KAPOSI'S SARCOMA O F T H E CONJUNCTIVA GEORGE M. HOWARD, M.D.,
FREDERICK A. JAKOBIEC,
AND ARTHUR G. DEVOE,
M.D.,
M.D.
New York, New York
Incidences of Kaposi's hemorrhagic sar coma, a tumor that had been considered unique and bizarre, have been reported more frequently. In 1960, Dutz and Stout1 col lected data on 1,256 patients with this tumor. Much of this experience originated in South and Central Africa where there is a high incidence among Bantu natives. The disease affects those between the ages of SO and 70, and occurs primarily in men (80-90%). 2-4 Herpes virus has been suspected as the cause, but there is no definite immunologie evidence to suggest spread by an infectious microorganism. Immunologie incompetence may be a factor that explains some of the features in Africans.5 The sarcoma originates in the skin, ini tially as a bluish-red macule on the lower extremities. Nonpitting edema of the ex tremities is common later in the course of the disease, apparently due to fluid accumulation in the corium. The condition seems to be a systemic disease of multicentric origin, pro longed duration, and rarely characterized by actual métastases. The cause of death is often a second primary tumor such as malignant lymphoma (notably Hodgkin's disease), leukemia, or a primary carcinoma with a separate histogenesis. Fortunately, the tu mors of Kaposi's sarcoma, especially in the early vascular stage, are radiosensitive. When the lesions become hardened and fibrotic, they lose their sensitivity. Treat ment has consisted of systemic chemotherapy and surgical excision of isolated nodules. Sacks,8 Mortada,7 and Lieberman and From the Edward S. Harkness Eye Institute, Columbia-Presbyterian Medical Center, New York, New York. Reprint requests to George M. Howard, M.D., College of Physicians and Surgeons of Columbia University, Department of Ophthalmology, 635 W. 165th St., New York, NY 10032.
Llovera8 reported cases of Kaposi's sar coma involving the conjunctiva. The disease rarely presents in the eyelids; a well-docu mented orbital case is not on record. The superficial locations of the lesions may re flect differential susceptibilities of the microvasculature to the disease. CASE REPORT
A 75-year-old man, examined in April 1971, had chronic injection of the left eye for three years (Fig. 1), which failed to improve with previous topical antibiotic corticosteroid therapy. The left eye had occasionally developed small external hemorrhages, and during the previous several months, a nodular, firm mass appeared in the lateral bulbar conjunctiva; this mass did not blanch with pressure. The tarsal conjunctiva was spared, and the tumefactions stopped short of the corneoscleral limbus (Fig. 2). Examination of the globe indicated surgically in duced aphakia with normal ocular tension ; corrected visual acuity was 20/25 in each eye. There was no proptosis, and no orbital bruit. Diffuse hemor rhagic conjunctival thickening was possibly due to carcinoma in situ, or to Kaposi's sarcoma. Kaposi's sarcoma of both legs had been estab lished histopathologically three years before the conjunctival involvement appeared. He had re ceived 3,100 rads of radiotherapy in divided doses over a three-month period to both lower ex tremities. A biopsy specimen of the conjunctival tumor in April indicated Kaposi's sarcoma. We ad ministered 1,000 additional rads to the left eye in September, during a three-week period, producing a moderate regression in the conjunctival tumor (Fig. 3). Six months later, corrected visual acuity in the left eye was 20/200. The optic disk was pale, possibly due to radiation changes. The conjunctival tumor continued to regress moderately. MICROSCOPIC
DESCRIPTION
The conjunctival tumor studied histopath ologically with hematoxylin and eosin, Fon tana, reticulum, and trichome stains revealed spindle-shaped cells with elongated oval nu clei, tending to group in fascicles (Fig. 4). Mitotic figures were rare, and melanin gran ules were not seen with the Fontana stain. 420
Fig. 1 (Howard, Jakobiec, and DeVoe) Chronic hemorrhagic thickening of the conjunctiva of the left eye (three-year duration) in a 75-year-old man.
Fig. 2 (Howard, Jakobiec, and DeVoe). The tarsal conjunctiva fails to exhibit the beefy thick ening characterizing the bulbar conjunctiva.
Fig. 3 (Howard, Jakobiec, and DeVoe). After radiotherapy, the conjunctival tumor regressed moderately at the corneoscleral limbus, where there is a zone surrounding the cornea partially free from injection and thickening.
Fig. 4 (Howard, Jakobiec, and DeVoe). Con junctiva at top. Spindle cells are grouped in inter lacing fascicles (hematoxylin and eosin, XI00).
Fig. 5 (Howard, Jakobiec, and DeVoe). Spindle cells tend to aggregate around clefts and round lumina (hematoxylin and eosin, X200).
Fig. 6 (Howard, Jakobiec, and DeVoe). Con junctiva at top. Clefts, lumina, and medium-sized blood vessels in the tumor. Erythrocytes and hemosiderin are present in the intercellular spaces (hematoxylin and eosin, X100).
VOL. 79, NO. 3
KAPOSI'S SARCOMA
Some areas were more fibroblastic and collagenized than others, as tumor cells tended to form irregular clefts, round lumina, and mantles around medium-sized vessels (Fig. S). Erythrocytes and hemosiderin were pres ent in intercellular spaces (Fig. 6). The con junctiva overlying the tumor was modified to a metaplastic nonkeratinized squamous epithelium (Fig. 5), indicating a Kaposi's sarcoma diagnosis. COMMENT
423
coma from squamous cetTëpîthelioma, since the latter tumor often invades the corneoscleral limbus and extends to the cornea early in its growth pattern. SUMMARY
A 7S-year-old man with known Kaposi's sarcoma of three-years' duration developed chronic hemorrhagic thickening of the con junctiva. Biopsy demonstrated histopathologically that the conjunctival thickening repre sented sarcoma involvement. Although sur gically untenable, the conjunctival lesion re sponds satisfactorily to radiotherapy.
Since Kaposi's sarcoma evolves in the subepithelial tissues, greater tumefaction is an ticipated in conjunctival sections possessing greater substantia propria. This supposition ACKNOWLEDGMENT is demonstrated by the patient's clinical ex Walter Schachat, M.D., referred the patient amination, showing significant thickening of and suggested the clinical diagnosis. the bulbar conjunctiva (Fig. 1 ) but strikingly little involvement of the tarsal conjunctiva REFERENCES (Fig, 2), The conjunctival mucosa is closely 1. Dutz, W., and Stout, A. P.: Kaposi's sar apposed over the tarsal plate without inter coma in infants and children. Cancer 13:684, 1960. 2. O'Brien, P. H., and Brasfield, R. D.: vening substantia propria. Kaposi's sarcoma. Cancer 19:1497, 1966. After radiotherapy, the perilimbal zone 3. Gordon, J. A. : Kaposi's sarcoma. A review where the substantia propria fades almost of 136 Rhodesian African cases. Postgrad. Med. completely regressed. Conjunctival involve J. 43:513, 1967. 4. Davis, J. : Kaposi's sarcoma. Present concept ment by Kaposi's sarcoma is characteristi of clinical course and treatment. N.Y. State J. cally more evident in the bulbar conjunctiva, Med. 68:2067, 1968. 5. Kaposi's sarcoma, editorial. Lancet 7798:300, sparing the immediate perilimbal zone and 1973. almost completely sparing the tarsal con 6. Sacks, I. : Kaposi's disease. Manifesting in junctiva. the eye. Br. J. Ophthalmol. 40:574, 19S6. 7. Mortada, A. : Conjunctival regressing Kaposi The tendency to completely involve the Br. J. Ophthalmol. 51:275, 1967. bulbar conjunctiva while sparing a narrow sarcoma. 8. Lieberman, P. H., and Llovera, I. N. : zone adjacent to the cornea can be a helpful Kaposi's sarcoma of the bulbar conjunctiva. Arch. clinical sign in differentiating Kaposi's sar Ophthalmol. 88:44, 1972.
FREDERICK A. JAKOBIEC,
AND ARTHUR G. DEVOE,
M.D.,
M.D.
New York, New York
Incidences of Kaposi's hemorrhagic sar coma, a tumor that had been considered unique and bizarre, have been reported more frequently. In 1960, Dutz and Stout1 col lected data on 1,256 patients with this tumor. Much of this experience originated in South and Central Africa where there is a high incidence among Bantu natives. The disease affects those between the ages of SO and 70, and occurs primarily in men (80-90%). 2-4 Herpes virus has been suspected as the cause, but there is no definite immunologie evidence to suggest spread by an infectious microorganism. Immunologie incompetence may be a factor that explains some of the features in Africans.5 The sarcoma originates in the skin, ini tially as a bluish-red macule on the lower extremities. Nonpitting edema of the ex tremities is common later in the course of the disease, apparently due to fluid accumulation in the corium. The condition seems to be a systemic disease of multicentric origin, pro longed duration, and rarely characterized by actual métastases. The cause of death is often a second primary tumor such as malignant lymphoma (notably Hodgkin's disease), leukemia, or a primary carcinoma with a separate histogenesis. Fortunately, the tu mors of Kaposi's sarcoma, especially in the early vascular stage, are radiosensitive. When the lesions become hardened and fibrotic, they lose their sensitivity. Treat ment has consisted of systemic chemotherapy and surgical excision of isolated nodules. Sacks,8 Mortada,7 and Lieberman and From the Edward S. Harkness Eye Institute, Columbia-Presbyterian Medical Center, New York, New York. Reprint requests to George M. Howard, M.D., College of Physicians and Surgeons of Columbia University, Department of Ophthalmology, 635 W. 165th St., New York, NY 10032.
Llovera8 reported cases of Kaposi's sar coma involving the conjunctiva. The disease rarely presents in the eyelids; a well-docu mented orbital case is not on record. The superficial locations of the lesions may re flect differential susceptibilities of the microvasculature to the disease. CASE REPORT
A 75-year-old man, examined in April 1971, had chronic injection of the left eye for three years (Fig. 1), which failed to improve with previous topical antibiotic corticosteroid therapy. The left eye had occasionally developed small external hemorrhages, and during the previous several months, a nodular, firm mass appeared in the lateral bulbar conjunctiva; this mass did not blanch with pressure. The tarsal conjunctiva was spared, and the tumefactions stopped short of the corneoscleral limbus (Fig. 2). Examination of the globe indicated surgically in duced aphakia with normal ocular tension ; corrected visual acuity was 20/25 in each eye. There was no proptosis, and no orbital bruit. Diffuse hemor rhagic conjunctival thickening was possibly due to carcinoma in situ, or to Kaposi's sarcoma. Kaposi's sarcoma of both legs had been estab lished histopathologically three years before the conjunctival involvement appeared. He had re ceived 3,100 rads of radiotherapy in divided doses over a three-month period to both lower ex tremities. A biopsy specimen of the conjunctival tumor in April indicated Kaposi's sarcoma. We ad ministered 1,000 additional rads to the left eye in September, during a three-week period, producing a moderate regression in the conjunctival tumor (Fig. 3). Six months later, corrected visual acuity in the left eye was 20/200. The optic disk was pale, possibly due to radiation changes. The conjunctival tumor continued to regress moderately. MICROSCOPIC
DESCRIPTION
The conjunctival tumor studied histopath ologically with hematoxylin and eosin, Fon tana, reticulum, and trichome stains revealed spindle-shaped cells with elongated oval nu clei, tending to group in fascicles (Fig. 4). Mitotic figures were rare, and melanin gran ules were not seen with the Fontana stain. 420
Fig. 1 (Howard, Jakobiec, and DeVoe) Chronic hemorrhagic thickening of the conjunctiva of the left eye (three-year duration) in a 75-year-old man.
Fig. 2 (Howard, Jakobiec, and DeVoe). The tarsal conjunctiva fails to exhibit the beefy thick ening characterizing the bulbar conjunctiva.
Fig. 3 (Howard, Jakobiec, and DeVoe). After radiotherapy, the conjunctival tumor regressed moderately at the corneoscleral limbus, where there is a zone surrounding the cornea partially free from injection and thickening.
Fig. 4 (Howard, Jakobiec, and DeVoe). Con junctiva at top. Spindle cells are grouped in inter lacing fascicles (hematoxylin and eosin, XI00).
Fig. 5 (Howard, Jakobiec, and DeVoe). Spindle cells tend to aggregate around clefts and round lumina (hematoxylin and eosin, X200).
Fig. 6 (Howard, Jakobiec, and DeVoe). Con junctiva at top. Clefts, lumina, and medium-sized blood vessels in the tumor. Erythrocytes and hemosiderin are present in the intercellular spaces (hematoxylin and eosin, X100).
VOL. 79, NO. 3
KAPOSI'S SARCOMA
Some areas were more fibroblastic and collagenized than others, as tumor cells tended to form irregular clefts, round lumina, and mantles around medium-sized vessels (Fig. S). Erythrocytes and hemosiderin were pres ent in intercellular spaces (Fig. 6). The con junctiva overlying the tumor was modified to a metaplastic nonkeratinized squamous epithelium (Fig. 5), indicating a Kaposi's sarcoma diagnosis. COMMENT
423
coma from squamous cetTëpîthelioma, since the latter tumor often invades the corneoscleral limbus and extends to the cornea early in its growth pattern. SUMMARY
A 7S-year-old man with known Kaposi's sarcoma of three-years' duration developed chronic hemorrhagic thickening of the con junctiva. Biopsy demonstrated histopathologically that the conjunctival thickening repre sented sarcoma involvement. Although sur gically untenable, the conjunctival lesion re sponds satisfactorily to radiotherapy.
Since Kaposi's sarcoma evolves in the subepithelial tissues, greater tumefaction is an ticipated in conjunctival sections possessing greater substantia propria. This supposition ACKNOWLEDGMENT is demonstrated by the patient's clinical ex Walter Schachat, M.D., referred the patient amination, showing significant thickening of and suggested the clinical diagnosis. the bulbar conjunctiva (Fig. 1 ) but strikingly little involvement of the tarsal conjunctiva REFERENCES (Fig, 2), The conjunctival mucosa is closely 1. Dutz, W., and Stout, A. P.: Kaposi's sar apposed over the tarsal plate without inter coma in infants and children. Cancer 13:684, 1960. 2. O'Brien, P. H., and Brasfield, R. D.: vening substantia propria. Kaposi's sarcoma. Cancer 19:1497, 1966. After radiotherapy, the perilimbal zone 3. Gordon, J. A. : Kaposi's sarcoma. A review where the substantia propria fades almost of 136 Rhodesian African cases. Postgrad. Med. completely regressed. Conjunctival involve J. 43:513, 1967. 4. Davis, J. : Kaposi's sarcoma. Present concept ment by Kaposi's sarcoma is characteristi of clinical course and treatment. N.Y. State J. cally more evident in the bulbar conjunctiva, Med. 68:2067, 1968. 5. Kaposi's sarcoma, editorial. Lancet 7798:300, sparing the immediate perilimbal zone and 1973. almost completely sparing the tarsal con 6. Sacks, I. : Kaposi's disease. Manifesting in junctiva. the eye. Br. J. Ophthalmol. 40:574, 19S6. 7. Mortada, A. : Conjunctival regressing Kaposi The tendency to completely involve the Br. J. Ophthalmol. 51:275, 1967. bulbar conjunctiva while sparing a narrow sarcoma. 8. Lieberman, P. H., and Llovera, I. N. : zone adjacent to the cornea can be a helpful Kaposi's sarcoma of the bulbar conjunctiva. Arch. clinical sign in differentiating Kaposi's sar Ophthalmol. 88:44, 1972.
