| OCR Text |
Show Journal of Neuro- Ophthtilmology 15( 2): 98- 101, 1995. © 1995 Raven Press, Ltd., New York Painful Ophthalmoplegia Caused by Hemangiopericytoma of the Cavernous Sinus Sherman McCalL M. D. and Bret B. Wagenhorst, M. D. Progressive, painful ophthalmoplegia developed in a 34- year- old man. MRI scan revealed an enhancing mass in the left cavernous sinus. Histologic examination of resected tumor revealed reticulin staining and cytologic features of hemangiopericytoma. Characteristics of intracranial hemangiopericytoma are reviewed. Key Words: Hemangiopericytoma- Cavernous sinus- CNS neoplasm- Ophthalmoplegia. From the Department of Ophthalmology, Shaw Air Force Base Hospital, Shaw Air Force Base, South Carolina U. S. A. Address correspondence and reprint requests to Dr. Sherman McCall, 1910 Evans Parkway, Silver Spring, MD 20902, U. S. A. Tumors of the cavernous sinus are rare, accounting for about 0.2% of all intracranial tumors ( 1). From 50% to 75% of tumors involving the cavernous sinus are chordomas, malignant nasopharyngeal extensions, or distant metastases ( 1). Of primary tumors of the cavernous sinus, saccular aneurysms account for about 80% and neurofibromas and meningiomas 10% each ( 1). Meningeal hemangiopericytomas are rarer still, with an incidence of only about 2% of central nervous system meningiomas ( 2). We report a case of primary hemangiopericytoma of the cavernous sinus. A review of the medical literature since 1966 revealed no other cases of hemangiopericytoma in this location. CASE REPORT A 34- year- old, previously healthy man presented with a several- week history of left retro-orbital pain and recent onset of horizontal, binocular diplopia on far left gaze. On examination, visual acuity was 20/ 15 OU. Pupillary responses and automated visual fields were normal. Hertel ex-ophthalmometry revealed no proptosis. There was no ptosis. Ocular movements were normal except there was slightly decreased abduction OS. No disk edema was present. The rest of the ocular, cranial nerve, and systemic neurological examination was normal. Magnetic resonance imaging ( MRI) scan of the brain and orbits with and without contrast revealed a slightly lobulated lesion in the left cavernous sinus with left deviation of the carotid artery ( Fig. 1). The lesion was isointense on Tl- weighted images and slightly hyperintense on T2- weighted images. The lesion enhanced vividly with gadolinium. There was no evidence of orbital involvement. Cerebral arteriogram revealed no evidence of tumor vascularity. The presumed diagnosis was HEMANGIOPERICYTOMA OF THE CAVERNOUS SINUS 99 FIG. 1. Preoperative magnetic resonance images. A: Coronal scan ( Tl = 0 ms, TE = 90 ms, TR = 2000 ms) showing a slightly hyperintense mass in the left cavernous sinus that displaces the internal carotid artery laterally. E3: Postgadolinium axial scan ( Tl = 0 ms, TE = 25 ms, TR = 570 ms) showing a vividly enhancing lesion in the left cavernous sinus, encroaching on the sella turcica and deviating the internal carotid artery laterally. cavernous sinus meningioma. Over the next 3 months the patient developed, on the left, partial third, fourth, and sixth cranial nerve palsies and decreased sensation over VI. The left pupil was noted to constrict on attempted adduction OS. Repeat MRI scan revealed more involvement of the cavernous sinus and invasion of the pituitary fossa. Repeat angiogram revealed narrowing of the intracavernous carotid artery. The patient underwent a frontotemporal craniotomy and orbital osteotomy for tumor excision. After resection the tumor was examined and reviewed by pathology staff and the Neurologic Tumor Board at Walter Reed Army Medical Center. To confirm the diagnosis, the case was also reviewed by the Armed Forces Institute of Pathology. This aggressive neoplasm was highly cellular, with scant cytoplasm and indistinct cellular borders ( Fig. 2). Its appearance was chaotic, with random nuclear orientation throughout. The tumor bulged into compressed, irregularly shaped " staghorn" vessels lined by endothelium reactive for factor VIII. Reticulin staining demonstrated the network of fibers surrounding each neoplastic cell which is highly characteristic of hemangiopericytomas. Clumps of large cells with foamy cytoplasm were present and there were geographic areas of lower cellularity. Cytological examination revealed oval nuclei with course chromatin and prominent nucleioli. Based on these features a pathologic diagnosis of hemangiopericytoma was made. ( 3- 5). Due to involvement of the sella turcica, immu-nohistochemical stains for chromogranin and pituitary hormones were obtained to rule out a primary pituitary tumor. Stains for luteinizing hormone, adrenocorticotropic hormone, growth hormone, thyroid- stimulating hormone, follicle-stimulating hormone, and chromogranin were all nonreactive. Hemangiopericytomas such as this one are distinguished from meningiomas by the lack of tight whorls and psammoma bodies. Likewise, immu-noreactivity for epithelial membrane antigen and the other less frequent meningioma markers ( S100 protein and cytokeratin) is appropriately absent in our case ( 4). Among the other tumors that may mimic hemangiopericytoma histologically, synovial sarcoma is ruled out by location and the absence of cytokeratin immunoreactivity. Mesenchymal chondrosarcoma is excluded by location and the absence of cartilaginous islands ( 6). Vascular neoplasms are excluded by the reactive pattern for factor VIII stain in this tumor, which was restricted to the vessel endothelium and did not extend to the tumor itself. Other neurogenic tumors are ruled out by the absence of chromogranin, glial fibrillary acidic protein, Leu- 7, neuron- specific enolase, and S100 protein ( 3). Post- operatively, the patient developed fatal pulmonary emboli. At autopsy the residual hemangiopericytoma was examined and found to be consistent with the original biopsy sample. No other primary tumor or distant metastasis was discovered. This corresponds with the absence of tumor necrosis and epithelial membrane antigen staining, to rule out metastatic carcinoma in the cavernous sinus. / Neuw- Ophtlmlmol, Vol. 15, No. 2, 1995 200 S. MCCALL AND B. B. WAGENHORST FIG. 2. Top: Low- power view of the tumor showing high cellu-larity with random orientation, scant cytoplasm, and indistinct cell borders. Bottom: High-power cytologic view showing oval nuclei with coarse chromatin and prominent nucleoli. DISCUSSION Hemangiopericytoma is a tumor composed of proliferations of capillaries surrounded by neoplastic perivascular cells presumed to arise from pericytes ( 7). The tumor may be found throughout the body. When found intracranially, it has been called various names including angioblastic meningioma ( hemangiopericytic variant), meningeal hemangiopericytoma, and, simply, hemangiopericytoma. Intracranial hemangiopericytomas are uncommon, representing less than 1% of all central nervous system tumors ( 2). They usually present as firm, vascular tumors attached to the meninges, compressing and displacing brain tissue rather than invading it ( 8,9). Over half the tumors are supratentorial, and they are commonly parasagittal or falcine ( 2). Computed tomography ( CT) scan of these lesions with contrast shows a homoge-nously enhancing tumor with scant surrounding edema ( 10). MRI scan reveals features similar to meningioma, the lesions being isointense with brain on Tl- weighted images. Angiography typically reveals tumors that fill rapidly and drain slowly ( 2). In our case, as in other reports of cavernous sinus tumors ( 11), the first sign of tumor was a partial abducens nerve palsy. This was preceded by several weeks by retro- orbital pain, most likely due to involvement of the trigeminal nerve. It is remarkable that despite the size of the tumor on the initial MRI scan, there were not more pronounced findings on the ocular and cranial nerve examination. It was not until 3 months after the onset of / Ncuro- Ophthalnwl, Vol. 15, No. 2, 1995 HEMANGIOPERICYTOMA OF THE CAVERNOUS SINUS 101 the abducens palsy that signs of a full sphenocav-ernous sinus syndrome, with cranial nerves 3, 4, 5, and 6 involvement, were present ( 12). By this time clinical signs of abnormal regeneration of the oculomotor nerve were evident. As seen in our patient, intracranial hemangiopericytomas may resemble meningioma in location, presenting symptoms and radiographic features, and their true identity may not be recognized until surgical resection and histological examination. However, the epidemiology of the two tumors differs. In one large series of intracranial hemangiopericytomas, 55% of patients were male, the average age at diagnosis was 38- 42 years, and the average survival period was 84 months ( 2). In contrast, meningioma is more common in women, typically presents in the fifties, and has an average survival period of greater than 100 months ( 13). From 20% to 60% of hemangiopericytomas display a low mitotic rate without necrosis or pleo-morphism. Imperfect attempts have been made to correlate this histological appearance to a benign clinical course ( 6). While this tumor has the first two features and did not metastasize clinically, the presence of nuclear pleomorphism places it outside this putative benign variant. It is important to distinguish intracranial hemangiopericytoma from meningioma because of the high recurrence rate and ability to metastasize often seen in hemangiopericytoma ( 10). The recurrence rates at 1, 5, and 10 years after surgery are 15%, 65%, and 76%, respectively. The rate of metastasis is 64% at 15 years ( 2). Recommended treatment is complete surgical resection and follow- up radiation ( 2). REFERENCES 1. Parkinson D, West M. Lesions of the cavernous plexus region. In: Youman JR, ed. Neurological surgery, 3rd Ed., Vol. 5. Philadelphia: WB Saunders, 1990: 3351- 2. 2. Guthrie BL, Ebersold M], Scheithauer BW, Shaw EG. Meningeal hemangiopericytoma: histopathological features, treatment, and long term followup of 44 cases. Neurosurgery 1989; 25: 514- 21. 3. McKeever PE, Blaivas M. The brain, spinal cord, and meninges. In: Sternberg SS, et al, eds. Diagnostic surgical pathology, 2nd Ed. New York: Raven Press; 1994: 409- 92. 4. Burger PC, Scheithauer BW, Vogel FS, Surgical pathology of the nervous system and its coverings. New York: Churchill Livingstone; 1991: 107- 14. 5. Rubinstein JL, Tumors of the central nervous system. In: Atlas of tumor pathology, 2nd ser., Vol 6. Washington, DC: Armed Forces Institute of Pathology; 1972: 180- 4. 6. Brooks JJ. Disorders of soft tissue. In: Sternberg SS, et al. Diagnostic surgical pathology, 2nd Ed. New York: Raven Press; 1994: 147- 229. 7. Stout AP, Murray MR, Hemangiopericytoma: a vascular tumor featuring Zimmerman's pericytes. Ann Surg 1942; 116: 26- 33. 8. Goellner JR, Laws ER, Soule EH, Okazaki H. Hemangiopericytoma of the meninges: Mayo Clinic experience. Am J Clin Pathol 1978; 70: 374- 80. 9. Mena H, Ribas JL, Pezeshkpour GH, et al. Hemangiopericytoma of the central nervous system: a review of 94 cases. Hum Pathol 1991; 22: 84- 91. 10. Servo A, Jaaskelainen J, Wahlstrom T, Haltia M. Diagnosis of intracranial hemangiopericytomas with angiography and CT scanning. Neuroradiology 1985; 27: 38- 43. 11. Sakalas R, Harbison JW, Vines FS, Becker DP. Chronic sixth nerve palsy: an initial sign of basisphenoid tumors. Arch Ophthalmol 1975; 93: 186- 90. 12. Miller N. Topical diagnosis of neuropathic ocular motility disorders. In: Walsh and Hoyt's clinical neuroophthalmology, 4th Ed., Vol. 2. Baltimore: Williams & Wilkins; 1985: 672. 13. Chan RD, Thompson GB. Morbidity, mortality, and quality of life following surgery for intracranial meningiomas. / Neurosurg 1984; 60: 52- 60. / Neuro- Ophthalmol, Vol. 15, No. 2, 1995 |
