415 Pineal Region Tumors: Computed Tomographic-Pathologic Spectrum Nancy N. Futrell' While several computed tomographic (CT) studies of posterior third ventricular Anne G. Osborn' neoplasms have included descriptions of pineal tumors, few reports have concentrated Bruce D. Cheson 2 on these uncommon lesions. Some authors have asserted that the CT appearance of many pineal tumors is virtually pathognomonic. A series of nine biopsy-proved pineal gland and eight other presumed tumors is presented that illustrates their remarkable heterogeneity in both histopathologic and CT appearance. These tumors included germinomas, teratocarcinomas, hamartomas, and other varieties. They had variable margination, attenuation, calcification, and suprasellar extension. Germinomas have the best response to radiation therapy. Biopsy of pineal region tumors is now feasible and is recommended for treatment planning. Tumors of the pineal region account for less th an 2% of all intracrani al neoplasms [1]. While several reports of computed tomography (CT) of third ventricular neoplasms have in cluded an occasi onal pineal tumor [2 , 3], few have focused on the radiographic spectrum of th ese uncommon lesions [4]. Some authors have asserted that the CT appearance of many pineal tumors is virtuall y pathognomonic [5]. We studied a series of nine biopsy-proven pineal gland tumors that demonstrated remarkable heterogeneity in both histopath ologic and CT appearance. Materials and Methods A total of 17 pineal gland tumors were detected in 15,000 consecutive CT scans. Four patients were female and 13 were male. Mean age for the fe males was 27 years; for the males, 15 years. Initial symptoms ranged from headache, nausea, and vomiting, to Parinaud syndrome, vi sual field defects, diabetes insipidus, and hypopituitari sm (table 1). Pl ain and contrast-enhanced CT scans were obtained in all cases. Nine of the 17 cases were proven by biopsy or autopsy. Two were germinomas (atypical teratomas), two were teratocarcinomas, and one each was pineal hamartoma, benign Received October 2 1, 1980 ; accepted after revision March 23, 1981 . teratoma, astrocytoma, and neurilemmoma. One other case was designated as a " mali gnant pineal tumor" by cerebrospinal fluid cytology. One case had insufficient tissue for patho­ Presented at th e annual meetin g of the Ameri­ can Roentgen Ray Society, San Francisco, CA, logic diagnosis. One case was diag nosed as a pineal lipoma from characteri stic CT findings. March 1981. The dramati c response of fi ve other cases to radiation therapy led to the presumptive clinical diagnosiS of germinoma (table 1); these had no evidence of residual or recurren t 1 Departm ent of Radiology, University of Utah College of Medicin e, 50 N. Medical Dr. , Salt Lake disease for a minimum of 3 years. One pati en t was lost to foll ow-up. City, UT 84132. Address reprint requests to A. G. Osborn . 2 Department of Medicine, University of Utah Results Coll ege of Medicine, Salt Lake City, UT 84132. This article appears in September/ October Germinoma (Atypical Teratoma) 1981 AJNR and November 198' AJR. The two documented and five presumed germ inomas were either isodense or AJNR 415-420, September/ October 1981 0195- 6 108/ 81 / 020 5 - 0415 $00.00 slightly hyperdense on plain CT scan (fig. 1 A) and showed marked, uniform © American Roentgen Ray Society increase in attentuation after contrast enhancement (fig. 18 ). Several were well 4 16 FUTRELL ET AL. AJNR:2, September / October 1981 TABLE 1: Pineal Gland Tumors: Case Summaries Case Ag e. Presenting Symptoms CT Appearance Biopsy Treatment/ Results No. Gend er 6, M Diplopi a, headache, nau- Isodense noncalcified Germinoma Complete resolution foll ow- sea, vomiting, CN III , posterior third ventricu- ing ventricular shunting, IV palsies lar mass, irregular mar- surgery, and radiation gins, inhomogeneous therapy contrast enhancement 2 .... 17, M Anorexia, diabetes insip- Well margin ated, hyper- Atypical teratoma with Died 2 years after initial idus, panhypopituitar- dense mass left basal prominent germinoma- presentation; atypi cal ter- ism ganglia with strong tous elements (au- atoma with suprasellar, contrast enhancement, topsy) pineal, basal ganglia, edema, obstructive hy- ependymal involvement droceph alus secondary diagnosed at postmortem to aqueduct stenosis; subtle ependymal en- hancement, third ven- tricle 3 18, M Vomiting, diabetes insip- Well delineated hyper- Complete resolution after idus, Parinaud syn- dense suprasellar, pos- ventricular shunt and ra- drome, panhypopitui- teri or third ventricular diation therapy; no recur- tarism masses; Ca++ in pineal rence after 4 years; pre- region. Strong contrast sumptive diagnosis: ger- enhancement of minoma masses and ventricular ependyma; obstructive hydrocephalus 4 .. 14 , F Headache, diplopia, pap- Well delineated slightly Ventricular shunt, radiati on illedema, seizu res hyperdense posterior therapy with tumor reso- third ventri cular mass lution; 1 year follow-up; without Ca++ ; strong, presumptive diagnosis: uniform contrast en- germinoma hancement; moderate obstructive hydroceph- alus 5 . 26, M Headache, vomiting, an- Calcified isodense poste- Complete resolution after orexia ri or third ventricular ventri cular shunting and mass with moderate radiation therapy; no re- contrast enhancement, currence after 5 years; obstruc ti ve hydroceph- presumpti ve diagnosis: alu s germinoma 6 . ... 17, M Headache, Parinaud syn- Isodene third ventricular Complete resolution after drome, papilledema mass with strong con- ventricular shunting and trast enhancement radiation th erapy; 3 year follow-up without recur- rence; presumptive diag- nosis: germinoma 7 .... 14, M ?Headache, Parinaud Well marginated hyper- Complete resolution after syndrome dense pineal region tu- ventricular shunting and mor with focal calcifi- radiation therapy; no re- cation, strong contrast currence after 5 years; enhancement, moder- presumed germinoma ate obstructive hydro- cephalus 8 .. 14 , M Headache, nausea, vom- Well delinated isodense, Teratocarcinoma Di ed from widespread iting, diabetes insip- calcified posterior third metastatic disease 1 year idus, Parinaud syn- ventricular mass with after diagnosis drome strong contrast en- hancement, moderate obstructive hydroceph- alus 9 18,M Diabetes insipidus, pan- Lobulated, hyperdense Teratocarcinoma Apparent resolution of both hypopitu itarism suprasellar, posteri or masses foll owing shunt th ird ventri c ular and radiation therapy; re- masses with foci of cal- currence of suprasellar cification in the pineal component after 6 tumor; strong but months; alpha-fetoprotein sli ghtly inhomogeneous in CSF contrast enhancement AJNR:2, September/ October 1981 PINEAL REGION TUMORS 417 Case Ag e. Presenting Symptoms CT Appearance Biopsy Treatment / Results No. Gender 10 6, M Anisocoria, Parinaud Well marginated posterior Pineal hamatoma Shunted with surgical re- syndrome discovered third ventricular mass moval; in coma 4 months after head trauma with densely calcified postop with major resid- rim, contrast-enhanc- ual neurologic defic its ing center; moderate 2'/4 years later hydrocephalus 11 8, M Decreased coordination Multicystic lobulated, ir- Benign teratoma (with Residual mass and neuro- regular third ventricular abundant neurogli al logic deficit following sur- tumor with foci of calci- elements) gery (3 year follow-up) fication, patchy periph- eral contrast enhance- ment 12 ... 63, M Memory loss, imbalance Well marginated isodense Neurilemmoma or pineo- Complete surgical removal partially calcified pos- cytoma with predomi- terior third ventricular nately neural el ements mass with moderate obstructive hydroceph- alus; uniform contrast enhancement 13 .... 33, M Dizziness, headache Slight irregular hyper- Midbrain astrocytoma Unsuccessful radiation dense posterior third therapy; died 1 year fol- ventricular mass with lowing biopsy focal calcification, patchy contrast en- hancement 14 .. .42, F Headache, vomiting Well delineated low at- Presumed lipoma because tenuation (-15 H) of CT characteristics mass with peripheral calcification; no con- trast enhancement; no hydrocephalus 15 .. 14, M Anorexia, nausea, vomit- Slightly irregular enhanc- " Small malignant cells" Complete resolution follow- ing, diabetes insipidus ing, partially calcified on CSF cytology ing ventricular shunting, posterior third ventricu- radiation therapy (16 lar mass; subtle supra- month follow-up) sellar mass identified on multiple thin , over- lapping axial scans; moderate obstructive hydrocephalus 16 .... 20, M Diplopia, Parinaud syn- Well demarcated hyper- In sufficient tissue Resolving after radiation drome dense posterior third therapy (2 month follow- ventricular mass, small up only) focus of Ca++ ; strong contrast enhancement 17 .... 22, M Diplopia, Parinaud syn- Irregular mixed density Lost to follow-up drome posterior third ventricu- lar mass with foci of calcification, patchy contrast enhancement Note.-eN = cranial nerve, CSF = cerebrospinal fluid. postop = postoperati ve . marginated while others appeared less well circumscribed. similar case of documented " ectopic pinealoma" in the Four cases had definite suprasellar involvement (fig . 2A). basal ganglia has been reported [6]. The present case was The suprasellar mass was quite subtle in two cases and diagnosed at autopsy. required several overlapping thin sections for detection. One case demonstrated striking subependymal enhance­ ment (fig . 28). When present, calcification appeared limited Teratocarcinoma