Cases reported "Orbital Neoplasms"

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1/27. 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.
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2/27. Extraorbital giant cell angiofibroma.

    A number of intriguing fibrovascular mesenchymal proliferations with benign or low grade malignant potential have recently been described. Giant cell angiofibroma was introduced as an entity by Dei Tos et al. in 1995 and initially considered to be a lesion of the orbit. We describe an extraorbital example, indicating that giant cell angiofibroma is not confined to the orbit. Immunologically, giant cell angiofibroma is positive for CD 34, bcl-2 and vimentin, and negative for epithelial and muscle markers, and S-100. The tumor shares several morphological and immunological properties with giant cell fibroblastoma and solitary fibrous tumor, yet it features a histology sufficiently characteristic to allow its categorization as a separate entity. The recommended treatment is complete but conservative excision. Metastases have not been reported.
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3/27. A giant intracranial mucocele associated with an orbitoethmoidal osteoma. Case report.

    The authors present a rare case of a giant intracranial mucocele associated with an orbitoethmoidal osteoma in a patient suffering from a generalized convulsive disorder. The broad pedicle of the osteoma had penetrated the cribriform plate and extended intracranially to form a nodular mass in the olfactory groove. The intracranial portion of the osteoma was surrounded by a mucocele. Both the cyst wall and multilayered intracystic septations of the mucocele were indented by layers of the osteoma. Although the extracranial portion adhered to the mucosa of the ethmoidal sinus, there were no signs of sinus obstruction. No direct communication other than the osteoma was identified between the mucocele and the ethmoidal mucosa. The large cerebral defect, which the mucocele occupied, communicated directly with the lateral ventricle without any intervening membranous structures. A frontal craniotomy is recommended for exposure of the lesion and plastic repair of the dural defect.
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4/27. Malignant changes in a giant orbital keratoacanthoma developing over 25 years.

    PURPOSE: To report a patient with a history over 25 years of a slowly growing, large, invasive crateriform tumour filling the anterior part of the orbit. methods: A 61-year-old male presented with a large tumour of the left orbit. Exenteration was performed with subsequent histological analysis of the excised mass. RESULTS: The main tumour showed the characteristic features of a keratoacanthoma. However, the posterior aspect of the tumour disclosed the morphology of a squamous cell carcinoma. Six months later, the patient presented with metastases to lymph nodes, lung and mediastinal tissue. A leukemoid reaction was diagnosed by fine needle biopsy. CONCLUSION: The giant variety of keratoacanthoma may fail to regress and can transform into a squamous cell carcinoma. In our patient, the development of a chronic lymphoid leukemia raises the possibility that it may be the underlying cause for the transformation of the posterior part of the keratoacanthoma into a frank squamous cell carcinoma.
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5/27. Orbital osteoclastoma of apparent extraskeletal origin in a pagetic patient: a case report.

    A large mass in the right orbit, causing proptosis, ptosis of the right upper eyelid, and limitation to eye movements, was surgically removed from a 51-year-old woman suffering from Paget's bone disease (PBD). Histologically, a giant cell tumor of the bone (osteoclastoma) was diagnosed. No bony involvement was apparent either operatively, microscopically, or on preoperative computed tomographic scans. The neoplasm has not recurred in a 3-year follow-up. In addition to the fact that osteoclastoma complicating PBD is rare, the extraskeletal origin of the tumor is a matter of interest and can be tentatively explained by an unusually powerful systemic stimulus acting on circulating osteoclast precursors. HUM PATHOL 31:1527-1531.
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6/27. Orbital intradiploic giant epidermoid cyst.

    We report a case of an intradiploic epidermoid cyst of the lateral orbital wall that resulted in proptosis and downward displacement of the left eye. Computed tomography and magnetic resonance imaging revealed a bone-destroying mass involving the frontal, sphenoid, and zygomatic bones that extended into the orbit, temporalis, and anterior cranial fossa. Complete surgical removal was performed through a lateral orbitotomy. The lateral wall was reconstructed with a porous polyethylene sheet. The cyst has not recurred after 18 months of follow-up.
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7/27. Dura and cranial base reconstruction by external oblique fascia and rectus abdominis muscle flap.

    The rectus abdominis muscle flap, combined with the external oblique fascia, were utilized in cranial-base reconstruction. In a wide defect caused by resection of a giant meningioma, the dura was reconstructed with the external oblique fascia and anterior sheath, and the cranial-base defect was reconstructed with the rectus abdominis muscle flap.
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8/27. Orbital lymphoma with concomitant sarcoid-like granulomas.

    A 41-year-old man presented with chronic eyelid swelling, conjunctival injection, and decreased ocular motility in all gaze directions. MRI showed bilateral enlarged extraocular muscles, including the tendons. Laboratory tests revealed elevated levels of angiotensin-converting enzyme. An orbital biopsy showed collections of monotonous small lymphocytes, and granulomatous inflammation that included multinucleated giant cells, predominantly Langhans type. Flow cytometric analysis of tissue demonstrated a light chain-restricted clonal population of B cells, a finding that confirmed the morphologic impression of lymphoma. This case demonstrates that elevated angiotensin-converting enzyme and granulomatous inflammation can occur in lymphoma. Careful histopathologic examination and flow cytometric analysis are essential to avoid an erroneous diagnosis that could lead to inappropriate management.
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9/27. Giant cell reparative granuloma of the orbit.

    This report describes a case of an orbital giant cell reparative granuloma in an 85-year-old woman. Giant cell reparative granulomas are fibro-osseous benign proliferations typically found in the jawbones and rarely in the orbital bones. All previously described cases in the orbit have been in younger patients, classically in the third to fourth decades.
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10/27. Orbital giant cell angiofibroma: immuno-histochemistry and differential diagnosis.

    CASE REPORT: A 57-year-old woman presented with a 16-month history of a slowly growing mass in the medial aspect of her right lower eyelid. On radiologic examination, the mass was seen to extend into the anterior orbit. Initial attempt at excision was complicated by problems with hemostasis and only a portion was removed. Histopathologic examination revealed a giant cell angiofibroma, which was subsequently excised. COMMENTS: Giant cell angiofibroma is a benign spindle cell neoplasm characterized by multinucleated giant cells and pseudovascular spaces with a propensity to occur in the anterior soft tissues of the orbit of middle-aged adults. It is part of a wide differential diagnosis of tumours that can be separated by various histologic and immunohistochemical features, which are discussed in the report. It is important to consider it preoperatively because the vascular nature of this tumour may create problems with hemostasis during excision.
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