- Email: [email protected]
Hemangiopericytoma of the Conjunctiva
Hemangiopericytoma of the Conjunctiva Two Cases HANS E. GROSSNIKLAUS, MD, W. RICHARD GREEN, MD, STEWART M. WOLFF, MD, NICHOLAS T. ILIFF, MD
Abstract: Two patients with hemangiopericytoma of the conjunctiva are reported. The first patient, a 40-year-old woman with a six-month history of a conjunctival mass, presented with a lesion confined to the conjunctiva. The second patient, a 43-year-old man, presented with a one-year history of a conjunctival mass. Excisional biopsy and histopathologic examination showed both lesions to be a hemangiopericytoma; an uncommon tumor of the conjunctiva. [Key words: conjunctiva, hemangiopericytoma, tumor.] Ophthalmology
93:265-267, 1986
Hemangiopericytomas, first reported by Stout and Murray, I are tumors that arise from vascular pericytes. These tumors may arise in the pelvic retroperitoneal spaces, soft tissues of the neck, limbs, trunk, paraspinal areas and, rarely, the orbit. 2,3 We recently observed the occurrence of hemangiopericytomas in the conjunctiva, an unusual location for the presentation of these lesions.
CASE REPORTS Case 1. A 40-year-old woman was evaluated for a right lower cul-de-sac conjunctival mass that had been present for six months and had recently enlarged. Examination showed a firm adherence of the mass to the conjunctiva (Fig 1). Computed tomography of the head showed no orbital involvement of the mass. The lesion was totally resected. PATHOLOGIC EVALUATION The specimen was a firm 14 X 14 X 12 mm ovoid, fleshy mass. Evaluation by light microscopy showed a dense subepithelial mass of spindle-shaped cells arranged in whorls and fascicles (Fig 2). The cells had oblong and slightly hyperchromatic
nuclei. Rare mitotic figures were present (one per 40 high power fields). There was no distinct capsule surrounding the tumor. Normal conjunctival epithelium containing goblet cells with moderate underlying chronic inflammation was present at one margin. Special stains showed reticulin present around normal vascular channels, and around and within tumor cell aggregations. The tumor cells were outside the basement membrane of the endothelial cells. Transmission electron microscopy of the tumor showed it to be composed of spindle-shaped cells arising from vascular pericytes (Fig 3). The tumor cells contained moderate amounts of rough endoplasmic reticulum, Golgi apparatus, mitochondria, and exhibited patchy areas of basement membrane production. In some areas, tumor cells were adjacent to normal endothelial cells. No distinct intracytoplasmic filaments were present in the tumor cells. A diagnosis of hemangiopericytoma was made. Case 2. A 43-year-old man had a one-year history of a gradually enlarging mass in the medial canthal area of the right eye underlying the conjunctiva. The mass was "needled" on two previous occasions without reduction in size. Examination showed a 1 cm mass in the medial canthal area protruding between the lids (Fig 4). The mass was smooth, pliable, and mobile. The overlying conjunctiva was injected. The lesion was resected and submitted for examination. PATHOLOGIC EVALUATION
From the Eye Pathology Laboratory, Wilmer Ophthalmological Institute, The Johns Hopkins Medical Institutions, Baltimore. Reprint requests to W. Richard Green, MD, Eye Pathology Laboratory, Wilmer Institute, Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, MD 21205.
The specimen was a homogenous, fleshy mass measuring 10.5 X 8.7 X 4.0 mm. Light microscopy showed a circumscribed mass composed ofa lacy-to-dense network of primarily spindleshaped cells with elongated basophilic nuclei and no apparent nucleoli and moderate eosinophilic cytoplasm (Fig. 5). Occa-
265
OPHTHALMOLOGY •
FEBRUARY 1986 •
VOLUME 93 •
NUMBER 2
Fig 1. Case I. Patient at presentation showing a large ovoid conjunctival lesion that protruded over her right lower lid.
Fig 3. Case I. Transmission electron micrograph of the tumor showing spindle-shaped cells (asterisks) in a perivascular configuration. These cells displayed patches of basement membrane production (left inset, arrow), and occasional junctional complexes (right inset) (X6000). Fig 2. Case I. Area shows relatively solid tumor composed of spindleshaped cells and inconspicuous small blood vessels (arrows) (hematoxylineosin, X450).
sional multinucleated giant cells, foci oflymphocytes, and plasma cells were scattered throughout the specimen. Transmission electron microscopy showed tumor cells in a perivascular configuration. The tumor cells were spindle-shaped and exhibited patches of basement membrane production and occasional junctional complexes. The lesion was diagnosed as a hemangiopericytoma.
DISCUSSION Hemangiopericytomas are vascular tumors that arise from pericytes. 3 Henderson and Farrow2 report that approximately 1.6% of all orbital tumors and approximately 3.1 % of all primary orbital neoplasms are hemangiopericytomas. In their series of 11 patients,z two, with incomplete excision, died of metastatic disease 35 years after initial diagnosis. Hemangiopericytomas metastasize in 12 to 45% of cases. 4 Sites of metastasis from orbital heman266
Fig 4. Case 2. Patient at presentation showing a right medial conjunctival mass in the interpalpebral fissure.
giopericytomas include bone, lung, and breast. 2 ,4 The tumor has an insidious presentation, is slow growing, presents between the ages of 20 and 40 years, and may metastasize late.
GROSSNIKLAUS, et al •
Differential diagnoses include angioblastic meningioma, hemangioendothelioma, vascular leiomyoma, glomus cell tumors, fibrosarcoma, and fibrous histiocytoma. A hemangioendothelioma can be differentiated from a hemangiopericytoma with the aid of reticulin stains. Hemangioendotheliomas have endothelial cells arranged in nests outlined by reticulin. Other tumors in the differential diagnoses can be distinguished by electron microscopy. Hemangiopericytomas are composed of nests of tumor cells outside the basement membrane of the endothelial cells. The cells have lighter cytoplasm than vascular smooth muscle or endothelial cells, lack the prominent desmosomes and cytoplasmic filamentation of angioblastic meningiomas, and produce patches of basement membrane. S The diagnosis is difficult in some instances because some authorsS believe that pericytes may differentiate toward fibroblasts or smooth muscle. The light microscopic appearance of our conjunctival hemangiopericytomas may resemble fibrous histiocytomas. Enzinger and Smith3 reported tumors that exhibited features of both hemangiopericytomas and fibrous histiocytomas, further suggesting a continuum of pericytes and fibroblasts. Our conjunctival hemangiopericytomas have the additional feature of lack of a capsule. Jakobiec and co-workers5 suggested that the formation of a capsule may result from tumor growth and pressure against orbital bone. Hemangiopericytomas elsewhere in the body tend not to be encapsulated, as in our cases, presumably due to lack of resistance to tumor growth. The location of a hemangiopericytoma in the conjunctiva is unusual. There have been previous reports of hemangiopericytomas of the eyelid,6,7 lacrimal sac,8 anterior orbit with subconjunctival involvement,S and lid and the orbit. 9 Searl and Ni lO include a report of a 25year-old woman with an epibulbar hemangiopericytoma in a series often patients with orbital and periocular hemangiopericytomas. The recommended method of treatment of hemangiopericytomas is surgical excision without violation of the surrounding fibrous tissue. 2 Incisional biopsy or piecemeal removal may cause seeding of tumor cells, thus predisposing to local recurrence or metastasis. If a recurrence is present, a radical excision is mandated. Radiotherapy is not curative, but it may produce some degree of palliation. 2
HEMANGIOPERICYTOMA
Fig 5. Case 2. Tumor is composed of swirls of spindle-shaped cells associated with and surrounding blood vessels (asterisks) (hematoxylineosin, X480).
REFERENCES 1. Stout AP, Murray MR. Hemangiopericytoma; a vascular tumor featuring Zimmermann's pericytes. Ann Surg 1942; 116:26-33. 2. Henderson JW, Farrow GM. Primary orbital hemangiopericytoma; an aggressive and potentially malignant neoplasm. Arch Ophthalmol1978; 96:666-73. 3. Enzinger FM, Smith SH. Hemangiopericytoma; an analysis of 106 cases. Hum Patho11976; 7:61-82. 4. Panda A, Dayal Y, Singhal V, Pattnaik NK. Haemangiopericytoma. Sr J Ophthalmol1984; 68:124-7. 5. Jakobiec FA, Howard GM, Jones IS, Wolff M. Hemangiopericytoma of the orbit. Am J Ophthalmol1974; 78:816-34. 6. Macoul KL. Hemangiopericytoma of the lid and orbit. Am J Ophthalmol 1968; 66:731-3. 7. Oshida N, Hisatomi U, Takemura M, Kobayashi Y. A hemangiopericytoma of the eye lid. Folia Ophthalmol Jpn 1970; 21 :269-72. 8. Gurney N, Chalkley T, O'Grady R. Lacrimal sac hemangiopericytoma. Am J Ophthalmol1971; 71 :757-9. 9. Stout AP. Hemangiopericytoma; a study of twenty-five new cases. Cancer 1949; 2:1027-54. 10. Searl SS, Ni C. Hemangiopericytoma. Int Ophthalmol Clin 1982; 22(1): 141-62.
267
Abstract: Two patients with hemangiopericytoma of the conjunctiva are reported. The first patient, a 40-year-old woman with a six-month history of a conjunctival mass, presented with a lesion confined to the conjunctiva. The second patient, a 43-year-old man, presented with a one-year history of a conjunctival mass. Excisional biopsy and histopathologic examination showed both lesions to be a hemangiopericytoma; an uncommon tumor of the conjunctiva. [Key words: conjunctiva, hemangiopericytoma, tumor.] Ophthalmology
93:265-267, 1986
Hemangiopericytomas, first reported by Stout and Murray, I are tumors that arise from vascular pericytes. These tumors may arise in the pelvic retroperitoneal spaces, soft tissues of the neck, limbs, trunk, paraspinal areas and, rarely, the orbit. 2,3 We recently observed the occurrence of hemangiopericytomas in the conjunctiva, an unusual location for the presentation of these lesions.
CASE REPORTS Case 1. A 40-year-old woman was evaluated for a right lower cul-de-sac conjunctival mass that had been present for six months and had recently enlarged. Examination showed a firm adherence of the mass to the conjunctiva (Fig 1). Computed tomography of the head showed no orbital involvement of the mass. The lesion was totally resected. PATHOLOGIC EVALUATION The specimen was a firm 14 X 14 X 12 mm ovoid, fleshy mass. Evaluation by light microscopy showed a dense subepithelial mass of spindle-shaped cells arranged in whorls and fascicles (Fig 2). The cells had oblong and slightly hyperchromatic
nuclei. Rare mitotic figures were present (one per 40 high power fields). There was no distinct capsule surrounding the tumor. Normal conjunctival epithelium containing goblet cells with moderate underlying chronic inflammation was present at one margin. Special stains showed reticulin present around normal vascular channels, and around and within tumor cell aggregations. The tumor cells were outside the basement membrane of the endothelial cells. Transmission electron microscopy of the tumor showed it to be composed of spindle-shaped cells arising from vascular pericytes (Fig 3). The tumor cells contained moderate amounts of rough endoplasmic reticulum, Golgi apparatus, mitochondria, and exhibited patchy areas of basement membrane production. In some areas, tumor cells were adjacent to normal endothelial cells. No distinct intracytoplasmic filaments were present in the tumor cells. A diagnosis of hemangiopericytoma was made. Case 2. A 43-year-old man had a one-year history of a gradually enlarging mass in the medial canthal area of the right eye underlying the conjunctiva. The mass was "needled" on two previous occasions without reduction in size. Examination showed a 1 cm mass in the medial canthal area protruding between the lids (Fig 4). The mass was smooth, pliable, and mobile. The overlying conjunctiva was injected. The lesion was resected and submitted for examination. PATHOLOGIC EVALUATION
From the Eye Pathology Laboratory, Wilmer Ophthalmological Institute, The Johns Hopkins Medical Institutions, Baltimore. Reprint requests to W. Richard Green, MD, Eye Pathology Laboratory, Wilmer Institute, Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, MD 21205.
The specimen was a homogenous, fleshy mass measuring 10.5 X 8.7 X 4.0 mm. Light microscopy showed a circumscribed mass composed ofa lacy-to-dense network of primarily spindleshaped cells with elongated basophilic nuclei and no apparent nucleoli and moderate eosinophilic cytoplasm (Fig. 5). Occa-
265
OPHTHALMOLOGY •
FEBRUARY 1986 •
VOLUME 93 •
NUMBER 2
Fig 1. Case I. Patient at presentation showing a large ovoid conjunctival lesion that protruded over her right lower lid.
Fig 3. Case I. Transmission electron micrograph of the tumor showing spindle-shaped cells (asterisks) in a perivascular configuration. These cells displayed patches of basement membrane production (left inset, arrow), and occasional junctional complexes (right inset) (X6000). Fig 2. Case I. Area shows relatively solid tumor composed of spindleshaped cells and inconspicuous small blood vessels (arrows) (hematoxylineosin, X450).
sional multinucleated giant cells, foci oflymphocytes, and plasma cells were scattered throughout the specimen. Transmission electron microscopy showed tumor cells in a perivascular configuration. The tumor cells were spindle-shaped and exhibited patches of basement membrane production and occasional junctional complexes. The lesion was diagnosed as a hemangiopericytoma.
DISCUSSION Hemangiopericytomas are vascular tumors that arise from pericytes. 3 Henderson and Farrow2 report that approximately 1.6% of all orbital tumors and approximately 3.1 % of all primary orbital neoplasms are hemangiopericytomas. In their series of 11 patients,z two, with incomplete excision, died of metastatic disease 35 years after initial diagnosis. Hemangiopericytomas metastasize in 12 to 45% of cases. 4 Sites of metastasis from orbital heman266
Fig 4. Case 2. Patient at presentation showing a right medial conjunctival mass in the interpalpebral fissure.
giopericytomas include bone, lung, and breast. 2 ,4 The tumor has an insidious presentation, is slow growing, presents between the ages of 20 and 40 years, and may metastasize late.
GROSSNIKLAUS, et al •
Differential diagnoses include angioblastic meningioma, hemangioendothelioma, vascular leiomyoma, glomus cell tumors, fibrosarcoma, and fibrous histiocytoma. A hemangioendothelioma can be differentiated from a hemangiopericytoma with the aid of reticulin stains. Hemangioendotheliomas have endothelial cells arranged in nests outlined by reticulin. Other tumors in the differential diagnoses can be distinguished by electron microscopy. Hemangiopericytomas are composed of nests of tumor cells outside the basement membrane of the endothelial cells. The cells have lighter cytoplasm than vascular smooth muscle or endothelial cells, lack the prominent desmosomes and cytoplasmic filamentation of angioblastic meningiomas, and produce patches of basement membrane. S The diagnosis is difficult in some instances because some authorsS believe that pericytes may differentiate toward fibroblasts or smooth muscle. The light microscopic appearance of our conjunctival hemangiopericytomas may resemble fibrous histiocytomas. Enzinger and Smith3 reported tumors that exhibited features of both hemangiopericytomas and fibrous histiocytomas, further suggesting a continuum of pericytes and fibroblasts. Our conjunctival hemangiopericytomas have the additional feature of lack of a capsule. Jakobiec and co-workers5 suggested that the formation of a capsule may result from tumor growth and pressure against orbital bone. Hemangiopericytomas elsewhere in the body tend not to be encapsulated, as in our cases, presumably due to lack of resistance to tumor growth. The location of a hemangiopericytoma in the conjunctiva is unusual. There have been previous reports of hemangiopericytomas of the eyelid,6,7 lacrimal sac,8 anterior orbit with subconjunctival involvement,S and lid and the orbit. 9 Searl and Ni lO include a report of a 25year-old woman with an epibulbar hemangiopericytoma in a series often patients with orbital and periocular hemangiopericytomas. The recommended method of treatment of hemangiopericytomas is surgical excision without violation of the surrounding fibrous tissue. 2 Incisional biopsy or piecemeal removal may cause seeding of tumor cells, thus predisposing to local recurrence or metastasis. If a recurrence is present, a radical excision is mandated. Radiotherapy is not curative, but it may produce some degree of palliation. 2
HEMANGIOPERICYTOMA
Fig 5. Case 2. Tumor is composed of swirls of spindle-shaped cells associated with and surrounding blood vessels (asterisks) (hematoxylineosin, X480).
REFERENCES 1. Stout AP, Murray MR. Hemangiopericytoma; a vascular tumor featuring Zimmermann's pericytes. Ann Surg 1942; 116:26-33. 2. Henderson JW, Farrow GM. Primary orbital hemangiopericytoma; an aggressive and potentially malignant neoplasm. Arch Ophthalmol1978; 96:666-73. 3. Enzinger FM, Smith SH. Hemangiopericytoma; an analysis of 106 cases. Hum Patho11976; 7:61-82. 4. Panda A, Dayal Y, Singhal V, Pattnaik NK. Haemangiopericytoma. Sr J Ophthalmol1984; 68:124-7. 5. Jakobiec FA, Howard GM, Jones IS, Wolff M. Hemangiopericytoma of the orbit. Am J Ophthalmol1974; 78:816-34. 6. Macoul KL. Hemangiopericytoma of the lid and orbit. Am J Ophthalmol 1968; 66:731-3. 7. Oshida N, Hisatomi U, Takemura M, Kobayashi Y. A hemangiopericytoma of the eye lid. Folia Ophthalmol Jpn 1970; 21 :269-72. 8. Gurney N, Chalkley T, O'Grady R. Lacrimal sac hemangiopericytoma. Am J Ophthalmol1971; 71 :757-9. 9. Stout AP. Hemangiopericytoma; a study of twenty-five new cases. Cancer 1949; 2:1027-54. 10. Searl SS, Ni C. Hemangiopericytoma. Int Ophthalmol Clin 1982; 22(1): 141-62.
267