A case of orbital solitary fibrous tumor

BACKGROUND: Solitary fibrous tumor is a spindle cell neoplasm that most commonly arises in the pleura and very rarely involves the orbit. CASE: A 38-year-old woman presented with slowly progressive proptosis of 3 months duration and optic nerve head edema in her right eye. Magnetic resonance imaging revealed a well-circumscribed, round mass lesion, which showed isointensity to the gray matter in a T1-weighted image, and variegated intensity in a T2-weighted image and contact with the optic nerve in her right orbit. The tumor was successfully removed by anterior orbitotomy. OBSERVATIONS: The tumor showed a "patternless pattern" of tumor cell arrangement, alternating hypercellular and hypocellular areas, a hemangiopericytoma-like pattern, and thickened strands of collagen. Immunohistochemically, the tumor cells were positive for CD34 and vimentin, and all were negative for other markers of epithelial, neural, muscular, histiocytic, and vascular endothelial cell elements. The tumor was diagnosed as a solitary fibrous tumor, and the patient was doing well with no evidence of recurrence 15 months after surgery. CONCLUSIONS: This case was the 19th reported case of solitary fibrous tumor in the orbital region. CD34 is a highly sensitive marker for solitary fibrous tumor.     

Corneal endothelial degeneration and fibrous proliferation after plana vitrectomy.

A 45-year-old woman with juvenile-onset diabetes had persistent corneal edema after a pars plana vitrectomy and lensectomy procedure. Phase contrast and electron microscopic observation of the patient's cornea revealed extreme attenuation of the endothelial cell layer and abnormal collagenous and basement membrane material interposed between Descemet's membrane and the endothelium. Endothelial fibrous proliferations in this case were consistent with the development of ultrastructurally identical fibrous proliferations in many other situations involving dysfunction of the corneal endothelium.     

Eyelid fibrous histiocytoma

A 62-year-old woman had a painless, asymptomatic, eyelid mass that progressively increased in size over a six-month period. Histologic study of the excisional biopsy specimen revealed a fibrous histiocytoma that contained areas of granuloma formation. A three-year follow-up has shown no recurrence of the tumor. The rarity of the lesion in the eyelid and further evidence for the histiocytic origin of the tumor are discussed.     

Identification of T lymphocytes in ocular adnexal neoplasms by hybridoma monoclonal antibodies

We performed indirect immunofluorescence with the OKT series of hybridoma monoclonal antibodies in order to determine the total number of T cells and their subset distribution, that is, the percentages of helper (OKT3+T4+T8-) and suppressor/cytotoxic (OKT3+T4-T8+) T cells, in 28 ocular adnexal lymphoid neoplasms. OKT3+T4+ (helper) T cells vastly predominated in each of ten benign, polyclonal ocular lymphoid proliferations. The helper-suppressor T-cell ratio (T4-8) ranged from 2.5 to 8.2 (mean, 5.4) in these ten cases. In comparison, the mean T4-T8 ratio in 24 benign reactive lymph nodes was 3.4. These findings strongly suggested that the polyclonal ocular lymphoid proliferations represent a T-cell antigen-dependent response characterized by a proliferation of helper T cells, which in turn drive B cells to proliferate and to differentiate, eventually resulting in the formation of a clinically detectable tumor. The mean T4-T8 ratio was 2.3 in 18 ocular and in 16 nodal monoclonal B-cell proliferations, suggesting that the benign T cells in these proliferations represent a residual cell population rather than a distinctive subset originating in response to the B-cell neoplasm.     

Lenticular fibroxanthomatous nodule

PURPOSE: To describe two patients with unique lenticular nodular proliferations. DESIGN: Observational case reports. METHODS: The clinical histories and pathologic findings of two patients with lenticular nodular proliferations were reviewed. RESULTS: One patient with persistent hyperplastic primary vitreous and another patient with trauma developed lenticular nodular proliferations. The nodules were vascularized collections of foamy histiocytes, multinucleated cells, lens capsule, and lens epithelium that had undergone fibrous metaplasia. The lesions were classified as lenticular fibroxanthomatous nodules. CONCLUSIONS: A lenticular fibroxanthomatous nodule is a unique clinicopathologic entity that should be differentiated from Soemmerring ring, Elschnig pearl, and other simulating entities such as juvenile xanthogranuloma.     

Solitary fibrous tumor of the lacrimal sac

PURPOSE: Solitary fibrous tumor is a rare spindle cell tumor arising in the pleura. We report two cases of this tumor occurring in the lacrimal sac. METHODS: A 23-year-old man and a 34-year-old woman presented with a medial canthal mass. They underwent surgical excision of their tumors. RESULTS: Solitary fibrous tumors were diagnosed by light microscopy, immunohistochemical study, and electron microscopy. Immunohistochemical staining showed that tumor cells were reactive with vimentin and CD34 and demonstrated no smooth muscle or neural differentiation (nonreactive with desmin and S-100 protein). CONCLUSIONS: The findings indicate that solitary fibrous tumor can occur in the lacrimal sac and may recur locally if it is removed incompletely. To our knowledge, these are the first reported cases of solitary fibrous tumors occurring in the lacrimal sac.     

Malignant fibrous histiocytoma of the conjunctiva

Fibrous histiocytomas are mesenchymal tumours composed of cells with fibroblastic to histiocytic differentiation. They can occur in any part of the body including the orbital tissues. To date, there are 18 cases of fibrous histiocytoma arising from the corneoscleral limbus reported in the literature. Eleven of these were classified as benign, and the rest were malignant fibrous histiocytomas. Benign fibrous histiocytomas have been reported in the orbit, eyelid, episclera and conjunctiva. Malignant fibrous histiocytoma has been well described in the orbit, but rarely as a primary conjunctival tumour. The rarity of the tumour makes its diagnosis and management a challenge. Herein, the clinicopathological features of a case of malignant fibrous histiocytoma are presented and its management with wide excision and cryotherapy followed by ocular reconstruction with amniotic membrane transplant is discussed.     

Fibrous histiocytoma of the lacrimal gland

Fibrous histiocytoma, a primary mesenchymal tumor of the orbit, is known to arise from various ocular and adnexal tissues. We are unable to find a published report of this tumor originating from the lacrimal gland. We report a case of a benign fibrous histiocytoma of the lacrimal gland in an 11 year old girl who presented with painless, progressive eyelid swelling and mild proptosis. Imaging studies revealed a discrete mass in the lacrimal gland region. The tumor was completely excised by anterolateral orbitotomy. Light microscopy showed a spindle cell tumor arising from the lacrimal gland. The tumor cells were arranged in a characteristic storiform (cartwheel) pattern with no pleomorphism or mitotic figures. Immunohistochemically, the tumor cells were focally positive for CD-68 and negative for S-100, smooth muscle actin, vimentin, and CD-34, which ruled out neurofibroma, leiomyoma, solitary fibrous tumor, and hemangiopericytoma. Based on these features, a diagnosis of benign fibrous histiocytoma was made.     

Orbital giant cell angiofibroma recurring as a solitary fibrous tumor

Giant cell angiofibroma has recently been hypothesized to be a clinicopathologic variant of solitary fibrous tumor. The authors report a case of an orbital giant cell angiofibroma that recurred as a solitary fibrous tumor 4 years later. The report strongly supports the hypothesis that giant cell angiofibroma and solitary fibrous tumor are related.     

Giant cell angiofibroma of the orbit and eyelid.

PURPOSE: To report the clinicopathologic features of a newly recognized tumor, giant cell angiofibroma. DESIGN: Observational case series. MAIN OUTCOME MEASURES: Clinical and histopathologic features of giant cell angiofibroma. METHODS: Light and electron microscopy and immunohistochemistry of five cases of giant cell angiofibroma. RESULTS: A total of five patients (4 women and 1 man) are described: two presented with a painless mass in the eyelid, two with a mass in the orbit, and one presented with a conjunctival lesion. All lesions were well demarcated with no capsule and were composed of blood vessels, a patternless spindle-shaped cell proliferation with a solid and pseudovascular appearance, and multinucleated giant cells. Both spindle-shaped and giant tumor cells were intensely positive for CD34 and vimentin. CONCLUSION: Giant cell angiofibroma resembles solitary fibrous tumor and giant cell fibroblastoma and should be considered in the differential diagnosis of spindle-cell tumors in the eyelid, orbit, and conjunctiva.     

Fibrous histiocytoma of the corneoscleral limbus and conjunctiva

An 11-year-old boy and a 28-year-old woman each had a painless, slowly enlarging, elevated, yellow lesion of the inferior corneoscleral limbus and adjacent cornea and conjunctiva of the left eye. Histopathologic examination of both lesions disclosed involvement of the conjunctival substantia propria and the adjacent limbal and peripheral corneal stroma by a nonencapsulated, dense infiltrate composed of elongated fibrocytic cells, plump histiocytic cells occasionally with a vacuolated cytoplasm, some multinucleated giant cells, and scattered lymphocytes. The diagnosis in each case was fibrous histiocytoma. Both patients were otherwise in good health with no dermatologic, systemic, or other ocular abnormalities.     

Malignant solitary fibrous tumor metastatic to the orbit

A 61-year-old man with a history of malignant solitary fibrous tumor of the chest had development of unusual sites of metastasis involving the sphenoid wing of the orbit and soft tissues of the cheek. He was found to have a solitary fibrous tumor, an uncommon type of spindle cell neoplasm that most often arises in the pleura, which was metastatic to the orbit. This is the first reported case of malignant solitary fibrous tumor metastatic to the orbit. The clinical and histopathologic findings of metastatic malignant solitary fibrous tumor are described.     

Solitary fibrous tumor of the orbit: a clinicopathologic study of six cases with review of the literature

Solitary fibrous tumor of the orbit is a rare spindle cell neoplasm. There are 42 cases of solitary fibrous tumor of the orbit available in the literature. We present six more cases of orbital solitary fibrous tumors, which presented to our institute between 1999 and 2001. We highlight the need for clinical recognition of these tumors as a distinct entity and inclusion of this tumor in the etiological differential diagnosis of well-circumscribed orbital lesions presenting as unilateral proptosis in both children and in adults. The diagnosis may be suspected based on radiological features supported by histopathologic and immunohistochemical study. The strong CD34 immunoreactivity of this tumor supports its diagnosis. Complete surgical resection is the most important prognostic factor of this tumor.     

Pathobiology of epiretinal and subretinal membranes: possible roles for the matricellular proteins thrombospondin 1 and osteonectin (SPARC)

Epiretinal and subretinal membranes are fibrocellular proliferations which form on the surfaces of the neuroretina as a sequel to a variety of ocular diseases. When these proliferations complicate rhegmatogenous retinal detachment (a condition known as proliferative vitreoretinopathy or PVR), the membranes often contain numerous retinal pigment epithelial (RPE) cells and a variety of extracellular proteins. The extracellular proteins include adhesive proteins like collagen, laminin and fibronectin. In addition, several matricellular proteins with potential counter-adhesive functions are present in the membranes. Two such matricellular proteins, thrombospondin 1 and osteonectin (or SPARC: Secreted Protein Acidic and Rich in Cysteine), tend to be co-distributed with the RPE cells in PVR membranes. By virtue of their counter-adhesive properties, thrombospondin 1 and SPARC may reduce RPE cell-matrix adhesion and so permit key RPE cellular activities (for example, migration or shape change) in periretinal membrane development. Furthermore, within a 'cocktail' containing other proteins such as the metalloproteinases and growth factors like the scatter factor/hepatocyte growth factor family, matricellular proteins may play a role in the RPE cell dissociation from Bruch's membrane, which characterises early PVR.     

Granular cell tumor (myoblastoma) of the orbit.

An 8-year-old white boy had a tumor in the left orbit with clinical manifestations of muscular impairment, exophthalmos, and reduced visual acuity. Five months after exenteration of the orbit the boy was healthy without tumor recurrence. Based on our observations with electron, light, and phase microscopy, and histochemical studies, we conclude that this tumor is benign, of histiocytic origin, and we propose the term granular cell histiocytoma be used to describe this entity.     

Microproliferations in proliferative diabetic retinopathy and their relationship to the vitreous: corresponding light and electron microscopic studies

Using a special embedding method, autopsied eyes with proliferative diabetic retinopathy were studied. In areas with no posterior vitreous detachment, small proliferations may arise multifocally and grow within the vitreous cortex. The fibrous material of the vitreous cortex is densely interconnected with and obviously incorporated into the newly formed proliferated tissue, a process which causes coarse traction lines on the vitreous cortex lamellae. The clinical consequences of these findings are discussed.     

Solitary fibrous tumor of the orbit in a child

PURPOSE: Solitary fibrous tumor is a rare spindle-cell tumor that usually is seen in the pleura. The orbit is one of the most common extrapleural sites. It is frequently misdiagnosed as hemangiopericytoma and is seen in older patients. We present the youngest case of this tumor, which was apparent in family photographs by age 10 and removed at age 15. The first reported echography findings are presented. METHODS: Case report and literature review. RESULTS: Solitary fibrous tumor was diagnosed by microscopy and immunohistochemical study that showed cells reactive with vimentin and CD34. CONCLUSIONS: Solitary fibrous tumor of the orbit has been diagnosed with increasing frequency in recent years as the result of improved methods of pathologic examination. It is important to be aware of this tumor and recognize that it must be included in the differential diagnosis of highly vascular spindle-cell tumors even in young children.     

Neoplastic angioendotheliomatosis. A variant of malignant lymphoma immunohistochemical and ultrastructural observations of three cases

Neoplastic angioendotheliomatosis (NAE) is a rare, fatal disease characterized by widespread intravascular proliferations of malignant cells of putative endothelial origin. Clinically, dermatologic and bizarre neurological manifestations predominate, but review of the reported cases of NAE reveals ophthalmic involvement to be frequent. To our knowledge, no reports of NAE have appeared in the ophthalmic literature. We describe three cases of NAE with the ocular manifestations of visual loss, cells in the vitreous, retinal artery occlusion, retinal vascular and pigment epithelial alterations, nystagmus, and cortical blindness. Autopsies (including eyes and central nervous system) revealed pancorporal involvement by intravascular anaplastic cells in each patient. In two patients massive extravascular involvement was also present. The tumor cells lacked ultrastructural features of endothelial cells and failed to stain for factor-VIII-related antigen. Common leukocyte antigen, a maker for hematopoietic cells, particularly lymphocytes, was detected on tumor cells in all cases, indicating that NAE is probably an extranodal lymphoma. The dramatic response of the central nervous system lesions to radiotherapy in one case supports this contention. It is suggested that this disorder be treated as a malignant lymphoma.