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Show Journal of Clinical NeuTo- ophthalmology 10( 2): 95- 99. 1990. © 1990 Raven Press, Ltd., New York Pinealoblastoma Metastatic to the Optic Nerve Richard W. HertIe, M. D. and Richard M. Robb, M. D. We report a lO- year- old white girl who developed a pinealoblastoma 2 years before presenting to us with metastatic spread to the left optic nerve. She was treated for the original tumor with irradiation and chemotherapy. She presented to us with decreased vision in the left eye, unilateral disk edema, and diffuse left optic nerve swelling without increased intracranial pressure. Biopsy of the posterior optic nerve sheath was required for diagnosis. Vision was lost in the left eye, but the optic nerve swelling diminished after chemotherapy and additional radiation therapy. Key Words: Pinealoblastoma- Optic nerve- Metastases. From the Departments of Ophthalmology, Bosto~ University School of Medicine ( RW. H.), and Children's HospItal ( RM. R) Boston, Massachusetts, U. S. A. . Address correspondence and reprint requests to Dr. Richard M. Robb at Department of Ophthalmology, Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, U. S. A. 95 Primary pineal tumors comprise 0.~ 1.0% of intracranial space- occupying lesions in European and American series ( 1). Seventy- five percent of these pineal tumors are malignant ( 2). They vary in their resectability, response to radiation or chemotherapy, and their tendency to disseminate along the neuraxis ( 2,3). They include pineal parenchymal tumors ( pinealoma, pinealoblastoma), germ cell tumors ( germinomas, embryonal cell carcinomas), and glial tumors. Pineal tumors tend to spread by extension along the leptomeninges rather than by distant hematogenous or lymphatic metastasis. We have had the opportunity to assist in the care of a patient with a pinealoblastoma that responded to initial treatment but then recurred in the meninges surrounding the optic nerve. CASE REPORT A 10- year- old white girl with a history of pinealoblastoma diagnosed by brain biopsy 2 years previously presented on July 20, 1987 with a 2- week history of episodes of " black vision" lasting for a few seconds at a time in the left eye. In August 1985 computerized tomography ( CT) of the head and orbits had delineated the pineal tumor and showed normal optic nerves ( Fig. 1A and B). At that time the patient's cerebrospinal fluid was positive for tumor cells. A ventriculoperitoneal shunt was placed and a chemotherapeutic regimen C9921 ( methylprednisone, vincristine, procarbazine, hydroxyurea, carmustine, cytarabine, and Cytoxan) was started. She was given 3,200 rad to the whole brain, excluding orbits, and 1,800 additional rad to the tumor locally. Her last chemotherapeutic regimen ended in November 1986, and last radiation treatment was given in February 1986. She was symptom free and had negative CT scans until the present complaints. General review of systems and past ocular history were noncontributory. Significant physical findings were limited to the ocular examination. 96 R. W. HERTLE AND R. M. ROBB FIG. 1. Axial computerized tomography scan of the head ( A) and orbits ( B) completed at the time of original diagnosis and treatment October 16, 1985. Scan A shows the original tumor and a ventriculoperitoneal shunt. Scan B shows symmetric, normal- appearing optic nerves. Uncorrected distance vision was 0.0.20/ 20 - 1 and 0.5. 20/ 40 + 1. Ishihara plate testing showed 11 of 15 correct O. U. dnd 1 of 15 correct 0.5. Confron~ 2~ inl' Vi" l1', 1 field" sh""" d d questionable superior altitudinal defect 0.5. The pupils were equal and measured 4.5 mm in the light and 6.0 mm in the dark. There was a relative afferent pupillary defect ( RAPO) 0.5. Results of motility testing were normal except for minimal limitation of vertical gaze. Goldmann applanation tonometry was 16 mm Hg O. U. Results of the remainder of the external and slit~ lampexaminations were normal. There was no proptosis, ptosis, palpable mass, or tenderness on reposition of the globe. The lenses and vitreous were clear O. U. The right disk was flat with a cup- to- disk ratio of 0.25, normal vessels and neuroretinal rim, and spontaneous venous pulsations. The macula, vessels, and periphery were normal. The left fundus showed 7- 9 diopters of pale disk edema. There were no spontaneous venous pulsations and the disk margin was obscured. There was retinal nerve fiber layer edema extending from the disk toward the macula. There were no hemorrhages or exudates and minimal disk hyperemia. The remainder of the fundus was normal. Lumbar puncture the same day revealed an opening pressure of 120 mm of water. The cerebrospinal fluid glucose was 42 mg% ( normal 5~ 75 mg%), and the total protein was 135 mg% ( normal 12- 25 mg%). Cytological findings showed lymphocytes, monocytes, and rare atypical, probably reactive mononuclear cells. CT scan of the head and orbits showed diffuse left optic nerve swelling from the optic foramen to the globe ( Fig. 2). The CT scan was otherwise without abnormalities. Electrolytes, complete blood count, liver function tests, blood urea nitrogen, creatinine, and urine analysis were normal. On July 22, 1987, 2 days later, there was no change in the eye examination. Goldmann visual fields showed an enlarged blind spot 0.5. Oral prednisone was started at 50 mg p. o. qd. On July 28, 1987 there was no change in the eye examination with a visual acuity 0.5. of 20/ 40 + 3 and a RAPO 0.5. A cisternogram was done, which showed no entrance of dye into the sheath of the left optic nerve. A magnetic resonance ( MR) imaging scan was done also, but it was not helpful in distinguishing between edema and infiltration of the optic nerve. On August 6, 1987, 4 days after the prednisone had been discontinued, the condition of the left eye had worsened. The visual acuity 0.0. was 201 20 and 0.5. 20/ 100. There was now a marked RAPO 0.5. and the disk edema had increased. Neurosurgical consultation was obtained. On August 11, 1987, a subfrontal craniotomy was performed with optic nerve decompression PINEALOBLASTOMA METASTATIC TO OPTIC NERVE 97 FIG. 2. Computerized tomography scan of the orbits on presentation July 20. 1987. It shows diffuse enlargement of the left optic nerve from the globe to the orbital apex. There is the impression that the globe is flattened posteriorly secondary to compression by the swollen left optic nerve. There is no other intraconal or extraconal abnormality. and biopsy of the optic nerve sheath at the orbital apex. The patient tolerated the procedure well. Pathological study of the nerve sheath biopsy showed tumor involvement consistent with metastatic pinealoblastoma ( Fig. 3). On the 1st postoperative day the visual acuity was 20/ 70 0.5. at near with a RAPD and pain with extraocular movement. On the 2nd postoperative day the left lids were swollen, and there was no light perception in the left eye. The left pupil showed no response to direet light. The intraocular pressure by applanation was 18 mm Hg. The fundus showed massive peripapillary subretinal, intraretinal, and subhyaloid bright red hemorrhage ( Fig. 4). The swelling of the optic disc was obscured by the peripapillary hemorrhage. An orbital CT scan on the same day showed no orbital hemorrhage, mass, or change in the size of the optic nerve. On August 15, 1987 the patient was discharged home with plans for radiation treatment, based on exclusion of the orbits from the original fields, and chemotherapy. On December 18, 1987 the patient was seen after four courses of VePesid ( VP- 16) and Cytoxan. Eye examination revealed a visual acuity 0.0. of 20/ 20 and no light perception 0.5. The left fundus FIG. 3. Photomicrograph of the histopathology of the optic nerve sheath biopsy. This shows metastatic small cell tumor involvement. I Gin Neuro- ophthalmol, Vol. 10, No. 2, 1990 98 R. W. HERTLE AND R. M. ROBB FIG. 4. Fundus photograph taken of the left eye 2 days after the subfrontal craniotomy. There is profound disk edema and massive peripapillary intraretinal and subretinal hemorrhage. showed decreased optic nerve edema with atrophy, resorbed hemorrhage, and residual peripapillary vitreoretinal folds ( Fig. 5). A CT scan done in December 1987 showed a reduction in the size of the left optic nerve. Goldmann visual field examination of the right eye was full. On February 26, 1988 the patient received 2,500 rad in one session to the left optic nerve and orbit. The patient responded to treatment temporarily, but had recurrence of the tumor in January 1989. She has been given further treatment with VP- 16 and Cytoxan, and more recently with carboplatinum, but the response of the tumor to treatment has been disappointing. DISCUSSION Pineal tumors usually present with increased intracranial pressure, papilledema, nausea, vomiting, and headaches ( 1,2). They can cause the Parinaud's syndrome ( paralysis of upward gaze, convergence nystagmus, convergence on attempted upward gaze, and light- near dissociation of the pupils) as well as sixth nerve palsy, deafness due to involvement of the auditory pathways, and cerebellar ataxia ( secondary to involvement of the superior cerebellar peduncle) ( 1,2,4). An uncommon but highly suggestive triad is precocious puberty anrl r, willpcj,' nn in d 111< 111' patient ( 1,5,6). In Borit FIG. 5. Fundus photograph taken of the left eye 3 months after surgical decompression and four courses of chemotherapy. There is remaining elevation of the disk with diffuse atrophy and peripapillary vitreoretinal folds. The vascular tree of the posterior pole is filled with blood. and Blackwood's report of eight cases the symptoms included headaches, visual changes ( usually blurring, decreased acuity, or visual field abnormalities), abnormal oculomotor findings, and cerebellar and auditory changes ( 7). Metastatic spread of pinealomas is usually to the leptomeninges ( 1- 6). Extensive leptomeningeal spread may be associated with invasion of the midbrain, epithalamus, and third ventricle ( 7). Local spread to the chiasm as well as primary ectopic suprasellar pinealomas were reported by Horrax and Wyatt, Backer and Rucker, Kageyama and Belsky ( 8- 10). The latter two authors differentiate three types of ectopic pinealomas: those that are metastatic to the floor of the third ventricle and grow into the pituitary stalk, the posterior lobe of the hypophysis, and the optic tract and chiasm; those that arise primarily in the third ventricle and invade the hypothalamus, the neurohypophysis, and the optic pathway; and last, those that arise primarily in the chiasm alone ( 10). Pinealoblastoma is a malignant tumor that belongs to the medulloblastoma- neuroblastoma group, but that, like retinoblastoma, bears signs of its special field of origin. Histologically patternless aggregates of small, uniform cells are present, interspersed with large areas of necrosis, frequent hemorrhages, and tiny foci of calcification. The PINEALOBLASTOMA METASTATIC TO OPTIC NERVE 99 cells have oval or elongated nuclei, and a variable amount of lightly eosinophilic cytoplasm. They have a small nucleolus, indistinct cell boundaries, frequent mitotic figures, and occasional tumor giant cells. Rare Homer- Wright and Flexner- Wintersteiner rosettes are seen ( 6). Historically, the surgical treatment of pineal tumors has been complicated by an operative mortality of 3~ 70% ( 6,7). Preferred treatment has been irradiation after a drainage procedure ( 6,7). This method has been associated with an overall mortality rate < 5% and a 5- year survival rate of 6~ 70% ( 3,6,7). Recently, with advanced techniques, surgical mortality has been brought down to < 5% ( 3). This is advantageous for the treatment of the 2~ 25% of tumors that are benign, encapsulated, or radioresistant. If adjuvant chemotherapy or immunotherapy is to be employed in the treatment of these tumors, precise identification of histologic type is necessary. Wara et al. in the Children's Cancer Study Group, reporting on pineal parenchymal tumors, state that patients with these tumors fare poorly, with an overall survival rate of 14% ( one of seven) ( 3). The incidence of spinal cord metastasis was 2% ( 3). Among 234 brain neoplasms seen at the Children's Hospital of Philadelphia, 11% were in the pineal region, 32% of those were pineal parenchymal tumors, and 50% ( 4 of 8) of these died of the disease ( 2). All the patients that died had evidence of subarachnoid dissemination at the time of death. The outcome of all their patients seemed to be directly dependent on the specific type of tumor. The authors conclude that surgical exploration and biopsy for specific diagnosis was advisable. Although measurements of cerebrospinal fluid n- fetoprotein and human chorionic gonadotropin had been suggested by others ( 11), the Philadelphia group concluded that these tests were unreliable ( 2). Our case of optic nerve sheath metastasis is consistent with the tendency for pinealoblastomas to spread via the leptomeninges. We are not aware of previously reported cases with unilateral disk edema, either as a presenting sign of the original tumor, or as a sign of recurrence. We know that there was no clinical or radiologic evidence of tumor in the optic nerve at the time of the original diagnosis. Subsequent CT scans did not include views of the orbits, and we do not know when the tumor disseminated to the left optic nerve. The diagnosis ultimately required surgical biopsy. Lumbar puncture, CT scan, and MR imaging established that there was no elevation of intracranial pressure. This implies that there was an obstruction of the subarachnoid space around the nerve at the apex of the orbit, a possibility supported by the failure of radiopaque dye to outline the optic nerve in the cisternogram. The disk edema was evidently secondary to a block of axonal flow at the orbital apex. A recent report of isolated medulloblastoma metastatic to the optic nerve confirms its susceptibility to local tumor spread ( 12). It is not clear to us why the vision of the left eye was lost after surgical exploration of the orbital apex. The vision was intact on the 1st postoperative day, but by the 2nd postoperative day it had been lost, perhaps as the result of a vascular event that was associated with massive hemorrhage around the disk. Although light perception was lost in the involved eye, treatment of the recurrent tumor was temporarily beneficial. We hope that this report will aid in the future diagnosis and treatment of pinealoblastomas. REFERENCES 1. Jooma R, Kendall BE. Diagnosis and management of pineal tumors. I Neurosurg 1983; 58: 654- 65. 2. Packer RJ, Sutton LN, Rosenstock JG, Rorke LB, Bilaniuk LT, Zimmerman RA, Littman PA, Bruce DA, Schut L. Pineal region tumors of childhood. Pediatrics 1984; 74: 97- 102. 3. Wara WM, Jenkin DT, Evans A, Erntel L Hittle R, Ortega J. 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