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Multinucleate Cell Angiohistiocytoma of the Orbit
histopathology of superior limbic keratoconjunctivitis. Am J Ophthalmol 1978;85:101-10. 4. Udell IJ, Kenyon KR, Sawa M, Dohlman C H . Treatment of superior limbic keratoconjunctivitis by thermocauterization of the superior bulbar conjunctiva. Ophthalmology 1986;93: 162-6. 5. Confino J, Brown SI. Treatment of superior limbic keratoconjunctivitis with topical cromolyn sodium. A n n Ophthalmol 1987;19:129-31.
Multinucleate Cell Angiohistiocytoma of the Orbit Jerry A. Shields, M.D., Ralph C. Eagle, Jr., M.D., Carol L. Shields, M.D., and Kenette K. Sohmer, M.D. PURPOSE: A 31-year-old woman had a five-year history of painless progressive swelling of her right upper eyelid. METHOD: A mass was removed from the superonasal aspect of the right orbit. RESULTS: The orbital mass was composed of a collagenous stroma with capillary channels and irregular giant cells. The findings were characteristic of multinucleate cell angiohistiocytoma. CONCLUSION: Multinucleate cell angiohistiocytoma should be included in the differential diagnosis of circumscribed orbital tumors. ULTINUCLEATE CELL ANGIOHISTIOCYTOMA IS A
rare cutaneous disorder first described by Smith and Wilson Jones.1,2 Only a few cases have been recorded.3'5 Characteristically, multinucleate cell angiohistiocytoma occurs in adult women as multiple, violaceous papules that usually involve the legs or hands and rarely the face.3 We studied a clinicopathologic correlation of multinucleate cell angiohistiocytoma that affected the orbital soft tissues. A 31-year-old woman had a five-year history of
Oncology Service (J.A.S., C.L.S.) and the Department of Pathology (R.C.E.), Wills Eye Hospital, Thomas Jefferson University; and Branchburg Eye Physicians (K.K.S.). Supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania. Inquiries to Jerry A. Shields, M.D., Ocular Oncology Service, Wills Eye Hospital, 900 Walnut St., Philadelphia, PA 19107; fax; (215) 928-1140.
402
painless progressive swelling of her right upper eyelid. Computed tomography showed an anterior orbital mass and she was referred for surgical excision of the lesion. Her ophthalmic and general medical history was otherwise noncontributory. General physical examination disclosed normal findings, with no abnormal cutaneous lesions. There was thickening of the medial aspect of the right upper eyelid and 2 mm of right blepharoptosis. A firm, nontender, orbital, soft-tissue mass was palpable superonasally to the right globe. There was no proptosis, displacement of the globe, choroidal folds, or optic disk edema. Orbital computed tomography showed an ovoid, well-circumscribed, soft-tissue mass in the superonasal aspect of the orbit anteriorly (Fig. 1 ). The tumor was removed intact through a superomedial orbitotomy. The mass was composed of collagenous connective tissue stroma, containing dilated, thin-walled, blood vessels (Fig. 2, left). Throughout the stroma were numerous bland multinucleated giant cells (Fig. 2, right). Sections of the mass were examined in consultation by pathologists in the Departments of Ophthalmic Pathology, Soft Tissue Tumor Pathology, and Dermatopathology at the Armed Forces Institute of Pathology, Washington, D.C. There was a consensus that the findings were characteristic of multinucleate cell angiohistiocytoma. The patient is well and without tumor recurrence more than two years after the operation. Multinucleate cell angiohistiocytoma is an idiopathic, benign vascular condition characterized clinically by multiple, grouped, violaceous, painless, cutaneous papules that generally occur in the arms and
Fig.
1 (Shields and associates).
C o m p u t e d tomogram
shows circumscribed mass i n superomedial portion of orbit (arrow).
AMERICAN JOURNAL OF OPHTHALMOLOGY
SEPTEMBER 1995
.».«***. v
Fig. 2 (Shields and associates). Left, Photomicrograph shows cellular vascular lesion with many irregular multinucleated giant cells (hematoxylin and eosin, X150). Right, Higher magnification photomicrograph shows blood vessels, histiocytes, and a characteristic multinucleated giant cell (arrow) (hematoxylin and eosin, X400).
legs of women over the age of 40 years. Histopathologically, multinucleate cell angiohistiocytoma is characterized by increased numbers of small blood vessels in the dermis, a sparse lymphocytic infiltration, histiocytes, and prominent multinucleated giant cells. Immunohistochemical studies have indicated that the dermal cells are compatible with histiocytes. Smith, Cerio, and Wilson Jones4 stated that multinucleate cell angiohistiocytoma is a distinct entity with different features from Kaposi's sarcoma and other cutaneous vascular entities. The pathogenesis of multinucleate cell angiohistiocytoma is uncertain. On the basis of their study of ten affected patients, Smith, Cerio, and Wilson Jones4 concluded that it is a specific chronic inflammatory disorder of unknown cause with histopathologic features of vascular hyperplasia and increased numbers of unusual connective tissue cells. The differential diagnosis of a circumscribed, round to ovoid, soft-tissue, orbital mass includes cavernous hemangioma, schwannoma, fibrous histiocytoma, hemangiopericytoma, and occasionally other less common lesions. The age and gender of our
VOL.120, N o . 3
patient is comparable with the age and gender of described patients with cutaneous multinucleate cell angiohistiocytoma, most of whom were adult women with a mean age of about 52 years.4 On the basis of our observations of this case, multinucleate cell angiohistiocytoma should be added to the list of conditions that can cause a well-circumscribed orbital mass. We anticipate that our patient should have an excellent prognosis. REFERENCES 1. Smith NP, Wilson Jones E. Multinucleate cell angiohistiocytoma—a new entity. Br J Dermatol 1985;113:15-20. 2. . Multinucleate cell angiohistiocytoma: a new entity. J Cutan Pathol 1986;13:77-81. 3. Smolle J, Auboeck L, Gogg-Retzer I, Soyer HP, Kerl H. Multinucleate cell angiohistiocytoma: a clinicopathological, immunohistochemical and ultrastructural study. Br J Dermatol 1989;121:113-21. 4. Smith NP, Cerio R, Wilson Jones E. Multinucleate cell angiohistiocytoma: an acquired vascular anomaly to be distinguished from Kaposi's sarcoma. Br J Dermatol 1990; 122:651-3. 5. Annessi G, Girolomoni G, Gianetti A. Multinucleate cell angiohistiocytoma. Am J Dermatopathol 1992;14:340-4.
BRIEF REPORTS
403
Multinucleate Cell Angiohistiocytoma of the Orbit Jerry A. Shields, M.D., Ralph C. Eagle, Jr., M.D., Carol L. Shields, M.D., and Kenette K. Sohmer, M.D. PURPOSE: A 31-year-old woman had a five-year history of painless progressive swelling of her right upper eyelid. METHOD: A mass was removed from the superonasal aspect of the right orbit. RESULTS: The orbital mass was composed of a collagenous stroma with capillary channels and irregular giant cells. The findings were characteristic of multinucleate cell angiohistiocytoma. CONCLUSION: Multinucleate cell angiohistiocytoma should be included in the differential diagnosis of circumscribed orbital tumors. ULTINUCLEATE CELL ANGIOHISTIOCYTOMA IS A
rare cutaneous disorder first described by Smith and Wilson Jones.1,2 Only a few cases have been recorded.3'5 Characteristically, multinucleate cell angiohistiocytoma occurs in adult women as multiple, violaceous papules that usually involve the legs or hands and rarely the face.3 We studied a clinicopathologic correlation of multinucleate cell angiohistiocytoma that affected the orbital soft tissues. A 31-year-old woman had a five-year history of
Oncology Service (J.A.S., C.L.S.) and the Department of Pathology (R.C.E.), Wills Eye Hospital, Thomas Jefferson University; and Branchburg Eye Physicians (K.K.S.). Supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania. Inquiries to Jerry A. Shields, M.D., Ocular Oncology Service, Wills Eye Hospital, 900 Walnut St., Philadelphia, PA 19107; fax; (215) 928-1140.
402
painless progressive swelling of her right upper eyelid. Computed tomography showed an anterior orbital mass and she was referred for surgical excision of the lesion. Her ophthalmic and general medical history was otherwise noncontributory. General physical examination disclosed normal findings, with no abnormal cutaneous lesions. There was thickening of the medial aspect of the right upper eyelid and 2 mm of right blepharoptosis. A firm, nontender, orbital, soft-tissue mass was palpable superonasally to the right globe. There was no proptosis, displacement of the globe, choroidal folds, or optic disk edema. Orbital computed tomography showed an ovoid, well-circumscribed, soft-tissue mass in the superonasal aspect of the orbit anteriorly (Fig. 1 ). The tumor was removed intact through a superomedial orbitotomy. The mass was composed of collagenous connective tissue stroma, containing dilated, thin-walled, blood vessels (Fig. 2, left). Throughout the stroma were numerous bland multinucleated giant cells (Fig. 2, right). Sections of the mass were examined in consultation by pathologists in the Departments of Ophthalmic Pathology, Soft Tissue Tumor Pathology, and Dermatopathology at the Armed Forces Institute of Pathology, Washington, D.C. There was a consensus that the findings were characteristic of multinucleate cell angiohistiocytoma. The patient is well and without tumor recurrence more than two years after the operation. Multinucleate cell angiohistiocytoma is an idiopathic, benign vascular condition characterized clinically by multiple, grouped, violaceous, painless, cutaneous papules that generally occur in the arms and
Fig.
1 (Shields and associates).
C o m p u t e d tomogram
shows circumscribed mass i n superomedial portion of orbit (arrow).
AMERICAN JOURNAL OF OPHTHALMOLOGY
SEPTEMBER 1995
.».«***. v
Fig. 2 (Shields and associates). Left, Photomicrograph shows cellular vascular lesion with many irregular multinucleated giant cells (hematoxylin and eosin, X150). Right, Higher magnification photomicrograph shows blood vessels, histiocytes, and a characteristic multinucleated giant cell (arrow) (hematoxylin and eosin, X400).
legs of women over the age of 40 years. Histopathologically, multinucleate cell angiohistiocytoma is characterized by increased numbers of small blood vessels in the dermis, a sparse lymphocytic infiltration, histiocytes, and prominent multinucleated giant cells. Immunohistochemical studies have indicated that the dermal cells are compatible with histiocytes. Smith, Cerio, and Wilson Jones4 stated that multinucleate cell angiohistiocytoma is a distinct entity with different features from Kaposi's sarcoma and other cutaneous vascular entities. The pathogenesis of multinucleate cell angiohistiocytoma is uncertain. On the basis of their study of ten affected patients, Smith, Cerio, and Wilson Jones4 concluded that it is a specific chronic inflammatory disorder of unknown cause with histopathologic features of vascular hyperplasia and increased numbers of unusual connective tissue cells. The differential diagnosis of a circumscribed, round to ovoid, soft-tissue, orbital mass includes cavernous hemangioma, schwannoma, fibrous histiocytoma, hemangiopericytoma, and occasionally other less common lesions. The age and gender of our
VOL.120, N o . 3
patient is comparable with the age and gender of described patients with cutaneous multinucleate cell angiohistiocytoma, most of whom were adult women with a mean age of about 52 years.4 On the basis of our observations of this case, multinucleate cell angiohistiocytoma should be added to the list of conditions that can cause a well-circumscribed orbital mass. We anticipate that our patient should have an excellent prognosis. REFERENCES 1. Smith NP, Wilson Jones E. Multinucleate cell angiohistiocytoma—a new entity. Br J Dermatol 1985;113:15-20. 2. . Multinucleate cell angiohistiocytoma: a new entity. J Cutan Pathol 1986;13:77-81. 3. Smolle J, Auboeck L, Gogg-Retzer I, Soyer HP, Kerl H. Multinucleate cell angiohistiocytoma: a clinicopathological, immunohistochemical and ultrastructural study. Br J Dermatol 1989;121:113-21. 4. Smith NP, Cerio R, Wilson Jones E. Multinucleate cell angiohistiocytoma: an acquired vascular anomaly to be distinguished from Kaposi's sarcoma. Br J Dermatol 1990; 122:651-3. 5. Annessi G, Girolomoni G, Gianetti A. Multinucleate cell angiohistiocytoma. Am J Dermatopathol 1992;14:340-4.
BRIEF REPORTS
403