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Show Journal of C/ i" ial/ Neuro- ophtha/ m% gy 10( 4): 239- 243. 1990. © 1990 Raven Press, Ltd., New York Pineal Germinoma with Unilateral Blindness Seeding of Germinoma Cells in Optic Nerve Sheath Riri S. Manor, M, D., Jacob Bar- Ziv, M. D., Rina Tadmor, M. D., Avi Eisbruch, M. D., and Gideon Rechavi, M. D. A 13- year- old boy presented with blindness and loss of the papillomacular bundle in the left eye, bilateral papilledema sparing the area of axonal loss, and Parinaud's syndrome. Computerized tomography ( CT) revealed an enlarged kinked left optic nerve and enlarged optic canal as well as a tumor in the pineal area producing hydrocephalus. Magnetic resonance ( MR) scans showed multiple spinal metastases. The histological diagnosis was germinoma. On completion of four courses of chemotherapy with cis- platin, vinblastine, and bleomycin, repeated CT of orbita and MR imaging of the spine demonstrated the disappearance of the tumor surrounding the left optic nerve and of the spinal metastases. This is believed to be the first case report of seeding of germinoma into the perioptic arachnoid space. Key Words: Blindness- Pinealis- Subarachnoid space of optic nerve sheath. From the Neuro- ophthalmology Unit, Beilinson Medical Center, Petah Tikva ( R. S. M.); Department of Radiology, Hasharon Hospital, Petah Tikva ( J. B.- Z); Departments of Radiology, ( R. T.), Oncology ( A. E.), and Hematology, Sheba Medical Center, Tel Hashomer ( C. R.); and the Sackler School of Medicine, Tel Aviv University, Ramat Aviv, Israel. . Presented at the International Neuro- ophthalmology SOCiety and Japanese Society of Neuro- ophthalmology meeting, Vancouver, British Columbia, Canada, May 1988. Address correspondence and reprint requests to Dr. R. S. Manor, Neuro- Ophthalmology Unit, Beilinson Medical Center, 49 100 Petah Tikva, Israel. 239 Seeding of pineal germinoma cells via the cerebrospinal fluid to the spinal subarachnoid space is well recognized. We examined a 13- year- old boy with visual loss in the left eye as the first presenting sign of a pineal germinoma with metastases to the perioptic arachnoid space. CASE REPORT A 13- year- old boy experienced abrupt deterioration of vision in his left eye over 2 weeks and some vague neck pain. He was urgently referred to the Neuro- ophthalmological Unit of the Beilinson Hospital because of bilateral papilledema and left eye blindness. Seven months earlier he had a visual acuity of 6/ 6 in both eyes in a routine school examination. On examination, visual acuity for the right eye was 6/ 6 and for the left eye only light projection superiorly. Visual field testing in the right eye showed an enlarged blind spot. The pupils, middilated, presented light- near dissociation. There was also retractory nystagmus and paralysis of pursuit and saccades on upward gaze. The fundi showed an elevated and hyperemic right optic disc ( Fig. 1A). The left optic disc was elevated and hyperemic mainly on its nasal side, whereas the temporal side between 2 and 5 o'clock had a sharp border and a pale color. There was a lack of the nerve fiber layer in the area of the papillomacular bundle ( Fig. 1B). An orbital CT scan revealed enlargement of the left intracranial optic nerve, particularly at the apex, with kinking of the nerve and intracranial extension of the space- occupying lesion. The optic canal was enlarged without signs of bone destruction ( Fig. 2). CT of the brain showed a large pineal mass and signs of hydrocephalus ( Fig. 3). A ventriculoperitoneal shunt was performed in 240 R. S. MANOR ET AL. A FIG. 1. The fundus picture of the optic discs in the initial examination. A: The right eye ( left) papilledema. 8: The left eye ( right). There is elevation and hyperemia of the nasal side of the disc and a sharp border of disc and pallor between 2 and 5 o · clock. The nerve fiber of the papillomacular bundle is lacking. the Neurosurgical Department of the Tel Hashomer Hospital, and during the 2- week interval before craniotomy there was a bilateral resolution of the papilledema. Cerebrospinal fluid analysis revealed no a- fetoprotein or 13- human chorionic gonadotropin. At operation there was a soft pink tumor, 3 em in size, covered by a thick arachnoidal membrane. The tumor was excised and the histological diagnosis was germinoma. Within a few days after the operation the boy began to experience progressively severe lower back pain and blurring of vision in the " good" right eye. MR imaging of the spine disclosed the presence of multiple spinal metastases. At this time examination showed visual acuity of the right eye to be 6/ 7, and the visual field presented a huge scotoma in the temporal field, located around the blind spot from degree 5 to 25 horizontally and from meridian 45 to 300 vertically. Fundus examination showed no papilledema. Chemotherapy was instituted and he received 4 cycles of cis- pIa tin ( 70 mg/ m2 every third week), bleomycin ( 15 mg once weekly), and vinblastine ( 3 mg/ m2 x 2 every third week). After 2 days of chemotherapy the back pain disappeared. After 5 days visual acuity and field of the right eye were normal, whereas in the left eye acuity improved to 2/ 60 and the field became measurable, showing a large cecocentral scotoma breaking out inferiorly. At the end of chemotherapy, CT scan of the orbit showed a normal- appearing optic nerve, with tumor remnants at the orbital apex ( Fig. 4). The tumoral mass was no longer evident in the pineal region ( Fig. 5). MR scans of the spine demonstrated resolution of the previous lesions. Following the chemotherapy the boy received JClin N/;' uruuphlhalllllJ/, Vul. III, r" I' f, jYY/ l radiotherapy ( 3500 R). He then felt well and returned to school. Eight months after the operation he complained of progressive visual blurring in the right eye and the visual field again showed a temporal field scotoma. One week later other neurological signs appeared, including bilateral involvement of nerves III and VII with weak right corneal reflex, difficulties in swallowing, loss of hearing first in the right ear and later in the left ear as well, and paralysis of vocal cords. CT and MR scans showed no evidence of pathology, but cerebrospinal fluid analysis at this time showed markers of a- fetoprotein and 13- human chorionic gonadotropin. These clinical and laboratory findings indicated widespread meningeal seeding of germinoma cells. A new series of chemotherapy led to some improvement in the oculomotor palsies, in hearing, and in the vocal cord palsy. Eleven months after the operation the patient's condition again deteriorated, and he died. Permission for autopsy was not obtained. DISCUSSION Edwards and co- authors ( 1) reported that among 33 children with pineal tumor ( 11 with germinoma) the presenting symptom was papilledema in five patients, abducens palsy in three, oculomotor palsy in one, and blurred vision in two. These authors provided no further explanation about the cause of the visual disturbances in the latter two patients. Jooma and Kendall ( 2) noted that in one of their 15 cases of pineal germinoma there was evidence of visual deterioration with optic atrophy, but orbital CT findings are not mentioned. PINEAL GERMINOMA SEEDING IN OPTIC NERVE SHEATH 241 . A c Johnson and Bell ( 3) found a relative afferent pupillary defect without any detectable visual dysfunction in a 23- year- old man with mixed germ cell tumor of the pineal area. In our case, rapid visual loss in the left eye brought this 13- year- old boy to the ophthalmologist. The findings of elements of Parinaud's syndrome focused clinical attention on the dorsal midbrain. In this context, optic nerve failure would usually be attributed to prolonged papilledema. In Our patient, however, the ophthalmoscopic findings were not those of secondary optic atrophy. The papilledema looked recent, visual acuity of the right eye was normal, and the blind spot of the right visual field was enlarged. In the left eye no papulomacular bundle was visible, and the papilledema developed only on the nasal half of the disc. This illustrated still further the findings of FIG. 2. Orbital CT ( computed tomography) scan in the initial examination. A, Showing an enlarged and kinked left optic nerve; with fusiform enlargement at the orbital apex; B, C, the enlarged optic canal without bone destruction, and the extension of the tumor intracranially. Wirtschafter et aI., ( 4) and Tso and Hayreh ( 5) that the development of papilledema with its arrest in axonal transport requires the presence of the axons. Paul and Hoyt ( 6), in a case of optic tract syndrome with retrograde hemioptic nerve fiber layer degeneration, observed that the subsequent development of papilledema in both eyes spared those portions of the discs affected by the axonal degeneration. In our patient, the CT scans of brain and orbits demonstrated the presence of a large pineal tumor with hydrocephalus but also an enlarged left optic canal and enlarged left optic nerve showing kinking and fusiform enlargement at the orbital apex and at the intracranial segment adjacent to the optic canal. Rothfus and co- authors ( 7) in 72 cases of enlargement of the optic nerves and sheaths, found that optic nerve glioma and meningioma caused I Cli" / IIt'" nH'l'lzlhal" wl, Vol. 10, / 110. 4, 1990 e 242 R. S. MANOR ET AL. FIG. 3. Brain CT ( computed tomography) scan showing a large pineal tumor producing hydrocephalus. fusiform enlargement of the optic nerve in their cases. In our patient, however, the finding of one of these two rare optic nerve tumors occurring coincidentally with a pineal region tumor seemed highly improbable. Peyster and co- workers ( 8) re- FIG. 4. Orbital CT ( computed tomography) scan after operation and the end of chemotherapy. The left optic nerve is no longer kinked or enlarged except for small remnants of tumor at the orbital apex. I Clin N,""" I'! llll< Ifmol, Vol lO, No. 4, 1990 FIG. 5. Brain CT ( computed tomography), post operative. There is no longer evidence of pineal tumor or hydrocephalus. viewed the conditions associated with optic nerve enlargement other than optic nerve glioma and meningioma and the nonneoplastic processes that may produce a similar or identical appearance in the CT scans. In a recent article on CT and MR imaging of the optic nerve and sheath, Azar- Kia and co- authors ( 9) noted that less common tumors such as hemangiopericytoma and hemangioblastoma as well as metastatic involvement of the optic nerves and nontumoral enlargement of the optic nerve sheath as the first manifestation of a systemic disease may all simulate an optic glioma or meningioma on both CT and MR imaging. In another paper, AzarKia and co- authors ( 10) concluded: " In most instances, clinical information plays a major role in differentiating between glioma, meningioma and other pathological entities involving the optic nerve." The progressively severe low back pain in the first postoperative week in our case, paralleling the onset of visual deterioration in the second eye, indicated the diagnosis of perioptic subarachnoid seeding as well as spinal seeding. Chemotherapy alone effected a spectacular resolution of back pain, visual loss, and tumorous enlargement of the left optic nerve. This response confirmed the diagnosis and the rapid efficacy of the chemotherapy given after surgery and before radiation ( 11). PINEAL GERMINOMA SEEDING IN OPTIC NERVE SHEATH 243 While the seeding of germinoma cells in the spinal arachnoid space is well known, we were not able to find in the literature a similar case of anterior seeding into the perioptic subarachnoid space of germinoma pineal tumor cells. In a case of midline cerebellar medulloblastoma that developed 28 months after operation and postoperative irradiation, an optic neuropathy with radiologic evidence of an enlarged optic nerve was described by Garrity and co- authors ( 12). In their case also the tumor likely spread through the subarachnoid space within the cerebrospinal fluid and lodged in the perioptic meninges. The intervening anatomic sites were not directly involved by the tumor. Another challenging aspect of this case was interpretation of the appearance of the papilledema. Was there genuine papilledema in the right eye ( produced by the transmission of the increased intracranial pressure) and disc edema in the left eye ( produced by tumor in the optic nerve)? With this alternative it is difficult to explain how the disc edema disappeared from the left eye after the shunt procedure in parallel with the disappearance of papilledema in the right eye. If we accept the alternative supposition that there was true papilledema also in the left eye, how could the orbital perioptic space infiltrated by tumoral cells have had free communication with the intracranial subarachnoid space? Our case also raises some interesting theoretical considerations about the tumoral behavior, with almost simultaneous recurrence of tumor activity 8 months after operation in anatomically remote areas such as the optic nerves and brain stem. This simultaneous recurrence may also be helpful in differentiating the effects of the tumor from that of postirradiation encephalopathy. Tumoral seeding of the optic nerve sheath at the onset of symptoms of a germinoma may indicate highly malignant behavior and a relatively poor prognosis. We advise orbital imaging in any case of pinealoma associated with a visual loss in order to search for evidence of perioptic meningeal seeding. REFERENCES 1. Edwards M5B, Hudgins RJ, Wilson CB, et al. Pineal region tumors in children. J Neurosurg 1988; 68: 689- 9. 2. Jooma R, Kendall B. Diagnosis and management of pineal tumors. f Neurosurg 1981; 58: 654- 65. 3. Johnson RE, Bell RA. Relative afferent pupillary defect in a lesion of the pretectaI afferent pupillary pathway. Can f Ophthalmol 1987; 22: 282- 4. 4. Wirtschafter JD, Rizzo FJ, 5miley Be. Optic nerve axoplasm and papilledema. Sun> OphthalmoI1975; 20: 157- 89. 5. Tso MOM, Hayreh 55. Optic disc edema in raised intracranial pressure. Arch Ophthalmol 1977; 95: 1458-- Q2. 6. Paul TO, Hoyt WF. Fundoscopic appearance of papilledema with optic tract atrophy. Arch OphthalmoI1976; 94: 467- 8. 7. Rothfus WE, Curtin HD, Slamovitz TL, et al. Optic nerve sheath enlargement. Radiology 1984; 150: 409- 15. 8. Peyster RG, Hershey BL, Haskin ME. High resolution CT of lesions of the optic nerve. Am J Neuroradiology 1983; 4: 16974. 9. Azar- Kia B, Mafee MF, Horowitz 5W, et al. CT and MRI of the optic nerve sheath. Semin Ultrasound, CT and MR 1988; 9: 454- 63. 10. Azar- Kia B, Nahedy MH, Elias DA, et al. Optic nerve tumors: role of magnetic resonance imaging and computed tomography. Radiol Clin North Am 1987; 25: 561- 81. 11. Allen Jc, Kim JH, Packer RJ. Neoadjuvant chemotherapy for newly diagnosed germ cell tumors of the central nervous system. I Neurosurg 1987; 67: 65- 70. 12. Garrity JA, Herman DC, Dinapoli RP, et al. Isolated metastasis to optic nerve from medulloblastoma. Ophthalmology 1989; 96: 207- 10. I Clil! NCl/ ro- 0l'lIthalmol, Vol. 10, No. 4, 1990 |
