Cases reported "Orbital Neoplasms"

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1/15. Primary orbital leiomyoma and leiomyosarcoma.

    A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.
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keywords = enophthalmos
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2/15. strabismus surgery complicated by "pulled in two syndrome" in a case of breast carcinoma metastatic to the medial rectus muscle.

    Metastatic carcinoma to the extraocular muscles is extremely rare; it is reported to occur from breast, lung, and gastric carcinoma as well as skin melanoma. (1-3) overall, intraocular metastases occur much more frequently than orbital metastases.(4) The most common primary tumors causing orbital metastases are breast and lung carcinomas.(5) strabismus due to orbital metastases from breast carcinoma usually results from fibrosis of the muscle, which often causes painful ophthalmoplegia and enophthalmos. (6,7) We report a case of presumed metastatic carcinoma to the medial rectus muscle causing restrictive strabismus in which surgery was complicated by the "pulled in two syndrome," or PITS.
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keywords = enophthalmos
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3/15. Fractionated, three-dimensional, planning-assisted proton-radiation therapy for orbital rhabdomyosarcoma: a novel technique.

    PURPOSE: Most children with orbital rhabdomyosarcoma will survive their disease. However, conventional photon-radiation treatment, as part of multimodality therapy, results in varying degrees of long-term functional and cosmetic side effects. This report introduces external beam proton radiation therapy (PRT) as a conformal, three-dimensional planned radiation technique for this disease, analyzes normal tissue dosimetry, and describes the technique's application in the first 2 patients. MATERIAL AND methods: Between January 1995 and February 1996, 2 patients underwent PRT following biopsy and chemotherapy for orbital rhabdomyosarcoma. Fifty and 55 cobalt Gray Equivalent (CGE) were delivered to the gross tumor volume and 40 CGE to clinical target volumes in both patients. A relative biologic effectiveness (RBE) of 1.1 was utilized to correlate proton dose calculations with CGE. To achieve dose conformity, a "patch technique" was utilized, where target regions were divided into segments, each treated by a separate proton field. Dose-volume histograms were obtained for target and nontarget regions, including lens, bony orbit, pituitary gland, optic chiasm, optic nerves, lacrimal gland, and ipsilateral frontal and temporal lobes. RESULTS: At 3.4 and 2.5 years after PRT, both patients are clinically and radiographically free of disease. visual acuity remains excellent, without signs of cataract formation; pituitary function is normal; cosmetically, only mild enophthalmos is noticeable. Doses to 90%, 50%, and 5% of lens volume were kept at less than 1%, less than 2%, and less than 8%, respectively. Fifty percent of lacrimal gland volume received less than 36% of the prescribed dose and 50% of the volume of the optic chiasm, pituitary gland, and hypothalamus were restricted to less than 2%. Proton conformity to orbital contents resulted in between 9% and 36% of the prescribed dose reaching the ipsilateral temporal and frontal lobes immediately adjacent to bony orbit (5% volume). CONCLUSION: PRT can offer excellent sparing of lens and selected intraorbital and ocular normal structures, while maintaining conformal target-dose coverage. The steep dose gradient beyond the orbit minimizes irradiation of normal brain parenchyma, with almost complete sparing of the pituitary gland. Reduction of integral irradiation exposure of the periorbital region will, hopefully, reduce the risk of second malignancy later in life. Reduced radiation dose to specific organs in close proximity to, but not part of the target region promises improved functional outcome and better cosmesis for childhood cancer survivors.
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ranking = 1
keywords = enophthalmos
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4/15. Intermittent visual loss and exophthalmos due to the Blue rubber bleb nevus syndrome.

    PURPOSE: To describe the occurrence of intermittent visual loss in a patient with orbital hemangioma due to the blue rubber bleb nevus syndrome. methods: Case report. RESULTS: A 70-year-old woman complained of intermittent blurred vision combined with pressure behind her left eye occurring after head bending or Valsalva maneuvers. Examination revealed 2 mm of enophthalmos on the left. After downward bending of the head the eye became exophthalmic by 12 mm and visual acuity decreased to finger counting. Neuro-ophthalmologic examination was otherwise normal. magnetic resonance imaging revealed an intraconal hemangioma. Bluish, soft, rubber-like, compressible and slowly refilling hemangiomas on the inferior lip, sublingually, on the hand, and vagina led to the diagnosis of BRBNS. CONCLUSIONS: Intermittent visual loss may be the presenting symptom of an orbital hemangioma in BRBNS. Recognition of this rare condition is important because of its potentially life-threatening complications.
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ranking = 1
keywords = enophthalmos
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5/15. enophthalmos associated with primary breast carcinoma.

    BACKGROUND AND OBJECTIVE: The most common source of metastases to the orbit is from breast carcinoma. The orbital presentation can be the presenting sign of the cancer. Proptosis or exophthalmos is the more usual way metastases present, associated with ocular motility problems or diplopia. We present a case of enophthalmos associated with breast carcinoma whereby there is as yet no radiological evidence of an actual orbital metastatic lesion. patients AND methods: A 51-year-old Caucasian woman with a known history of breast cancer presented with a 10-month history of vertical diplopia, particularly on upgaze. She noted that her left eye was becoming more "sunken" and examination confirmed an 8-mm enophthalmos on that side with restricted vertical eye movements and abduction. RESULTS: A provisional diagnosis of metastatic breast cancer was made. CT and MRI scans were performed. The main feature noted was that of orbital fat atrophy with no evidence of an orbital mass. She has been followed up for a period of 2(1/2) years with 6-monthly scans. There has been no progression of her clinical signs and no orbital lesion found so far. We are continuing to review her. CONCLUSION: Although no actual orbital mass has been found yet, we are treating her case as enophthalmos associated with scirrhosing breast carcinoma; she may, however, have spontaneous orbital fat atrophy, triggered by the cancer.
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keywords = enophthalmos
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6/15. Downgaze "hang-up" of the upper eyelid in patients with adult-onset ptosis: an important sign of possible orbital malignancy.

    PURPOSE: To highlight, in patients with adult-onset ptosis, the importance of upper eyelid hang-up during downgaze as a clinical sign of serious orbital disease. DESIGN: Retrospective, observational case series. patients AND methods: Retrospective review of the presenting clinical symptoms and signs for a series of seven patients, some referred for treatment of adult-onset ptosis, with hang-up of the upper eyelid on down-gaze. RESULTS: All patients had symptoms of upper eyelid ptosis at presentation, starting at ages ranging from 29 to 81 years (mean, 64 years; median, 70 years). All patients displayed marked hang-up of the affected upper lid on downgaze, and the levator excursion was reduced (range, 2-13 mm; mean, 9 mm) compared with the asymptomatic side. All patients with downgaze hang-up had associated orbital malignancy, but they otherwise had only minimal or no underlying orbital disease. Exophthalmometry varied between subtle (1 mm) relative enophthalmos in two cases, to mild relative proptosis (3/7 cases; range, 2-3 mm). Ipsilateral up gaze was reduced in 4 of 7 patients (57%). CONCLUSION: In contrast to patients with adult-onset blepharoptosis (resulting from dehiscence of the levator muscle aponeurosis), adults presenting with ptosis resulting from malignant infiltration of the upper orbital tissues may display hang-up of the upper eyelid in downgaze. This important sign should alert the clinician to the possibility of serious underlying orbital disease.
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ranking = 1
keywords = enophthalmos
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7/15. Orbital metastasis as the initial finding of breast carcinoma: a ten-year survival.

    A 50-year-old woman with a clinically negative breast examination was diagnosed with metastatic breast cancer only after pathologic evidence of an orbital metastasis. She presented with pressure and pain above the right eye associated with enophthalmos and pseudo-Brown syndrome. The diagnosis of breast cancer based on initial ophthalmic findings requires a strong level of suspicion. Multiple reports show that survival in these cases is limited, usually only 1 year after diagnosis. We present a case of 10-year survival after the diagnosis of breast cancer presenting as an orbital mass. These new findings provoke us to continue aggressive systemic and ophthalmic treatment for such patients.
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ranking = 1
keywords = enophthalmos
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8/15. Congenital unilateral ocular fibrosis syndrome secondary to benign congenital tumor.

    Congenital unilateral fibrosis, blepharoptosis and enophthalmos syndrome is one of the rare forms of congenital fibrosis of extraocular muscles. The nature and cause of such unilateral inflammation leading to fibrosis is unknown. Prenatal orbital penetration has been proposed as a cause of this syndrome. We report a case of this rare syndrome associated with an adjacent sinus tumor.
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ranking = 1
keywords = enophthalmos
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9/15. A surgical treatment of severe late posttraumatic enophthalmos using sliced costal cartilage chip grafts.

    The efficacy of sliced costal cartilage chip grafts for the treatment of late posttraumatic enophthalmos was investigated. Surgery was conducted based on the method reported by Matsuo et al. in 1989. After making an incision in the lower eyelid, dissecting the subperiosteum of the medial orbital wall, orbital floor and lateral orbital wall was performed to the posterior of the orbit, and then costal cartilage chips were gradually grafted in a step-like configuration to the subperiosteum from a location posterior to the equatorial plane of the eyeball. At this time, as well as to the area of concave depression in the orbital bone caused by the fracture, grafts were made to the subperiosteum of the non-deformed medial and lateral orbital wall, to move all of the orbital tissue, including the eyeball, forward. This was performed for five cases of severe late posttraumatic enophthalmos. Among the five cases, there were four cases of severe orbital fracture and one case for which malignant orbital tumor extirpation and radiation therapy had been performed. Following surgery, although mild enophthalmos remained in three of the five cases, esthetically satisfactory results were obtained for all cases. Costal cartilage chip grafts were shown to be an effective method for the treatment of late posttraumatic enophthalmos.
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ranking = 8
keywords = enophthalmos
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10/15. breast carcinoma metastatic to the orbit.

    breast carcinoma metastatic to the orbit presented in four patients as a diffuse mass lesion. Clinical findings included restricted ocular motility, palpable mass, enophthalmos, and ptosis. The delay in diagnosis in three cases was due to subtle early findings, the long time interval from the primary breast lesion, lack of other metastases, and, in each of the four cases, the patient's reluctance to disclose any history of breast disease. Pathologic examination of the orbital breast metastases revealed two types: an adenocarcinomatous pattern with nests of pleomorphic malignant appearing cells and a histiocytoid variant with bland, large cells similar to histiocytes.
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ranking = 1
keywords = enophthalmos
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