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PubMedDate not supplied

Embryonal rhabdomyosarcoma of orbit--a case report.

Embryonal rhabdomyosarcoma of orbit presenting as a case of rapid proptosis of the right eye is reported in a 4 year old male child. There was no evidence of recurrence during a follow up of 6 months. Rhabdomyosarcoma is one of the most common primary malignant orbital neoplasms of child hood. It usually produces a precipitously progressing unilateral proptosis of sudden onset. It is a highly malignant neoplasm of pleuripotential embryonic mesoderm, which commonly differentiates to form cells similar to rhabdomyoblasts of the foetus. Because of the presence of elongated cells that contain abundance of eosinophilic glycogen rich cytoplasm, it is generally referred to as embryonal form of rhabdomyosarcoma.

Child, Preschool↗
PubMed1987

[Pseudotumoral amyloidosis of the orbit].

A case of orbital amyloid pseudotumour is reported; this rare condition typically present as a ptosis followed by slowly growing orbital neoplasm, in middle-aged women. CT-scan disclose an extra-conal, extra-osseous, calcified mass; preoperative diagnosis of this rare condition might help in avoiding recurrence, probably due to incomplete excision. Pathophysiology of these amyloid deposits remain unknown.

Amyloidosis↗
PubMed1980

Palliative orbital decompression for metastatic melanoma to the orbit.

Although relatively rare, metastatic carcinomas to the orbit are the second most common orbital neoplasm in the adult. There are only ten cases reported in the literature of cutaneous melanoma metastatic to the orbit and, of these, only two had disease involving both orbits. This paper presents the third case of bilateral orbital melanoma; however, in this case, the primary site was the uveal tract in the first eye, with contralateral involvement being a metastasis of the orbital primary. The patient developed severe unilateral proptosis in his only seeing eye with progressive loss of vision. A palliative orbital decompression was performed which successfully restored visual acuity for an extended period of time. A brief discussion of the rationale for this procedure in this type of case is presented.

Ciliary Body↗
PubMed1999

Peripheral nerve tumors of the orbit.

Neurofibromas and schwannomas are orbital neoplasms with similar clinical and radiologic features. Solitary or circumscribed neurofibromas and schwannomas demonstrate slow progressive growth with ocular displacement and are amenable to surgical resection. Plexiform and diffuse neurofibromas are diffusely infiltrative in the orbit and periocular region, they are difficult to resect, and they have a high rate of recurrence. Malignant peripheral nerve tumors are rare, but they have a very high rate of recurrence and a low 5-year survival rate because of extension through the superior orbital fissure to the midbrain region. This article describes the clinical and radiologic features of peripheral nerve sheath tumors.

Disease Progression↗
PubMedDate not supplied

Canine lobular orbital adenoma: a report of 15 cases with distinctive features.

OBJECTIVE: To describe a unique orbital neoplasm in dogs, of lacrimal or salivary gland origin. ANIMALS STUDIED: Fifteen dogs with lesions consistent with a diagnosis of lobular adenomas involving the orbit were identified from the Comparative Ophthalmic Pathology Laboratory of Wisconsin from 1994 to 2001. RESULTS: The neoplasm occurred in nine females and six males. Affected dogs ranged in age from 7 to 17 years (mean = 9.7 years). Follow-up information was available for 13 of the 15 cases. The clinical presentation included swollen/hyperemic eyelids (4/15), third eyelid protrusion (3/15), conjunctival mass (6/15), exophthalmos (4/15), resistance to retropulsion (2/15), or strabismus (1/15). In 13 cases the masses were composed of nodular, friable tissue and they were solid in two cases. Histologically, the tissue was found in encapsulated lobules resembling well differentiated lacrimal or salivary glands but completely lacking ducts. Granular PAS-positive material was found within the cytoplasm. There was recurrence in 10 of the 13 cases available for follow-up. Of those cases in which enucleation or exenteration was performed (3/15), there was recurrence of disease in one case. In three cases the dogs were euthanized before recurrence at 3 months, 5 months and 3 years post surgery. None of the deaths was related to the tumor. CONCLUSION: In the 15 cases reviewed, lobular adenomas of the orbit presented clinically and histologically as a benign neoplasm of lacrimal or salivary gland origin. Recurrence was likely unless the mass was completely excised, at times requiring orbital exenteration.

Adenoma↗
PubMed1992

Epithelioid hemangioendothelioma of the orbital bones.

PURPOSE: The authors report a case of an epithelioid hemangioendothelioma arising in the orbital bones. A review of the literature related to this rare orbital neoplasm identified eight well-documented cases, one of which occurred in a patient younger than that reported here, but none of which originated in bone. METHODS: A 3 1/2-month-old boy had a left inferior orbital mass that had grown rapidly over a 3-day period. An emergency computed tomography scan showed a large neoplasm with significant bone destruction of the zygoma and maxilla. Initial examination suggested a rhabdomyosarcoma, and a transconjunctival biopsy was performed, which was complicated by significant blood loss. The final pathologic diagnosis was an epithelioid hemangioendothelioma, or grade 2 hemangioendothelioma, of bone origin. No other sites of disease were found on metastatic survey. Subsequent treatment consisted of an en bloc tumor resection sparing the orbital soft tissues and globe. RESULTS: The patient is free of disease and has normal visual fixation and ocular motility 20 months after surgery. CONCLUSION: Epithelioid hemangioendothelioma, a vascular malignancy of endothelial cell origin, very rarely involves the orbit. This case is notable for its early development, rapid growth, bony origin, and epithelioid histology.

Biopsy↗
PubMed1988

Management of paranasal sinus neoplasms invading the orbit.

Sinus tumors can readily invade the orbit through their shared walls. The initial symptoms of a sinus neoplasm are often due to orbital invasion. A comprehensive overview of the pathology, diagnosis, and treatment of these tumors is presented.

Adult↗
PubMedDate not supplied

CT characteristics of orbital pseudotumors and other orbital inflammatory processes.

Orbital pseudotumor is a nonspecific inflammatory process of unknown etiology that may mimic a true orbital neoplasm, specifically lymphoma. It exhibits a highly variable clinical and radiographic presentation. Thirty-nine patients with a presumptive diagnosis of orbital inflammatory disease were examined with CT and evaluated with respect to 13 findings associated with inflammatory disease of the orbit. Pseudotumor was included as part of the differential diagnosis in 25 patients. Twenty-one cases received a final (clinical) diagnosis of pseudotumor. The remainder were comprised of a broad range of orbital inflammatory conditions. Key radiographic features required to entertain or exclude the diagnosis of pseudotumor included establishing the presence of an orbital mass, extraocular muscle enlargement, bony erosion, enhancement, and associated paranasal sinus disease.

Adult↗
PubMed1979

Vascular problems of the orbit.

Vascular neoplasms and malformations of the orbit are unusual and fascinating problems. The benign capillary and cavernous hemangiomas are the most common vascular tumors and are classified separately because of distinct clinical and pathologic differences. The capillary hemangiomas progress rapidly in early childhood but have a tendency to regress. The cavernous hemangiomas occur later in life, progress slowly, and often require surgical excision. The orbital aneurysm, venous malformation, arteriovenous malformation, and carotid-cavernous fistula all may cause exophthalmos, visual loss, and other ocular abnormalities. They can be differentiated clinically; however, recent advances in ultrasonography, venography, and arteriography usually permit an exaxt diagnosis. They may be treated surgically, but the mortality and morbidity associated with such surgery must be balanced against the threat of loss of vision.

Adult↗