimal rim enhancement (25%)– DWI: markedly reduced
• DDx: arachnoid cyst, neurocysticercosis, cystic neoplasm, dermoid cyst
Epidermoid Cyst
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3. Smith AB, Rushing EJ, Smirniotopoulos JG. From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation. Radiographics. 2010 Nov;30(7):2001-20.
4. Osborn AG. Diagnostic Neuroradiology: A Text/Atlas. Elsevier Health Sciences. 1994.
5. Yousem DM, Grossman RI, Zimmerman RD. Neuroradiology : The Requisites, 3rd Edition. Elsevier Health Sciences. 2010.
6. Chang AH, Fuller GN, Debnam JM, Karis JP, Coons SW, Ross JS, Dean BL. MR imaging of papillary tumor of the pineal region. AJNR Am J Neuroradiol. 2008 Jan;29(1):187-9.
7. Amirsys STATdx