Mucoepidermoid carcinoma of the conjunctiva with lung metastasis

A 36-year-old lady presented with redness and decreased vision in right eye since 6 months. She was earlier diagnosed of cavitary lung lesion, presumed secondary to tuberculosis and treated with anti-tubercular treatment for 4 months. Examination of affected right eye revealed nil light perception, conjunctival congestion with an exuberant mass in the inferotemporal bulbar conjunctiva, proptosis, iris neovascularization, 360° closed angles, intraocular pressure of 48 mm Hg, exudative retinal detachment, uveal mass and orbital extension. A diagnostic needle biopsy of uveal mass revealed malignant cells. Computed tomography-guided lung biopsy revealed squamous cell carcinoma (SCC), indicating metastatic spread from the orbit. She underwent lid-sparing exenteration of the right eye. Histopathological examination of the orbital tissue revealed mucoepidermoid carcinoma arising from the conjunctiva with extensive invasion into the orbital tissue, muscle fibers, sclera, choroid and optic nerve. Multiple tumor emboli were seen in the lumen of orbital blood vessels. In conclusion, mucoepidermoid carcinoma of the conjunctiva is a rare, aggressive variant of SCC. Early intervention is essential to prevent intraocular invasion and systemic metastasis.     

Eosinophilic granuloma of the orbit with spontaneous healing

A 13-year-old boy presented with a 2-month history of swelling of the right upper eyelid and displacement of the right eye. Computed tomography (CT) showed a tumor in the right orbit and a large osteolytic defect of the orbital frontal bone. At surgery a yellowish-tan mass was found replacing the orbital roof and abutting the dura. Incisional biopsy established the diagnosis of eosinophilic granuloma. In the absence of further therapy, the mass resolved, and the bony defect completely reossified within 14 months. During more than 2 years of follow-up, there has been no evidence of local recurrence or systemic disease. This case demonstrates that eosinophilic granuloma may resolve spontaneously with healing of bony defects, thus eliminating the need for adjunctive resection or radiation therapy in selected patients.     

Orbital myxoma comorbid with acute myelomonocytic leukemia

We report the first case of orbital myxoma in a 10-year-old girl with a history of acute myelomonocytic leukemia diagnosed at the age of 10 months. She presented with a mass in the right orbit, which was excised completely. There was no recurrence during the 6 months of follow-up.     

Solitary fibrous tumor of the orbit presenting in pregnancy

A 32-year-old woman, three months pregnant, reported with the complaint of protrusion of the right eye for six months. She gave history of rapid protrusion of eyeball for the last two months along with the history of double vision for the last one month. Computer tomography (CT) scan revealed a well-defined mass lesion in the intraconal space of the right orbit which was excised through a lateral orbitotomy approach. Histological examination and immunohistochemistry revealed a solitary fibrous tumor, which showed a rapid progression in pregnancy.     

A case of uveal, palpebral, and orbital invasions in adult T-Cell leukemia

BACKGROUND: Patients with adult T-cell leukemia (ATL) may have eyelid lymphoma, uveitis, or cytomegalovirus retinitis due to being immunocompromised. However, there have been few reports on the invasion of multiple ocular lesions. We treated 1 unusual ATL patient with uveitis in whom multiple ocular invasions were suspected. CASE: A woman in whom ATL was diagnosed 10 years previously complained of blurred vision and decreased visual acuity in the right eye. Anterior uveitis of the right eye was suspected. One week later the cells increased in the anterior chamber, and fibrin exudates and hyphema appeared. She was admitted to our hospital. OBSERVATIONS: The visual acuity was 0.04 in the right eye and finger-counting from 30 cm in the left. She was treated with systemic steroid therapy. Inflammation disappeared, but both eyelids became swollen and multiple ocular lesions appeared. She was given carcinostatic therapy once more and the mass lesions decreased. Mass lesions appeared in the iris and in the bulbar conjunctiva. Computed tomography and magnetic resonance imagining (MRI) showed that the mass lesions extended to the right orbit and both nasal cavities. MRI also demonstrated choroidal thickening in the left eye. CONCLUSION: This case documents that ATL cells may cause severe uveitis and invade multiple ocular tissues such as the iris, eyelid, choroids, and orbit.     

Orbital Rosai-Dorfman disease with subperiosteal bone involvement mimicking eosinophilic granuloma

The presentation of RDD as an anterior subperiosteal orbital mass with bone involvement has, to the authors' knowledge, not been previously reported. We describe a case of Rosai-Dorfman disease (RDD) presenting as an anterior superolateral subperiosteal orbital mass with erosion of overlying bone mimicking eosinophilic granuloma. It was debulked using endoscopic-guided curettage and the patient was given both local and systemic corticosteroids. Careful histological analysis revealed the diagnosis of RDD and the patient remains asymptomatic and recurrence free at 16 months follow-up. Involvement of the pituitary gland, a recognized yet unusual finding in this condition was also noted. RDD should be considered in the differential diagnosis of a soft tissue mass of the superolateral orbit.     

Orbital tuberculosis manifesting with enophthalmos and causing a diagnostic dilemma

A 60-year-old woman with no known systemic disease was referred with a hard mass in the left orbit and enophthalmos of two months duration. Differential diagnoses of metastasis from an undetected scirrhous carcinoma and sclerosing nonspecific orbital inflammatory disease were considered and a biopsy was performed. Histopathology demonstrated granulomatous inflammation with fibrosis. Subsequent polymerase chain reaction was positive for Mycobacterium tuberculosis deoxyribonucleic acid. There was no evidence of systemic tuberculosis. The patient was treated with four-drug combination anti-tubercular therapy for 6 months. The mass regressed and there was no local recurrence at two years follow-up. Orbital tuberculosis generally manifests with proptosis or osteomyelitis. However, enophthalmos may be caused by the fibrosing variant. Biopsy with histopathologic and microbioloic evaluation is essential to distinguish it from other more common causes of an orbital mass with paradoxical enophthalmos such as metastatic scirrhous carcinoma and sclerosing nonspecific orbital inflammatory disease.     

Treatment of recurrent eosinophilic granuloma with systemic therapy

PURPOSE: To describe the systemic treatment of recurrent eosinophilic granuloma. METHODS: Case report and review of Langerhans cell histiocytosis. RESULTS: A 9-year-old boy had painful, right upper eyelid swelling. A computed tomography scan showed a right superolateral orbital mass with evidence of bony erosion. Frozen section evaluation was consistent with eosinophilic granuloma. Biopsy and curettage were performed. Systemic work-up was negative for extraorbital disease. Recurrence of the orbital mass 6 weeks after surgery was confirmed by magnetic resonance imaging (MRI). Four weeks after treatment with prednisone and vinblastine, MRI showed a marked decrease in the size of the orbital lesion. Additional treatment with 6-mercaptopurine and methotrexate was initiated, and MRI 6 months later showed complete resolution of the lesion. CONCLUSIONS: The treatment of recurrent eosinophilic granuloma is challenging. The goal is to effectively cure the lesion with minimal short-term and long-term side effects. Systemic steroids, vinca alkaloids, antimetabolites, and antifols are preferred because they have less toxicity and mutagenicity. Combination chemotherapy used in conjunction with treatment by a pediatric hematologist/oncologist appears to be an effective method of treating recurrent disease.     

Coup de sabre presenting with worsening diplopia and enophthalmos

A middle-aged woman with a diagnosis of linear scleroderma and systemic sclerosis presented with an atrophic skin lesion of the forehead extending in the right orbit and progressive diplopia in the right gaze. On exam, she had enophthalmos with a small, manifest esotropia in the right gaze. Orbital MRI revealed fat atrophy of the right upper eyelid and orbit not previously described. Inflammation and progressive collagen fibrosis involving subcutaneous tissue, fat, and muscle secondary to linear scleroderma may result in enophthalmos and diplopia.     

Infratemporal inflammatory myofibroblastic tumour with orbital extension

Inflammatory myofibroblastic tumour (IMT) is a rare distinctive neoplasm of intermediate biological potential with a predilection for the abdominopelvic region and lung of children and young adults. It is histologically composed of spindle cells (myofibroblasts) in a myxoid to collagenous stroma with a prominent inflammatory infiltrate composed primarily of plasma cells and lymphocytes. Its pathogenesis is controversial. Arising most commonly in the lungs, only two cases of histopathologically confirmed IMT originating from the infratemporal and pterygopalatine fossae have been documented in the literature. Neither had orbital involvement. We now report the first case of IMT originating from the infratemporal fossa with orbital extension in a previously well 31‐year‐old woman. The patient presented with a 5‐month history of intermittent right‐sided headaches, progressive trismus and right lower lid swelling. She then developed right proptosis, diplopia and paraesthesia in the ophthalmic and maxillary divisions of her trigeminal nerve. Magnetic resonance imaging showed a soft tissue mass occupying most of the right infratemporal fossa with extension into pterygopalatine fossa and orbit. Provisional diagnosis from an open biopsy was nodular fasciitis. She underwent surgical debulking of the infratemporal fossa and lateral orbit through a coronal and trans‐oral approach with trans‐zygomatic access via total zygomatic osteotomy. Review of intraoperative specimens revised the diagnosis to IMT. Further management included systemic corticosteroids and adjuvant radiotherapy.