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Cavernous Hemangiomas of the Orbit
Cavernous Hemangiomas of the Orbit MARK C. RUCHMAN, MD, * JOSEPH FLANAGAN, MDt
Abstract: Patients with cavernous hemangioma of the orbit seen at the Wills Eye Hospital for the 1a-year period, 1972-1981 were reviewed in a retrospective manner. This benign vascular tumor most typically presented as unilateral painless proptosis in a middle-aged woman. The duration of symptoms was between 6 months and 2 years. CT scanning demonstrated a well-circumscribed, homogeneous tumor without bony erosion. Ultrasonography typically showed good sound transmission with moderate internal reflectivity. It is not possible to distinguish clinically a benign cavernous hemangioma from the rare and potentially lethal hemangiopericytoma. Since recurrence and metastasis of hemangiopericytoma may b!"l related to incomplete excision, fastidious dissection and removal of all tumors thought to be cavernous hemangioma before surgery is advised for fear the final pathology may indicate hemangiopericytoma. [Key words: cavernous hemangioma, orbital turn or, proptosis, vascular tumor.] Ophthalmology 90: 1328-1336, 1983
Cavernous hemangiomas are a common orbital tumor. 1•2 Despite their histologically benign nature, their frequent location in the muscle cone may compromise optic nerve function and produce visual loss in otherwise healthy adults. A retrospective study was performed to help define the natural history and clinical features of this tumor. We add our observations to those of Harris 3 and Kopelow,4 who published the two principle reviews of this tumor already in the literature. One patient in our series had many of the clinical features of cavernous hemangioma of the orbit. Histologically, however, the tumor was a hemangiopericytoma. We reviewed the literature of this rare orbital tumor to stress the manner in which its management differs from that of cavernous hemangioma.
From the Southern New England Regional Eye Center, Waterbury, Connecticut: and the Oculoplastic Service, Wills Eye Hospital, Philadelphia, Pennsylvania. t Presented at the International Symposium on Eyelid, Orbital and Lacrimal Surgery, San Francisco, California, October 29-30, 1982.
MATERIALS AND METHODS Complete medical records of all patients with cavernous hemangiomas of the orbit seen at the Wills Eye Hospital for the IO-year period 1972-1981 were reviewed in a retrospective manner. In order to not overlook any cases, the initial medical records search was performed for the general entity "hemangioma." Many of these patients were infants whose hemangiomas often showed spontaneous regression during the first years of life. Though these patients were usually not biopsied, they were, on clinical findings alone, obvious cases of capillary hemangiomas of the lid and orbit. We accept the findings of Haik,5 Flanagan,6 and Reese 7 that these represent a distinct clinical entity. All such patients were excluded from the study. . All patients in this series with cavernous hemangiomas of the orbit came to orbitotomy. Histopathologic specimens were available for correlation with preoperative photographs, ultrasonography, plain skull radiographs, and computed tomography.
This paper is a thesis submitted in partial fulfillment of the requirements for Fellowship in the American Society of Ophthalmic Plastic and Reconstructive Surgeons.
RESULTS
Reprint requests to Mark Ruchman, Southern New England Regional Eye Center, 87 Grandview Ave., Waterbury, CT 06708.
Thirteen histologically confirmed cases of cavernous hemangioma of the orbit were seen at the Wills Eye Hos-
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Table 1. Cavernous Hemangiomas of the Orbit Wills Eye Hospital 1972-1981 Age
Sex
Side of Involvement
Duration of Symptoms
36 31
Male Female
Left Right
5 months Unknown
21
Female
Left
1 Year
30
Female
Right
2 Years
67
Female
Left
2 Years
53
Female
Right
6 months
59 47
Male Female
Left Right
18 months 5 Years
22
Male
Left
32
Male
58 65 36
Location of Tumors
Vision
Proptosis
Intraconal Intraconal
20/20 20/20
3 mm 3 mm
Lower lid Anterior orbit Superotemporal Orbital rim Lower lid Anterior orbit Intraconal
20/20
None
20/20
None
20/30
None
20/200
12 mm
20/20 20/40
4 mm 8 mm
1 Year
Intraconal Sup nasal Orbital rim Intraconal
20/200
6 mm
Right
1 Year
Intraconal
20/20
6 mm
Female
Right
4 Months
20/25
None
Female Female
Right Right
18 Months 1 Year
Lower lid Anterior orbit Intraconal Infero lateral orbit
20/30 20/20
None 4 mm
pital during the lO-year period, 1972-1981 (Table 1). Nine were women, four were men. The average age was 43, range 21-67. Seven had right-sided involvement, six had left. Seven of 13 tumors were located within the muscle cone (Fig 1), and six of 13 were located outside the muscle cone or anteriorly in the orbit (Fig 2). The complaints that brought the patient to medical attention were proptosis, diminished visual acuity, diplopia, palpable mass, or swelling of the lid. Typically, the patient was bothered by several of the above complaints and it was difficult to determine which was first or preeminent. Twelve of 13 patients were able to date the onset of their symptoms. Nine of 12 patients noted the beginning of symptoms 8 months or less prior to seeking medical attention. Two patients noted their onset 2 years and one patient 5 years before consulting a physician. These latter three patients had tumors that were located more anteriorly. Generally, those patients that had tumors located within the muscle cone tended to present earlier. Nine of the 13 patients presented with proptosis. The average amount of proptosis was 6 mm, (range 3-12 mm, median 4 mm). Though the progression of the proptosis was slow (average 3 mm per year) its rate was also highly variable. One patient developed 12 mm of proptosis in 6 months, another 8 mm in 5 years. In none of our patients was the proptosis intermittent. One patient claimed the proptosis increased when her head was placed in a dependent position. This finding was not corroborated by other observers. Pain was not a prominent complaint in any of our patients with proptosis. Eight of 13 patients presented objective signs of impaired visual function: reduced acuity, field disturbance,
Comment Disc edema Afferent pupil defect constricted field
Disc edema afferent pupil Disc edema Disc edema Disc edema afferent pupil No visual disturbance, only proptosis Disc edema Choroidal folds
afferent pupil defects, disc edema, or choroidal folds. Six of these eight had tumors located within the muscle cone. Visual acuity was 20/20 in three of these six patients with their current refraction. In the remaining three patients with intraconal tumors, visual acuity was 20/30 in one and 20/200 in two. All patients with intraconal tumors had the expected associated findings of disc edema, desaturation to red and Marcus Gunn pupils. Two patients had impaired visual function with tumors outside the muscle cone. One of these two patients had a tumor located in the superonasal aspect of the orbit. The mass was palpable along the orbital rim. Diplopia was present in upgaze. There was "mild disc edema." The visual acuity was 20/40. In the second of these two patients, vision was 20/70 but improved to 20/20 with pinhole, suggesting an acquired anisometropic hyperopia. This tumor was located in the inferolateral aspect of the orbit. Choroidal folds were present though their precise location was not noted in the record. Seven of 13 patients had preoperative photographs to document their proptosis or lid swelling. None of these patients had any cutaneous vascular anomalies or stigmata that might suggest prior, although regressed, hemangiomas of the skin in association with their orbital tumor. Computed tomographic scanning of the orbit was the single most useful test in evaluating patients with cavernous hemangiomas of the orbit. Nine of 13 patients had this study performed. The four patients in whom this study was not performed either had very anterior, small, easily palpable lesions or else presented during the early days of computed tomographic scanning when either the machines were not readily available or the resolution had 1329
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Fig 1. A 65-year-old woman presented with slowly progressive proptosis of the right globe. A, computed tomographic scans demonstrated a wellcircumscribed contrast enhancing mass lesion within the muscle cone. Axial view. B, coronal view. C, the tumor was removed with a cryoprobe through a lateral orbitotomy. Histologic examination revealed a cavernous hemangioma.
not improved to the point where it was useful in the diagnosis of orbital disease. The lesions in our patients had characteristic findings on computed tomographic scanning. Regardless of their location, all tumors were 1330
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well circumscribed, contrast enhancing mass lesions. Tissue borders were delineated sharply from surrounding structures. Where the tumor was adjacent to bone, normal bony contour was not disturbed nor was bony erosion present. Five of 13 patients had A and B mode ultrasonograms performed as part of their preoperative evaluation (Fig 3). Four of these studies were performed by ophthalmologists with special training in ultrasonography, and one was performed by a radiologist. Three of five also underwent computed tomographic scanning of the orbit. The two patients who had ultrasonograms performed without computed tomographic scans were evaluated at the time when computed tomography was in its infancy. B-mode ultrasonography demonstrated sharply defined echoes surrounding the tumor, especially at its most anterior extent. The body of the tumor was sonolucent with good sound transmission. A-mode documented a moderate degree of internal reflectivity. Imaging of the tumor was technically difficult in anterior orbital locations. The test was most useful when performed with a water bath in evaluating intraconal lesions. In two of the five cases where ultrasonography was performed the study was not considered diagnostic and the examiner would not venture a tissue diagnosis. In three of five cases, the findings were considered representative enough that the examiner specifically listed cavernous hemangioma as a likely diagnosis. In reviewing the ultrasonograms of patients with cavernous hemangiomas of the orbit, a study was shown to us as demonstrating typical features of this tumor. A 52year-old woman presented with a 3-month history of proptosis of the right globe. Vision was 20/20. Fundus examination was normal. There were no striae. B-scan demonstrated a mass lesion just superior to the optic nerve, within the muscle cone. Ultrasonographically it had a well-demarcated anterior border echo. Sound transmission through the tumor was good. There was moderate to low internal reflectivity. With compression on the globe, the volume of the mass demonstrably decreased (Fig 4). Though the history, clinical features, and ultrasonogram were all consistent with a cavernous hemangioma, histologic examination revealed a cystic neurilemoma. When plain skull radiographs were performed, they were invariably normal and were not considered helpful. None of our patients had arteriography or venography. All 13 patients came to surgical exploration. Twelve had orbitotomies performed by ophthalmic surgeons. Tumor location in the orbit was such that six required a Kronlein procedure (Fig 5). In all 12 cases, the tumor was removed completely. Review of operative reports revealed that the tumor was described repeatedly as "red," "purplish," "spongy," and "encapsulated." It could often be "shelled out" without significant bleeding. Extraction from surrounding soft tissues was often facilitated by the use of a cryoprobe. One of the 13 patients had the roof of her orbit removed (Naffzinger approach) by a neurosurgeon. The mass was biopsied but because of its close proximity to apical orbital structures the attending neurosurgeon considered en-bloc removal dangerous.
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Fig 2. A, 59-year-old white man presented with an 18-month history of swelling of the left lower lid and a well-defined mass palpable along the inferior orbital rim. This tumor was removed through an anterior orbitotomy. Histologic examination revealed a cavernous hemangioma. B, computed tomographic scan demonstrated a well circumscribed round tumor. Axial view. C, coronal view.
In 12 of 13 patients, there was only one tumor present at orbitotomy. In one patient, however, we suspect two separate tumors may have been present. This 30-yearold woman presented with a 2-year history of a nontender
mass slowly increasing in size along the superotemporal orbital rim. Ultrasonography revealed a defect in the nasal side of the retro-orbital fat pad that was considered characteristic of a cavernous hemangioma. Ultrasonograph-
Fig 3. A, B-mode ultrasonogram of cavernous hemangioma demonstrates sharply defined border echoes with good sound transmission. B, A-mode ultrasonogram of cavernous hemangioma demonstrating moderate internal reflectivity.
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Fig 4. A, B-mode ultrasonogram of cystic neurilemoma has a sonic appearance similar to cavernous hemangioma. B, size of tumor decreases on compression of orbital contents.
ically, this deep orbital tumor was not contiguous with the very anterior lesion that brought the patient to medical attention. Because the deep orbital tumor was not causing any visual compromise, orbitotomy for its removal was not considered indicated. Since the deep orbital lesion was interpreted by experienced ultrasonographers as being consistent with a cavernous hemangioma, we postulate that this orbit may have harbored two such tumors, one anterior and the second posterior. Each of the orbitotomies was performed by an experienced orbital surgeon. Complications of the en-bloc resection of the tumor are few. One patient with a superonasal tumor had a postoperative underaction of the superior oblique muscle. One patient with an intraconal tumor requiring a Kronlein procedure had a postoperative lateral rectus paresis. Each of these deficits were transient. There were no cases of postoperative orbital cellulitis or hemorrhage. In those patients undergoing total resection of the tumor, postoperative visual acuities were equal to, or better than the preoperative acuity in all patients. No patients lost central vision in association with the surgery. The poorest preoperative visual acuity was 20/200 in a 22-year-old man with an intraconal tumor and disc edema. After Kronlein orbitotomy, vision in that eye improved to 20/20. Surgical specimens were fixed in formalin and processed in the standard manner. Hematoxylin-eosin prepared sections were reviewed by ophthalmic pathologists who made a histologic diagnosis of cavernous hemangioma in each case (Fig 6). The tumors were composed of widely dilated vascular channels lined by generally thin or attentuated endothelial cells. Red blood cells were often present in the vascular channels. A thin capsule of fibrous tissue was often present. The histologic appearances of these tumors were similar to those noted by Harris 3 and Kopelow.4 Thirteen patients included in our review had histologically confirmed cavernous hemangiomas of the orbit. An additional patient had many of the clinical and radiographic features of this tumor, however, histologically 1332
this was a hemangiopericytoma. This 30-year-old woman presented with a I-year history of a shadow across the nasal aspect of her right eye. Ophthalmic evaluation at that time was normal. Two months prior to presentation at the Wills Eye Hospital, the right eye began to protrude. Skull films and thyroid evaluation were normal. On examination, vision was 20/25 in the right eye and 20/15 in the left eye with 3 mm of proptosis of the right globe. Chorioretinal striae were present in the temporal periphery. Computed tomographic scan documented a wellcircumscribed mass lesion in the lateral aspect of the orbit (Fig 7A). Ultrasonography revealed a mass lesion with well-demarcated border echoes. Sound transmission through the tumor was excellent, allowing good delineation of the posterior tumor edge. There was moderate to high internal reflectivity (Fig 7B). A Kronlein orbitotomy was performed to remove this well-encapsulated tumor. The histopathologic diagnosis was hemangiopericytoma.
DISCUSSION Cavernous hemangiomas are a common benign tumor of the orbit. I •2 In a consecutive series of 520 patients presenting to a neurosurgical service with orbital disease, Wende 9 found it to be the most common primary orbital tumor. Yet in reality, it is an uncommon finding and most clinicians will see few, if any, in their practice. In a retrospective study of orbital vascular tumors seen at Moorfields Eye Hospital, the major referral center for the United Kingdom, Wright,8 reported ten cases seen over a 5-year period. In a review of surgical pathology specimens accumulated over a to-year period at the UCLA Medical Center4 six were cavernous hemangiomas of the orbit. In the largest retrospective study, Harris and Jakobiec lO reported 66 cases accumulated over a 40-year period, largely from the practice of Dr. Algernon Reese at the Institute of Ophthalmology in New York. The
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Fig S. 31-year-old woman required a Kronlein lateral orbitotomy for removal of an intraconal cavernous hemangioma. A, incision sight is delineated with marking pen. A silk suture is passed below the lateral rectus muscle. A moist Weck-cell sponge maintains corneal hydration. B, the lateral wall is removed with a Stryker saw. C,the tumor is removed from surrounding soft tissues with a cryoprobe. D, encapsulated tumor removed completely. E, postoperative photograph demonstrating intact lateral rectus function.
incidence of this disease as reported from major referral centers in London, Los Angeles, and New York is 2.0, 0.6, and 1.65 per year respectively. Our results of 13 cases in 10 years, an incidence of 1.3 new cases per year is in keeping with other reports. Harris3 noted a clear predilection for this tumor to affect middle-aged women. Seventy percent of his patients were women. The average age was 42 years (range 1867). The demography of our patient population is remarkably similar. Sixty-nine percent (9 of 13) of our
patients were women. The average age was 43 years (range 21-67). Similar findings appear in the studies ofWright8 and Kopelow. 4 All of our patients were Caucasian as were the patients studied by Harris. 3 We concluded from these similarities that our patient population is representative of the disease. The predilection of this tumor for middleaged women is genuine. In reviewing studies similar to our own, we can find no data to support geographic or ethnic factors as being significant vectors in this disease. Our tumors were about evenly divided between those 1333
Fig 6. A, Light photomicrograph of cavernous hemangioma demonstrating widely dilated blood filled vascular channels (hematoxylin-eosin, X 10). 8, High power view demonstrating endothelial cells lining the vascular lumens (hematoxylin-eosin, x 125).
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Fig 7. A, computed tomographic scan of a 30-year-old woman with a histologically confirmed hemangiopericytoma. B, A-mode ultrasonogram of the same patient demonstrating moderate internal reflectivity. Neither computed tomography nor ultrasonography can distinguish a hemangiopericytoma from a cavernous hemangioma.
located within the muscle cone and those located more anteriorly in the orbit. This is somewhat at variance with the findings of Wright,S where nine of ten tumors originated at the superior orbital fissure, and of Kopelow4 where five of six were located in the posterior aspect of the orbit. We do not believe that this difference defines a particular subpopulation of patients. Rather, it more likely represents a sampling error in a small series. Additionally, it is also likely that the more anterior tumors were removed ". . . in the community" and only the deep orbital tumors were referred to the major academic center. Twelve of our 13 tumors were solitary masses in the orbit. We reported one case where a deep tumor occurred in the same orbit as a biopsy confirmed anterior tumor. The deep tumor had the ultrasonographic characteristics of a cavernous hemangioma and was distinct from the more anterior lesion. We concluded that this may have represented two cavernous hemangiomas in the same orbit. Interestingly, additional reports of such tumor multiplicity have appeared. In Kopelow's smaller series4 of six patients, one had two distinct tumors in the same orbit. Harris3 describes one patient with two tumors in the same orbit. In this case, they were each removed in separate surgeries. He also reports one patient who had five separate hemangiomas removed at one sitting. We conclude that though tumor multiplicity is rare, it may occur. The orbital surgeon should be mindful of this uncommon variant in evaluating patients with this tumor. In the preoperative evaluation of patients with orbital tumors, we found computed tomography with both axial and coronal reconstruction to be invaluable. The tumor had a characteristic rounded, well-circumscribed appearance. There was no associated inflammation or infiltration of adjacent structures. When the tumor was adjacent to bone there was no contiguous erosion or fossa formation. The tumor typically enhanced with contrast. II Radiographically, the appearance could also be consistent with a neurilemoma or hemangiopericytoma. When the
tumor is adjacent or contiguous with the optic nerve, meningioma or glioma must also be considered. The ultrasonographic characteristics of orbital cavernous hemangioma have been described by Coleman. 12 We reviewed the ultrasonograms of a middle-aged woman with proptosis (the population at risk for a cavernous hemangioma of the orbit). Her tumor had well-circumscribed borders, good sound transmission and moderate internal reflectivity: all features characteristic of a cavernous hemangioma. However, histologically, the tumor was a cystic neurilemoma. We present this to stress the point that neither computed tomography nor ultrasonography can translate a radiographic or sonic image into a tissue diagnosis. For best results, both should be employed in evaluating patients with orbital tumors. Computed tomographic scanning will define the size, shape, and relationship of the tumor to adjoining structures, while ultrasonography will define internal tissue characteristics. However, even with both these modalities, errors may occur. Hemangiopericytoma, a tumor whose histology and biologic behavior is very different from cavernous hemangioma, cannot be distinguished from it by either computed tomographic scanning or ultrasonographyY Arteriography and venography have both been evaluated in the diagnosis of this tumor. Despite initial interest,13,14 their utility has not been demonstrated and they add little to the evaluation that is not already known by the combined use of the less invasive modalities of computed tomography and ultrasonography. On venography, the tumor mass is indicated indirectly by displacement of the superior or inferior ophthalmic vein. If the tumor is not largely thrombosed, a stain of contrast material may be seen. Similarly, arteriography l5 only gives indirect evidence of tumor mass by displacing branches of the ophthalmic artery. The absence of an arterial "blush" corroborates the clinical observation that these tumors are low flow systems largely independent of the general circulation. 1335
OPHTHALMOLOGY •
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One of our patients had a histologically confirmed hemangiopericytoma. Preoperative computed tomography and ultrasonography failed to distinguish this tumor from the more benign cavernous hemangioma (Fig 7). Since the natural history of this tumor is often ominous, it is worthy of special comment and must always be considered when the differential diagnosis includes cavernous hemangioma. Prior to the work of Stout,16 neoplastic proliferations of pericytes were not recognized as entities distinct from endothelial cell proliferations. He classified the hemangiopericytoma as an entity to be differentiated from the hemangioendothelioma. The distinction is now accepted by most pathologists and is corroborated by the characteristic appearance with reticulin stain of each tumor. The details of these staining characteristics are beyond the scope of this paper and the reader is referred to the excellent review by Jakobiec. 17 Hemangiopericytoma of the orbit is a rare tumor. It comprised only 1.7% of 764 orbital tumors reviewed at the Mayo Clinic. 18 It is more commonly seen in such extraorbitallocations as the retroperitoneum, pelvis, and soft tissues of the trunk and limbs. Unlike cavernous hemangiomas of the orbit it afflicts both sexes equally. The problematic feature of this tumor is that a benign or malignant biologic activity often cannot be predicted on the basis of current histopathologic techniques. 19.20 In a large review of nonorbital hemangiopericytomas, a high rate of either metastasis or local recurrence was found. 21 In a separate study BackwinkeJ22 stated that 50% of the tumors had a malignant course with either local recurrence or metastasis. In Jakobiec's series l7 of seven cases of orbital hemangiopericytomas, two patients had late recurrences (25 years and 6 years) that eventually required exenteration. In Henderson's review l8 of 11 cases seen at the Mayo Clinic, two patients died of metastatic disease and two had local recurrences, one intracranially. Preoperative computed tomography and ultrasonography will give the surgeon no clue as to whether he is dealing with a benign cavernous hemangioma or a potentially lethal hemangiopericytoma. Similarly, the intraoperative appearance is not helpful. The tumor is well encapsulated and looks grossly like a cavernous hemangioma. Since local recurrence may be related to incomplete excision,20 we recommend fastidious surgical technique and careful en-bloc resection of all tumors thought to be cavernous hemangiomas for the fear they may actually be the potentially lethal hemangiopericytoma. Signs of local recurrence or metastasis typically develop years or decades later. 2o Thus these patients require careful postoperative follow-up for the rest of their lives.
ACKNOWLEDGMENTS The authors wish to thank Dr. Peter J. Savino and the late Dr. Gerard Shannon for allowing us to review their medical
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records. We also wish to thank Dr. Mary A. Stefanyszyn and Dr. Andrew Eller for preparing the light photomicrographs. Mrs. Nancy Cimino provided assistance in the preparation of this manuscript. This project was supported, in part, by a grant from the Research to Prevent Blindness Foundation.
REFERENCES 1. Henderson JW. Orbital Tumors, 2nd ed. New York: Brian C. Decker, 1980; 128. 2. Reese AB. Tumors of the Eye, 3rd ed. Hagerstown: Harper & Row, 1976; 264. 3. Harris GJ, Jakobiec FA. Cavemous hemangiomas of the orbit. J Neurosurg 1979; 51 :219-28. 4. Kopelow SM, Foos RY, Straatsma BR, et al. Cavernous hemangioma of the orbit. Int Ophthalmol Clin 1971; 11(3):113-25. 5. Haik BG, Jakobiec FA, Ellsworth RM, Jones IS. Capillary hemangioma of the lid and orbit: an analysiS of the clinical features and therapeutic results in 101 cases. Ophthalmology 1979; 86:760-92. 6. Flanagan JC. Vascular problems of the orbit. Ophthalmology 1979; 86:896-913. 7. Reese AB. Expanding lesions of the orbit. Trans Ophthalmol Soc UK, 1971; 91 :85-1 04. 8. Wright JE. Orbital vascular anomolies. Trans Am Acad Ophthalmol Otolaryngol 1974; 78:606-16. 9. Wende S, Kazner E, Grumme T. The diagnostic value of computed tomography and orbital diseases: a cooperative study of 520 cases. Neurosurg Rev 1980; 3:43-9. 10. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: a clinicopathologic analysiS of sixty-six cases. In: Jakobiec FA, ed. Ocular and Adnexal Tumors. Birmingham: Aesculapius Publishing Co, 1978; 741-81. 11. Forbes GS, Sheedy PF II, Waller RR. Orbital tumors evaluated by computer tomography. Radiology 1980; 136: 101-11. 12. Coleman DJ, Jack RL, Franzen LA. High resolution B-scan ultrasonography of the orbit. II. Hemangiomas of the orbit. Arch Ophthalmol 1972; 88:368-74. 13. Krayenbuhl H. The value of orbital angiography for diagnosis of unilateral exophthalmos. J Neurosurg 1962; 19:289-301. 14. Vignaud T, Clay C, Bilaniuc LT. Venography of the orbit; an analytical report of 413 cases. Radiology 1974; 110:373-82. 15. Dilenge D. Arteriography in angiomas of the orbit. Radiology 1974; 113:355-61. 16. Stout AP. Hemangiopericytoma: a study of twenty-five new cases. Cancer 1949; 2:1027-35. 17. Jakobiec FA, Howard GM, Jones IS, Wolff M. Hemangiopericytoma of the orbit. Am J Ophthalmol1974; 78:816-34. 18. Henderson JW, Farrow Gw. Primary orbital hemangiopericytoma; an aggressive and potentially malignant neoplasm. Arch Ophthalmol1978; 96:666-73. 19. Tomik F, Vojacek K, Horvath A. Hemangiopericytoma. Neoplasma 1977; 24:445-51. 20. Croxatto JO, Font RL. Hemangiopericytoma of the orbit: a clinicopathologic study of 30 cases. Hum Pathol 1982; 13:210-8. 21. McMaster MJ, Soule EH, Ivins JC. Hemangiopericytoma; a clinicopathologic study and long-term followup of 60 patients. Cancer 1975; 36:2232-44. 22. Backwinkel KD, Diddams JA. Hemangiopericytoma; report of a case and comprehensive review of the literature. Cancer 1970; 25:896901.
Abstract: Patients with cavernous hemangioma of the orbit seen at the Wills Eye Hospital for the 1a-year period, 1972-1981 were reviewed in a retrospective manner. This benign vascular tumor most typically presented as unilateral painless proptosis in a middle-aged woman. The duration of symptoms was between 6 months and 2 years. CT scanning demonstrated a well-circumscribed, homogeneous tumor without bony erosion. Ultrasonography typically showed good sound transmission with moderate internal reflectivity. It is not possible to distinguish clinically a benign cavernous hemangioma from the rare and potentially lethal hemangiopericytoma. Since recurrence and metastasis of hemangiopericytoma may b!"l related to incomplete excision, fastidious dissection and removal of all tumors thought to be cavernous hemangioma before surgery is advised for fear the final pathology may indicate hemangiopericytoma. [Key words: cavernous hemangioma, orbital turn or, proptosis, vascular tumor.] Ophthalmology 90: 1328-1336, 1983
Cavernous hemangiomas are a common orbital tumor. 1•2 Despite their histologically benign nature, their frequent location in the muscle cone may compromise optic nerve function and produce visual loss in otherwise healthy adults. A retrospective study was performed to help define the natural history and clinical features of this tumor. We add our observations to those of Harris 3 and Kopelow,4 who published the two principle reviews of this tumor already in the literature. One patient in our series had many of the clinical features of cavernous hemangioma of the orbit. Histologically, however, the tumor was a hemangiopericytoma. We reviewed the literature of this rare orbital tumor to stress the manner in which its management differs from that of cavernous hemangioma.
From the Southern New England Regional Eye Center, Waterbury, Connecticut: and the Oculoplastic Service, Wills Eye Hospital, Philadelphia, Pennsylvania. t Presented at the International Symposium on Eyelid, Orbital and Lacrimal Surgery, San Francisco, California, October 29-30, 1982.
MATERIALS AND METHODS Complete medical records of all patients with cavernous hemangiomas of the orbit seen at the Wills Eye Hospital for the IO-year period 1972-1981 were reviewed in a retrospective manner. In order to not overlook any cases, the initial medical records search was performed for the general entity "hemangioma." Many of these patients were infants whose hemangiomas often showed spontaneous regression during the first years of life. Though these patients were usually not biopsied, they were, on clinical findings alone, obvious cases of capillary hemangiomas of the lid and orbit. We accept the findings of Haik,5 Flanagan,6 and Reese 7 that these represent a distinct clinical entity. All such patients were excluded from the study. . All patients in this series with cavernous hemangiomas of the orbit came to orbitotomy. Histopathologic specimens were available for correlation with preoperative photographs, ultrasonography, plain skull radiographs, and computed tomography.
This paper is a thesis submitted in partial fulfillment of the requirements for Fellowship in the American Society of Ophthalmic Plastic and Reconstructive Surgeons.
RESULTS
Reprint requests to Mark Ruchman, Southern New England Regional Eye Center, 87 Grandview Ave., Waterbury, CT 06708.
Thirteen histologically confirmed cases of cavernous hemangioma of the orbit were seen at the Wills Eye Hos-
1328
0161-6420/83/1100/1328/$1.25 © American Academy of Ophthalmology
RUCHMAN AND FLANAGAN
•
CAVERNOUS HEMANGIOMAS
Table 1. Cavernous Hemangiomas of the Orbit Wills Eye Hospital 1972-1981 Age
Sex
Side of Involvement
Duration of Symptoms
36 31
Male Female
Left Right
5 months Unknown
21
Female
Left
1 Year
30
Female
Right
2 Years
67
Female
Left
2 Years
53
Female
Right
6 months
59 47
Male Female
Left Right
18 months 5 Years
22
Male
Left
32
Male
58 65 36
Location of Tumors
Vision
Proptosis
Intraconal Intraconal
20/20 20/20
3 mm 3 mm
Lower lid Anterior orbit Superotemporal Orbital rim Lower lid Anterior orbit Intraconal
20/20
None
20/20
None
20/30
None
20/200
12 mm
20/20 20/40
4 mm 8 mm
1 Year
Intraconal Sup nasal Orbital rim Intraconal
20/200
6 mm
Right
1 Year
Intraconal
20/20
6 mm
Female
Right
4 Months
20/25
None
Female Female
Right Right
18 Months 1 Year
Lower lid Anterior orbit Intraconal Infero lateral orbit
20/30 20/20
None 4 mm
pital during the lO-year period, 1972-1981 (Table 1). Nine were women, four were men. The average age was 43, range 21-67. Seven had right-sided involvement, six had left. Seven of 13 tumors were located within the muscle cone (Fig 1), and six of 13 were located outside the muscle cone or anteriorly in the orbit (Fig 2). The complaints that brought the patient to medical attention were proptosis, diminished visual acuity, diplopia, palpable mass, or swelling of the lid. Typically, the patient was bothered by several of the above complaints and it was difficult to determine which was first or preeminent. Twelve of 13 patients were able to date the onset of their symptoms. Nine of 12 patients noted the beginning of symptoms 8 months or less prior to seeking medical attention. Two patients noted their onset 2 years and one patient 5 years before consulting a physician. These latter three patients had tumors that were located more anteriorly. Generally, those patients that had tumors located within the muscle cone tended to present earlier. Nine of the 13 patients presented with proptosis. The average amount of proptosis was 6 mm, (range 3-12 mm, median 4 mm). Though the progression of the proptosis was slow (average 3 mm per year) its rate was also highly variable. One patient developed 12 mm of proptosis in 6 months, another 8 mm in 5 years. In none of our patients was the proptosis intermittent. One patient claimed the proptosis increased when her head was placed in a dependent position. This finding was not corroborated by other observers. Pain was not a prominent complaint in any of our patients with proptosis. Eight of 13 patients presented objective signs of impaired visual function: reduced acuity, field disturbance,
Comment Disc edema Afferent pupil defect constricted field
Disc edema afferent pupil Disc edema Disc edema Disc edema afferent pupil No visual disturbance, only proptosis Disc edema Choroidal folds
afferent pupil defects, disc edema, or choroidal folds. Six of these eight had tumors located within the muscle cone. Visual acuity was 20/20 in three of these six patients with their current refraction. In the remaining three patients with intraconal tumors, visual acuity was 20/30 in one and 20/200 in two. All patients with intraconal tumors had the expected associated findings of disc edema, desaturation to red and Marcus Gunn pupils. Two patients had impaired visual function with tumors outside the muscle cone. One of these two patients had a tumor located in the superonasal aspect of the orbit. The mass was palpable along the orbital rim. Diplopia was present in upgaze. There was "mild disc edema." The visual acuity was 20/40. In the second of these two patients, vision was 20/70 but improved to 20/20 with pinhole, suggesting an acquired anisometropic hyperopia. This tumor was located in the inferolateral aspect of the orbit. Choroidal folds were present though their precise location was not noted in the record. Seven of 13 patients had preoperative photographs to document their proptosis or lid swelling. None of these patients had any cutaneous vascular anomalies or stigmata that might suggest prior, although regressed, hemangiomas of the skin in association with their orbital tumor. Computed tomographic scanning of the orbit was the single most useful test in evaluating patients with cavernous hemangiomas of the orbit. Nine of 13 patients had this study performed. The four patients in whom this study was not performed either had very anterior, small, easily palpable lesions or else presented during the early days of computed tomographic scanning when either the machines were not readily available or the resolution had 1329
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Fig 1. A 65-year-old woman presented with slowly progressive proptosis of the right globe. A, computed tomographic scans demonstrated a wellcircumscribed contrast enhancing mass lesion within the muscle cone. Axial view. B, coronal view. C, the tumor was removed with a cryoprobe through a lateral orbitotomy. Histologic examination revealed a cavernous hemangioma.
not improved to the point where it was useful in the diagnosis of orbital disease. The lesions in our patients had characteristic findings on computed tomographic scanning. Regardless of their location, all tumors were 1330
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well circumscribed, contrast enhancing mass lesions. Tissue borders were delineated sharply from surrounding structures. Where the tumor was adjacent to bone, normal bony contour was not disturbed nor was bony erosion present. Five of 13 patients had A and B mode ultrasonograms performed as part of their preoperative evaluation (Fig 3). Four of these studies were performed by ophthalmologists with special training in ultrasonography, and one was performed by a radiologist. Three of five also underwent computed tomographic scanning of the orbit. The two patients who had ultrasonograms performed without computed tomographic scans were evaluated at the time when computed tomography was in its infancy. B-mode ultrasonography demonstrated sharply defined echoes surrounding the tumor, especially at its most anterior extent. The body of the tumor was sonolucent with good sound transmission. A-mode documented a moderate degree of internal reflectivity. Imaging of the tumor was technically difficult in anterior orbital locations. The test was most useful when performed with a water bath in evaluating intraconal lesions. In two of the five cases where ultrasonography was performed the study was not considered diagnostic and the examiner would not venture a tissue diagnosis. In three of five cases, the findings were considered representative enough that the examiner specifically listed cavernous hemangioma as a likely diagnosis. In reviewing the ultrasonograms of patients with cavernous hemangiomas of the orbit, a study was shown to us as demonstrating typical features of this tumor. A 52year-old woman presented with a 3-month history of proptosis of the right globe. Vision was 20/20. Fundus examination was normal. There were no striae. B-scan demonstrated a mass lesion just superior to the optic nerve, within the muscle cone. Ultrasonographically it had a well-demarcated anterior border echo. Sound transmission through the tumor was good. There was moderate to low internal reflectivity. With compression on the globe, the volume of the mass demonstrably decreased (Fig 4). Though the history, clinical features, and ultrasonogram were all consistent with a cavernous hemangioma, histologic examination revealed a cystic neurilemoma. When plain skull radiographs were performed, they were invariably normal and were not considered helpful. None of our patients had arteriography or venography. All 13 patients came to surgical exploration. Twelve had orbitotomies performed by ophthalmic surgeons. Tumor location in the orbit was such that six required a Kronlein procedure (Fig 5). In all 12 cases, the tumor was removed completely. Review of operative reports revealed that the tumor was described repeatedly as "red," "purplish," "spongy," and "encapsulated." It could often be "shelled out" without significant bleeding. Extraction from surrounding soft tissues was often facilitated by the use of a cryoprobe. One of the 13 patients had the roof of her orbit removed (Naffzinger approach) by a neurosurgeon. The mass was biopsied but because of its close proximity to apical orbital structures the attending neurosurgeon considered en-bloc removal dangerous.
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Fig 2. A, 59-year-old white man presented with an 18-month history of swelling of the left lower lid and a well-defined mass palpable along the inferior orbital rim. This tumor was removed through an anterior orbitotomy. Histologic examination revealed a cavernous hemangioma. B, computed tomographic scan demonstrated a well circumscribed round tumor. Axial view. C, coronal view.
In 12 of 13 patients, there was only one tumor present at orbitotomy. In one patient, however, we suspect two separate tumors may have been present. This 30-yearold woman presented with a 2-year history of a nontender
mass slowly increasing in size along the superotemporal orbital rim. Ultrasonography revealed a defect in the nasal side of the retro-orbital fat pad that was considered characteristic of a cavernous hemangioma. Ultrasonograph-
Fig 3. A, B-mode ultrasonogram of cavernous hemangioma demonstrates sharply defined border echoes with good sound transmission. B, A-mode ultrasonogram of cavernous hemangioma demonstrating moderate internal reflectivity.
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Fig 4. A, B-mode ultrasonogram of cystic neurilemoma has a sonic appearance similar to cavernous hemangioma. B, size of tumor decreases on compression of orbital contents.
ically, this deep orbital tumor was not contiguous with the very anterior lesion that brought the patient to medical attention. Because the deep orbital tumor was not causing any visual compromise, orbitotomy for its removal was not considered indicated. Since the deep orbital lesion was interpreted by experienced ultrasonographers as being consistent with a cavernous hemangioma, we postulate that this orbit may have harbored two such tumors, one anterior and the second posterior. Each of the orbitotomies was performed by an experienced orbital surgeon. Complications of the en-bloc resection of the tumor are few. One patient with a superonasal tumor had a postoperative underaction of the superior oblique muscle. One patient with an intraconal tumor requiring a Kronlein procedure had a postoperative lateral rectus paresis. Each of these deficits were transient. There were no cases of postoperative orbital cellulitis or hemorrhage. In those patients undergoing total resection of the tumor, postoperative visual acuities were equal to, or better than the preoperative acuity in all patients. No patients lost central vision in association with the surgery. The poorest preoperative visual acuity was 20/200 in a 22-year-old man with an intraconal tumor and disc edema. After Kronlein orbitotomy, vision in that eye improved to 20/20. Surgical specimens were fixed in formalin and processed in the standard manner. Hematoxylin-eosin prepared sections were reviewed by ophthalmic pathologists who made a histologic diagnosis of cavernous hemangioma in each case (Fig 6). The tumors were composed of widely dilated vascular channels lined by generally thin or attentuated endothelial cells. Red blood cells were often present in the vascular channels. A thin capsule of fibrous tissue was often present. The histologic appearances of these tumors were similar to those noted by Harris 3 and Kopelow.4 Thirteen patients included in our review had histologically confirmed cavernous hemangiomas of the orbit. An additional patient had many of the clinical and radiographic features of this tumor, however, histologically 1332
this was a hemangiopericytoma. This 30-year-old woman presented with a I-year history of a shadow across the nasal aspect of her right eye. Ophthalmic evaluation at that time was normal. Two months prior to presentation at the Wills Eye Hospital, the right eye began to protrude. Skull films and thyroid evaluation were normal. On examination, vision was 20/25 in the right eye and 20/15 in the left eye with 3 mm of proptosis of the right globe. Chorioretinal striae were present in the temporal periphery. Computed tomographic scan documented a wellcircumscribed mass lesion in the lateral aspect of the orbit (Fig 7A). Ultrasonography revealed a mass lesion with well-demarcated border echoes. Sound transmission through the tumor was excellent, allowing good delineation of the posterior tumor edge. There was moderate to high internal reflectivity (Fig 7B). A Kronlein orbitotomy was performed to remove this well-encapsulated tumor. The histopathologic diagnosis was hemangiopericytoma.
DISCUSSION Cavernous hemangiomas are a common benign tumor of the orbit. I •2 In a consecutive series of 520 patients presenting to a neurosurgical service with orbital disease, Wende 9 found it to be the most common primary orbital tumor. Yet in reality, it is an uncommon finding and most clinicians will see few, if any, in their practice. In a retrospective study of orbital vascular tumors seen at Moorfields Eye Hospital, the major referral center for the United Kingdom, Wright,8 reported ten cases seen over a 5-year period. In a review of surgical pathology specimens accumulated over a to-year period at the UCLA Medical Center4 six were cavernous hemangiomas of the orbit. In the largest retrospective study, Harris and Jakobiec lO reported 66 cases accumulated over a 40-year period, largely from the practice of Dr. Algernon Reese at the Institute of Ophthalmology in New York. The
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Fig S. 31-year-old woman required a Kronlein lateral orbitotomy for removal of an intraconal cavernous hemangioma. A, incision sight is delineated with marking pen. A silk suture is passed below the lateral rectus muscle. A moist Weck-cell sponge maintains corneal hydration. B, the lateral wall is removed with a Stryker saw. C,the tumor is removed from surrounding soft tissues with a cryoprobe. D, encapsulated tumor removed completely. E, postoperative photograph demonstrating intact lateral rectus function.
incidence of this disease as reported from major referral centers in London, Los Angeles, and New York is 2.0, 0.6, and 1.65 per year respectively. Our results of 13 cases in 10 years, an incidence of 1.3 new cases per year is in keeping with other reports. Harris3 noted a clear predilection for this tumor to affect middle-aged women. Seventy percent of his patients were women. The average age was 42 years (range 1867). The demography of our patient population is remarkably similar. Sixty-nine percent (9 of 13) of our
patients were women. The average age was 43 years (range 21-67). Similar findings appear in the studies ofWright8 and Kopelow. 4 All of our patients were Caucasian as were the patients studied by Harris. 3 We concluded from these similarities that our patient population is representative of the disease. The predilection of this tumor for middleaged women is genuine. In reviewing studies similar to our own, we can find no data to support geographic or ethnic factors as being significant vectors in this disease. Our tumors were about evenly divided between those 1333
Fig 6. A, Light photomicrograph of cavernous hemangioma demonstrating widely dilated blood filled vascular channels (hematoxylin-eosin, X 10). 8, High power view demonstrating endothelial cells lining the vascular lumens (hematoxylin-eosin, x 125).
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Fig 7. A, computed tomographic scan of a 30-year-old woman with a histologically confirmed hemangiopericytoma. B, A-mode ultrasonogram of the same patient demonstrating moderate internal reflectivity. Neither computed tomography nor ultrasonography can distinguish a hemangiopericytoma from a cavernous hemangioma.
located within the muscle cone and those located more anteriorly in the orbit. This is somewhat at variance with the findings of Wright,S where nine of ten tumors originated at the superior orbital fissure, and of Kopelow4 where five of six were located in the posterior aspect of the orbit. We do not believe that this difference defines a particular subpopulation of patients. Rather, it more likely represents a sampling error in a small series. Additionally, it is also likely that the more anterior tumors were removed ". . . in the community" and only the deep orbital tumors were referred to the major academic center. Twelve of our 13 tumors were solitary masses in the orbit. We reported one case where a deep tumor occurred in the same orbit as a biopsy confirmed anterior tumor. The deep tumor had the ultrasonographic characteristics of a cavernous hemangioma and was distinct from the more anterior lesion. We concluded that this may have represented two cavernous hemangiomas in the same orbit. Interestingly, additional reports of such tumor multiplicity have appeared. In Kopelow's smaller series4 of six patients, one had two distinct tumors in the same orbit. Harris3 describes one patient with two tumors in the same orbit. In this case, they were each removed in separate surgeries. He also reports one patient who had five separate hemangiomas removed at one sitting. We conclude that though tumor multiplicity is rare, it may occur. The orbital surgeon should be mindful of this uncommon variant in evaluating patients with this tumor. In the preoperative evaluation of patients with orbital tumors, we found computed tomography with both axial and coronal reconstruction to be invaluable. The tumor had a characteristic rounded, well-circumscribed appearance. There was no associated inflammation or infiltration of adjacent structures. When the tumor was adjacent to bone there was no contiguous erosion or fossa formation. The tumor typically enhanced with contrast. II Radiographically, the appearance could also be consistent with a neurilemoma or hemangiopericytoma. When the
tumor is adjacent or contiguous with the optic nerve, meningioma or glioma must also be considered. The ultrasonographic characteristics of orbital cavernous hemangioma have been described by Coleman. 12 We reviewed the ultrasonograms of a middle-aged woman with proptosis (the population at risk for a cavernous hemangioma of the orbit). Her tumor had well-circumscribed borders, good sound transmission and moderate internal reflectivity: all features characteristic of a cavernous hemangioma. However, histologically, the tumor was a cystic neurilemoma. We present this to stress the point that neither computed tomography nor ultrasonography can translate a radiographic or sonic image into a tissue diagnosis. For best results, both should be employed in evaluating patients with orbital tumors. Computed tomographic scanning will define the size, shape, and relationship of the tumor to adjoining structures, while ultrasonography will define internal tissue characteristics. However, even with both these modalities, errors may occur. Hemangiopericytoma, a tumor whose histology and biologic behavior is very different from cavernous hemangioma, cannot be distinguished from it by either computed tomographic scanning or ultrasonographyY Arteriography and venography have both been evaluated in the diagnosis of this tumor. Despite initial interest,13,14 their utility has not been demonstrated and they add little to the evaluation that is not already known by the combined use of the less invasive modalities of computed tomography and ultrasonography. On venography, the tumor mass is indicated indirectly by displacement of the superior or inferior ophthalmic vein. If the tumor is not largely thrombosed, a stain of contrast material may be seen. Similarly, arteriography l5 only gives indirect evidence of tumor mass by displacing branches of the ophthalmic artery. The absence of an arterial "blush" corroborates the clinical observation that these tumors are low flow systems largely independent of the general circulation. 1335
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One of our patients had a histologically confirmed hemangiopericytoma. Preoperative computed tomography and ultrasonography failed to distinguish this tumor from the more benign cavernous hemangioma (Fig 7). Since the natural history of this tumor is often ominous, it is worthy of special comment and must always be considered when the differential diagnosis includes cavernous hemangioma. Prior to the work of Stout,16 neoplastic proliferations of pericytes were not recognized as entities distinct from endothelial cell proliferations. He classified the hemangiopericytoma as an entity to be differentiated from the hemangioendothelioma. The distinction is now accepted by most pathologists and is corroborated by the characteristic appearance with reticulin stain of each tumor. The details of these staining characteristics are beyond the scope of this paper and the reader is referred to the excellent review by Jakobiec. 17 Hemangiopericytoma of the orbit is a rare tumor. It comprised only 1.7% of 764 orbital tumors reviewed at the Mayo Clinic. 18 It is more commonly seen in such extraorbitallocations as the retroperitoneum, pelvis, and soft tissues of the trunk and limbs. Unlike cavernous hemangiomas of the orbit it afflicts both sexes equally. The problematic feature of this tumor is that a benign or malignant biologic activity often cannot be predicted on the basis of current histopathologic techniques. 19.20 In a large review of nonorbital hemangiopericytomas, a high rate of either metastasis or local recurrence was found. 21 In a separate study BackwinkeJ22 stated that 50% of the tumors had a malignant course with either local recurrence or metastasis. In Jakobiec's series l7 of seven cases of orbital hemangiopericytomas, two patients had late recurrences (25 years and 6 years) that eventually required exenteration. In Henderson's review l8 of 11 cases seen at the Mayo Clinic, two patients died of metastatic disease and two had local recurrences, one intracranially. Preoperative computed tomography and ultrasonography will give the surgeon no clue as to whether he is dealing with a benign cavernous hemangioma or a potentially lethal hemangiopericytoma. Similarly, the intraoperative appearance is not helpful. The tumor is well encapsulated and looks grossly like a cavernous hemangioma. Since local recurrence may be related to incomplete excision,20 we recommend fastidious surgical technique and careful en-bloc resection of all tumors thought to be cavernous hemangiomas for the fear they may actually be the potentially lethal hemangiopericytoma. Signs of local recurrence or metastasis typically develop years or decades later. 2o Thus these patients require careful postoperative follow-up for the rest of their lives.
ACKNOWLEDGMENTS The authors wish to thank Dr. Peter J. Savino and the late Dr. Gerard Shannon for allowing us to review their medical
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records. We also wish to thank Dr. Mary A. Stefanyszyn and Dr. Andrew Eller for preparing the light photomicrographs. Mrs. Nancy Cimino provided assistance in the preparation of this manuscript. This project was supported, in part, by a grant from the Research to Prevent Blindness Foundation.
REFERENCES 1. Henderson JW. Orbital Tumors, 2nd ed. New York: Brian C. Decker, 1980; 128. 2. Reese AB. Tumors of the Eye, 3rd ed. Hagerstown: Harper & Row, 1976; 264. 3. Harris GJ, Jakobiec FA. Cavemous hemangiomas of the orbit. J Neurosurg 1979; 51 :219-28. 4. Kopelow SM, Foos RY, Straatsma BR, et al. Cavernous hemangioma of the orbit. Int Ophthalmol Clin 1971; 11(3):113-25. 5. Haik BG, Jakobiec FA, Ellsworth RM, Jones IS. Capillary hemangioma of the lid and orbit: an analysiS of the clinical features and therapeutic results in 101 cases. Ophthalmology 1979; 86:760-92. 6. Flanagan JC. Vascular problems of the orbit. Ophthalmology 1979; 86:896-913. 7. Reese AB. Expanding lesions of the orbit. Trans Ophthalmol Soc UK, 1971; 91 :85-1 04. 8. Wright JE. Orbital vascular anomolies. Trans Am Acad Ophthalmol Otolaryngol 1974; 78:606-16. 9. Wende S, Kazner E, Grumme T. The diagnostic value of computed tomography and orbital diseases: a cooperative study of 520 cases. Neurosurg Rev 1980; 3:43-9. 10. Harris GJ, Jakobiec FA. Cavernous hemangioma of the orbit: a clinicopathologic analysiS of sixty-six cases. In: Jakobiec FA, ed. Ocular and Adnexal Tumors. Birmingham: Aesculapius Publishing Co, 1978; 741-81. 11. Forbes GS, Sheedy PF II, Waller RR. Orbital tumors evaluated by computer tomography. Radiology 1980; 136: 101-11. 12. Coleman DJ, Jack RL, Franzen LA. High resolution B-scan ultrasonography of the orbit. II. Hemangiomas of the orbit. Arch Ophthalmol 1972; 88:368-74. 13. Krayenbuhl H. The value of orbital angiography for diagnosis of unilateral exophthalmos. J Neurosurg 1962; 19:289-301. 14. Vignaud T, Clay C, Bilaniuc LT. Venography of the orbit; an analytical report of 413 cases. Radiology 1974; 110:373-82. 15. Dilenge D. Arteriography in angiomas of the orbit. Radiology 1974; 113:355-61. 16. Stout AP. Hemangiopericytoma: a study of twenty-five new cases. Cancer 1949; 2:1027-35. 17. Jakobiec FA, Howard GM, Jones IS, Wolff M. Hemangiopericytoma of the orbit. Am J Ophthalmol1974; 78:816-34. 18. Henderson JW, Farrow Gw. Primary orbital hemangiopericytoma; an aggressive and potentially malignant neoplasm. Arch Ophthalmol1978; 96:666-73. 19. Tomik F, Vojacek K, Horvath A. Hemangiopericytoma. Neoplasma 1977; 24:445-51. 20. Croxatto JO, Font RL. Hemangiopericytoma of the orbit: a clinicopathologic study of 30 cases. Hum Pathol 1982; 13:210-8. 21. McMaster MJ, Soule EH, Ivins JC. Hemangiopericytoma; a clinicopathologic study and long-term followup of 60 patients. Cancer 1975; 36:2232-44. 22. Backwinkel KD, Diddams JA. Hemangiopericytoma; report of a case and comprehensive review of the literature. Cancer 1970; 25:896901.