"PINEALOMAS”and

Other Pineal Region Masses

James G. Smirniotopoulos, M.D.

Professor of Radiology and NeurologyChairman, Department of Radiology and Nuclear

MedicineUniformed Services University of the Health

SciencesBethesda, MD 20814 USAEmail: jsmirnio@usuhs.mil

Voice: 301-295-3145 Fax: 301-295-3145

Visit us on the WEB at http://rad.usuhs.mil

Disclaimer

The opinions expressed herein are those of the author(s), and are not necessarily representative of the Uniformed Services University of the Health Sciences (USUHS), the Department of Defense (DOD); or the World Health Organization (WHO). Medicine is a constantly changing field, and medical information is subject to frequent correction and revision. Therefore the reader is entirely responsible for verifying the accuracy and relevance of the information contained herein. Portions copyright 1997 James G. Smirniotopoulos, M.D.

Pineal Region

• Anatomy/Physiology• Sx and Signs • Germ Cell Tumors• Non-GCT• Regional Masses

PINEAL GLAND:Introduction

"Seat of the Soul"• Daily (Diurnal) Biorhythms• Life‑cycles

– Puberty, Migration, Mating• Responds to Light/Dark• Third Eye in some Animals (Tuatara)• Melatonin levels• Accessory Optic Pathway• Retinohypothalamic tract, RAS, Sympathetics)

NORMAL PINEAL CALCIFICATION

• Radiology 142:659-66, 1982– 725 Normal Patients– "Youngest was 6 1/2”– 8 ‑ 11% from 8 to 14 yrs.– 30% for 15 y.o.– 39 ‑ 40% from 17 to 20 yrs.

PINEAL REGION MASS - SYMPTOMS/SIGNS

• Specific For Pineal Region– Parinaud Syndrome

• Suggestive Of Pineal-Hypothalamic Axis– Precocious Puberty

• Non-Specific– Headache, Nausea, Vomiting

PARINAUD'S SYNDROME

• Aqueduct/Tectal Syndrome• Failure of conjugate vertical gaze

– lose upward >> downward• Mydriasis, dilated fixed pupils• Failed ocular convergence

– lateral midbrain tegmentum• Blepharospasm (eyelids)

PINEAL REGION:Differential

• Germ cell neoplasms• Pineal cell neoplasms• Gliomas• Non‑neuroglial masses

PINEAL REGION:Why Germ Cells?

• Germ Cells– Multipotential

• "Pure" (Few Divisions)– Sequestered in yolk Stalk

• Migrate to Urogenital Ridge– Gonadal Stem Cells

WHO Classification

• Germinoma (Seminoma)• Embryonal Carcinoma• Yolk Sac (Endodermal sinus)• Choriocarcinoma• Teratoma

– Immature, Mature, Malignant• Mixed Germ Cell

PINEAL REGION MASS

• 369 pts. Reported by HOFFMAN• TUMOR TYPE %• GERM CELL 59 (all types)• Germinoma 39• Teratoma ‑ Malignant 11• Teratoma ‑ Benign 2• Yolk Sac 2• Choriocarcinoma 2• Embryonal CA 1• Mixed Germ Cell 2+

• TRUE PINEAL 14• Pineoblastoma 12• Pineocytoma 2• OTHER NEOPLASMS 27• Gliomas 26• Non‑Glial (Meningioma, etc.) 1• NON‑NEOPLASTIC MASSES

• Epidermoid 1+

GERM CELL TUMORS

• Calcification– Calcification In Pineal Gland– Not in Neoplasm– Premature Pineal Calcification?– Due to Precocious Puberty?

• Neoplasm Surrounds Pineal• Neoplasm Surrounds Calcification

PINEAL REGION NEOPLASMS:Calcification

• PINEAL Ca++ vs. TUMOR Ca++• Germinoma 11/14 0/14• Yolk‑sac 2/6 0/6• Choriocarcinoma 3/4 0/4• Pineocytoma 0/3 2/3

GERMINOMA• Synonyms: Pinealoma, Seminoma, and

Dysgerminoma.• Cell of Origin: Germ Cell rests, 2‑cell histology• Associations: None• Incidence: 1‑2% of ALL Cranial, 2‑4% of Child• upto 14% of Child in Japan and Taiwan• Age: 5‑35 (15)• Sex: 2‑7M/1F for pineal & M=F for suprasellar• Location: 60‑ 80% Pineal, 22% Suprasellar• Treatment: Bx, Radiation, and ChemoTx

Prognosis: >50% at 5yrs. (Radiosensitive)

INTRACRANIAL GERMINOMA

• Central:– Pineal (para‑pineal)– Suprasellar cistern

• Homogeneously Solid– Hyperdense on NCT– Isointense on T1W– Hormonally silent (no AFP/HCG)– immuno+ for PLAP

GERMINOMA

• CT Imaging– Sharply circumscribed,midline mass– Surrounds/Engulfs Pineal Ca++– Less often Thalamic, 3rd vent.,

suprasellar– NCT ‑ Homogeneous Hyperdense– ECT ‑ Homogeneous Enhancement– +/‑ CSF Spread, tumor Ca++– MR ‑ Isointense to Gray

PINEAL REGION NEOPLASMS MR

• AJNR 11:557-565, 1990• Germinoma:

– Iso on T1W– Slightly Hyper on T2W

• Choriocarcinoma:– Hyper on T1W (blood)

• Dermoid, Teratoma:– Hyper on T1W (lipid)

PINEAL REGION Teratoma

• Sharply circumscribed• Lobulated and Lobulated• HETEROGENEOUS

– (mixture of lipid, soft‑tissue, Ca++)

• Enhancement of solid areas

TERATOMA vs. DERMOID

• Teratoma is a Neoplasm – From Multipotential Cells/Tissues– Ectoderm (Skin, Occasionally. Brain)

Common– Lipid from Mesodermal FAT/Sebaceous– Multiloculated, Lobulated

• Dermoid Inclusion Cyst– Only Skin (Ectoderm)– Sebaceous Lipid, Unilocular Mass

PINEAL NEOPLASM

• AJNR 1989;10:1039-1044

Hyperdense on Plain CT Homogeneous Enhancement

• Germinoma 11/14 11/14 (1 min.)• Yolk‑sac 3/6 5/6• Chorio Ca. 4/4 3/4• Embryonal Ca. 1/1 1/1• Pineocytoma 2/3 2/2

• Pineoblastoma 1/1 1/1 (minimal)

Pineal Neoplasms Serum Laboratory Tests

• Neoplasm HCG AFP PLAP• Germinoma -- -- Inc.• Yolk‑sac -- inc.• Chorio Ca. inc. --• Embryonal Ca. inc. inc.

PINEAL PARENCHYMA

• Pineoblastoma (PNET)– Young Patients – Tumor itself calcifies– "Exploded" Pineal Ca++

• Pineocytoma (Mature)– Young or Old (Avg. 35 y.o.)

• Trilateral Retinoblastoma– Heritable, Rb gene, chromosome 13

Pineal Region Masses Other Lesions

• Glioma– Splenium of Corpus Callosum– Tectum of Midbrain – Thalamus– RARE in Pineal itself

Congenital

• Lipoma• Inclusion Cyst

(Epidermoid/Dermoid)• Galen Malformation

Meningioma

• Inferior Falx• Tentorial edge

Vein Of Galen Malformation

• Symptoms, Signs– Childhood– High Output Failure– Persistent Ductus – Hydrocephalus– Cranial Bruit/Thrill

• Adult– Asymptomatic – Pineal Symptoms

Vein Of Galen Malformation Types/Causes

• Parenchymal AVM (Shunt)• Direct Fistulae To Vein• Dural Fistula Drains To Vein• Sinus Thrombosis (fetal)• Hypoplastic Straight Sinus

HYDROCEPHALUS

• MECHANICAL– Aqueductal Obstruction

• Impaired CSF Resorption– Venous Hypertension– Impaired development of

arachnoid villi

INTRACRANIAL LIPOMA

• Congenital, Not Neoplasm• Midline (Usually)• Often Related To Corpus Or BS• Abnormal Differentiation of Meninx

Primativa into Fat

PINEAL REGION SUMMARY

• Germ Cell Tumors are Most Common– Engulfed Pineal Ca++

• Seminoma?– Male, Young, homogeneous, etc.

• Teratoma? (Heterogeneous, Lobulated)

• Pineal Parenchymal Tumors– "Exploded" Ca++

Bibliography

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