Pineal region masses: differential diagnosis.
Transcription
Pineal region masses: differential diagnosis.
From the Archives of the AFIP This article meets the criteriafor 1.0 credit hour in Category 1 of theAMA Physician’s Recognition Award. To obtain credit, see the questionnaire at the end ofthe article. Pmeal Region Masses Differential : #{149} Diagnosis James G. Smirniotopoulos, Hernando Mena, LTC, Most young pineal male neous mass with signal the mass engulfs matter; tilocular region patients. masses bryonal or be seen histologic in patients diagnosis phologic logic have verification neoplastic. U INTRODUCTION pineal region is a central cisterns ventricle; of the and the overlying occur splenium in this area ofchildhood occurrence terms: (krm #{149} Pineal From ccli c opinions RSNA. pmneal location corpus that and the Institute 147.3629 Pincal the of all intracranial CSF lxdy. that pineal = Pincal #{149} to gland; the the poste- thalami, and lesions and that about 4% States (1). However, the the reported rate cx- cc-rcbrospinal 1-17.1346 few histo- appear various masses in the United in Asia, where of Pathology, #{149} masses Combined, 1%-3% Thus, and velum interpositum; of the brain stem, the callosum. than very pattern. the may the correct of the mor- characteristics, includes calcifiand Although evaluation region plate tissues pineal fluid body. CT. 147.121 1 Pincal #{149} body. MR. 147.3621 of Radiologic Md (J.G.S.); or as reflecting ncoplasm. DC 20306-6000 and DC (J.G.S.). Receivedjanuary Address reprint requests The intensity empineo- 12:577-596 I)cpartmcnt Washington, Bethesda. Forces or older. a careful mul- and either predilection imaging most midline less neoplasms, 1992; the Sts, signal quadrigemmnal adjacent solid of the Armed = body. RadioGraphics I for represent AFIP 147.1214 and intracranial masses encountered of pineal masses is notably higher Abbreviations: Index of age with sexual are of pineal tumor, usually preexisting no of gray types sinus are “explode” have pathognomonic is necessary be may that occur in is a homoge- Teratomas Other endodermal 20 years suggested a truly areas. neoplasms they are be USN to those gland. lipid parenchymal attenuation, tumors surrounding nor third containing which MC, similar pineal choriocarcinoma, who may features, of these attenuation calcified gei-minomas, LCDR, germ cell neoplasms is a germinoma, which and pineoblastomas, the Rushing malignant common a densely Pineal Unlike the are most intensity include cations. #{149} ElisabetbJ. masses carcinoma. cytomas Fern masses The heterogeneous region The MD USA MC, assertions views the Pathology. (J.G.S.. E)epartments Forces H.M.); ofRadiology 2, 1992; toJ.G.S. revision contained herein of the Armed E.J.R., Departments Institute the and Neurology. requestedJanuary are of the the views or the Navy Bldg Services received of the or the authors Rm M- 1 2 1. Ala.ska 54 Universityofthc Georgetown 30 and private Army of Pathology, Uniformed Health University February and Department are Hospital. 11: accepted not to he construed and Sciences, Washington. February 14. as official of Defense. 1992 577 ceeds of the 9% of all intracranial neoplasms seen here unique to this tinctly uncommon tumor to the a limited region list. U NORMAL of the brain elsewhere. pineal and tic masses (1). are peculiar and are dis- Localizing region yet Most or allows accurate a one differential ANATOMY, to offer scan diagnos- quadrigeminal plate cistern is a U-shaped structure that surrounds the pineal gland and the dorsal brain stem and has attenuation equal to that ofCSF. However, on MR and CT images obtained in a true horizontal plane, the quadrigeminal plate cistern is a triangle pointing posteriorly, with its base against the tectal plate. The adjacent fluid-filled spaces provide good imaging contrast on both Tiweighted MR images and CT scans (Fig 1). Another important anatomic landmark of the pineal region is the pair ofvessels imrnediately above the pineal gland; these are the internal cerebral veins. Just behind the pineal gland, these join with the basal veins to form the great vein of Galen, and, above these veins, is the splenium of the corpus callosum. The internal cerebral veins are readily identifled as tubular enhancement on CT scans obtamed after contrast material is administered and as curvilinear signal intensity voids cxtending anteriorly into the cistern of the yelum interpositum on plain MR images. Pineal and parapineal masses elevate these PHYSIOLOGY, AND EMBRYOLOGY The pineal gland is a small reddish-brown structure that is usually approximately 8 mm long and 4 mm wide and is attached to the upper aspect of the posterior border of the third ventricle within the (Fig 1). It lies cerebrospinal quadrigeminal in the fluid plate cistern midline, (CSF) and of the behind the third ventricle. Anterior to the pineal gland is the cistern of the velum interpositum (cistern of the transverse fissure). The pineal gland develops from the most caudal portion of the roof of the third ventricle, from an area of ependymal thickening that evaginates tion (2). within the ventricle tion of the that pineal control 7th week is a patent of gestacavity connects is lined to the third by ependyma. gland of certain and the there gland and regulation els during Initially, The in humans func- is twofold: circulating of long-term hormone (eg, onset 1evof pu- berty) and short-term (eg, diurnal or circadian) biologic rhythms. The relationship between tumors of the pineal region and the precocious onset of puberty was documented at the end suggested ited of the that sexual past the century, normal when pineal it was gland inhib- pineal gland development. In many lower is superficial and vertebrates, the has photoreceptor a direct function; in some species of lizard, the formation of a lens overlying a portion of the gland transforms the pineal gland into a dorsal “third eye. Even in humans, the regulation of biologic rhythms by the pineal gland responds to photoperiodic cues. The pineal gland receives this light or dark information “ via an accessory optic pathway that One landmark for imaging the pineal region with computed tomography (CT) and magnetic resonance (MR) is the quadrigeminal plate cistern (Fig 1). On a routine axial CT begins as the retinohypothalamic tract, passes through the spinal cord by means of the reticular activating system, and reaches the gland through both sympathetic and parasympathetic innervation (3). The pineal gland has two populations ofcells. About 95% are a neuronal type cell or pinealocyte with dendritic processes, and the other 5% are neuroglial supporting cells that resemble astrocytes. obtained veins, at the whereas usual callosal inclined masses angle, depress them. Just below rigeminal the pineal gland are the four quadbodies composed of the two supenor and two inferior colliculi. These form the roof of the dorsal midbrain, which is also called the tectum of the mesencephalon. The normal pineal gland may not always be visualized within the quadrigeminal plate cistern because of its small size and volume averaging. It may appear isointense to gray matter on MR images and have attenuation equal to that ofgray matter on CT scans. When the pineal gland is calcified, the effects of volume averaging are minimized on CT scans, and it is invariably visualized. Normal pineal calcifications are scans than (Fig observed more they are on frequently plain on CT radiographs 1). In a large tients, review physiologic of over calcification 700 healthy pa- at CT was set of puberty, the prevalence of normal RadioGraphics #{149} Smirniotopoulos Ct a! pi- neal calcification increases from only 8% to 1 1% at age 8-14 years to 40% by age 20 years (4). Pathologic studies suggest that pineal caldo not change substantially with age, and, although some glands never most develop at least some microscopic U not noted in patients younger than age 5 years but increased in prevalence dramatically and almost logarithmically with age. After the on- cifications 578 the Volume calcify, calci- 12 Number 3 fr’ :r .-.. .,.. ‘s ‘, -.., ‘4p . P - C- d. 1. (a) Normal pineal gland. Axial dissection is a downward view ofthe third ventricle and the thalami; the velum interpositum has been removed. The pineal gland (*) is a pinecone-shaped mass at the posterior margin of the third ventricle. Directly underneath are the two superior colliculi of the midbrain roof (tectum or tectal plate, arrowheads). The space behind the midbrain is the quadrigeminal plate cistern. The triangular space anterior to the pineal gland is the cistern of the velum interpositum, bounded laterally by the curving medial borders of the pulvinar (P) ofthe thalami. This cistern extends anteriorly over the roofofthe third ventricle (removed) to the foramen of Monro and the paired fomices (F). The septum pellucidum connects the fornices with the genu of the corpus callosum (GC). (b) Normal pineal gland in a 17-year-old girl (who also has tuberous sclerosis). CT scan shows calcification in a normal pineal gland (*). (The calcifications in the right frontal horn are subependymal nodules of tuberous sclerosis.) Note the triangular space anterior to the pineal gland, bound by the pulvinar of the thalami (arrowheads). The axial plane of the CT section is actually at an angle to the true horizontal plane, and the quadrigeminal plate cistern (Q) appears U shaped. Axial Ti- (c) and T2-weighted (d) MR images show the quadrigeminal plate cistern as a triangle pointing backward (Q) from the pineal gland. The great vein ofGalen lies within the quadrigeminal plate cistern. The anterior extension of the quadrigeminal plate cistern is the cistern of the velum interpositum (or cistern ofthe transverse fissure). It is a triangle pointing forward and contains the tubulax signal intensity voids of the paired internal cerebral veins (arrowhead). Figure May 1992 Smirniotopoulos Ct a! U RadioGraphics U 579 fications at puberty (5). However, the pres- AFIP ence of pineal calcification does not correlate with any change in pineal hormone activity and is not a sign of dysfunction (5). Although calcifications within the pineal gland are usually normal, they are also often seen in association plasms. with Calcifications in the masses monly pineal within calcium the deposits However, and displace preexisting the region occur gland within the may pineal types neoplastic have normal Embryonal Teratoma as teratoma, dysgermi- carcinoma Benign, mature Benign, immature (> 10% immature Malignant Malignant may calcifications multipotential tissues) calcifi- tumors from Masses noma) tissue. tumoral (derived Embryonic differentiation Germinoma (atypical comthan cell tumors Region cells) with most rather parenchymal Germ of neo- in association pineat teratomas cations, various seen of Pineal Classification immature teratoma (histologi- caily benign but metastatic) Extraembryonic differentiation to periphery. Choriocarcinoma SYMPTOMS MASSES Pineal signs twisting enough rial masses and headache. may symptoms the nonspe- in this normal or may be severe transtentoarea may function Neoplasms tissues ‘ ‘ (intrinsic) Less commonly, with pineal region hypogonadism masses rather gland) elements or adjacent Ependymoma pi- Meningioma and three associated of supporting the Astrocytoma also of the Lymphoma Nonneoplastic Pineal cyst Lipoma Arachnoid Dermoid Vascular Metastatic masses cyst or epidermoid malformations (inclusion cysts) neoplasms are than precocious puberty. Diabetes insipidus may occur if the mass has either extended directly downward or seeded the CSF to involve the anterior or inferior recesses of the third yentricle and hypothalamus. Pineal region masses may produce a classic U PINEAL REGION GERM CELL TUMORS which the patient is unable to upwardly dcviate the eyes and has impaired ocular convergence. This syndrome results from the effects of pressure on the reflex nuclei of the quad- Masses of the pineal region are commonly referred to as pinealomas. This term was introduced by Krabbe as a generic name for pineal masses. This seemed appropriate at the time because, with routine light microscopy, the histologic features of many of these tumors appeared to resemble pineal cells. However, later research in pineal masses, coupled with the advent of immunohistochernical rigeminal stains neurologic syndrome, plate. defined In more advanced by Parmnaud, cases, in both upward and downward gaze may be impaired. When a patient presents with a paralysis of upward gaze, the possibility of a pineal region mass should be considered. Health nervous RadioGraphics U Smirniotopoulos Ct a! and ultrastructural studies (electron microscopy) revealed that most of these pineal region tumors were not of pineal parenchymal origin (6). Therefore, pinealoma is a misnomer. The Table summarizes the current terminology used at the Armed Forces Institute of Pathology (AFIP) for pineal tumors. This U ( ‘yolk sac’ ) tumor neoplasms Astrocytoma (within Mixed neoplasms lead to precocious puberty. theories to explain precocious puberty: (a) interference with the norma! antigonadotropic effect of the gland, (b) secondary invasion into the diencephalon with destruction of the sexual inhibitory function of the median eminence, and (c) ectopic production of gonadotropins by the neoplasm. 580 sinus parenchymal Pineobtastoma and obstruction aqueduct downward Masses with neal gland There are Pineal as seizures from of the sylvian to precipitate herniation. produce such Hydrocephalus interfere Endodermal PINEAL Pineocytoma region cific TO RELATED U classification is modified from Organization classification system tumors. Volume the World of central 12 Number 3 Figure pecially splenium year-old virtually internal 2. (a) Semischematic of a pineal mass. The sagittal The most common lesions of this region (over two-thirds) are neoplastic derivatives of multipotential embryonic germ cells that are similar, if not identical, to gonadal germ cell tumors. The germ cell tumors are classified by cell type and may be benign or malignant. Within the germ cell tumor group, there is a separation between neoplasms that form embryonic tissue and those that form extraembryonic tissues. Primordial germ cells in mammals develop from relatively undivided embryonic cells of the yolk sac wall, near the allantois. In normal development, they move from the yolk sac, along its wall, and through the dorsal tery of the hindgut. During week, they invaginate into (2). This movement occurs neously with the development cephalon (eg, thalamus, mesen- the 6th gestational the genital ridges virtually simultaof the dien- hypothalamus) and pineal gland (at 5-8 weeks). Thus, it is possible that misguided germ cells could become embedded in or persist to surround the pineal gland. Aberrant migration may also cxplain other extragonadal germ cell tumors that arise in the retroperitoneum, sacrococcygeal area, and mediastinum (1,6,7). May 1992 plane is ideal those in the pineal and sellar regions. The internal cerebral veins ofthe corpus callosum from the pineal gland below. (b) Sagittal boy with a germinoma demonstrates a homogeneous mass in the equal to that of gray matter. The germinoma is just inferior to the cerebral veins (arrowhead). for imaging alt midline masses, es- (heavy black line) separate the Ti-weighted MR image ofa 13pineal region, with signal intensity linear signal intensity void of the Germinoma Germinorna, formerly called atypical teratoma, is histologically identical to ovarian dysgerminorna and testicular seminoma. Germinorna (Figs 2, 3) is the most common type of pineal region mass, accounting for twothirds of the germ cell tumors and 40% or more of all pineal region neoplasms. About 80% of intracranial germinornas are in the pincal area; the other 20% localize near the suprasellar region, either in the third ventricle or in the cistern. Male patients are affected two to 17 times more often than female patients. The peak age of presentation is in the 2nd decade, and few patients are older than age 30 years at initial observation. A substantial number ofyoung children ( < 10 years of age) have germinorna in association with precocious puberty. I Germinoma is a malignant tumor corn- posed of a mixture oflarge multipotential primitive germ cells about 15-30 pm in diameter and smaller cells that resemble lymphocytes (some pathologists believe they arc reac- Smirniotopou!os et a! U RadioGraphics U 581 C. C. Figure 3. Pineal germinoma in a 2 1-year-old man. (a) Nonenhanced CT scan demonstrates a mass in the region of the pineal gland, extending into the dorsal midbrain (tectum). The right side ofthe mass has higher attenuation (arrowhead) relative to brain. (b) Axial Ti-weighted MR image demonstrates a hypointense mass (*) involving the tectum. (c) The mass appears highly enhanced after administration ofgadopentetate dimeglumine (Magnevist; Berlex, Wayne, NJ). (d) Plain sagittal Ti-weighted MR image shows slight heterogeneity. (e) After administration of gadopentetate dimeglumine, the MR image shows homogeneous enhancement, with compression and possible extension into the brain stem. 582 U RadioGraphics U Smirniotopoulos Ct a! Volume 12 Number 3 ma with seeding. (a) Sagittal Tiof a 22-year-old man demonstrates tat are homogeneous and are equal at of gray matter. There is a domical region and a second smaller mass tern. (b) Coronal Ti-weighted MR atient amine after administration of gademonstrates abnormal enarged pituitary infundibulum, which rom the pineal germinoma. (c) Sagit- brained at autopsy of a different paLtes two prominent masses. The origie large mass in the pineal region. The suprasellar cistern, represents CSF ther, these two masses compress and Th The pineal mass extends well be- nd encroaches on the roof of the iso spreads anteriorly, to the foramen he cistern of the velum interpositum tive lymphocytes). Because germinomas Germinomas are homogeneous not encapsulated, they may invade the adjacent structures of the brain (eg, thalamus) and easily spread along the surfaces of the brain or through the flow of CSF (Fig 4). At initial presentation, disseminated the patient commonly has disease. Germinomas are plasms and respond therapy. Currently, very radiosensitive well to specific cytotoxic agents vinblastine Adriamycin sulfate, neochemosuch as (doxorubicin hydrochloride; Adria Laboratories, Columbus, Ohio), cisplatin, and cyclophosphamide are used for both intracranial and testicular germ cell tumors (1). The overall prognosis has irnproved with combined therapy and is relatively good, with a 5-year survival of 75% after radiation therapy alone. May 1992 have a strong masses that tendency have either to be attenu- ation higher tamed Ganti pineal equal to that of gray matter or slightly than that of brain on CT scans obwithout the use of contrast material. et al (8) reported that all 15 cases of germinomas studied without contrast material had attenuation higher than that of brain (8). They also noted that in six cases the neoplasm was clearly outside the pineal gland but had surrounded and engulfed it. The pineal gland was seen usually as a very prominent and calcified structure within the tumor mass. Smlrniotopou!os Ct a! U RadioGraphics U 583 The normal tions prevalence detected at CT of pineal This is true even noma is in the in the pineal ports (1 1) have indicated tions seen germinomas when suprasellar area with the cistern (9,10). to a ger- germi- rather Although that miliar 40%, but, for unknown reasons, this increases almost 100% when the patient also has minoma. This calcifica- is approximately than most the re- calcifica- represent the engulfed pineal gland, Ganti et at (8) reported calcification within the substance of the tumor in 10 of their 16 cases. CSF seeding of the subarachnoid space or the ventricles was also On noted in five MR images, nomas tends of their the to be 16 cases signal equal (Fig intensity to that 4). of germi- of gray matter on images obtained with both the short and long pulse sequences; they are relatively homogeneous masses. Small cystic areas were noted in only two of 10 cases reported by Zee et at (1 1). The findings ofa noncalcified, homogeneous pineal region mass with signal intensity equal to that of gray matter seen in a young male patient are strongly suggestive of a germinoma histologically. Germinomas ap- pear highly enhanced and usually homogeneous on both CT and MR images obtained with contrast material. However, this enhancement is rather nonspecific, since the majority of neoplasms in this region do not have an intact blood-brain barrier. Gadolinium-enhanced MR imaging is recommended to help identify metastases from CSF seeding. . is more a male predilection Teratomas cells are that usually ture of two and tissues or more organogenesis, representing of the and simplistic a mix- embryologic mesoderm, even 2: 1 to 8:1. of multipotential normal producing of ectoderm, sues neoplasms recapitulate layers endoderm. organs Tis- derived from all three germ cell layers are possible. The spectrum of differentiation in a teratoma can extend from a multicystic mass composed primarily of ectodermal derivatives, sues to a complex mass representing to a primitive (a fetus or abortive in fetu). U RadioGraphics additional and the seen at twinning most common in mature (including U tis- endoderm, attempt However, type of differentiation mas is in ectodermal dermat) structures. 584 with mesoderm Smit-niotopoulos is often cyst.” produces that called Normal or terato- neuroecto- the mimics skin (erroneously) skin epidermoid) rather than erogeneous teratoma is the second most common region tumor, accounting for approxi1 5% of all masses. Teratomas also have from teratoma faand a “der- elements seen in a dermoid include squamous epithelium and complicated dermal appendage structures such as hair follicles and sebaceous and sweat glands. (Epidermoids only have squamous epithelium.) However, careful histologic study of these lesions usually reveals small components of diverse tissues, confirming their origin from multipotential cells rather than only from ectoderm. Although most sacrococcygeal, retroperitoneal, and gonadal dermoids are actually welldifferentiated teratomas, as described above, many pineal and suprasellar dermoids are not. Instead, most intracranial dermoids (and epidermoids) are in reality merely inclusion cysts derived only from ectoderm rather than from multipotential cells. These intracranial “true” dermoids arise when the ectoderm becomes abnormally folded into the developing head of the embryo. This occurs as the neural tube closes and the eye, ear, and face moid Teratoma varies moid of development begin to form at 3-5 weeks. These inclusion cysts of skin Pineal pineat mately that hence path mature are rnultilocular multiloculated likely to be (the typically true masses. Thus, intracranial a teratoma der- unilocular than a hetmass a more benign epithelial inclusion cyst. The true inclusion cyst dermoid usually occurs in the suprasellar cistern and posterior fossa and is uncommon in the pineal region. Teratomas frequently produce neuroectodermal tissues (with variable degrees of differentiation) that may resemble the embryologic neural tube or a neuroblastoma. Occasionally, these teratomas with neuroblastic cells may mature spontaneously into fully differentiated neural tissue (6). Alternatively, a teratoma with immature neural tissue may metastasize. These metastases may consist entirely of neuroblastic elements. Pineal region teratomas can be partially or totally encapsulated; however, they may also be unencapsulated and locally invasive. In imaging studies, teratomas tend to be heterogeneous, multilocular, ring or ring-enhanced lesions. They may have areas of mixed CSF, lipid, and soft-tissue characteristics, as well as calcification (Figs 5-8) (10-13). All five teratomas reported by Ganti et al (8) were sharply circumscribed masses with areas oflow (fatty) attenuation on CT scans, a feature notably absent from all the other tumor types in their series. They also noted turnoral calcification in four of their five cases. et a! Volume 12 Number 3 Figure 5. Teratoma. (a) CT scan of a 4-year-old boy demonstrates gion extending anteriorly into the cistern of the velum interpositum. a heterogeneous mass in the pineal reThe mass contains several large chunks of calcification and a darker, cystic-appearing area (arrowhead) . Heterogeneity like this, especially when there is lipid material and calcification, is a hallmark of a mature teratoma. (b) After contrast material is administered, there is relatively homogeneous enhancement of the noncalcified solid portions of the tumor. The cystic region does not appear enhanced. (c) Ti-weighted MR image of the same patient demonstrates a mildly heterogeneous mass largely isointense relative to gray matter. However, there are focal areas of Tiweighted shortening (arrowhead) from lipid material (eg, sebaceous). The cystic region (*) has higher signal intensity than that of CSF because of proteinaceous material. (d) Sagittal Ti-weighted MR image of an 8-yearold boy demonstrates a grossly heterogeneous mass with large amounts of hyperintense lipid material. It extends anteriorly toward the cistern of the velum interpositum and posterior third ventricle. Note the cystic region (*) . The signal intensity void of the internal cerebral veins (arrowhead) is superior to the mass, but, in addition, there is a thin rim of hypointensity encircling the mass, suggesting a tumor capsule. (Courtesy of L. Baker, MD, University of California, San Francisco.) (e) Sagittal gross specimen of a mature pineal teratoma from a different patient shows a grossly heterogeneous mass that is well encapsulated (arrowhead). The varied contents of this partially cystic mass include a superior portion with a ‘ ‘cheesy’ ‘ consistency from sebaceous material. In the sagittal plane, it is clear that much of this mass is below the tentorium. (From the L. Rubinstein collection, AFIP.) May 1992 Smirniotopoulos Ct a! U RadioGrapbics U 585 Figures 6, 7. (6) Teratoma. (a) Axial CT scan obtained without contrast material shows a multicystic heterogeneous mass extending from the pineal region anteriorly into the cistern of the velum interpositum. Note the multiple rings of contrast material enhancement that surround nonenhanced cystic areas on both the axial (b) and the coronal (C) CT scans. This appearance is suggestive of teratoma. (7) Pineal teratoma in a 37-year-old man. Axial Ti-weighted MR image demonstrates a heterogeneous mass with two high-signal-in. tensity foci ofTi shortening from lipid material. However, the rest ofthe mass is homogeneous and only slightly hypointense compared with the signal intensity of gray matter. In this case, the radiologic diagnosis of teratoma would be less confident, since hemorrhage into other pineal neoplasms (eg, choriocarcinoma) could have a similar appearance. 586 U RadioGraphics U Smirniotopoulos et a! Volume 12 Number 3 Figure 8. Ruptured pineal region teratoma in a 38-year-old man. (a) Sagittal Ti-weighted MR image dem- onstrates a heterogeneous lesion in the pineal region. There are multiple irregular high-signal-intensity foci from tumor lipid material. Note the extension of the lipid signal intensity below the tentorium and into the fourth ventricle (arrow) . (b) Axial CT scan demonstrates lipid material floating in a layer in the superior portion of the frontal horns of both lateral ventricles. There are multiple low-attenuation lipid droplets in the sulci of the visual cortex and in the quadrigeminal plate cistern. (C) Axial Ti-weighted MR image shows multiple high-signal-intensity foci, corresponding to the lipid droplets seen on the CT scan. Axial Ti- (d) and T2weighted (e) MR images demonstrate a supernatant lipid layer floating on the heavier CSF in the superior portions of both lateral ventricles. On the T2-weighted image, there are high- and low-signal-intensity bands at the lipid interfaces caused by a chemical shift artifact. On Ti-weighted show evidence areas of signal mas may also limited solid-tissue to the teratomas surgery, a chemical planting 1992 the cystic may rupture their meningitis the may components contrast as Terato- material CT and MR images. heterogeneous, either areas spaces spilling along teratomas lipid (1 1 , 1 3). demonstrate on both is usually lining or hyperintensity enhancement Enhancement walls May MR images, of fatty or (Fig along 6). Pineal spontaneously varied (Fig or at contents, 8), or, the . Choriocarcinoma Choriocarcinomas all pineal account masses and lection. Intracranial from differentiation cells into They may both CSF dotropin. for also have associated placentalike with and plasma human Their appearance than a male choriocarcinomas of the pluripotential extraembryonic be less elevated 5% of predi- arise germ tissues. levels of chorionic gonaat CT is nonspe- causing rarely, im- meninges. Smirniotopoulos Ct a! U RadioGraphics U 587 C. ci. Figure 9. Yolk sac tumor in a 9-year-old boy. (a) Axial CT scan obtained without the use of contrast mate- nat demonstrates a round, relatively homogeneous, low-attenuation mass, engulfing the calcifications within the central pineal gland. Germinomas are usually high-attenuation masses; thus, other diagnoses should be considered. However, the appearance is neither specific for nor suggestive ofyolk sac tumor. There is an incidental dural osteoma (arrow) . (b) Axial CT scan obtained after contrast material is administered shows homogeneous enhancement, which is also nonspecific. (c) Axial T2-weighted MR image demonstrates nonspecific homogeneous hyperintensity (higher than the signal intensity ofgray matter) ofthe mass. (d) Sagittal MR image obtained which is slightly gadopentetate cific; like sented plain germinomas, by areas CT without scan the use of contrast hypointense dimeglumine relative shows they of high and are often attenuation show prominent with 588 U hyperintensity short RadioGrapbics on repetition U MR images time or matter. but repre- on the contrast enhancement. However, the common ence of subacute hemorrhage in these vascular neoplasms may produce areas cal material to gray prominent preshighly of fo- Smirniotopoulos time pulse Ct a! minimal heterogeneity obtained after enhancement. sequences This (1 1, 13). in the mass, administration latter differentiate the more the lipid atoma. common germinoma but signal intensity suggestive Endoderma! of feature tentially . obtained echo demonstrates (e) Sagittal MR image slightly heterogeneous can choriocarcinoma Sinus po- from may mimic of a ter- Tumor The endodermal sinus tumor (or yolk sac tumor) is another example of differentiation of the totipotential germ cells from cxtraernbryonic tissues (Fig 9). These uncommon tumors Volume 12 Number 3 a. b. Figure 10. Mixed germ cell tumor (germinoma and yolk sac tumor) obtained without contrast material demonstrates a heterogeneous cystic regions and three areas of calcification. The mass is equal parenchymal neoplasms and other germ cell tumors could have hanced CT scan shows homogeneous enhancement ofthe solid have histologic features of both yolk sac endo- derm and mesoblasts (14). They may be associated with elevated levels of a-fetoprotein in both CSF and serum. Although they are more common than embryonal carcinoma, at least 50% occur other as mixed germ specific cell tumors elements radiologic combined (Fig features 10) have with (14). been No Embryona! Carcinoma Embryonal carcinoma malignant, bryonal-type gested that totipotential, subsequent of large, undifferentiated emIt has been sugare derived from a cells. all teratomas wholesale differentiation of these elements (6) . Embryonal cell tumors develop focal regions of differentiation embryonic (somatic) or extraembryonic cental or vitelline) tissues. Therefore, surprising that embryonal carcinoma quently found component in mixed may also from (jlait is not is a fregerm cell tumors. When embryonal carcinoma mixed germ cell tumor, it can be the most gressive be the only ment May 1992 component to produce and systemic hemorrhage, and necrosis are frequently assays may PINEAL PARENCHYMAL NEOPLASMS Pineal parenchymal neoplasms be helpful in U is composed epithelial boy. (a) Axial CT scan occurring histologic features (14). There are no particular imaging characteristics that suggest embryonal carcinoma of the pineal region. However, because these tumors are capable of producing elevated levels of both a-fetoprotein and human chorionic gonadotropin, serum or CSF differential diagnosis. de- scribed. . ses, in a 17-year-old mass in the pineal region. There are focal in attenuation compared with brain. Pineal a similar appearance. (b) Axial contrast-enportions ofthe lesion. may is part metastases. less than 15% ofall pineal account region for masses. Un- like the germinomas, they have no sexual predilection and may be found in older patients who arc beyond their 2nd decade. There are basically two types that arise from the pineal cells: the pineocytoma and the pineoblastoma. In addition, however, pineal parenchymal tumors may have ganglionic and astrocytic differentiation, and various cornbinations of these types are also possible. of a agdc- Mito- Smirniotopoulos Ct a! U RadioGraphics #{149}589 d. shows C. Figure 11. Pineocytoma. (a) Plain CT scan a large and relatively homogeneous mass in the pineal region, with peripheral displacement of pineal calcification (arrows). The mass has extended anteriorly along the velum interpositum. This is the exploded pineal appearance that suggests an intrinsic pineal parenchymat neoplasm. (b) Contrast-enhanced CT scan shows homogeneous enhancement in the mass, which assumes a triangular shape as it conforms to the contours of the pulvinar of the thalami and velum interpositurn. (C) Axial proton-density-weighted MR image shows the mass is homogeneously hyperintense; it is diamond shaped because it fills the two opposing triangles of the velum interpositum (anterior) and quadrigeminal plate cistern (posterior). (d) Sagittal T2-weighted image shows the mass is under the internal cerebral veins separating the fourth The (arrow) and extends anteriorly the cerebellum from the brain ventricle). pineocytorna is a tumor well-differentiated ally indistinguishable normal 590 U mature RadioGraphics pineal cells composed that histologically parenchyma U (3). Smirniotopoulos along stem, of are virtu- from Often the velurn interpositum. and encroaches on the the the only Ct a! The mass also extends inferiorly, superior medullary velum (the roof of feature suggesting a neoplasm is the presence of a mass or a clearly enlarged pineal gland. The pineocytoma is a circumscribed, though unencapsulated tumor, that may remain to- Volume 12 Number 3 Figure 12. trast material Pineocytoma demonstrates of brain yet dimeglumine in a 27-year-old a homogeneous is slightly higher was administered. than woman. mass that of CSF. (b) Anteriorly, there catty confined and is unlikely to spread by means of the CSF. Because it is a benign neoplasm, hemorrhage and necrosis arc uncommon. The pineoblastoma is a malignant tumor composed of undifferentiated or immature pineal cells. It is a type ofprimitive neuroectodermal tumor similar to the medulloblastoma, neuroblastoma, and retinoblastoma. Occasionally, pineoblastomas produce ing both eyes and the pineal intermediate mature coblastoma, has prognosis diate neoplasm, pineocytoma for also this between the blastoma and outcome possible the been latter dismal relatively identified. lesion is also outcome more of immature pin- of pincofavorable pineocytoma. As the pineal version of the primitive neuroectodermal tumor, the pineoblastoma would be expected to have heterogeneous morphologic features; whereas, the benign pincocytoma, without histologic changes of necrosis and hemorrhage, should be homogeneous. May 1992 with a mixture and there after gadopentetate (*). is considerable overlap in may A pineoblastoma is unencapsulated and often invades directly into the adjacent brain or may spread by means of the CSF (either through the ventricles or along the meninges). An However, is seen interme- (trilateral retinoblastoma). both enhancement cystic region conthat The of retinoretinoblasin the retina sites, includ- gland Heterogeneous is a nonenhanced MR image obtained without Signal intensity is lower than their appearance at imaging. Zee et al (11) reported eight cases, four each of pineocytoma and pineoblastoma; two of each type had calcifications. When images were obtamed after gadolinium contrast material infusion (two pineocytomas and one pineoblastoma), both types were enhanced homogeneously; neither type had cystic areas (Fig ii) (11). In a review of our own pathologic material consisting of 2 1 pineocytomas and 1 1 pincoblastomas, however, cystic changes were identified in one case only, which was a pineocytorna (Fig 12); the others were solid tumors. Tien et al (13) also described a heterogeneous pattern, but it was seen in two of their three cases of pineoblastoma. Pineocytomas and pineoblastomas may contain intrinsic calcifications within the neoplasm in contrast to the germinoma: In the latter cases, the tumor is usually not calcified but the pineal gland is (8-10,12,15). In addition, both types of pineal parenchymal tumor histologic structures (rosettes) reminiscent blastoma. Patients with inherited toma may have multifocal lesions and may have tumors in multiple (a) Sagittal Ti-weighted (*) in the pineal gland. peripherally displace preexisting normal pineal calcifications. This produces an cxploded pincal pattern rather than the engulfed pincal gland seen with germinomas (Fig 1 1). This latter feature may be valuable the differential neoplasms. Smirniotopoulos diagnosis of these et a! U in uncommon RadioGrapbics U 591 Figure 13. Pineal cyst in a 32-year-old woman. (a) Axial MR image obtained after gadopentetate dirneglumine was administered demonstrates a cystic mass with a postenor peripheral rim of contrast enhancement, representing compressed normal pineal tissue. The cyst fluid has slightly higher signal intensity than that of CSF. (b) Sagittal image also demonstrates a uni- formly hypointense mass, with a small postenor crescent of gadolinium enhancement in the residual pineal parenchyma. (c) Photomicrograph (hematoxylin-eosin stain) of a specimen from a different patient shows a cystic space rimmed by residual pineal tissue. C. U NONNEOPLASTIC Pinea! Cyst Pineal cysts may MASSES . mechanisms: lined pineal tation, pathologic arise from persistence two of the different ependymal- diverticulum and secondary cavi- within an area ofgtiosis. In perhaps studies, pineal cysts of variable are reported to occur in 25%-40% lected patients; however, this high includes small cysts only a few size of unsefrequency millimeters in diameter (5). Pineal cysts may be lined by ependyma, rimmed with gliosis, or (most often) merely surrounded by compressed nor- mal pincal may be may resect cally, reflect with RadioGrapbics U Smirniotopoulos Ct a! a bias only pineal Pathologically, pincal multiloculated of the symptomatic cysts are tissue surrounded remnants U or and cysts are filled with a protcinaceous material. Although they are usually an incidental finding at imaging or at autopsy examination, when large, they may produce symptoms. According to our own surgical pathologic material, 12 of 16 pineal cysts were found in patients who presented with symptoms directly related to mass effect that was severe enough to require resection. However, this gliotic 592 tissue. single with neurosurgeons lesions. most often Rosenthal fibers to Microscopilined with and arc by a peripheral rim of residual of the normal pineal tissue, often microscopic hemorrhage. Volume 12 Number 3 a. b. Figure 14. Lipoma. (a) Axial CT scan shows a fatty attenuating mass in the pineal region that does hance. With its homogeneity and lack of enhancement, the mass is most likely not a teratoma. It most of the quadrigeminal plate cistern. (b) Sagittal Ti-weighted MR image shows a mass in the nat plate cistern with homogeneous high signal intensity, similar to the signal intensity from the ous fat and clival marrow. As with many other pineal region masses, it extends infenorly (below rium) and pushes the cerebellum away from the brain stem. Pineal ages, cysts are occurring tients (16). cysts (without ripheral or rim to the cause the brain barrier, may outside (Fig pineal tive into hancement the fluid neoplasm a bloodnoted material rim This may also that ofCSF show can sequences, content rim Intracranial ence of lipid matically The fluid higher signal obtained probably because in in a mass Most lesions fat that subarachnoid space differentiation or develop as a result maldevelopment and of does not lipomas the primitiva differentiates arachnoid, and the making removal confirming virtually im- a developmental (19). intracranial pineal lipomas, region, are anomalies or fewer cases mas are with various in only (20). more often as those with about In contrast, anterior associated of agenesis other developmental of the lipocorpus defects. Lipomas typically on pres- mogeneous weighted high signal intensity on the TiMR image (Fig 14). Unlike most auto- pineal or any region contrast have other one-third (50%-80%) degrees and such associated tenuation differentiated (adipocytes) intracranial lipoma, possible congenital in- The mater, genital callosum dermoids. to either mesoderm. be and material refer congenital 1992 must teratomas pia the the meninx normally the tento- inner meningeal layer of the dura mater. Unlike acquired lipomas and other neoplastic masses, the developmental lipomas usually do not grow as expansile masses. Instead of displacing the adjacent structures to the periphcry, normal vessels and cranial nerves are often intimately incorporated within the con- in the (16,18). lipomas both into Posterior Lipoma from structure, The meninx cause en- images on of bryologic (19,20). sugges- than cyst. to On residual center. ofa than protein Be- lacks rather suggestive all pulse tissues enhancement cysts . May enhanced tensity a high inter- (17). 13) contrast the pineal with pe- normally homogeneous of a solid pineal pineal tissue images, from tissue pa- on MR images. sequence produce im- 20 was normal it is therefore diffuse pineal ofsix enhancement be gadolinium-enhanced delayed MR confirmation), the normal on of every series pathologic to represent displaced seen one In a recent ring preted frequently in almost not en- occupies quadrigemisubcutane- CT homogeneous scans and neoplasms, low usually they have may not atho- show enhancement. are within the of abnormal of an em- Smirniotopoulos Ct a! U RadioGrapbics U 593 ::#{149}r . I I #{149}:--. N LflCe(l ‘pr1a(llng n ti tert.brti trans ‘cins callosum are diflu’i ftht.. brain tric enlarge- ij [scanshow 11, n of the I astrocv- iO\\ ‘a j; itta1 MR . in contrast S spriad . by . mat- #{149}n : 594 U RadioGraphics #{149} Smirniotopoulos et a! Volume 12 Number 3 Figure 17. Vein ofGalen aneurysm. (a) and sagittal (b) MR images show Axial a large rounded hypointensity (flow void) in the pineal region (*). The straight sinus (arrow in b) is also dilated. (c) Vertebral angiogram shows shunting into the vein ofGalen (*), which is massively enlarged. C. stem and duce gross and thalamic gliomas (Fig enlargement vertical extension infiltration of the 16) of those of the white may graphic pro- structures tumor matter tion through long tracts (eg, S Vascular Vascular Malformation malformations mass in the pineal largement of the as a pineal region an adult the (Fig vein bus region. malformation venous system; 1992 to the or from or CT, distal sigmoid) MR, and fistuas arterio- into the obstruction, of the transverse, of vein; deep thrombo- dural . bosis is present. an abnormal dilata- as a response to in- pressure, or both. of the mass on CT is a nonspecific a characteristic finding, flow MR void, images unless re- throm- SUMMARY The as in direct dural show usually flow, increased enhancement veal U manifest as well from draining or en. may vein, a enlargement occur connections venous ultrasonography, Aneurysmal Aneurysmal of a parenchymal produce in a child, may a result (straight, also of Galen mass 17). sis, or hypoplasia May may vein of Galen arterial creased Although scans corticospinal). techniques of the most common germinoma, from pluripotential pineal region a malignant neoplasm germ cells. mass is the derived Germinomas are homogeneous masses that engulf a calcifled pineal gland and have signal intensity equal to that of gray matter. The second most common pineal region mass is another germ cell tumor, the teratoma. These are usually sinuses Doppler various angio- Smirniotopoulos Ct a! U RadioGrapbics U 595 heterogeneous and multiloculated containing calcification and lipid The true neoplasms of the pincal include mature malignant pineocytomas ated and pineoblastomas. inseparable the displace but two germ cell preexisting may be tumors pincal 5. ance of the human tooldage.J Pathol may be 6. differenti- because from distinguished is infiltration atlas pro- stem; adjacent they can be relative to fat. routine MR imaging in depicting cations and of the accurately making localizing a differential tamed be gion masses 8. of small calcifi- importance use of contrast in the of pineal be correct histologic suspected after characteristics, have very a truly the of these pathognomonic Acknowledgments: Williams for manuscript Kruger for photography. We are grateful preparation sist Tomogr 1991; Futrell NN, Osborn 2. cranial germ-cell tumors: natural pathogenesis. J Neurosurg 1985; LangmanJ. Medical embryology. 3. Baltimore: Williams & Wilkins, 178, 318-364. PreslockJP. The pineal gland: tions 1984; 4. MT, Gelman and clinical 5:282-308. Zimmerman incidence R, Hochberg correlations. RA, Bilaniuk of pineal Wilkins, LT. 1. 596 U b RadioGraphics 2. d 1992; 3. U for a Smirniotopoulos a 1984; 15:56-63. AG, Cheson BD. As- Pineal MR AC, TowfighiJ. 1986; Pineal AC, Yarnell pineal on MR images. cysts cysts: 7: 1081-1086. Mamourian Tamaki T. Enhancement AJNR 1991; of 12: N, Shirataki tients.AJNR 20. by are d 1990; Cysts M, of the pi- 11:665-674. Test Education published given 6. S. Tart RP, Quisling RG. Curvilinear and tubulonodular varieties of lipoma of the corpus callosum: an MR and CT study. J Comput Assist Tomogr 1991 ; 15:805-810. Medical Syndrome, K, Lin T, Masumura 5, Matsumoto neal gland: a new clinical entity to be distinguished from tumors of the pineal region. Childs Nerv Syst 1989; 5:172-176. Truwit CL, Barkovich AJ. Pathogenesis of intracranial lipoma: an MR study in 42 pa- 19. Ct a! MR imagJ Comput 31-60. AJNR Katayama Rev 5. M, et al. neoplasms. 773-774. 12:365-378), 4. M. Mamourian 18. Continuing on Peutz-Jegbers Negoro 16. implica- detected T, YoshidaJ, CT Age-related calcification test basic Suprasellar Chang T, Teng MMH, Guo W, Sheng W. of pineal tumors and intracranial germ-cell tumors. AJNR 1989; 10: 1039-1044. Intra- history and 63:155-167. 3rd ed. 1975; 175- Endocr Answers The answers for the Graphics (RadioGraphics F. MR. 18. of 15. 17. Jennings Fasc Institute region tumors: computed tomographicpathologic spectrum. AJR 1981; 137:951-956. Tien RD, Barkovich AJ, Edwards MSB. MR imaging of pineal tumors. AJNR 1990; 11: 557-565. Herrick MK. Pathology ofpineal tumors. In: Neuwalt Ei, ed. Diagnosis and treatment of pineal region tumors. Baltimore: Williams & to Clarice and Steve 1. series. H, Apuzzo 14. REFERENCES I, Abel CG, Kageyama imaging. U 2nd Forces region imaging pattern. Therefore, it has been repeatedly emphasized that histologic verification is necessary for almost every pineal region mass that appears to be neoplastic (11-13). pathology. DC: Armed ing of pineal 13. tu- puberty 1972; 108:137-144. F. Extragonadal teratomas: Zee C, Segall diagnosis few appear- from 1 1. re- reviewing gland 10. material evaluation histological 142: Pineal tumors: clinical diagnosis, with special emphasis on the significance of pineal calcification. Neurosurgery 1981; 8:656-668. Zimmerman RA, Bilaniuk LT, WOOdJH, Bruce DA, Schut L. Computed tomography of pineal, parapineal, and histologically related tumors. Radiology 1980; 137:669-677. 12. the often Chang of (ii). Although mors insensitivity 9. tumor calcification in diagnosis, a CT scan ob- the helpful imaging of the because without may may Because The 1982; germinomas. Cancer 1968; 22:533-544. Ganti SR, Hilal SK, Stein BM, Silver AJ, Mawad M, Sane P. CT ofpineal region tumors. AJNR 1986; 7:97-104. and spread along the white matter tracts. Pincal region lipomas are usually developmental rather than neoplastic, are homogeneous, and arc equal in attenuation and signal intensity Radiology pineal Pathology, 1980. Simson LR, Lampe 7. the oftumor Washington, appearance. there Gonzalez-Crussi they calcifications, ducing an exploded pineal Astrocytomas may arise corpus callosum and brain when computed tomography. 659-662. Tapp E, Huxley M. primitive These at imaging from masses material. parenchyrna in the March 1992 issue of Radio- below. c 7. c 8. c 9. Volume d 10. 12 c Number 3