Cases reported "Orbital Neoplasms"

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1/170. Immunohistochemical examination of an orbital alveolar soft part sarcoma.

    BACKGROUND: A 32 year-old male patient had a 6-week history of left-sided proptosis. Computer tomography revealed a 16x15x15 mm smooth and well-defined mass between the optic nerve and the medial and superior rectus muscles in the left orbit. methods: The tumour was excised via a cranio-medial orbitotomy approach. RESULTS: Histopathological examination, immunohistochemistry and electron microscopy findings were consistent with an alveolar soft part sarcoma. Immunohistochemical staining showed positive immunoreactivity for neuronespecific enolase, vimentin, p53 (30%), p21 (10%) and cyclin d1 (20%), and negative immunoreactivity for CD45, cytokeratins, S-100 protein, glial fibrillary acidic protein, synaptophysin, chromogranin, calcitonin, serotonin, thyreoglobulin, desmin, myosin, actin, HMB-45, pRB, p16 and BCL-2. The growth fraction of the tumour cells was 3%. At examination 4 years after surgical excision, there was no evidence of local recurrence or for metastases. CONCLUSION: Alveolar soft part sarcoma of the orbit is a rare malignant tumour best controlled by surgery. The unpredictable behaviour of these neoplasms, however, indicates the need for long-term follow-up.
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2/170. Primary orbital Ewing's sarcoma: report of a case and review of the literature.

    Primary orbital Ewing's sarcoma is a very rare condition. Since the first case was reported in 1950, only 7 other cases have been reported in the English literature. Herein we describe the ninth, the only bilateral and the youngest case of primary orbital Ewing's sarcoma occurring in a 2-year-old boy, who presented to us with bilateral painless proptosis. Tissue biopsy of the tumour was obtained through the nasal sinus. Immunohistological studies of the biopsy tissue confirmed the diagnosis of Ewing's sarcoma. No distant site of the tumour was found so this was considered a primary orbital tumour. Combined chemotherapy and radiotherapy without surgical resection achieved an encouraging result in that the patient has remained in remission for 30 months after completion of treatment. The successful use of combined chemotherapy and radiotherapy, without surgery, adds further support to evidence that surgical excision may be avoided in selected cases of primary orbital Ewing's sarcoma.
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3/170. Congenital malignant rhabdoid tumor of the orbit.

    Malignant rhabdoid tumor is a rare and highly malignant renal tumor of infancy. Extrarenal tumors involving the orbit have been reported, but never at birth. The authors describe a primary malignant rhabdoid tumor of the orbit in a neonate who had massive unilateral proptosis at birth. Clinical, radiographic, and histologic features of the tumor are discussed.
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4/170. Recurrent proptotic diplopia due to congestive expansion of cavernous haemangioma with relapsing right-sided cardiac failure.

    A 75-year-old man with a recent history of pulmonary embolism, presented with collapse followed by a gran mal seizure and right-sided non-pulsatile proptosis. On recovery, he had diplopia on lateral and upward gaze and signs of congestive cardiac failure. Further pulmonary embolism was proven by lung scintigraphy. Computed tomography of his orbits confirmed a contrast-enhancing space-occupying lesion of the medial wall of the right orbit, with no intracranial abnormality. The patient was investigated for metastatic tumour as a possible cause of the space-occupying lesion and the unprovoked thromboembolic event, but no evidence of malignancy was found. The orbital lesion was not biopsied because of the risk of bleeding from anticoagulation. Three weeks later, the patient represented with recurrent cardiac failure, proptosis, and diplopia. A transorbital ultrasound confirmed an encapsulated, well-defined vascular lesion, with typical appearances and Doppler flow characteristics of a cavernous haemangioma. Diuretic therapy abolished the proptosis and diplopia in tandem with relief of the cardiac failure. This is the first description of recurrent proptosis with diplopia due to recurrent congestive expansion of an orbital cavernous haemangioma.
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5/170. Orbital rhabdomyosarcoma presenting as an apparent orbital subperiosteal abscess.

    We present a patient with acute sinusitis whose CT scan showed a ring-enhancing lesion within the orbit typical of an orbital subperiosteal abscess. On exploration of the orbit, there was no pus present but a tumour was found, which on histological examination was found to be a rhabdomyosarcoma. The clinical signs and CT characteristics of orbital subperiosteal abscesses and rhabdomyosarcomas are discussed. A ring-enhancing lesion within the orbit on CT scan should not be regarded as being pathognomonic for a subperiosteal abscess and the possibility of other diagnoses should be considered.
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6/170. Schwannoma of infratemporal fossa in a young child.

    Schwannomas in children are rare. A nine-year-old boy presented with swelling right side of face and proptosis for 4 months. CT scan showed a heterogenous mass in the right infratemporal fossa and extending to the posterior ethmoid. There was erosion of the bony walls of the infratemporal fossa. A biopsy through the sublabial approach was reported as schwannoma. The tumour was approached by Weber-Ferguson approach and excised in toto with complete relief of symptoms.
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7/170. 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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8/170. Solitary fibrous tumour of the orbit. Report of a new case.

    PURPOSE: Solitary fibrous tumour (SFT) of the orbit is a very rare lesion that may be misdiagnosed as fibrous histiocytoma, haemangiopericytoma, or other orbital tumour. We studied a case of SFT of the orbit in a 35-year-old woman. methods: The patient, with a 1-year history, reported a 'pressure' sensation behind her right eye, but no pain, vision changes, or other associated symptoms. Ocular examination was normal. magnetic resonance imaging (MRI) showed a well-circumscribed medial mass in the right orbit. The tumour was excised. RESULTS: The tumour was a cellular spindle-cell neoplasm with a storiform pattern. The tumour cells were spindle-shaped with bland nuclei and rare mitoses. Immunostaining was positive for vimentin and CD34, but negative for cytokeratin (AE1-AE3), EMA, desmin, smooth muscle actin, S-100, CD31, CD45 and bcl-2. CONCLUSION: SFT can infrequently involve the orbit. The tumour must be removed entirely to avoid recurrences. Careful and continued follow-up is important because orbital recurrence may occur several years after the excision of the primary tumour.
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9/170. Management of orbital lymphangioma using intralesional injection of OK-432.

    AIM: To treat orbital lymphangioma with an intralesional injection of OK-432 (group A streptococcus pyogenes of human origin). METHOD: A 14 year old boy had a right orbital cystic lymphangioma. The visual acuity in the eye was 20/28. In an initial treatment, 0.02 mg of OK-432, was injected into the tumour after aspiration of the fluid contents, but no effect was seen. The second treatment was performed with 0.04 mg of OK-432. RESULT: 4 months later, the lesion had totally shrunk to fibrous tissue. The side effects were fever, a local inflammatory reaction lasting 3 days, and increased intraocular pressure, which was managed by draining the fluid contents. visual acuity improved to 20/15, and the visual field defect and restriction of eye movement seen before treatment disappeared. No recurrence was noted 1 year after treatment. CONCLUSION: An intralesional injection of OK-432 shrunk the lymphangioma without functional disturbance and scar in the facial skin. OK-432 may be useful for orbital lymphangioma, but further studies are still warranted to determine efficacy, complications, and the optimal dose for safe treatment.
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10/170. Subtotal reconstruction of the orbit after destruction by benign tumour.

    We present five cases of reconstruction of the orbital walls after destruction by tumours. Treatment is based upon wide exposure through a bicoronal scalp incision and immediate reconstruction with autogenous iliac bone grafts. we do not recommend using foreign materials for reconstructing the orbit.
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