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3. Intraocular Reticulum Cell Sarcoma
Ocular Pathology for Clinicians Edited by Frederick A. Jakobiec, MD
3. Intraocular Reticulum Cell Sarcoma MICHAEL D. WAGONER, MD,* JOHN R. GONDER, MD,* DANIEL M. ALBERT, MD,* CHRISTOPHER L. CANNY, MDt ,.._ a: w co ~
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Abstract: Reticulum cell sarcoma is a form of malignant lymphoma that may arise systemically in the reticuloendothelial cells of the spleen or lymph nodes, or in the microglial cells of the brain. Intraocular reticulum cell sarcoma occurs most frequently in association with central nervous system reticulum cell sarcoma. Although relatively uncommon, this diagnosis should be considered in any adult patient with vitreitis or chorioretinitis of unknown etiology, particularly if the inflammation does not respond to the usual therapy. The diagnosis of reticulum cell sarcoma must be considered with any uveitis or vitreitis associated with neurologic symptoms. Prompt diagnosis is of importance because the tumor is radiosensitive, and improved visual acuity and survival may follow radiation therapy. [Key words: diffuse histiocytic lymphoma, intraocular tumors, lymphoma, microgliomatosis, ocular inflammation, reticulum cell sarcoma.] Ophthalmology 87: 724-727, 1980
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Reticulum cell sarcoma is a form of malignant lymphoma that may arise systemically in the reticuloendothelial cells of the spleen or lymph nodes 1 or in the microglial cells of the brain. 2 •3 Intraocular involvement with reticulum cell sarcoma, typically presenting with uveitis or vitreitis, occurs most frequently in association with the central nervous system form of the disease.4-7
CASE REPORT A 65-year-old white man initially presented to another hospital in February 1977 with left arm and leg weakness. A right frontoparietal tumor was detected by computed tomography. A brain biopsy performed at the time of craniotomy was interpreted as oligodendroglioma. He was treated with partial exciFrom the Harvard Medical School, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston,* and the University of Western Ontario, Department of Ophthalmology, London, Ontario. t Reprint requests to Daniel M. Albert, MD, Howe Laboratory of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114.
724
sion of the tumor and irradiation, with complete resolution of his symptoms. The patient presented for ophthalmic evaluation in October 1978, complaining of gradually decreased vision associated with numerous floaters and occasional photopsia. His best corrected visual acuity was 6/30 (20/100) in the right eye and hand movements in the left eye. Intraocular tension was lO mm Hg in the right eye and 11 mm Hg in the left eye by applanation tonometry. No flare or cells were present in the anterior chamber of either eye. In both eyes, a prominent cellular reaction with fibrillar infiltrates was present in the vitreous. The appearance of the right fundus was unremarkable. The left fundus contained diffuse infiltrates at the level of the retinal pigment epithelium and choriocapillaris in the superior temporal quadrant. A chorioretinal scar was present inferiorly. The patient was admitted to the University Hospital in London, Ontario, for further evaluation. Neurologic examination showed no abnormality, but computed tomography showed an area of encephalomalacia in the right frontal lobe without evidence of recurrent tumor. The results of chest radiography were normal. A complete blood count was within normal limits. The sedimentation rate was not elevated, and serum protein electrophoresis was normal. Toxoplasmosis, histoplasmosis, and coccidioidococcus titers were not elevated, and skin testing with PPD was nonreactive.
0161-6420/80/0700/0724/$00.70 ©American Academy of Ophthalmology
Fig 1. Left, previtrectomy photograph with vitreous opacities in the left eye. Fig 2. Right, hemorrhagic infiltrative retinitis of the left eye (photographed following vitrectomy).
When the thorough evaluation failed to disclose an etiology of the patient's uveitis, a tentative diagnosis of reticulum cell sarcoma was made because of a prior history of a brain tumor. A therapeutic trial of steroids was initiated with little effect, and the patient was subsequently lost to follow-up until February 1979. At that time, his best corrected visual acuity had decreased to hand movements vision in each'-eye. His vitreous cellular reaction had worsened in both eyes, and prominent vitreous opacities were now present (Fig 1). There was still no evidence of involvement of the anterior segment. A hemorrhagic infiltrative retinitis was present in the inferior temporal vascular arcade of the left eye (Fig 2). A diagnostic pars plana vitrectomy was performed, with cytologic examination revealing reticulum cell sarcoma (Fig 3). By that time the original blocks of tissue from his brain biopsy had been obtained, and re-examination of this specimen confirmed the diagnosis of reticulum cell sarcoma rather than the original diagnosis of oligodendroglioma (Fig 4). The patient was treated with 4,000 rads of irradiation to each eye, with improvement in visual acuity to 6/ 15-2 (20/50-2) in both eyes. There was almost complete clearing of his vitreous opacities and resolution of his infiltrative retinitis. The patient's general condition deteriorated and he was readmitted to the neurology service at the University Hospital in November 1979. On admission he was moribund and unresponsive, precluding meaningful evaluation of his visual acuity. He pursued an inexorably downhill course and died of his disease on November 29, 1979. His left eye was submitted to the Pathology Department of the Massachusetts Eye and Ear Infirmary for histopathologic examination. Gross examination revealed a slightly flattened globe measuring 22 mm horizontally, 23 mm vertically, and 23 mm anteroposteriorly. A vitrectomy scar was present at ten o'clock, 5 mm posterior to the limbus. Upon opening the globe, multiple small areas of chorioretinal scarring were present. Two large areas of scarring were present: one near the vitrectomy site and one near the ora serrata inferiorly. Multiple white intraretinal infiltrates were present in the submacular area. Histopathologically, the cornea was unremarkable. The anterior chamber was deep and did not contain inflammatory cells or tumor cells. The chamber angle was open. The iris and ciliary body were unremarkable. There were multiple small areas of dense chorioretinal adhesions with retinal pigment
epithelium necrosis (Fig 5). Two large areas of chorioretinal scarring were present. One was located near the ora serrata near the vitrectomy site, with detachment of the choroid and retina at that site. The other was located inferiorly near the ora serrata and corresponded to the site previously described in the gross and clinical examination. The choroid was diffusely infiltrated with chronic, nongranulomatous inflammatory cells. The retina was thin and atrophic with disorganization of the layers. The retina was diffusely infiltrated with pleomorphic tumor cells containing large hyperchromatic nuclei and scant cytoplasm (Fig 6). In some sections, characteristic · perivascular involvement with these tumor cells could be seen (Fig 7). The vitreous cavity contained a few remnant fibrils but no cells. The optic nerve showed mild optic atrophy. The pathologic diagnosis was reticulum cell sarcoma with retinal atrophy and chorioretinal scarring.
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DISCUSSION Reticulum cell sarcoma is a form of malignant lymphoma that may be primary in the eye, or secondary to generalized malignant lymphoma or to reticulum cell sarcoma of the central nervous system. Although intraocular involvement has been reported with systemic reticulum cell sarcoma, 4 •8- 10 it is most frequently seen in association with reticulum cell sarcoma of the central nervous system. 4 - 7 Three cases have been described as involving the eyes alone. 5 • 1 L 12 With regard to the relationship of ocular involvement to CNS and systemic involvement, it is not clear whether this process (1) involves the central nervous system primarily with intraocular extension; (2) involves the eye primarily with central nervous system extension; or (3) is a multifocal process diffusely involving the microglial elements of the retina and central nervous system simultaneously. The multifocal involvement seen in reticulum cell sarcoma of the brain, 13 as well as the isolated reports of primary reticulum cell sarcoma of the eye alone, tends to support the latter hypothesis. With intraocular involvement, the differential diagnosis includes other causes of retinal tumors in
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Fig 3. Top leji, vitrectomy specimen showing reticulum cell sarcoma (hematoxylin and eosin, original magnification x400). Fig 4. Top right, brain biopsy tissue revealing reticulum cell sarcoma with typical perivascular pattern (hematoxylin and eosin, original magnification x40). Fig· 5. Middle left, focal area of chorioretinal adhesions (hematoxylin and eosin, original magnification x40). Fig 6. Middle right, diffuse infiltration of tumor cells through all retinal layers (hematoxylin and eosin, original magnification x 100). Fig 7. Bottom, retinal perivascular infiltration with tumor cells (hematoxylin and eosin, original magnification X 160).
adults, including leukemic infiltrates, other lymphomas, and metastatic tumors to the retina. A recent review of the literature from this laboratory 4 reported 23 histologically confirmed cases of reticulum cell sarcoma of the eye, as well as 22 nonconfirmed cases. The age range was 27 to 81 years with an average of 58 years. There was no sex predilection. Typically, the patient presents with decreased visual acuity and eye pain. Although not present in our patient, increased intraocular
726
pressure has been noted in some cases. 5 • 8• 11 Uveitis, predominantly posterior, was present in all cases reviewed by Kiin et al. 4 It usually presents as a unilateral disease, but becomes bilateral in 50% of the cases. Uveitis usually precedes the onset of neurologic symptoms by a period ranging up to nine years, 8 • 14 although it may postdate the development of neurologic deficits, 15 as in our patient. Posterior pole involvement includes vitreous cellular reaction, retinal infiltrates, chorioretinallesions, and subretinal masses. The cells in
the vitreous may be inflammatory, neoplastic, or both. The cells may clump on vitreous fibrils and form opaque transvitreal sheets. 14 Aspiration of vitreous fluid may establish the diagnosis and may improve visual acuity by removing the opacified vitreous. 3 • 14 Exudative retinal detachment, as well as retinal and vitreous hemorrhages, has been observed. Papilledema may occur secondary to direct tumor involvement, increased intracranial pressure, or hypotony resulting from uveitis. 15 The tumor cells are generally pleomorphic with scant cytoplasm and prominent nuclear membranes, and have round, oval, or indented nuclei, many of which have prominent eccentrically located nucleoli. Distinct outpouchings are seen in some of these nucleoli which, when having a finger-like configuration, are considered characteristic of this tumor. The cells in the retina, like those in the brain, may have a perivascular location with fine, concentric reticular fibers surrounding them. Choroidal infiltration is diffuse, in contrast to metastatic carcinoma, which typically forms columns of cells separated by connective tissue. 8 There is a characteristic tendency for reticulum cell sarcoma to form a mass separating Bruch's membrane from the retinal pigment epithelium. 4 •15 Electron-microscopic studies by Horvafl demonstrated the frequent occurrence of intranuclear inclusions and cytoplasmic crystalloids. Pseudopodl:!.l cytoplasmic extensions and cytosomes are occasionally seen, as are autophagic vacuoles. Immature cells lack these features. In addition, electron-dense bundles can sometimes be seen in the intercellular spaces; these are thought to represent a fibrin polymer. The reticulum seen by light microscopy appears on electron microscopy as thin collagen fibers in the intercellular spaces. Reticulum cell sarcoma of the brain is extremely radiosensitive. 16 •17 Intraocular involvement with the tumor often responds to radiotherapy with improved visual acuity. Therapy is palliative and not curative, with the interval between the onset of eye signs and death ranging from six months to ten years. 4 In summary, a case of intraocular reticulum cell sarcoma associated with intracerebral reticulum sell sarcoma has been presented. This diagnosis should be considered in all adults with posterior uveitis, vitreitis, or chorioretinitis of unknown etiology, particularly when seen in association with neurologic symptoms. The importance of proper diagnosis lies in the radiosensitivity of these tumors and the im-
provement in visual acuity and prolonged survival that frequently accompany therapy.
REFERENCES 1. Boyd W. Textbook of Pathology. An Introduction to Medicine. 7th ed. Philadelphia. Lea and Fepiger, 1961; 1109-14. 2. Schaumburg HH, Plank CR, Adam RD. The reticulum cell sarcoma-microglioma group of brain tumors. Brain 1972; 95:199-212. 3. Horvat B, Pena C, Fisher ER. Primary reticulum cell sarcoma (microglioma) of brain. An electron microscopic study. Arch Pathol 1969; 87:609-16. 4. Kim EW, Zakov ZN, Albert OM, et al. Intraocular reticulum cell sarcoma: a case report and literature review. Albrecht von Graefes Arch Klin Exp Ophthalmol 1979; 209:167-78. 5. Klingele TG, Hogan MJ. Ocular reticulum cell sarcoma. Am J Ophthalmol 1975; 79:39-47. 6. Kennerdell JS, Johnson BL, Wisotzky HM. Vitreous cellular reaction associated with reticulum cell sarcoma of the brain. Arch Ophthalmol1975; 93:1341-5. 7. Neault RW, VanScoy RE, Okazaki H, et al. Uveitis associated with isolated reticulum cell sarcoma of the brain. Am J Ophthalmol 1972: 73:431-6. 8. Vogel MH, Font RL, Zimmerman LE, et al. Reticulum cell sarcoma of the retina and uvea; report of six cases and review of the literature. Am J Ophthalmol 1968; 66:205-15. 9. Cooper EL, Riker JL. Malignant lymphoma of the uveal tract. Am J Ophthalmol1975; 34:1153-8. 10. Nevins RC Jr, Frey WW, Elliot JH. Primary, solitary intraocular reticulum cell sarcoma (microgliomatosis): a clinicopathologic case report. Trans Am Acad Ophthalmol Otolaryngol 1968; 72:867-76. 11. Barr CC, Green WR, Payne JW, et al. Intraocular reticulum-cell sarcoma: clinicopathologic study of four cases and review of the literature. Surv Ophthalmol 1975; 19:224-39. 12. Sullivan SF, Dallow RL. Intraocular reticulum cell sarcoma: its dramatic response to systemic chemotherapy and its angiogenic potential. Ann Ophthalmol 1977; 9:401-406. 13. Burstein SO, Kernahan JW, Uihlein A. Neoplasms of the reticuloendothelial system of the brain. Cancer 1963; 16:289-305. 14. Michels RG, Knox DL, Erozan YS, et al. Intraocular reticulum cell sarcoma: Diagnosis by pars plana Vitrectomy. Arch Ophthalmol 1975; 93:1331 -5. 15. Minckler OS, Font RL, Zimmerman LE. Uveitis and reticulum cell sarcoma of brain with bilateral neoplastic seeding of vitreous without retinal or uveal involvement. Am J Ophthalmol 1975; 80:433-9. 16. Fisher D, Mantell BS, Urich H. The clinical diagnosis and treatment of microgliomatosis: report of a case. J Neural Neurosurg Psychiatry 1969; 32:474-8. 17. Kernahan JW, Uihlein A. Sarcomas of the Brain. Springfield: Charles C Thomas, 1962.
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3. Intraocular Reticulum Cell Sarcoma MICHAEL D. WAGONER, MD,* JOHN R. GONDER, MD,* DANIEL M. ALBERT, MD,* CHRISTOPHER L. CANNY, MDt ,.._ a: w co ~
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Abstract: Reticulum cell sarcoma is a form of malignant lymphoma that may arise systemically in the reticuloendothelial cells of the spleen or lymph nodes, or in the microglial cells of the brain. Intraocular reticulum cell sarcoma occurs most frequently in association with central nervous system reticulum cell sarcoma. Although relatively uncommon, this diagnosis should be considered in any adult patient with vitreitis or chorioretinitis of unknown etiology, particularly if the inflammation does not respond to the usual therapy. The diagnosis of reticulum cell sarcoma must be considered with any uveitis or vitreitis associated with neurologic symptoms. Prompt diagnosis is of importance because the tumor is radiosensitive, and improved visual acuity and survival may follow radiation therapy. [Key words: diffuse histiocytic lymphoma, intraocular tumors, lymphoma, microgliomatosis, ocular inflammation, reticulum cell sarcoma.] Ophthalmology 87: 724-727, 1980
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Reticulum cell sarcoma is a form of malignant lymphoma that may arise systemically in the reticuloendothelial cells of the spleen or lymph nodes 1 or in the microglial cells of the brain. 2 •3 Intraocular involvement with reticulum cell sarcoma, typically presenting with uveitis or vitreitis, occurs most frequently in association with the central nervous system form of the disease.4-7
CASE REPORT A 65-year-old white man initially presented to another hospital in February 1977 with left arm and leg weakness. A right frontoparietal tumor was detected by computed tomography. A brain biopsy performed at the time of craniotomy was interpreted as oligodendroglioma. He was treated with partial exciFrom the Harvard Medical School, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston,* and the University of Western Ontario, Department of Ophthalmology, London, Ontario. t Reprint requests to Daniel M. Albert, MD, Howe Laboratory of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114.
724
sion of the tumor and irradiation, with complete resolution of his symptoms. The patient presented for ophthalmic evaluation in October 1978, complaining of gradually decreased vision associated with numerous floaters and occasional photopsia. His best corrected visual acuity was 6/30 (20/100) in the right eye and hand movements in the left eye. Intraocular tension was lO mm Hg in the right eye and 11 mm Hg in the left eye by applanation tonometry. No flare or cells were present in the anterior chamber of either eye. In both eyes, a prominent cellular reaction with fibrillar infiltrates was present in the vitreous. The appearance of the right fundus was unremarkable. The left fundus contained diffuse infiltrates at the level of the retinal pigment epithelium and choriocapillaris in the superior temporal quadrant. A chorioretinal scar was present inferiorly. The patient was admitted to the University Hospital in London, Ontario, for further evaluation. Neurologic examination showed no abnormality, but computed tomography showed an area of encephalomalacia in the right frontal lobe without evidence of recurrent tumor. The results of chest radiography were normal. A complete blood count was within normal limits. The sedimentation rate was not elevated, and serum protein electrophoresis was normal. Toxoplasmosis, histoplasmosis, and coccidioidococcus titers were not elevated, and skin testing with PPD was nonreactive.
0161-6420/80/0700/0724/$00.70 ©American Academy of Ophthalmology
Fig 1. Left, previtrectomy photograph with vitreous opacities in the left eye. Fig 2. Right, hemorrhagic infiltrative retinitis of the left eye (photographed following vitrectomy).
When the thorough evaluation failed to disclose an etiology of the patient's uveitis, a tentative diagnosis of reticulum cell sarcoma was made because of a prior history of a brain tumor. A therapeutic trial of steroids was initiated with little effect, and the patient was subsequently lost to follow-up until February 1979. At that time, his best corrected visual acuity had decreased to hand movements vision in each'-eye. His vitreous cellular reaction had worsened in both eyes, and prominent vitreous opacities were now present (Fig 1). There was still no evidence of involvement of the anterior segment. A hemorrhagic infiltrative retinitis was present in the inferior temporal vascular arcade of the left eye (Fig 2). A diagnostic pars plana vitrectomy was performed, with cytologic examination revealing reticulum cell sarcoma (Fig 3). By that time the original blocks of tissue from his brain biopsy had been obtained, and re-examination of this specimen confirmed the diagnosis of reticulum cell sarcoma rather than the original diagnosis of oligodendroglioma (Fig 4). The patient was treated with 4,000 rads of irradiation to each eye, with improvement in visual acuity to 6/ 15-2 (20/50-2) in both eyes. There was almost complete clearing of his vitreous opacities and resolution of his infiltrative retinitis. The patient's general condition deteriorated and he was readmitted to the neurology service at the University Hospital in November 1979. On admission he was moribund and unresponsive, precluding meaningful evaluation of his visual acuity. He pursued an inexorably downhill course and died of his disease on November 29, 1979. His left eye was submitted to the Pathology Department of the Massachusetts Eye and Ear Infirmary for histopathologic examination. Gross examination revealed a slightly flattened globe measuring 22 mm horizontally, 23 mm vertically, and 23 mm anteroposteriorly. A vitrectomy scar was present at ten o'clock, 5 mm posterior to the limbus. Upon opening the globe, multiple small areas of chorioretinal scarring were present. Two large areas of scarring were present: one near the vitrectomy site and one near the ora serrata inferiorly. Multiple white intraretinal infiltrates were present in the submacular area. Histopathologically, the cornea was unremarkable. The anterior chamber was deep and did not contain inflammatory cells or tumor cells. The chamber angle was open. The iris and ciliary body were unremarkable. There were multiple small areas of dense chorioretinal adhesions with retinal pigment
epithelium necrosis (Fig 5). Two large areas of chorioretinal scarring were present. One was located near the ora serrata near the vitrectomy site, with detachment of the choroid and retina at that site. The other was located inferiorly near the ora serrata and corresponded to the site previously described in the gross and clinical examination. The choroid was diffusely infiltrated with chronic, nongranulomatous inflammatory cells. The retina was thin and atrophic with disorganization of the layers. The retina was diffusely infiltrated with pleomorphic tumor cells containing large hyperchromatic nuclei and scant cytoplasm (Fig 6). In some sections, characteristic · perivascular involvement with these tumor cells could be seen (Fig 7). The vitreous cavity contained a few remnant fibrils but no cells. The optic nerve showed mild optic atrophy. The pathologic diagnosis was reticulum cell sarcoma with retinal atrophy and chorioretinal scarring.
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DISCUSSION Reticulum cell sarcoma is a form of malignant lymphoma that may be primary in the eye, or secondary to generalized malignant lymphoma or to reticulum cell sarcoma of the central nervous system. Although intraocular involvement has been reported with systemic reticulum cell sarcoma, 4 •8- 10 it is most frequently seen in association with reticulum cell sarcoma of the central nervous system. 4 - 7 Three cases have been described as involving the eyes alone. 5 • 1 L 12 With regard to the relationship of ocular involvement to CNS and systemic involvement, it is not clear whether this process (1) involves the central nervous system primarily with intraocular extension; (2) involves the eye primarily with central nervous system extension; or (3) is a multifocal process diffusely involving the microglial elements of the retina and central nervous system simultaneously. The multifocal involvement seen in reticulum cell sarcoma of the brain, 13 as well as the isolated reports of primary reticulum cell sarcoma of the eye alone, tends to support the latter hypothesis. With intraocular involvement, the differential diagnosis includes other causes of retinal tumors in
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Fig 3. Top leji, vitrectomy specimen showing reticulum cell sarcoma (hematoxylin and eosin, original magnification x400). Fig 4. Top right, brain biopsy tissue revealing reticulum cell sarcoma with typical perivascular pattern (hematoxylin and eosin, original magnification x40). Fig· 5. Middle left, focal area of chorioretinal adhesions (hematoxylin and eosin, original magnification x40). Fig 6. Middle right, diffuse infiltration of tumor cells through all retinal layers (hematoxylin and eosin, original magnification x 100). Fig 7. Bottom, retinal perivascular infiltration with tumor cells (hematoxylin and eosin, original magnification X 160).
adults, including leukemic infiltrates, other lymphomas, and metastatic tumors to the retina. A recent review of the literature from this laboratory 4 reported 23 histologically confirmed cases of reticulum cell sarcoma of the eye, as well as 22 nonconfirmed cases. The age range was 27 to 81 years with an average of 58 years. There was no sex predilection. Typically, the patient presents with decreased visual acuity and eye pain. Although not present in our patient, increased intraocular
726
pressure has been noted in some cases. 5 • 8• 11 Uveitis, predominantly posterior, was present in all cases reviewed by Kiin et al. 4 It usually presents as a unilateral disease, but becomes bilateral in 50% of the cases. Uveitis usually precedes the onset of neurologic symptoms by a period ranging up to nine years, 8 • 14 although it may postdate the development of neurologic deficits, 15 as in our patient. Posterior pole involvement includes vitreous cellular reaction, retinal infiltrates, chorioretinallesions, and subretinal masses. The cells in
the vitreous may be inflammatory, neoplastic, or both. The cells may clump on vitreous fibrils and form opaque transvitreal sheets. 14 Aspiration of vitreous fluid may establish the diagnosis and may improve visual acuity by removing the opacified vitreous. 3 • 14 Exudative retinal detachment, as well as retinal and vitreous hemorrhages, has been observed. Papilledema may occur secondary to direct tumor involvement, increased intracranial pressure, or hypotony resulting from uveitis. 15 The tumor cells are generally pleomorphic with scant cytoplasm and prominent nuclear membranes, and have round, oval, or indented nuclei, many of which have prominent eccentrically located nucleoli. Distinct outpouchings are seen in some of these nucleoli which, when having a finger-like configuration, are considered characteristic of this tumor. The cells in the retina, like those in the brain, may have a perivascular location with fine, concentric reticular fibers surrounding them. Choroidal infiltration is diffuse, in contrast to metastatic carcinoma, which typically forms columns of cells separated by connective tissue. 8 There is a characteristic tendency for reticulum cell sarcoma to form a mass separating Bruch's membrane from the retinal pigment epithelium. 4 •15 Electron-microscopic studies by Horvafl demonstrated the frequent occurrence of intranuclear inclusions and cytoplasmic crystalloids. Pseudopodl:!.l cytoplasmic extensions and cytosomes are occasionally seen, as are autophagic vacuoles. Immature cells lack these features. In addition, electron-dense bundles can sometimes be seen in the intercellular spaces; these are thought to represent a fibrin polymer. The reticulum seen by light microscopy appears on electron microscopy as thin collagen fibers in the intercellular spaces. Reticulum cell sarcoma of the brain is extremely radiosensitive. 16 •17 Intraocular involvement with the tumor often responds to radiotherapy with improved visual acuity. Therapy is palliative and not curative, with the interval between the onset of eye signs and death ranging from six months to ten years. 4 In summary, a case of intraocular reticulum cell sarcoma associated with intracerebral reticulum sell sarcoma has been presented. This diagnosis should be considered in all adults with posterior uveitis, vitreitis, or chorioretinitis of unknown etiology, particularly when seen in association with neurologic symptoms. The importance of proper diagnosis lies in the radiosensitivity of these tumors and the im-
provement in visual acuity and prolonged survival that frequently accompany therapy.
REFERENCES 1. Boyd W. Textbook of Pathology. An Introduction to Medicine. 7th ed. Philadelphia. Lea and Fepiger, 1961; 1109-14. 2. Schaumburg HH, Plank CR, Adam RD. The reticulum cell sarcoma-microglioma group of brain tumors. Brain 1972; 95:199-212. 3. Horvat B, Pena C, Fisher ER. Primary reticulum cell sarcoma (microglioma) of brain. An electron microscopic study. Arch Pathol 1969; 87:609-16. 4. Kim EW, Zakov ZN, Albert OM, et al. Intraocular reticulum cell sarcoma: a case report and literature review. Albrecht von Graefes Arch Klin Exp Ophthalmol 1979; 209:167-78. 5. Klingele TG, Hogan MJ. Ocular reticulum cell sarcoma. Am J Ophthalmol 1975; 79:39-47. 6. Kennerdell JS, Johnson BL, Wisotzky HM. Vitreous cellular reaction associated with reticulum cell sarcoma of the brain. Arch Ophthalmol1975; 93:1341-5. 7. Neault RW, VanScoy RE, Okazaki H, et al. Uveitis associated with isolated reticulum cell sarcoma of the brain. Am J Ophthalmol 1972: 73:431-6. 8. Vogel MH, Font RL, Zimmerman LE, et al. Reticulum cell sarcoma of the retina and uvea; report of six cases and review of the literature. Am J Ophthalmol 1968; 66:205-15. 9. Cooper EL, Riker JL. Malignant lymphoma of the uveal tract. Am J Ophthalmol1975; 34:1153-8. 10. Nevins RC Jr, Frey WW, Elliot JH. Primary, solitary intraocular reticulum cell sarcoma (microgliomatosis): a clinicopathologic case report. Trans Am Acad Ophthalmol Otolaryngol 1968; 72:867-76. 11. Barr CC, Green WR, Payne JW, et al. Intraocular reticulum-cell sarcoma: clinicopathologic study of four cases and review of the literature. Surv Ophthalmol 1975; 19:224-39. 12. Sullivan SF, Dallow RL. Intraocular reticulum cell sarcoma: its dramatic response to systemic chemotherapy and its angiogenic potential. Ann Ophthalmol 1977; 9:401-406. 13. Burstein SO, Kernahan JW, Uihlein A. Neoplasms of the reticuloendothelial system of the brain. Cancer 1963; 16:289-305. 14. Michels RG, Knox DL, Erozan YS, et al. Intraocular reticulum cell sarcoma: Diagnosis by pars plana Vitrectomy. Arch Ophthalmol 1975; 93:1331 -5. 15. Minckler OS, Font RL, Zimmerman LE. Uveitis and reticulum cell sarcoma of brain with bilateral neoplastic seeding of vitreous without retinal or uveal involvement. Am J Ophthalmol 1975; 80:433-9. 16. Fisher D, Mantell BS, Urich H. The clinical diagnosis and treatment of microgliomatosis: report of a case. J Neural Neurosurg Psychiatry 1969; 32:474-8. 17. Kernahan JW, Uihlein A. Sarcomas of the Brain. Springfield: Charles C Thomas, 1962.
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