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Show Jounwl of Clinical Neuro- ophthalmology 10( 1): 9- 17. 1990. Primary Chiasmal Germinoma A Case Report and Review of the Literature C. Bradley Bowman, M. D. and Bradley K. Farris, M. D. © 1990 Raven Press, Ltd., New York A case is presented in which an adult man with painless progressive loss of vision subsequently was found to have a primary supraseUar/ perichiasmal germinoma ( ectopic pinealoma). A review of the literature revealed 93 similar cases of genninoma occurring in the perichiasmal region and these are tabulated. The diagnosis and management of this lesion are discussed, induding the recognition of the characteristic neuroendocrinologic triad of diabetes insipidus, visual changes, and hypopituitarism. Of 64 cases from the literature wherein presenting symptoms were reported, 56 ( 87.5%) had diabetes insipidus, 53 ( 82.8%) visual changes, and 36 ( 56%) hypopituitarism. A review of the literature suggests that diabetes insipidus is usually the initial symptom in suprasellar genninoma. However, we wish to emphasize the ophthalmologic presentation of this entity, because patients in the age group most affected ( adolescents) will often not recognize symptoms of diabetes inSipidus, but will first seek medical attention for painless progressive loss of vision suggestive of chiasmal compression. The radiosensitivity of this lesion is also discussed. Of 61 patients receiving irradiation therapy, 42 ( 68%) were surviving at the time of their individual case report. No patient in the review survived without irradiation therapy. Key Words: Atypical teratoma- Chiasm- Chiasmal compression syndrome-- Ectopic pinealoma- Junctional scotoma. From the University of Oklahoma College of Medicine, Dean A. McGee Eye Institute, Oklahoma City, Oklahoma, U. S. A. Address correspondence and reprint requests to Dr. Bradley K. Farris, Assistant Professor of Ophthalmology, at Uruverslty of Oklahoma College of Medicine, Dean A. McGee Eye Inshtute, 608 Stanton L. Young Blvd., Oklahoma City, OK 73104, U. S. A. 9 Suprasellar germinomas, also referred to as ectopic pinealomas or atypical teratomas, are relatively rare tumors that have attracted considerable attention during the past few years. Reports of the remarkable sensitivity of these germ cell tumors to irradiation therapy, and improved imaging of midline central nervous system ( eNS) lesions with magnetic resonance imaging ( MRI) have contributed to the importance of making a rapid and correct tisssue diagnosis ( 1- 4). In 1961, Kageyama and Belsky described a neuroendocrinologic triad associated with suprasellar germinoma consisting of diabetes insipidus, visual disturbances, and hypopituitarism ( 5). This same triad has been well documented by several authors in the neurosurgical and radiological literature ( 1,2,~). However, the more common ophthalmologic presentation of this entity, with visual loss suggestive of chiasmal compression, has not yet been appropriately emphasized. CASE REPORT A 44- year- old white male oil field worker presented to a local ophthalmologist in September 1988, complaining of painless, progressive loss of vision in both eyes over the previous 5 months. One week before our examination his visual acuity had been noted to be 20/ 40 right eye, and light perception left eye. A routine computerized axial tomographic scan ( CT) of the brain was obtained and was stated to be normal. He was subsequently referred to the Dean A. McGee Eye Institute for further evaluation. Neuro- ophthalmologic examination on September 19, 1988, revealed a best corrected visual acuity of 20/ 20 + 2 RE, and 20/ 400 LE. Pupils were 6.0 mm RE and 5.5 mm LE, with 3 + and equal reaction to light in both eyes. There was no afferent 10 C. B. BOWMAN AND B. K. FARRIS pupillary defect noted. Color vision by Ishihara plates was 11.0/ 15 correct right eye, and 1.5/ 15 correct left eye. Tangent screen testing demonstrated a complete temporal hemianopsia RE, and a cecocentral scotoma LE, suggestive of a left junctional scotoma. Funduscopic examination demonstrated temporal atrophy of the right optic nerve, and diffuse atrophy of the left. The macula, vessels, and periphery were normal bilaterally. The remainder of the neuro- ophthalmological examination was unremarkable. Suspecting a left anterior chiasmal compression syndrome, an MRI of the brain was obtained that demonstrated a diffuse high intensity signal emmanating from a thickened optic chiasm. The thickening was more prominent on the left with marked gadolinium enhancement ( Fig. 1). A lumbar puncture was not performed. Additional history subsequently obtained from the patient re- FIG. 1. T1- weighted MRI scans of the brain. Top left: sagittal pre- infusion, preirradiation, demonstrating the thickened optic chiasm. Top right: sagittal postinfusion ( gadolinium), preirradiation, showing significant gadolinium uptake in the area of the enlarged chiasm. Bottom left: Coronal preinfusion, preirradiation, again demonstrating a large chiasm. Bottom right: postinfusion. preirradiation, showing m;:. Hked enhancement of the chiasmal mass with gadolinium infusion. I Clm Neuru- ophthalmol, Vl''- 1/), \( 1 1, l ': lY/ I PRIMARY CHIASMAL GERMINOMA 11 vealed a 30- pound weight gain over the past year. Also, when questioned specifically, the patient admitted t~, b~ t did not complain of, a 3- year history of polydIpsIa, and polyuria. In fact, he recalled a daily water consumption of 2- 3 gallons. He denied any changes in hair distribution, hat or shoe size, or libido. The patient also denied headaches. Endocrinologic evaluation confirmed diabetes insipidus, hypotestosteronism, and hypothyroidism. On October 21, 1988, the patient underwent a left frontal craniotomy. On gross inspection in situ, the chiasm was noted to be markedly thickened just posterior to the left optic nerve, and the left carotid artery was somewhat displaced. Several small biopsy specimens taken from the outer portion of the mass subsequently revealed " gliosis and chronic inflammation suggestive of germinoma" ( Fig. 2). The mass was felt to be nonresectable and the patient recovered well from the procedure. Subsequently, the patient received 5,040 rad of total ventricular irradiation therapy over a period of 6 weeks, after which the chiasmal mass was felt to have almost completely resolved by MRI ( Fig. 3). On March 22, 1989, follow- up neuro- ophthalmologic examination revealed a best corrected visual acuity of 20/ 20 right eye and 20/ 400 left eye. Pupils were equal and reactive to light in both eyes, with a subtle afferent pupillary defect on the left eye. Tangent screen and Goldmann visual field testing revealed a right homonomous hemianopsia with a coexisting ceco- central scotoma LE. Funduscopic examination was unchanged. The remainder of the neuro- ophthalmological examination was normal. Symptoms of diabetes insipidus had cleared. REVIEW OF THE LITERATURE A review of the literature revealed 93 cases of primary suprasellar ( ectopic) germinoma ( Table 1). Suprasellar germinoma secondary to metastasis from a primary in the pineal body will not be discu~ sed. Primary tumors of the pineal region compnse a separate and distinct entity and do not usually present with the neuroendocrinologic triad previously described. The patients ranged in age from 6 to 41 years with the average age being 14.9 years. The male/ female ratio for the group as a whole was near 1: 1, with 49 women and 44 men. Fig. 4 illustrates the frequency of this tumor at various ages. Of the 64 cases in which the presenting symptoms were specified by the authors, 56 ( 87.5%) had diabetes insipidus, 53 ( 82.8%) had visual changes ( decreased acuity, diplopia, or visual field defects), and 36 ( 56%) had hypopituitarism ( Fig. 5). Of the 61 patients who received irradiation therapy, 42 ( 69%) were reported to be surviving to date of the individual case report, and of all 93 case reports, no patient survived without irradiation therapy. The average amount of irradiation given was 5,255 rad over 5- 6 weeks, with eight patients receiving spinal irradiation. DISCUSSION Pierce ( 9) and Ramsey ( 10) showed that the fine structure of the pineal and ectopic germinoma is identical to that of the classic testicular seminoma. Many theories have been proposed as to how these germ cell tumors find their way into the cranial vault ( 11- 15). However, it seems that most authors now accept the proposal of Witschi, which FIG. 2. Histologic section of the biopsy specimen taken from the outer posterior portion of the thickened chiasm. These sections demonstrate the seminomata- like appearance of the large cell component with a diffuse parenchymatous infiltration characteristic of germinoma. ( Hematoxylin and eosin; left, x 19, right x38.) JClin Neuro- ophthalmol, Vol. 10, No. 1, 1990 12 C. B. BOWMAN AND B. K. FARRIS FIG. 3. T1- weighted MRI scans of the brain. Top: saggital postinfusion ( gadolinium), postirradiation, demonstrating near complete resolution of tumor with only residual gadolinium uptake. Bottom: coronal postinfusion, postirradiation, also demonstrating tumor resolution. suggests that germ cells, which originate in the yolk sac endoderm and migrate widely throughout the embryo before localizing in the gonadal ridges, sometimes find their way into the head of the embryo ( 16). Simson et al. go further to state that the pineal, 3rd ventricle, and suprasellar regions seem to have in common the properties necessary to sequester and maintain aberrant germ cells ( 2). These cells may then produce neoplasms that are I ClUJ Netift/- tJpllt! W{ HlII/, ~ ( 1/ III ,,<, I I II III identical to germ cell tumors found elsewhere in the body. The classification of intracranial germ cell tumors has not been formally unified. However, Takakura has recently categorized them into three types: germinoma ( two- cell pattern pinealoma), mature teratoma ( benign), and immature teratoma ( malignant, including choriocarcinoma, endodermal sinus tumor, embryonal carcinoma, etc.) ( 4). Kageyama and Belsky have devised a classification that divides these tumors of the infundibularsuprasellar region into three types. One reaches the pituitary stalk and optic pathways after arising in the pineal region and metastasizing anteriorly. A second originates within the anterior part of the third ventricle and spreads to involve the optic nerves, chiasm, pituitary, and hypothalamus. The last type, located primarily extra- axially is the ectopic pinealoma of the chiasmal region ( 5). This separation is important, as patients with primary chiasmal and perichiasmal germinomas will often notice visual field defects and/ or decreased visual acuity, and thus present initially to the ophthalmologist. In 1961, Kageyama ( 8) was one of the first authors to describe the characteristic triad of diabetes insipidus, hypopituitarism, and visual disturbances that has since defined ectopic pinealoma as a distinct diagnostic entity ( 5,8). This same triad has subsequently been re- emphasized and documented by several other authors ( 1- 4,6,7). Most authors report diabetes insipidus as the initial symptom, sometimes preceding the onset of visual disturbances and hypopituitarism by 2- 3 years ( 1,2,7,17). Others, however, emphasize that symptoms of diabetes insipidus often go unrecognized, especially in children, and it is usually the visual disturbance that brings the patient to seek medical attention ( 1,2,8). Most authors agree with Kageyama when he states that this syndrome rarely presents with signs or symptoms indicating increased intracranial pressure ( 5,8). Age distribution was studied in 70 cases of suprasellar germinomas by Camins and Mount in their literature review ( 17). They found that female subjects were more affected under the age of 10 years, while male subjects were more affected over the age of 20 years. Between the ages of 10 and 20 years, both sexes were affected equally. These findings are also supported by our literature review ( Fig. 4). Takeuchi noted that this may suggest that the growth of suprasellar germinomas might be influenced by a sex factor ( 7). Interestingly, intracranial germ cell tumors are much more com- PRIMARY CHIASMAL GERMINOMA 13 TABLE 1. Primary suprasellar germinoma: literature review Ref. Case AgelSex Presentation Treatment Outcome Starck et al. ( 27) Case 1 251m 01. · visual changes, O Oeath due to renal hypopituitarism insufficiency Gautier et al. ( 28) Case 1 14/ f 01, visual changes Anesthetic death Ford and Muncie ( 29) Case 1 151m 01, visual changes Oeath 4 yr after onset Case 2 231m 01 Oeath 5 yr after onset Case 3 211m Visual changes Oeath 1 yr after onset Baggenstos et al. ( 30) Case 1 18/ f 01, visual changes. Biopsy and irradiation Oeath 1 yr later with spinal hypopituitarism, therapy, C no specifics metastases headache given Akamatsu ( 31) Case 1 221m 01, visual changes, Oeath, time not reported hypopituitarism, headache Russell et al. ( 32) Case 1 10/ f Oi, visual changes, Incomplete resection Oeath 13 mo later with hypopituitarism, metastases to spinal headache cord Russell ( 33) Case 1 141m Oi, visual changes, Post- op death 1 mo later hypopituitarism, headache Horrax and Wyatt ( 34) Case 1 121m 01, visual changes, Incomplete resection, Survivi ng 4 yr after hypopituitarism, 4,000 rads diagnosis headache Troland and Brown ( 35) Case 1 141m Headache, Incomplete resection, Surviving 2 yr after precocious puberty 2,000 rad? diagnosis Baker and Rucker ( 14) Case 1 171m 01, visual changes, Biopsy, irradiation, Oeath 1.5 yr after headache amount unspecified diagnosis with spinal cord metastases Rand and Lemmen ( 36) Case 1 15/ f 01, visual changes, hypopituitarism, headache Russell ( 37) Case 1 10/ f 01, visual changes Oeath before surgery Kahn et al. ( 38) Case 1 13/ f 01, visual changes, hypopituitarism Sasano et al. ( 39) Case 1 201m 01, hypopituitarism Loken ( 40) Case 2 41/ m 01, visual changes Oeath, time not noted Case 3 15/ f 01, visual changes, Oeath, time not noted hypopituitarism, headache Busch ( 41) Case 1 321m 01, visual changes, hypopituitarism Heon and Scoville ( 42) Case 1 121f 01 Biopsy and incomplete Oeath, 36 h post- op resection Kageyama and Belsky ( 5) Case 1 13/ 1 01, visual changes, Biopsy and irradiation, Oeath 4 yr after diagnosis hypopituitarism. amount unspecified headaches Case 2 16/ 1 01, visual changes Biopsy with no resection Oeath 1 mo post- op Lewis and Baxter ( 43) Case 1 16/ 1 01, visual changes, Biopsy Oeath 11 d post- op hypopituitarism Case 2 191m 01, visual changes Biopsy and irrad. of head Oeath 3 yr post- op and spine, amount unspecified Rubin and Kramer ( 1) Case 1 9/ 1 01, visual changes, Incomplete resection, Oeath 5 yr later with renal hypopituitarism 4,109 rad insufficiency Case 2 81m 01, visual changes, Biopsy and 4.050 rad Oeath 1 yr later with precocious puberty minengeal metastases Case 3 121f 01, visual changes, Biopsy and 6,000 rad Surviving 7 yr after hypopituitarism, diagnosis headaches Case 4 8/ f 01, visual changes, Biopsy and 5,000 rad Surviving 5 yr after headache diagnosis Case 5 121f 01, visual changes, Biopsy and 5,000 rad Surviving 3 yr after hypopituitarism, diagnosis headache Nakamura et al. ( 44) Case 1 281m 01, headache Tumor undiagnosed until Oeath 2 yr after initial autopsy presentation Dayan et al. ( 45) Case 4 11/ f Hypopituatarism, Death 4 yr after diagnosis headache Nishiyama et al. ( 46) Case 1 111m 01, hypopituitarism Biopsy and irradiation, Surviving 3.5 yr after amount unspecified diagnosis Case 6 4/ 1 Biopsy Oeath 3 d post- op Case 8 11/ f 01 Biopsy and irradiation, Surviving 1 yr later amount unspecilied Simson et al. ( 2) Case 1 10/ 1 01, visual changes Biopsy, partial excision, Surviving 18 yr after 5,900 rad diagnosis Case 2 111m 01, visual changes, Biopsy and Surviving 7 yr after hypopituitarism decompression, 4,148 diagnosis rad continued 1elin Neuro- ophlhalmol. Vol. 10. No. I, 1990 14 C. B. BOWMAN AND B. K. FARRIS TABLE 1-( Continued) ReI. Case AgelSex Presentation Treatment Outcome Case 3 ll/ f 01, visual changes Biopsy and partial Surviving 4 yr after excision, 4,500 rad diagnosis Case 4 111m Visual changes Biopsy and partial Surviving 5 yr after excision, 4,190 rad diagnosis Case 5 7/ m 01, visual changes, Biopsy and partial Surviving 3 yr after headaches excision, 3,700 rad diagnosis Case 6 6/ f Visual changes, Biopsy and partial Oeath 5 yr later with hypopituitarism excision, total of 7,600 widely infiltrative tumor rad with recurrence Case 7 13/ f 01, visual changes, Biopsy and partial Oeath 1 yr later Irom hypopituitarism excision, 6,000 rad residual suprasellar tumor Case 8 121m 01, visual changes Biopsy Oeath 14 d post- op Case 9 23/ f 01, visual changes. Biopsy and partial Oeath 12 h post- op hypopituitarism, excision headaches Case 10 161m 01, visual changes, " Total excision" Oeath 6 mo later with headaches recurrence Case 11 10/ f 01, visual changes, Subtotal excision Oeath 14 h post- op headaches Ghatak et al. ( 4) Case 1 191m Visual changes, Biopsy Oeath 8 d post- op hypopituitarism Case 2 13/ f Hypopituitarism, Oiagnosis at autopsy Oeath 3 dafter headache presentation Kageyama ( 8) Case 1 141m 01, visual changes, Biopsy, 5,500 rad Surviving 11 yr after headaches diagnosis Case 2 28/ f 01, visual changes, Craniotomy without Surviving 5 yr after hypopituitarism, biopsy, 6,000 rad surgery headaches Case 3 211m 01, visual changes, Biopsy and partial Surviving 7 yr after hypopituitarism, excision, 6,000 rad presentation headaches Case 4 221m 01, visual changes, Biopsy and partial Surviving 10 yr after hypopituitarism excision, 6,000 rad diagnosis Cohen et al. ( 6) Case 1 14/ f 01, visual changes, Craniotomy without Oeath 2 yr later with hypopituitarism, biopsy, 5,000 rad recurrence headaches Camins and Mount ( 17) Case 1 151m 01, visual changes Biopsy and 7,500 rad Oeath 19 yr later, cause not specified Case 2 281m 01, visual changes, Biopsy and 1,800 rad Surviving 25 yr after hypopituitarism diagnosis Case 3 8/ 1 01, visual changes, Biopsy and 840 rad Oeath 1 yr post- op hypopituitarism ( incomplete) Case 4 7/ f Visual changes Biopsy and 3,200 rad Oeath 7 mo post- op Case 5 111m 01, visual changes Biopsy and 5,850 rad to Oeath 6 mo post- op, cause head. 2,150 rad to spine not specified Case 6 17/ 1 01, visual changes, Biopsy Oeath 4 yr post- op hypopituitarism Case 7 13/ f 01, visual changes, Biopsy and 8,500 rad Surviving 9 yr after hypopituitarism diagnosis Case 8 161m 01, visual changes, Biopsy and 3,220 rad to Surviving 5 yr after hypopituitarism head, 4,000 rad to spine diagnosis Case 9 11/ f 01, visual changes Biopsy and 5,000 rad Surviving 1 yr later Case 10 18/ 1 01, visual changes. Biopsy and 5,000 rad Oeath 6 mo post- op, cause hypopituitarism, not specilied headaches Case 11 21/ f 01, visual changes, Biopsy, 3,920 rad Surviving 9 mo post- op hypopituitarism Sung et al. ( 3) Case 1 7/ 1 Biopsy and partial Surviving 6 yr after excision, 4,000 rad diagnosis Case 2 111m Presenting symptoms Total excision and 4.000 Oeath 7 mo post- op with not mentioned in rad spinal metastases these cases Case 3 101m Biopsy and 5,500 rad Oeath 7 mo post- op with adrenal insufficiency Case 4 ll/ f Partial excision and 5,000 Surviving 5 yr after rad initially; 3,500 rad diagnosis and 1 yr after later with recurrence recurrence Case 5 161m Partial excision and 4,000 Surviving 6 yr after rad to brain; also diagnosis and 2 yr after irradiation ( unspecified) therapy for spinal to spine metastases Case 6 15/ f Biopsy and 5,000 rad Surviving 4 yr alter diagnosis Case 7 20/ 1 Partial excision and 4,000 Surviving 14 yr alter rad diagnosis continued I Clin Ref. Takeuchi et at ( 7) Neuwelt et al. ( 48) PRIMARY CHIASMAL GERMINOMA 15 TABLE l-( Continued) Case AgelSex Presentation Treatment Outcome Case 8 15/ f Partial excision and 4,000 Surviving 7 yr after rad initially, 4,500 rad diagnosis; surviving additional to brain; after spinal recurrence Case 9 3,000 rad to spine 121m Biopsy and 5,000 rad Death 1.5 yr later with Case 10 spinal metastases 10lf Presenting symptoms Partial excision and 5,000 Surviving 2.5 yr later not specified rad Case 11 201m Biopsy and 4,000 rad Surviving 4 yr after diagnosis Case 1 161m Biopsy Death 4 mo post- op Case 2 11/ f Biopsy Death 18 mo post- op Case 3 141m Biopsy and 5,500 rad Surviving 16 yr after diagnosis Case 4 211m Biopsy and 6,000 rad Surviving 14 yr after diagnosis Case 5 221m Biopsy and 11,000 rad Death 11 yr later due to hypothalamic crisis and brain radiation necrosis Case 6 281m Biopsy and 6,000 rad Surviving 13 yr afler diagnosis Case 7 121m Biopsy and 5,500 rad Surviving 6 yr after diagnosis Case 8 15/ f Biopsy and 6,000 rad Death 9 mo after diagnosis Case 9 8/ f Diagnosis by CSF Surviving 5 yr after cy1010gy, 6,000 rad diagnosis Case 10 121f Biopsy and 6,000 rad to Death 1 yr later wilh spinal brain, 2,000 to spine metastases Case 11 6/ f Biopsy and 6,000 rad to Surviving 2 yr after brain, 2,000 to spine diagnosis Case 12 121f Biopsy and 6,000 rad Surviving 3 yr after diagnosis Case 13 111m Biopsy and 6,000 rad to Surviving 21 mo after brain, 3,000 to spine diagnosis Case 14 271m Biopsy and 6000 rad Surviving 18 mo after diagnosis Case 15 101m Biopsy and 6,000 rad to Surviving 17 mo after brain, 3,000 spinal diagnosis Case 16 15/ f Diagnosis by CSF cy1ology Death before treatment Case 17 9/ f Diagnosis by CSF Surviving 1 y after cytology, 6,000 rad diagnosis Case 18 14/ f Diagnosis by CSF Surviving 1 y after cytology, 5,500 rad to diagnosis brain, 3,000 spinal Case 1 221m DI, headaches Craniotomy without Surviving 12 yr after biopsy, 4,500 rad, diagnosis and 1 yr after recurrence treated with recurrence chemotherapy • Diabeles insipidus. b Decreased visual acuity, visual field defects, or diplopia. c All irradialion therapy is to brain only unless further specified. J~ Jl) 7-; Illlmttei' ~ I ; n ~ I. l " Ib .~ ID ~! i•• 1J- 9 go. 10" 20 FIG. 4. Age and sex incidence of 93 patients wi~ h suprasellar germinoma. Black bars, male patIents; striped bars, female patients. mon in Japan than in North America or Europe ( 18,19). The differential diagnosis of perichiasmal germinoma should include chiasmal glioma, craniopharyngioma, pituitary adenoma, infundibuloma, astrocytoma of the anterior portion of the floor of the third ventricle, tuberculum sellae meningioma, Schuller- Christian disease, and metastatic neoplasm. Diabetes inSipidus has been shown to be present in up to 95% of all patients with suprasellar germinoma ( 18). By comparison, diabetes insipidus is a rare phenomenon in pituitary adenoma, chiasmal glioma and infundibuloma. In Matson's I Clin Neurcrophlhalmol, Vol. 10, No. 1, 1990 C. B. BOWMAN AND B. K. FARRIS FIG. 5. Preoperative signs and symptoms in 64 cases of primary suprasellar germinoma. series ( 1969) of 57 craniopharyngiomas, only 8% of the cases were associated with diabetes insipidus ( 18). It therefore seems appropriate that diabetes insipidus of unexplained etiology should be carefully followed during the first and second decades of life for associated visual disturbances and hypopituitarism. The optimal approach to diagnosis and treatment of intracranial germinomas has been a much debated topic in the literature. As early as 1950, Rao et al. emphasized the value of irradiation therapy in the treatment of ectopic pinealomas ( 20). In 1965, Rubin and Kramer published a review of 36 cases of ectopic pinealoma, re- emphasizing the radiocurable characteristics of these tumors ( 1). The remarkable sensitivity of intracranial germinoma to irradiation therapy, combined with the relatively high morbidity and mortality previously associated with craniotomy and biopsy, has prompted some authors to recommend a radiotherapeutic trial without biopsy when germinoma is suspected ( 1,2,8,20). Others, however, point out that initial biopsy of these lesions will permit more therapy decisions to be based on tissue diagnoses and reduce the frequency of potentially harmful radiotherapy trials ( 21). Also, recent advances in microsurgical and stereotactic techniques have significantly decreased the surgical morbidity and mortality previously associated with biopsy or resection of these lesions, and biopsy can usually be obtained without further visual loss ( 4,21). The metastatic spread of intracranial germinoma through the spinal fluid to other spinal axis locations has been described, although the incidence ':", 1.' fllr primary chiasmalle- CONCLUSION The inclusion of germinoma in the differential diagnoses of patients presenting with chiasmaI field defects is extremely important. The characteristic triad of visual loss consistant with chiasmal compression, diabetes insipidus, and hypopituitarism define this presentation as a distinct diagnostic entity. Review of the literature suggests that diabetes insipidus is usually the initial symptom in suprasellar germinoma. However, in most instances this tumor occurs in the adolescent age sions, has not been well established ( 1,3,22,23). This has prompted some authors to recommend routine craniospinal radiotherapy for all patients with biopsy- proven germinomas, as well as those with clinical and radiographic evidence suggestive of germinoma ( 20,23). However, due to the low incidence of spinal metastases and the associated morbidity of irradiation to the spinal axis, most authors do not currently recommend such aggressive treatment ( 21,24). Camins and Mount recommend that patients be followed regularly with cerebrospinal fluid millipores, one before discharge, and then at yearly intervals. Accordingly, only patients with clinical evidence of subarachnoid metastasis or positive millipores should receive entire neuroaxis irradiation ( 17). Currently, the most accepted recommendation for treatment of suprasellar germinoma is irradiation of the tumor field and entire ventricular system with a dose of 4,500- 5,000 rad, given over a period of 5- 6 weeks ( 1,3,21,24). Consideration must be given to the postoperative, postradiation management of the patient's endocrine function. Patients may need maintenance with hydrocortisone, testosterone, thyroid extract, as well as pitressin. Replacement therapy and continued management is important so that death from neuro- endocrine complications may be avoided. The outcome of treatment for intracranial germ cell tumors differs according to the histologic type of tumor and the location, i. e., suprasellar versus pineal. Overall, the radiocurability of germinoma has produced an excellent survival outcome with most authors reporting a 10- year survival rate of 55- 72% for all intracranial germinomas ( pineal and suprasellar) ( 4,25). If one narrrows this down to only suprasellar lesions, the rate improves to 84.4% at 10 years ( 26). These survival rates are from data over the past 20 years, with mortality and morbidity of surgial intervention being much higher during the initial10- year period than in the following 10 years. visueJ. h ypopit-changes uitarism Diabetes insipidus 16 100 90 10 70 ~ of patients 60 50 40 30 20 10 J (.' 1",. 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