FIBROUS HISTIOCYTOMA OF THE TARSUS FREDERICK A. JAKOBIEC, M.D., ARTHUR GERARD DEVOE, M.D., AND JAMES BOYD, M.D. New York, New York
Neoplastic causes of pseudochalazion have been limited to epithelial tumors such as meibomian gland carcinoma1 and sebaceous adenoma.2 We believe this is the first report of a pseudochalazion re sulting from a proliferation of the fibrous portion of the tarsus. CASE REPORT In the fall, 1975, a 22-year-old white man was noted by his mother to have an asymptomatic lump in the medial aspect of his left upper eyelid. After one year of slowly progressive, painless enlargement of the tumor, the patient was examined by an oph thalmologist. The lesion was within the tarsus (Fig. 2) and appeared as a lobular, firm growth on the conjunctival aspect of the eyelid (Fig. 1). Visual acuity was 6/5^(20/15) in both eyes without correc tion; the remainder of the ocular examination was unremarkable. The clinical diagnosis was a chalazion, although one observer thought the lesion might be composed of cartilage because of its firm consistency. The lesion was excised, and there has
Fig. 2 (Jakobiec, DëVœ, and Boyd). Photomacrograph of the excised lesion showing its solid homo geneous character, as well as remnants of com pressed tarsus stained darker at the periphery. (Masson's trichrome, x7).
been no recurrence during a 12-month follow-up. The lesion was composed of spindle cells and xanthoma cells (Fig. 3). In some fields, the spindle cells exhibited a matted or spiral nebular pattern
Fig. 1 (Jakobiec, DeVoe, and Boyd). A wellcircumscribed, subconjunctival tumor in the medial aspect of the left upper eyelid. Note the smooth, lobular contour and the noninflamed overlying con junctival epithelium. From the Edward S. Harkness Eye Institute and the Algernon B. Reese Laboratory of Ophthalmic Pathology, New York, New York. Reprint requests to Frederick A. Jakobiec, M.D., Edward S. Harkness Eye Institute, Box 57, New York, NY 10032.
Fig. 3 (Jakobiec, DeVoe, and Boyd). The tumor below displays a well-developed matted pattern. Evenly dispersed throughout the tumor are darker staining, multinucleated giant cells. The arrow points to surviving lamellae of the tarsal collagen. The conjunctiva above is lightly inflamed, but uninvolved with tumor. (Masson's trichrome, x76).
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Fig. 4 (Jakobiec, DeVoe, and Boyd). Higher power of the matted pattern, which is created by twisting bundles of fibrohistiocytes displaying vesicular nu clei and opaque elongated cytoplasm, (hematoxylin and eosin, x94). (Fig. 4). The xanthoma cells were vacuolated and either interspersed among the spindle cells or ar ranged in small clusters (Fig. 5). Multinucleated giant cells were evenly scattered throughout the tumor; many displayed the classic features of Touton giant cells as they consisted of a central ring of nuclei enclosing eosinophilic cytoplasm and an out side rim of vacuolated cytoplasm (Fig. 5, inset). The reticulin stain disclosed a moderately heavy deposi tion of this fiber in the compact spindle cell areas (Fig. 6, left), but only a few fibers were present in
795
Fig. 5 (Jakobiec, DeVoe, and Boyd). Area of the tumor featuring xanthoma cells and multinucleated giant cells. Inset, Two Touton giant cells exhibiting an annulus of nuclei separating central eosinophilic cytoplasm from a peripheral rim of highly vacuolat ed cytoplasm, (hematoxylin and eosin; x350; inset, x375). the areas dominated by xanthoma cells (Fig. 6,
right).
DISCUSSION
Neither Duke-Elder and MacFaul 3 nor Reese4 mention fibrous tumors of the tar sus in their reviews of neoplasms of the eyelids. Fibrous histiocytomas have been described in the orbit,5 at the corneoscleral limbus, 6,7 and in the conjunctiva.8 Familiarity with the yellow appearance of acquired anterior subconjunctival fibrous
Fig 6. (Jakobiec, DeVoe, and Boyd). Left, Heavy deposition of reticulin fibers in the more fibroblastic regions of the tumor. Right, Lower fiber content characterizes a zone of heavily lipidized cells. The arrow points to a multinucleated Touton giant cell. (Laidlaw-reticulin; left, x80; right, xl80).
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AMERICAN JOURNAL OF OPHTHALMOLOGY
histiocytomas should assure their clinical diagnosis, since lipomatous lesions are usually congenital. Several clinical as pects of the present intratarsal lesion should be useful in future preoperative diagnosis. Most chalazia begin with either pain or inflammation and, in common with other reactive or inflammatory conditions, reach a detectable size in a short period of time (days or weeks), causing the patient to seek ophthalmic consultation. None of these features characterized our patient's history. The onset of his disease could not be pinpointed and the tumor's growth was so painless that another person dis covered it. When chalazia are subconjunctival, they normally provoke an in flammatory reaction in the overlying con junctiva and produce some irregularity of the surface epithelium; in this case, the overlying conjunctiva was smooth and noninflamed. The sharp delimitation of the lesion from uninvolved adjacent tis sue differs from the appearance of cha lazia, which tend to blend imperceptibly with the surrounding tissue. Finally, the texture of the lesion was distinctive and gave the impression of cartilage to one experienced observer; chalazia may be come firm with healing, but are more yielding on pressure. There are several histopathologic rea sons for considering this lesion idiopathic and not an unusual reaction pattern to an earlier chalazion. There was a compara tive dearth of chronic inflammatory cells and a denser cellularity than would occur in a fibrous chalazion. The well devel oped matted pattern created by the tumor cells indicates fibrous histiocytoma, and is not a feature of reparative fibrous tis sue. The even distribution of the giant cells throughout the lesion is also in keep ing with a primary neoplasm, since repar ative processes featuring giant cells ex hibit a zonal pattern of uneven distribu tion and concentration of the giant cells at
DECEMBER, 1977
various foci. Lastly, one would expect foreign-body-type giant cells in a repara tive phase of a chalazion rather than Touton giant cells, with their circular ar rangement of nuclei separating central opaque eosinophilic cytoplasm from an outside rim of foamy vacuolated cyto plasm. In this case, the presence of Touton giant cells suggested the possibility of an isolated xanthogranuloma, but the matted pattern and the absence of associated in flammatory cells such as plasma cells, eosinophils, or lymphocytes in appreciable numbers argue more in favor of a fibrous histiocytoma. Xanthogranulomas com monly affect the dermis of the eyelids, but have not been reported to originate in the tarsus. 9 Pseudosarcomatous nodular fasciitis may also develop rapidly in the dermis of the eyelids,10 or appear subconjunctivally,11 but again the tarsus is spared. In the orbit, fibrous histiocytomas are usually benign and often circumscribed, but they tend to recur and may display an infiltrative growth pattern.5 In contrast, solitary lesions of the anterior adnexal structures are more easily managed and appear to be lower grade neoplasms. A simple excision of the tumor is usually curative,6·8 although one instance of a corneoscleral fibrous histiocytoma re curred after an initial, incomplete exci sion, but was totally removed in subse quent surgery.7 The present tumor was easily shelled out because of its pseudocapsule formed by surviving elements of the tarsus at the periphery. SUMMARY
A 21-year-old white man had an asymp tomatic eyelid mass that had slowly en larged for one year. On clinical examina tion the lesion was well circumscribed; it appeared subconjunctivally but provoked no conjunctival inflammation, and had a consistency suggestive of cartilage. Mi-
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croscopic examination showed it was matted and composed of elongated fibrohistiocytes and an evenly distributed population of xanthoma cells and Touton giant cells. The lesion was easily removed by local excision because of a pseudocap sule formed by remnants of the tarsus; there has been no recurrence during a 12-month follow-up. REFERENCES 1. Boniuk, M., and Zimmerman, L. E.: Sebaceous carcinoma of the eyelid, eyebrow, caruncle, and orbit. Trans. Am. Acad. Ophthalmol. Otolaryngol. 72:619, 1968. 2. Jakobiec, F. A.: Sebaceous adenoma of the eyelid and visceral malignancy. Am. J. Ophthalmol. 78:952, 1974. 3. Duke-Elder, S., and MacFaul, P. A.: The Ocu lar Adnexa. Diseases of the Eyelids. In Duke-Elder,
797
S. (ed.): System of Ophthalmology, vol. 13, pt. 1. St. Louis, C. V. Mosby, 1974, pp. 467-468. 4. Reese, A. B.: Tumors of the Eye. Hagerstown, Harper and Row, 1976, pp. 38-62; 314-317. 5. Jakobiec, F. A., Howard, G., and Tannenbaum, M.: Fibrous histiocytomas of the orbit. Am. J. Oph thalmol. 77:333, 1974. 6. Jakobiec, F. A.: Fibrous histiocytoma of the corneoscleral limbus. Am. J. Ophthalmol. 78:700, 1974. 7. Faludi, J. E., Kenyon, K., and Green, W. R.: Fibrous histiocytoma of the corneoscleral limbus. Am. J. Ophthalmol. 80:619, 1975. 8. Albert, D. M., and Smith, R. S.: Fibrous xanthomas of the conjunctiva. Arch. Ophthalmol. 80: 474, 1968. 9. Zimmerman, L. E.: Ocular lesions of juvenile xanthogranuloma. Nevoxanthoendothelioma. Trans. Am. Acad. Ophthalmol. Otolarnygol. 69:412, 1965. 10. Font, R. L., and Zimmerman, L. E.: Nodular fascitis of the eye and adnexa. A report of 10 cases. Arch. Ophthalmol. 75:475, 1966. 11. Ferry, A. P., and Sherman, S. E.: Nodular fascitis of the conjunctiva apparently originating in the fascia bulbi. Am. J. Ophthalmol. 78:514, 1974.
Neoplastic causes of pseudochalazion have been limited to epithelial tumors such as meibomian gland carcinoma1 and sebaceous adenoma.2 We believe this is the first report of a pseudochalazion re sulting from a proliferation of the fibrous portion of the tarsus. CASE REPORT In the fall, 1975, a 22-year-old white man was noted by his mother to have an asymptomatic lump in the medial aspect of his left upper eyelid. After one year of slowly progressive, painless enlargement of the tumor, the patient was examined by an oph thalmologist. The lesion was within the tarsus (Fig. 2) and appeared as a lobular, firm growth on the conjunctival aspect of the eyelid (Fig. 1). Visual acuity was 6/5^(20/15) in both eyes without correc tion; the remainder of the ocular examination was unremarkable. The clinical diagnosis was a chalazion, although one observer thought the lesion might be composed of cartilage because of its firm consistency. The lesion was excised, and there has
Fig. 2 (Jakobiec, DëVœ, and Boyd). Photomacrograph of the excised lesion showing its solid homo geneous character, as well as remnants of com pressed tarsus stained darker at the periphery. (Masson's trichrome, x7).
been no recurrence during a 12-month follow-up. The lesion was composed of spindle cells and xanthoma cells (Fig. 3). In some fields, the spindle cells exhibited a matted or spiral nebular pattern
Fig. 1 (Jakobiec, DeVoe, and Boyd). A wellcircumscribed, subconjunctival tumor in the medial aspect of the left upper eyelid. Note the smooth, lobular contour and the noninflamed overlying con junctival epithelium. From the Edward S. Harkness Eye Institute and the Algernon B. Reese Laboratory of Ophthalmic Pathology, New York, New York. Reprint requests to Frederick A. Jakobiec, M.D., Edward S. Harkness Eye Institute, Box 57, New York, NY 10032.
Fig. 3 (Jakobiec, DeVoe, and Boyd). The tumor below displays a well-developed matted pattern. Evenly dispersed throughout the tumor are darker staining, multinucleated giant cells. The arrow points to surviving lamellae of the tarsal collagen. The conjunctiva above is lightly inflamed, but uninvolved with tumor. (Masson's trichrome, x76).
794
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FIBROUS HISTIOCYTOMA
Fig. 4 (Jakobiec, DeVoe, and Boyd). Higher power of the matted pattern, which is created by twisting bundles of fibrohistiocytes displaying vesicular nu clei and opaque elongated cytoplasm, (hematoxylin and eosin, x94). (Fig. 4). The xanthoma cells were vacuolated and either interspersed among the spindle cells or ar ranged in small clusters (Fig. 5). Multinucleated giant cells were evenly scattered throughout the tumor; many displayed the classic features of Touton giant cells as they consisted of a central ring of nuclei enclosing eosinophilic cytoplasm and an out side rim of vacuolated cytoplasm (Fig. 5, inset). The reticulin stain disclosed a moderately heavy deposi tion of this fiber in the compact spindle cell areas (Fig. 6, left), but only a few fibers were present in
795
Fig. 5 (Jakobiec, DeVoe, and Boyd). Area of the tumor featuring xanthoma cells and multinucleated giant cells. Inset, Two Touton giant cells exhibiting an annulus of nuclei separating central eosinophilic cytoplasm from a peripheral rim of highly vacuolat ed cytoplasm, (hematoxylin and eosin; x350; inset, x375). the areas dominated by xanthoma cells (Fig. 6,
right).
DISCUSSION
Neither Duke-Elder and MacFaul 3 nor Reese4 mention fibrous tumors of the tar sus in their reviews of neoplasms of the eyelids. Fibrous histiocytomas have been described in the orbit,5 at the corneoscleral limbus, 6,7 and in the conjunctiva.8 Familiarity with the yellow appearance of acquired anterior subconjunctival fibrous
Fig 6. (Jakobiec, DeVoe, and Boyd). Left, Heavy deposition of reticulin fibers in the more fibroblastic regions of the tumor. Right, Lower fiber content characterizes a zone of heavily lipidized cells. The arrow points to a multinucleated Touton giant cell. (Laidlaw-reticulin; left, x80; right, xl80).
796
AMERICAN JOURNAL OF OPHTHALMOLOGY
histiocytomas should assure their clinical diagnosis, since lipomatous lesions are usually congenital. Several clinical as pects of the present intratarsal lesion should be useful in future preoperative diagnosis. Most chalazia begin with either pain or inflammation and, in common with other reactive or inflammatory conditions, reach a detectable size in a short period of time (days or weeks), causing the patient to seek ophthalmic consultation. None of these features characterized our patient's history. The onset of his disease could not be pinpointed and the tumor's growth was so painless that another person dis covered it. When chalazia are subconjunctival, they normally provoke an in flammatory reaction in the overlying con junctiva and produce some irregularity of the surface epithelium; in this case, the overlying conjunctiva was smooth and noninflamed. The sharp delimitation of the lesion from uninvolved adjacent tis sue differs from the appearance of cha lazia, which tend to blend imperceptibly with the surrounding tissue. Finally, the texture of the lesion was distinctive and gave the impression of cartilage to one experienced observer; chalazia may be come firm with healing, but are more yielding on pressure. There are several histopathologic rea sons for considering this lesion idiopathic and not an unusual reaction pattern to an earlier chalazion. There was a compara tive dearth of chronic inflammatory cells and a denser cellularity than would occur in a fibrous chalazion. The well devel oped matted pattern created by the tumor cells indicates fibrous histiocytoma, and is not a feature of reparative fibrous tis sue. The even distribution of the giant cells throughout the lesion is also in keep ing with a primary neoplasm, since repar ative processes featuring giant cells ex hibit a zonal pattern of uneven distribu tion and concentration of the giant cells at
DECEMBER, 1977
various foci. Lastly, one would expect foreign-body-type giant cells in a repara tive phase of a chalazion rather than Touton giant cells, with their circular ar rangement of nuclei separating central opaque eosinophilic cytoplasm from an outside rim of foamy vacuolated cyto plasm. In this case, the presence of Touton giant cells suggested the possibility of an isolated xanthogranuloma, but the matted pattern and the absence of associated in flammatory cells such as plasma cells, eosinophils, or lymphocytes in appreciable numbers argue more in favor of a fibrous histiocytoma. Xanthogranulomas com monly affect the dermis of the eyelids, but have not been reported to originate in the tarsus. 9 Pseudosarcomatous nodular fasciitis may also develop rapidly in the dermis of the eyelids,10 or appear subconjunctivally,11 but again the tarsus is spared. In the orbit, fibrous histiocytomas are usually benign and often circumscribed, but they tend to recur and may display an infiltrative growth pattern.5 In contrast, solitary lesions of the anterior adnexal structures are more easily managed and appear to be lower grade neoplasms. A simple excision of the tumor is usually curative,6·8 although one instance of a corneoscleral fibrous histiocytoma re curred after an initial, incomplete exci sion, but was totally removed in subse quent surgery.7 The present tumor was easily shelled out because of its pseudocapsule formed by surviving elements of the tarsus at the periphery. SUMMARY
A 21-year-old white man had an asymp tomatic eyelid mass that had slowly en larged for one year. On clinical examina tion the lesion was well circumscribed; it appeared subconjunctivally but provoked no conjunctival inflammation, and had a consistency suggestive of cartilage. Mi-
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FIBROUS HISTIOCYTOMA
croscopic examination showed it was matted and composed of elongated fibrohistiocytes and an evenly distributed population of xanthoma cells and Touton giant cells. The lesion was easily removed by local excision because of a pseudocap sule formed by remnants of the tarsus; there has been no recurrence during a 12-month follow-up. REFERENCES 1. Boniuk, M., and Zimmerman, L. E.: Sebaceous carcinoma of the eyelid, eyebrow, caruncle, and orbit. Trans. Am. Acad. Ophthalmol. Otolaryngol. 72:619, 1968. 2. Jakobiec, F. A.: Sebaceous adenoma of the eyelid and visceral malignancy. Am. J. Ophthalmol. 78:952, 1974. 3. Duke-Elder, S., and MacFaul, P. A.: The Ocu lar Adnexa. Diseases of the Eyelids. In Duke-Elder,
797
S. (ed.): System of Ophthalmology, vol. 13, pt. 1. St. Louis, C. V. Mosby, 1974, pp. 467-468. 4. Reese, A. B.: Tumors of the Eye. Hagerstown, Harper and Row, 1976, pp. 38-62; 314-317. 5. Jakobiec, F. A., Howard, G., and Tannenbaum, M.: Fibrous histiocytomas of the orbit. Am. J. Oph thalmol. 77:333, 1974. 6. Jakobiec, F. A.: Fibrous histiocytoma of the corneoscleral limbus. Am. J. Ophthalmol. 78:700, 1974. 7. Faludi, J. E., Kenyon, K., and Green, W. R.: Fibrous histiocytoma of the corneoscleral limbus. Am. J. Ophthalmol. 80:619, 1975. 8. Albert, D. M., and Smith, R. S.: Fibrous xanthomas of the conjunctiva. Arch. Ophthalmol. 80: 474, 1968. 9. Zimmerman, L. E.: Ocular lesions of juvenile xanthogranuloma. Nevoxanthoendothelioma. Trans. Am. Acad. Ophthalmol. Otolarnygol. 69:412, 1965. 10. Font, R. L., and Zimmerman, L. E.: Nodular fascitis of the eye and adnexa. A report of 10 cases. Arch. Ophthalmol. 75:475, 1966. 11. Ferry, A. P., and Sherman, S. E.: Nodular fascitis of the conjunctiva apparently originating in the fascia bulbi. Am. J. Ophthalmol. 78:514, 1974.
