Isolated neurofibroma of the orbit with extensive myxoid changes: a clinicopathologic study including MRI and electron microscopic findings.

Isolated orbital neurofibroma (INO) without systemic neurofibromatosis is rare. Previous reports including MRI with elaboration of the ultrastructural features of the cellular and extracellular components in INO do not exist. A 44-year-old male presented with painless, slowly progressive proptosis (6 mm) and downward displacement (3 mm) of the left eye of two years duration. MRI revealed a suprabulbar mass hypointense to fat, isointense to brain on T1-weighted images with small hyperintense areas following contrast enhancement. T2-weighted images showed a predominantly hyperintense mass with a few hypointense islands. The lesion was excised using an anterior superior transseptal orbitotomy. Histopathologically, large areas of a paucicellular myxoid stroma containing mucopolysaccharides made up approximately three-quarters of the mass. Focal hypercellular areas showed marked collagen deposition. Blood vessels were distributed at the periphery of the lesion and in association with nodules of nerve sheath cells. Ultrastructurally, perineural and fibroblast-like cells were loosely arranged in a myxoid stroma. Schwann cells were embedded in a dense collagenous matrix. Variable differentiation of Schwann cells, perineural cells and fibroblast-like cells associated with a more solid (collagenous) or loose (myxoid) extracellular matrix may determine the characteristic, though not specific, MRI features in isolated neurofibromas of the orbit.

July 2002: 66-year-old female with a one-year history of progressive left proptosis.

The July 2002 Case of the Month (COM). This 66-year-old Caucasian female presented with gradually increasing protrusion of her left eye over a one-year period. She complained of increased tearing and foreign body sensation. The physical examination revealed a visual acuity of 20/20, normal color testing, full vision field with motility of her left eye limited in lateral gaze. Pupils were round, symmetric, with no afferent pupillary defect noted. On external examination, her left eye was grossly proptotic with resistance to retropulsion. She had 4 mm proptosis of the left eye. Computed tomography and MR imaging demonstrated a left retro-orbital mass with gadolinium enhancement and focal remodeling of orbital bones. She underwent surgical resection of tumor with a diagnosis of solitary fibrous tumor, and postoperatively she was symptom free. The histopathological differential diagnoses of spindle cell neoplasms of the orbit are discussed. Five months after surgery, no evidence of tumor recurrence was seen on neuroimaging and her vision was 20/20.

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.

Malignant fibrous histiocytoma of the lateral wall of the orbit.

A 58-year-old male presented with malignant fibrous histiocytoma originating from the lateral wall of the orbit, manifesting as left exophthalmos and diplopia on left lateral gaze. Magnetic resonance (MR) imaging demonstrated the tumor extending into the left orbit and under the temporalis muscle. The tumor was isointense with muscle on T1-weighted MR images and was surrounded by a low-intensity rim, which was proved to be the capsule after histological examination. The tumor was completely removed after detaching the lateral orbital rim and zygomatic arch using the dismasking flap method. He underwent one course of CYVADIC chemotherapy and has demonstrated no recurrence for 1 year and 6 months postoperatively.

Solitary fibrous tumor of the nasal cavity and orbit.

Solitary fibrous tumors (SFT) are rare spindle cell neoplasms generally associated with the serosal surface, especially the pleura. Two SFT are reported arising in the nasal cavity and orbit, occurring in a 33 year old female and a 21 year old male, respectively. The lesions lacked the characteristic features of other recognized neoplasms that occur in these regions. Immunohistochemically, the tumors cells were diffusely and strongly positive for vimentin and CD34 and some or many tumor cells expressed neuron-specific enolase and Leu-7. Ultrastructurally, the tumor cells showed no distinct features although they were suggested to be primitive mesenchymal or fibroblast-like cells. Both tumors were diploid by flow cytometry. The two patients have had benign clinical courses with limited follow-up. Our findings suggest that SFT is of mesenchymal cell origin and support another location for SFT, indicating additional evidence against mesothelial origin. SFT should be included in the differential diagnosis of spindle cell tumors arising in the nasal cavity and orbit.

Spontaneous intraorbital hematoma mimicking orbital neoplasm--case report.

A 66-year-old female presented with spontaneous intraorbital hematoma manifesting as left exophthalmos after left eye discomfort persisting for 5 months. She had no history of head injury. Computed tomography revealed a round mass in her left orbit which was slightly high density with no postcontrast enhancement. A blood cyst was extirpated through frontotemporal craniotomy. Histological findings showed no tumor or vascular anomaly. Spontaneous intraorbital hematoma with insidious symptoms is extremely rare and should be considered in the differential diagnosis of orbital mass lesions.

Primary post-traumatic intraorbital meningioma.

Intraorbital meningiomas are uncommon tumours that usually represent extension from a primary intracranial tumour. Trauma has been proposed by Cushing and Eisenhardt as an etiologic factor in the development of meningioma following head injury. We report a case of primary post-traumatic intraorbital meningioma and discuss its pathogenesis and management.

Solitary fibrous tumor of the orbit.

We report three cases of an orbital soft tissue lesion that fulfills the histologic, immunohistochemical, and electron microscopic criteria for solitary fibrous tumor, an entity previously described as a pleural tumor, but recently reported to occur in other locations. All three patients presented with proptosis. Two of the patients were cured by simple excision, and one patient had two recurrences, the last recurrence incompletely excised. The findings indicate that solitary fibrous tumor can occur in the orbit and, like solitary fibrous tumors of other anatomic sites, may behave in a nonaggressive or occasionally, locally aggressive fashion, with as yet no metastatic potential demonstrated in orbital lesions.

Granular cell tumor of the eye (myoblastoma): ultrastructural and immunohistochemical studies.

The clinical and pathological characteristics of a myoblastoma of the orbital are described. The tumor presented as a painless hard mass in the left orbit of a 56-year-old caucasian male, causing diplopia and hypertrophy of the left eye in the primary visual position. Light microscopy studies revealed a typical granular cell tumor with PAS-positive intracytoplasmic granules. Immunoperoxidase studies showed positivity for S-100 protein and desmin. Electron microscopy studies showed numerous intracellular myelin degenerative bodies. The patient developed an epidermoid cyst at the site of initial surgery, but has no signs of local or distant disease two years after initial treatment.

Primary malignant ectomesenchymoma of the orbit.

Malignant ectomesenchymoma (MEM) is a rare soft tissue tumour believed to arise from a pluripotential migratory neural crest cell and composed of both a mesenchymal element (most often rhabdomyosarcoma) and a neuroectodermal element (often neuroblastoma). Reported sites of origin are the abdomen, perineum or scrotum, the extremities, the middle ear, nasopharynx, face, and neck. We report the first case of an orbital MEM, with a review of the 17 cases previously reported from other sites.

Leiomyosarcoma of the orbit: diagnosis of its recurrence by fine-needle aspiration cytology.

An 8 1/2-yr-old boy with proptosis was diagnosed to have low-grade leiomyosarcoma of the orbit following lateral orbitotomy and histology of an incompletely excised intraconal mass. He received chemotherapy but had recurrence of proptosis at the age of 12 yr. Ultrasonography revealed a solid mass and its fine-needle aspiration cytology features were consistent with recurrence of leiomyosarcoma. A year later, the cytodiagnosis was confirmed by histology of the excised tumor from a second lateral orbitotomy. Masson's trichrome stain confirmed the smooth muscle nature of the neoplasm and ultrastructural features were in favor of leiomyosarcoma. The patient received intermittent chemotherapy, and is clinically free from disease at the age of 17 yr.

Intraocular and orbital malignant schwannomas in F344 rats.

Intraocular and orbital malignant Schwannomas in two F344 rats are presented. The two Schwannomas were identified among approximately 60,000 male and 60,000 female F344 rats. The intraocular malignant Schwannoma occurred in the iris, invading the corneal stroma through the destroyed Descemet membrane. The malignant orbital Schwannoma occurred in the left orbit, invading the contralateral orbit along the optic nerve. Histologically, the intraocular Schwannoma consisted predominantly of a perivascular fascicular pattern of plump spindle cells associated with marked cytoplasmic vacuolization. The orbital Schwannoma consisted of Antoni type A and B pattern, but Antoni B tissues predominated. Antoni A tissues consisted of closely packed, elongated spindle cells arranged in interlacing fascicles, while Antoni B tissues were highly cellular and consisted of anaplastic, small cells associated with marked cyst formation. Immunohistochemically, the intraocular Schwannoma had a positive immunoreactivity for S-100 protein, while the orbital Schwannoma had a negative immunoreactivity. Ultrastructurally, the cells of both intraocular and orbital Schwannomas had long, thin cell processes and pericytoplasmic basal laminae. Particularly, the plump spindle cells of the intraocular Schwannoma were most strikingly characterized by the well developed, extremely attenuated cell processes arranged in a lamellar or spiral pattern. These cell processes and cell bodies were associated with numerous desmosomes. Intracytoplasmic filamentous granules and bodies, consisting of intermediate filaments approximately 7 nm in width, were additional characteristics of the plump spindle cells.

Orbital squamous cell carcinoma after retinal detachment surgery.

We report on a 76-year-old patient with a squamous cell carcinoma of the left orbit. The tumour had no connection with the conjunctiva but was located at the site of an encircling band which had been inserted 13 years before. A major part of the tumour presented as a well circumscribed solid mass within the extraocular tissues next to the inferior equator, but the exenteration specimen also showed tumour extension within the adjacent choroid. Histological examination showed a well differentiated keratinising squamous cell carcinoma with numerous mitotic figures and many epithelial pearls. A thorough examination in search of a primary carcinoma of the lacrimal gland or the sinus, with invasion into the orbit, or an epithelial neoplasm elsewhere suggestive of metastatic disease into the choroid did not reveal any specific pathological findings. Thus the most probable origin of the tumour seems to be epithelium which had been misplaced during retinal detachment surgery and had subsequently undergone malignant transformation.

Poorly differentiated primary orbital sarcoma (presumed malignant rhabdoid tumor). Radiologic and histopathologic correlation.

A 47-year-old man presented with acquired hyperopia and gaze-evoked visual loss. Computed tomography and magnetic resonance imaging demonstrated a well-circumscribed intraconal tumor. Histopathologic studies indicated that the tumor was a poorly differentiated primary orbital sarcoma compatible with malignant fibrous histiocytoma or malignant rhabdoid tumor. To our knowledge, no case of primary orbital malignant rhabdoid tumor has been previously described in an adult.

Juvenile fibromatosis of the orbit: a case report with review of the literature.

Proliferation of fibrous tissues to form a localised mass with infiltrating margins may occur anywhere in the body; involvement of the orbit is rare. Children and young adults are most commonly affected. The term 'juvenile fibromatosis' is one of several synonyms. Of the two main forms of this entity, the solitary form is the more common and the prognosis is good. The multicentric form has a poor prognosis. The patients described, a 2 1/2-year-old boy, had a solitary nodule of juvenile fibromatosis removed from the right orbit and was asymptomatic 2 3/4 years postoperatively.