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Orbital Hemangiopericytoma or Vascular Meningioma?
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preservation of color vision : Report of a case fol lowing asphyxiation. Arch. Ophth. 9:957, 1933. 5. Schlaegel, T. F. : Psychosomatic Ophthalmology. Baltimore, Williams and Wilkins, 1957. 6. Granger, G. W. : Personality with visual per ception : A review. J. Ment. Sei. 99:8, 1953.
7. Krill, A. E., and Newell, F. W. : The diagnosis of ocular conversion reaction involving visual function. Arch. Ophth. 79:254, 1968. 8. Kinsbourne, M., and Warrington, E. K. : Ob servations on colour agnosia. J. Neurol. Neurosurg. Psychiat. 27 :296, 1964.
ORBITAL HEMANGIOPERICYTOMA OR VASCULAR H. SAUL SUGAR, M.D.,
GORDON R. F I S H M A N , M.D., AND PAUL GOODMAN,
MENINGIOMA?
SIDNEY KOBERNICK,
M.D.,
M.D.
Detroit, Michigan Definite delineation of pathologic entitiesS from their histologie appearance and stain ing characteristics is often difficult andJ sometimes impossible. Experience with aa case which highlighted the controversial dif ferential diagnosis between orbital hemangiopericytoma and vascular meningioma hasS led to the presentation of this paper for its teaching value.
granuloma were removed through a conjunctival in cision just medial to the lateral canthus. Healing was uneventful, but the proptosis failed to diminish. On January 23, 1968, a mass was felt in the upper nasal portion of the anterior orbit. The proptosis had increased to 7 mm (Fig. 2). The patient was referred for consultation (to H.S.S.). Because only five months had passed since the surgical procedure, further observation was advised. The visual field related to the retinitis sclopeteria is shown in Figure 3. One year later, on February 11, 1969, the visual actuity was reduced to 20/200, corrected, and the proptosis had increased to 10 mm. Surgery was ad vised. Roentgenography at that time showed an inCASE HISTORY A 32-year-old Caucasian man was first seen on„ creased density over the left orbit and left maxil August 14, 1967 (by G. R. F.), because of promi lary antrum with some decrease in the dimensions nence of his left eye for about six weeks. The onsett of the left optic foramen. On March 5, 1969, was apparently sudden, and the condition had(j through an incision over the palpable mass in the changed little since it was first noticed. Dryness andj upper nasal portion of the left upper eyelid, the mass was exposed and dissected free of its surpain around the left eye, usually in the morning,, were associated with the proptosis. The patient'[ rounding tissue. It was found to extend vertically complained of headaches and vague retro-orbital1 to the orbital apex, being narrow at each end and discomfort. His history revealed that, at the age of£ widest at its center. The capusle was removed with seven, a BB shot had penetrated the left orbit. Itj. the tumor except at the orbital apex where it was had been localized by roentgenography, but no at freed separately. A frozen section diagnosis of probtempt had been made to remove it. During the pastt able neurofibroma was made. The wound was then two years, a nasal field defect had been noted in thee closed and a pressure bandage applied. The tumor weighed 10 g and measured 4χ2.5χ2 left eye, associated with chorioretinal scarring„ (Fig. 1). cm. It was fairly soft with a sharply demarcated, Examination showed the right eye to be normalj bosselated surface and a slightly bulging cut surwith visual acuity of 20/20 corrected with —3.00D) face (Fig. 4). Sections of the main tumor showed sphere. Visual acuity of the left eye was 20/20 with[j it to be highly cellular and very vascular with nu correction (—1.75 C +0.75 cyl X 75°), but chorio merous small, closely spaced channels lined by uniform, flattened endothelial cells. Some of the chanretinal scarring was present temporally. There wass weakness of elevation of the left eye and theree nels contained erythrocytes, but most were empty. was a small scar in the nasal portion of the left(. Between the vascular channels were masses of cells (pericytes) having uniform oval vesicular nuclei upper eyelid where the BB had entered. The prop tosis of the left eye measured 4 mm. A subconjunc- and relatively scanty lightly acidophilic cytoplasm tival hemorrhage was present in the left lateralj (Fig. 5). In many areas, nuclei were arranged in canthal region. A small nodule was palpable justj palisade pattern (Fig. 6). Extremely rare mitoses were found after much searching. Occasional thin behind the globe laterally. On August 23, 1967, the BB and an encapsulatedj collagen bands were present. Reticulum stain showed the small blood vessels outlined by fairly From Sinai Hospital of Detroit and Wayne Statee dense sheets of reticulin with delicate reticulin University School of Medicine. fibers extending outward between groups as well as Reprint requests to H. Saul Sugar, M.D., 970D single cells (Fig. 7). Fisher Bldg., Detroit, Michigan 48202. The tumor contained a diffuse light infiltrate of
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Fig. 1 (Sugar, Fishman, Kobernick, and Goodman). Appearance of left fundus showing retinitis sclopetaria resulting from the passage of BB lateral to left macula.
lymphocytes, histiocytes, and rare plasma cells. Also present were moderate numbers of focal small granulomas composed of rounded masses of closely packed histiocytes of epithelioid type (Figs. 6, 8). No multinucleated giant cells or foreign material were noted in any of the granulomas. There was no necrosis within or adjacent to the focal granulomas. They were distributed fairly evenly throughout the tumor with one or two found in most low power fields at various levels of sectioning. The capsule of the tumor was removed sep arately and consisted of a thin layer of compressed collagen. Small amounts of fat and skeletal muscle were adherent to its external surface. No tumor cells appeared to penetrate the capsule. Despite inflammatory areas, the tumor appeared distinctly neoplastic to all observers. A diagnosis of hemangiopericytoma of the orbit was made initially because of the pattern of vascular channels sur rounded by groups of cells thought to be pericytes. The reticulin pattern was thought to be distinctive for hemangiopericytoma. The role of the focal and diffuse inflammatory component is uncertain, but possibly related to the old injury and subsequent or bital surgery. Because of the rarity of the tumor, unique in our experience, consultation was obtained with Dr. Raffaele Lattes of Columbia University. Dr. Lattes
diagnosed the tumor as an orbital meningioma of the vascular type. He felt that the tumor resembled a hemangiopericytoma, but that the reticulin compo nent was too scanty to substantiate the diagnosis and some areas showed cells with a more epithelial than mesenchymal appearance. Further therapy appeared warranted because of uncertainty of completeness of excision at the deep area of the tumor which lay near the optic fora men. Recurrence of tumor was thought to be highly probable regardless of the precision of the diagno sis. Cobalt radiation was given to the left orbit to a total dose of 4250 r over a period of several weeks. Recovery was uneventful and without recurrence over an eight-month period of followup. On May 8,
Fig. 2 (Sugar, Fishman, Kobernick, and Good man). Appearance of patient in February, 1969, before surgery.
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Fig. 3 (Sugar, Fishman, Kobernick, and Goodman). Visual field of left eye in January 1968.
first described by Rouget.2 The pericytes are contractile cells but have no myofibrils. They are variable in size and shape; some appear more related to smooth muscle cells while DISCUSSION others seem more epithelioid. Gallivan3 de The hemangiopericytoma was first de scribed cytological variations ranging from scribed by Stout and Murray in 19421 as a smaller immature cells with oval nuclei and tumor derived from the capillary pericyte minimal cytoplasm arranged in compact masses to mature spindle cells with more abundant cytoplasm and a tendency to whorl formation. The tumor is characterized by endothelial sprouts surrounded by sheaths of pericytic cells. With the reticulin stain, argyrophilic fibrils are found surrounding the pericytes, and separating them from the endothelium. In man most of these rare tumors have most often been found in soft tissues, particularly the thigh, buttock, colon, omenturn, uterus, retroperitoneum, oral cavity and pharynx, mediastinum, orbit, méninges, choroid plexus and spinal canal. About a third are locally invasive and about half produce ^ sugar, Fishman, tusnman, Kobernick, and Good métastases.1 There are no reliable criteria to Fig. 4t (Sugar, man). Gross appearance of tumor in cross section.
1969, exophthalmometer readings were equal in both eyes (Fig. 9). On August 15, 1969, the left visual acuity was 20/30 corrected. The optic disks were normal.
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Fig. 5 (Sugar, Fishman, Kobernick, and Goodman). Highly vascular area showing uniform flattened endothelial cells and groups of larger cells between the vascular channels (hematoxylin, phloxin, and saffron, X370).
Fig. 6 (Sugar, Fishman, Kobernick, and Goodman). Overall view of tumor showing highly vascular cellular areas and few granulomas (hematoxylin, phloxin, and saffron, X17S).
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Fig. 7 (Sugar, Fishman, Kobernick, and Goodman). Reticulum stain showing deposits of reticulin fibers about the vascular channels and delicate fibrils extending between the groups of perivascular cells (Wilder's reticulum, χ370).
Fig. 8 (Sugar, Fishman, Kobernick, and Goodman). Granuloma with tumor (hematoxylin, phloxin, and saffron, X370).
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Fig. 9 (Sugar, Fishman, Kobernick, and Good man). Appearance of patient in 1969, following surgical removal of tumor.
distinguish histologically between benign and malignant tumors. 4 The first orbital tumor of this type re ported in the ophthalmic literature was de scribed in a paper by Goodman.5 The paper was entitled, "Hemangiopericytoma of the orbit," but the histopathologic diagnosis by Irvine was possible hemangiopericytoma, possible angioplastic form of meningioma. This emphasizes exactly the same diagnostic problem which our case presented. Seven other cases involving the orbit, all called he mangiopericytoma, were reported by Stout and Murray 1 (1 case), Stout6 (2), Fox 7 (1), Spaeth and Valdes-Dapena8 ( 1 ), and Nover9 (2). It is difficult to classify orbital meningeal tumors because of the pleomorphism of the cells ensheathing the clusters of small ves sels. If the vessels are ensheathed by meningothelial cells, the tumors are considered to be angioblastic meningiomas. Some of these tumors are identical in appearance with hemangioblastoma of the brain. Bailey and Ford, 10 Bailey and Cushing,11 del Rio Hortega,12 and Courville13 separate the an gioblastic meningiomas from the general group of meningiomas. All of these authors wrote on the subject before Stout's work. Later, in 1950, Courville14 classified the an gioblastic meningiomas without reference to hemangiopericytoma. Begg and Garret,15 and Kruse, 16 considered these tumors of the mé ninges to be hemangiopericytomas. Kruse reported eight cases among 900 tumors of
JULY, 1970
the méninges. He described the pathologic features as extreme vascularity, small, rela tively uniform, oval or elongated nuclei hav ing moderate amounts of reticulated chromatin. The cytoplasm is sparse, as a rule, and the cells tend to spindle appearance, ar ranged in suggestive whorls or pinwheels. The tumor cells lie external to an abundance of thin-walled vascular channels lined by a single layer of endothelial cells. This picture differs in no essential from the description of hemangiopericytomas in other organs. Neither diagnosis, as applied to the tu mors discussed here, is entirely satisfactory. The term hemangiopericytoma assumes ori gin from a specific cell which may occur any place in the body in association with blood vessels. The term "angiomatous menin gioma" implies origin from meningeal cells. However, these distinctions are assumptions based on suggestive morphologic appear ances which cannot be substantiated with present morphologic methods alone. Further more, if a tumor arises from the vascular pericytes of the méninges rather than those of orbital fat, it could be considered a men ingioma of the hemangiopericytoma type. The difficulty posed is more than semantic, since it involves problems of biologic origin of such tumors which, as yet, are poorly un derstood. REFERENCES
1. Stout, A. P., and Murray, M. R.: Hemangio pericytoma: A vascular tumor featuring Zimmermann's pericytes. Ann. Surg. 116:26, 1942. 2. Rouget, C. : Mémoire sur le développment la structure et las propriétés physiologiques des capil laires sanguins et lymphatiques. Arch. Physiol. Norm, et Path. 5:603, 1873. 3. Gallivan, W. F. : Hemangiopericvtoma. Cancer 7 :59S, 1954. 4. Stout, A. P., and Lattes, R. : Tumors of the soft tissues. Fascicle 1 of the second series of the Atlas of Tumor Pathology. Armed Forces Inst. Pa thology, 1967. 5. Goodman, S. A. : Hemangiopericytoma of the orbit. Am. J. Ophth. 40:237,1955. 6. Stout, A. P. : Hemangiopericytoma : A study of 25 new cases. Cancer 2 :1027, 1949. 7. Fox, S. A. : Hemangiopericytoma of the orbit. Am. J. Ophth. 40:786, 1955.
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8. Spaeth, E. B., and Valdes-Dapena, A.: Hemangiopericytoma. Arch. Ophth. 60:1070, 1958. 9. Nover, A. : Beitrag zur pathologische Anato mie der malignen Orbitaltumoren. Ophthalmologica 126:3S, 1953. 10. Bailey, O. T., and Ford, R. F. : Sclerosing hemangiomas of the central nervous system. Progres sive tissue changes in hemangioblastomas of the brain and in so-called angioblastic meningiomas. Am. J. Path. 18:1, 1942. 11. Bailey, P., Cushing, H., and Eisenhardt, L. Arch. Path. 6:953, 1928. 12. del Rio Hortega, P., Prado, J. M., and Polak, M. : Respecta a la estructura especifica de los menin-
goexoteliomas. Rev. Assoc. Med. Argent. 58:568,1944. 13. Courville, C. B., and Abbott,. K. H. : The an gioblastic group of meningiomas. A study of 13 verified cases. Bull. Los Angeles Neurol. Soc. 5:47, 1940. 14. Courville, C. B. : Pathology of the Central Nervous System, 3rd ed. Mountain View, Califor nia, Pacific Press, 1950. 15. Begg, C. F., and Garret, R. : Hemangiopericytoma occurring in the méninges. Cancer 7:602, 1954. 16. Kruse, F., Jr. : Hemangiopericytoma of the méninges (angioblastic meningioma of Cushing and Eisenhardt). Neurology 11:771, 1961.
SURGICAL MANAGEMENT O F ORBITOFRONTAL VARIX IN KLIPPEL-TRÉNAUNAY-WEBER SYNDROME J. E A R L R A T H B U N , M.D.,
W I L L I A M F . H O Y T , M.D.,
A N D C R O W E L L BEARD,
M.D.
San Franchco, California
Varicosities of the legs, cutaneous hemangiectases, and hypertrophy of all tissues in the affected limbs characterize a rare neurocutaneous syndrome termed naevus varicosus osteo-hypertrophicus by Klippel and Trénaunay in 1900,1 and hemangiectatic hy pertrophy by F. Parkes Weber in 1907.2 Re cently, Haberland and Pérou 3 (1966) stressed that the venous malformations of the syndrome occur solely in the limbs. This report describes the unique occurrence, and surgical management, of a varix in the fore head and orbit of a teenage girl who had typical stigmata of the Klippel-TrénaunayWeber syndrome. CASE REPORT
This 15-year-old girl was admitted to the Uni versity of California Medical Center in May, 1969, for evaluation of unilateral headaches and marked hypertrophy of her legs. Her headaches (1) had From the Department of Ophthalmology and the Division of Neurological Surgery, University of California Medical Center, San Francisco, Califor nia. Dr. Rathbun is a Fellow in Ophthalmic Plastic Surgery supported by USPH Training Grant No. EY 00011-15. Reprint requests to J. Earl Rathbun, M.D., De partment of Ophthalmology, University of Califor nia Medical Center, San Francisco, California 94122.
been present for six months; (2) were accompa nied by a sensation of fullness in the right brow, forehead, and orbit; (3) were caused by straining, stooping, bending, or sleeping with less than two pillows; and (4) disappeared when she held her head upright. They were not accompanied by eyelid or cohjunctival swelling. History—She was born two months prematurely and had multiple flat red birthmarks on her left leg and trunk. By three months of age, her left leg ap peared to be growing more rapidly than the right. During early childhood, several cutaneous hemangiomas were excised from her trunk and left leg. Enlargement of the left leg progressed slowly ac companied by similar enlargement of her right lower leg and foot; because of these deformities, she had trouble walking and wore elastic stockings at all times. When she was six years old, her right second toe had grown so long that its distal phal anx was amputated so that she could wear correctly fitting shoes (Fig. 1). Physical examination—Her left leg was gro tesquely enlarged. She had large, raised, soft areas on her back, left chest, and left abdomen. Multiple serpiginous erythematous patches with punctate telangiectatic centers were scattered over the flexor surface of her left leg, her left buttocks and her back. An oversized right second toe was missing its distal phalanx. Multiple, hard, nontender, subcuta neous biopsy-proven angiomas, 2 to 3 mm in di ameter, were present over the interphalangeal joints of both hands. All of her fingers were grotesquely hyperextensible (Fig. 1). Orbital and ocular findings—The shape and size of her head were normal. Three large veins were visible and palpable on her right forehead, begin ning above the hairline superiorly and laterally and merging into one large vein medial to the right eye-
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DISSOCIATION OF COLOR AND FORM SENSE
preservation of color vision : Report of a case fol lowing asphyxiation. Arch. Ophth. 9:957, 1933. 5. Schlaegel, T. F. : Psychosomatic Ophthalmology. Baltimore, Williams and Wilkins, 1957. 6. Granger, G. W. : Personality with visual per ception : A review. J. Ment. Sei. 99:8, 1953.
7. Krill, A. E., and Newell, F. W. : The diagnosis of ocular conversion reaction involving visual function. Arch. Ophth. 79:254, 1968. 8. Kinsbourne, M., and Warrington, E. K. : Ob servations on colour agnosia. J. Neurol. Neurosurg. Psychiat. 27 :296, 1964.
ORBITAL HEMANGIOPERICYTOMA OR VASCULAR H. SAUL SUGAR, M.D.,
GORDON R. F I S H M A N , M.D., AND PAUL GOODMAN,
MENINGIOMA?
SIDNEY KOBERNICK,
M.D.,
M.D.
Detroit, Michigan Definite delineation of pathologic entitiesS from their histologie appearance and stain ing characteristics is often difficult andJ sometimes impossible. Experience with aa case which highlighted the controversial dif ferential diagnosis between orbital hemangiopericytoma and vascular meningioma hasS led to the presentation of this paper for its teaching value.
granuloma were removed through a conjunctival in cision just medial to the lateral canthus. Healing was uneventful, but the proptosis failed to diminish. On January 23, 1968, a mass was felt in the upper nasal portion of the anterior orbit. The proptosis had increased to 7 mm (Fig. 2). The patient was referred for consultation (to H.S.S.). Because only five months had passed since the surgical procedure, further observation was advised. The visual field related to the retinitis sclopeteria is shown in Figure 3. One year later, on February 11, 1969, the visual actuity was reduced to 20/200, corrected, and the proptosis had increased to 10 mm. Surgery was ad vised. Roentgenography at that time showed an inCASE HISTORY A 32-year-old Caucasian man was first seen on„ creased density over the left orbit and left maxil August 14, 1967 (by G. R. F.), because of promi lary antrum with some decrease in the dimensions nence of his left eye for about six weeks. The onsett of the left optic foramen. On March 5, 1969, was apparently sudden, and the condition had(j through an incision over the palpable mass in the changed little since it was first noticed. Dryness andj upper nasal portion of the left upper eyelid, the mass was exposed and dissected free of its surpain around the left eye, usually in the morning,, were associated with the proptosis. The patient'[ rounding tissue. It was found to extend vertically complained of headaches and vague retro-orbital1 to the orbital apex, being narrow at each end and discomfort. His history revealed that, at the age of£ widest at its center. The capusle was removed with seven, a BB shot had penetrated the left orbit. Itj. the tumor except at the orbital apex where it was had been localized by roentgenography, but no at freed separately. A frozen section diagnosis of probtempt had been made to remove it. During the pastt able neurofibroma was made. The wound was then two years, a nasal field defect had been noted in thee closed and a pressure bandage applied. The tumor weighed 10 g and measured 4χ2.5χ2 left eye, associated with chorioretinal scarring„ (Fig. 1). cm. It was fairly soft with a sharply demarcated, Examination showed the right eye to be normalj bosselated surface and a slightly bulging cut surwith visual acuity of 20/20 corrected with —3.00D) face (Fig. 4). Sections of the main tumor showed sphere. Visual acuity of the left eye was 20/20 with[j it to be highly cellular and very vascular with nu correction (—1.75 C +0.75 cyl X 75°), but chorio merous small, closely spaced channels lined by uniform, flattened endothelial cells. Some of the chanretinal scarring was present temporally. There wass weakness of elevation of the left eye and theree nels contained erythrocytes, but most were empty. was a small scar in the nasal portion of the left(. Between the vascular channels were masses of cells (pericytes) having uniform oval vesicular nuclei upper eyelid where the BB had entered. The prop tosis of the left eye measured 4 mm. A subconjunc- and relatively scanty lightly acidophilic cytoplasm tival hemorrhage was present in the left lateralj (Fig. 5). In many areas, nuclei were arranged in canthal region. A small nodule was palpable justj palisade pattern (Fig. 6). Extremely rare mitoses were found after much searching. Occasional thin behind the globe laterally. On August 23, 1967, the BB and an encapsulatedj collagen bands were present. Reticulum stain showed the small blood vessels outlined by fairly From Sinai Hospital of Detroit and Wayne Statee dense sheets of reticulin with delicate reticulin University School of Medicine. fibers extending outward between groups as well as Reprint requests to H. Saul Sugar, M.D., 970D single cells (Fig. 7). Fisher Bldg., Detroit, Michigan 48202. The tumor contained a diffuse light infiltrate of
104
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Fig. 1 (Sugar, Fishman, Kobernick, and Goodman). Appearance of left fundus showing retinitis sclopetaria resulting from the passage of BB lateral to left macula.
lymphocytes, histiocytes, and rare plasma cells. Also present were moderate numbers of focal small granulomas composed of rounded masses of closely packed histiocytes of epithelioid type (Figs. 6, 8). No multinucleated giant cells or foreign material were noted in any of the granulomas. There was no necrosis within or adjacent to the focal granulomas. They were distributed fairly evenly throughout the tumor with one or two found in most low power fields at various levels of sectioning. The capsule of the tumor was removed sep arately and consisted of a thin layer of compressed collagen. Small amounts of fat and skeletal muscle were adherent to its external surface. No tumor cells appeared to penetrate the capsule. Despite inflammatory areas, the tumor appeared distinctly neoplastic to all observers. A diagnosis of hemangiopericytoma of the orbit was made initially because of the pattern of vascular channels sur rounded by groups of cells thought to be pericytes. The reticulin pattern was thought to be distinctive for hemangiopericytoma. The role of the focal and diffuse inflammatory component is uncertain, but possibly related to the old injury and subsequent or bital surgery. Because of the rarity of the tumor, unique in our experience, consultation was obtained with Dr. Raffaele Lattes of Columbia University. Dr. Lattes
diagnosed the tumor as an orbital meningioma of the vascular type. He felt that the tumor resembled a hemangiopericytoma, but that the reticulin compo nent was too scanty to substantiate the diagnosis and some areas showed cells with a more epithelial than mesenchymal appearance. Further therapy appeared warranted because of uncertainty of completeness of excision at the deep area of the tumor which lay near the optic fora men. Recurrence of tumor was thought to be highly probable regardless of the precision of the diagno sis. Cobalt radiation was given to the left orbit to a total dose of 4250 r over a period of several weeks. Recovery was uneventful and without recurrence over an eight-month period of followup. On May 8,
Fig. 2 (Sugar, Fishman, Kobernick, and Good man). Appearance of patient in February, 1969, before surgery.
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Fig. 3 (Sugar, Fishman, Kobernick, and Goodman). Visual field of left eye in January 1968.
first described by Rouget.2 The pericytes are contractile cells but have no myofibrils. They are variable in size and shape; some appear more related to smooth muscle cells while DISCUSSION others seem more epithelioid. Gallivan3 de The hemangiopericytoma was first de scribed cytological variations ranging from scribed by Stout and Murray in 19421 as a smaller immature cells with oval nuclei and tumor derived from the capillary pericyte minimal cytoplasm arranged in compact masses to mature spindle cells with more abundant cytoplasm and a tendency to whorl formation. The tumor is characterized by endothelial sprouts surrounded by sheaths of pericytic cells. With the reticulin stain, argyrophilic fibrils are found surrounding the pericytes, and separating them from the endothelium. In man most of these rare tumors have most often been found in soft tissues, particularly the thigh, buttock, colon, omenturn, uterus, retroperitoneum, oral cavity and pharynx, mediastinum, orbit, méninges, choroid plexus and spinal canal. About a third are locally invasive and about half produce ^ sugar, Fishman, tusnman, Kobernick, and Good métastases.1 There are no reliable criteria to Fig. 4t (Sugar, man). Gross appearance of tumor in cross section.
1969, exophthalmometer readings were equal in both eyes (Fig. 9). On August 15, 1969, the left visual acuity was 20/30 corrected. The optic disks were normal.
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Fig. 5 (Sugar, Fishman, Kobernick, and Goodman). Highly vascular area showing uniform flattened endothelial cells and groups of larger cells between the vascular channels (hematoxylin, phloxin, and saffron, X370).
Fig. 6 (Sugar, Fishman, Kobernick, and Goodman). Overall view of tumor showing highly vascular cellular areas and few granulomas (hematoxylin, phloxin, and saffron, X17S).
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Fig. 7 (Sugar, Fishman, Kobernick, and Goodman). Reticulum stain showing deposits of reticulin fibers about the vascular channels and delicate fibrils extending between the groups of perivascular cells (Wilder's reticulum, χ370).
Fig. 8 (Sugar, Fishman, Kobernick, and Goodman). Granuloma with tumor (hematoxylin, phloxin, and saffron, X370).
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Fig. 9 (Sugar, Fishman, Kobernick, and Good man). Appearance of patient in 1969, following surgical removal of tumor.
distinguish histologically between benign and malignant tumors. 4 The first orbital tumor of this type re ported in the ophthalmic literature was de scribed in a paper by Goodman.5 The paper was entitled, "Hemangiopericytoma of the orbit," but the histopathologic diagnosis by Irvine was possible hemangiopericytoma, possible angioplastic form of meningioma. This emphasizes exactly the same diagnostic problem which our case presented. Seven other cases involving the orbit, all called he mangiopericytoma, were reported by Stout and Murray 1 (1 case), Stout6 (2), Fox 7 (1), Spaeth and Valdes-Dapena8 ( 1 ), and Nover9 (2). It is difficult to classify orbital meningeal tumors because of the pleomorphism of the cells ensheathing the clusters of small ves sels. If the vessels are ensheathed by meningothelial cells, the tumors are considered to be angioblastic meningiomas. Some of these tumors are identical in appearance with hemangioblastoma of the brain. Bailey and Ford, 10 Bailey and Cushing,11 del Rio Hortega,12 and Courville13 separate the an gioblastic meningiomas from the general group of meningiomas. All of these authors wrote on the subject before Stout's work. Later, in 1950, Courville14 classified the an gioblastic meningiomas without reference to hemangiopericytoma. Begg and Garret,15 and Kruse, 16 considered these tumors of the mé ninges to be hemangiopericytomas. Kruse reported eight cases among 900 tumors of
JULY, 1970
the méninges. He described the pathologic features as extreme vascularity, small, rela tively uniform, oval or elongated nuclei hav ing moderate amounts of reticulated chromatin. The cytoplasm is sparse, as a rule, and the cells tend to spindle appearance, ar ranged in suggestive whorls or pinwheels. The tumor cells lie external to an abundance of thin-walled vascular channels lined by a single layer of endothelial cells. This picture differs in no essential from the description of hemangiopericytomas in other organs. Neither diagnosis, as applied to the tu mors discussed here, is entirely satisfactory. The term hemangiopericytoma assumes ori gin from a specific cell which may occur any place in the body in association with blood vessels. The term "angiomatous menin gioma" implies origin from meningeal cells. However, these distinctions are assumptions based on suggestive morphologic appear ances which cannot be substantiated with present morphologic methods alone. Further more, if a tumor arises from the vascular pericytes of the méninges rather than those of orbital fat, it could be considered a men ingioma of the hemangiopericytoma type. The difficulty posed is more than semantic, since it involves problems of biologic origin of such tumors which, as yet, are poorly un derstood. REFERENCES
1. Stout, A. P., and Murray, M. R.: Hemangio pericytoma: A vascular tumor featuring Zimmermann's pericytes. Ann. Surg. 116:26, 1942. 2. Rouget, C. : Mémoire sur le développment la structure et las propriétés physiologiques des capil laires sanguins et lymphatiques. Arch. Physiol. Norm, et Path. 5:603, 1873. 3. Gallivan, W. F. : Hemangiopericvtoma. Cancer 7 :59S, 1954. 4. Stout, A. P., and Lattes, R. : Tumors of the soft tissues. Fascicle 1 of the second series of the Atlas of Tumor Pathology. Armed Forces Inst. Pa thology, 1967. 5. Goodman, S. A. : Hemangiopericytoma of the orbit. Am. J. Ophth. 40:237,1955. 6. Stout, A. P. : Hemangiopericytoma : A study of 25 new cases. Cancer 2 :1027, 1949. 7. Fox, S. A. : Hemangiopericytoma of the orbit. Am. J. Ophth. 40:786, 1955.
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HEMANGIOPERICYTOMA OR MENINGIOMA?
8. Spaeth, E. B., and Valdes-Dapena, A.: Hemangiopericytoma. Arch. Ophth. 60:1070, 1958. 9. Nover, A. : Beitrag zur pathologische Anato mie der malignen Orbitaltumoren. Ophthalmologica 126:3S, 1953. 10. Bailey, O. T., and Ford, R. F. : Sclerosing hemangiomas of the central nervous system. Progres sive tissue changes in hemangioblastomas of the brain and in so-called angioblastic meningiomas. Am. J. Path. 18:1, 1942. 11. Bailey, P., Cushing, H., and Eisenhardt, L. Arch. Path. 6:953, 1928. 12. del Rio Hortega, P., Prado, J. M., and Polak, M. : Respecta a la estructura especifica de los menin-
goexoteliomas. Rev. Assoc. Med. Argent. 58:568,1944. 13. Courville, C. B., and Abbott,. K. H. : The an gioblastic group of meningiomas. A study of 13 verified cases. Bull. Los Angeles Neurol. Soc. 5:47, 1940. 14. Courville, C. B. : Pathology of the Central Nervous System, 3rd ed. Mountain View, Califor nia, Pacific Press, 1950. 15. Begg, C. F., and Garret, R. : Hemangiopericytoma occurring in the méninges. Cancer 7:602, 1954. 16. Kruse, F., Jr. : Hemangiopericytoma of the méninges (angioblastic meningioma of Cushing and Eisenhardt). Neurology 11:771, 1961.
SURGICAL MANAGEMENT O F ORBITOFRONTAL VARIX IN KLIPPEL-TRÉNAUNAY-WEBER SYNDROME J. E A R L R A T H B U N , M.D.,
W I L L I A M F . H O Y T , M.D.,
A N D C R O W E L L BEARD,
M.D.
San Franchco, California
Varicosities of the legs, cutaneous hemangiectases, and hypertrophy of all tissues in the affected limbs characterize a rare neurocutaneous syndrome termed naevus varicosus osteo-hypertrophicus by Klippel and Trénaunay in 1900,1 and hemangiectatic hy pertrophy by F. Parkes Weber in 1907.2 Re cently, Haberland and Pérou 3 (1966) stressed that the venous malformations of the syndrome occur solely in the limbs. This report describes the unique occurrence, and surgical management, of a varix in the fore head and orbit of a teenage girl who had typical stigmata of the Klippel-TrénaunayWeber syndrome. CASE REPORT
This 15-year-old girl was admitted to the Uni versity of California Medical Center in May, 1969, for evaluation of unilateral headaches and marked hypertrophy of her legs. Her headaches (1) had From the Department of Ophthalmology and the Division of Neurological Surgery, University of California Medical Center, San Francisco, Califor nia. Dr. Rathbun is a Fellow in Ophthalmic Plastic Surgery supported by USPH Training Grant No. EY 00011-15. Reprint requests to J. Earl Rathbun, M.D., De partment of Ophthalmology, University of Califor nia Medical Center, San Francisco, California 94122.
been present for six months; (2) were accompa nied by a sensation of fullness in the right brow, forehead, and orbit; (3) were caused by straining, stooping, bending, or sleeping with less than two pillows; and (4) disappeared when she held her head upright. They were not accompanied by eyelid or cohjunctival swelling. History—She was born two months prematurely and had multiple flat red birthmarks on her left leg and trunk. By three months of age, her left leg ap peared to be growing more rapidly than the right. During early childhood, several cutaneous hemangiomas were excised from her trunk and left leg. Enlargement of the left leg progressed slowly ac companied by similar enlargement of her right lower leg and foot; because of these deformities, she had trouble walking and wore elastic stockings at all times. When she was six years old, her right second toe had grown so long that its distal phal anx was amputated so that she could wear correctly fitting shoes (Fig. 1). Physical examination—Her left leg was gro tesquely enlarged. She had large, raised, soft areas on her back, left chest, and left abdomen. Multiple serpiginous erythematous patches with punctate telangiectatic centers were scattered over the flexor surface of her left leg, her left buttocks and her back. An oversized right second toe was missing its distal phalanx. Multiple, hard, nontender, subcuta neous biopsy-proven angiomas, 2 to 3 mm in di ameter, were present over the interphalangeal joints of both hands. All of her fingers were grotesquely hyperextensible (Fig. 1). Orbital and ocular findings—The shape and size of her head were normal. Three large veins were visible and palpable on her right forehead, begin ning above the hairline superiorly and laterally and merging into one large vein medial to the right eye-