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Management of orbital primary malignancies
This paper reviews current concepts in the management of primary nonlymphoid orbital malignancies, reexamined on the basis of a large personal series (54 cases). Therapeutic control of orbital primary nonlymphoid malignancies can be achieved only in a limited number of cases. We advise the complete removal of all suspected epithelial tumors of the lacrimal gland; if a malignancy is not completely removed, the choice is between tumor excision and exenteration
Epithelial malignancies of the lacrimal gland: survival rates after extensive and conservative therapy
We reviewed 96 cases of lacrimal gland tumors, 20 patients had epithelial malignancies (11 adenoid cystic carcinomas, 7 carcinomas in pleiomorphic adenomas, 1 mucoepidermoid tumor, 1 adenocarcinoma, and 1 carcinoma in an epidermoid cyst). Classic computed tomographic scan changes associated with lacrimal gland malignancies were found in only 55% of cases. The median survival was 6.75 years in the seven patients treated with extensive surgery (orbital exenteration, possibly associated with osseous resections) and nine years in the 12 cases treated with eye-saving procedures (tumor excision, possibly followed by radiotherapy). Our data demonstrated that extensive surgery for lacrimal gland malignancies does not improve survival. Therefore, a therapeutic protocol is suggested, in which all suspected epithelial tumors (discriminated from inflammatory and lymphoid lesions by computed tomographic scan findings) undergo dacryoadenectomy, without a previous biopsy. Subsequently, in the case of carcinomas, the choice between extensive and eye-saving procedures is based on the extent and aggressiveness of the tumor and clinical findings
Prognosis of orbital lymphoid hyperplasia
Background: Orbital lymphoid hyperplasia can be associated with systemic non-Hodgkin lymphoma (NHL), even when polyclonal proliferation is found in the orbit. Although irradiation is recommended, some orbital lymphoid hyperplasias are treated by steroids (when inflammation is clinically presumed) or left untreated. Methods: The incidence of concurrent NHL and the incidence of future NHL after oral prednisone, radiotherapy, or no treatment were retrospectively evaluated in 33 cases of lymphoid hyperplasia (22 benign lymphoid hyperplasias, BLH, and 11 atypical lymphoid hyperplasias, ALH), after follow-up of 2-13 years. Results: NHL occurred in 12 of 33 cases (36.4%). In seven patients it was concurrent; in five patients it occurred 2-6 years later. In the actuarial curve, at 5 years 55% of patients were free of lymphoma, at 10 years, 46%. NHL was more commonly observed when the lacrimal gland was involved (57% vs 21%; P=0.03). Of the 13 patients treated with oral steroids, 46% had complete response, 39% partial response, and 15% future NHL. Of the seven irradiated patients, five (71%) had complete response, two (29%) partial response, and none future NHL. Of the eight untreated patients, five (63%) had partial response and three (37%) future NHL. Irradiated lacrimal gland BLHs only achieved partial response, one having radiation-induced orbital inflammation. Conclusion: Because of a high risk of NHL, in all orbital lymphoid tumors systemic staging and follow-up are mandatory. The advised management is irradiation, except for Sjogren syndrome, an initially inflammatory lacrimal gland BLH, where a course of steroid is suggested before considering radiotherapy
CT & MRI reliability in the diagnosis of lacrimal fossa tumors
This prospective study sought to verify whether computed tomography (CT) and magnetic resonance imaging (MRI) can be used as reliable means for planning a therapeutic approach to lacrimal fossa tumors. Twenty-six cases of lacrimal of a gland mass at first observation were studied. After clinical and radiologic evaluation, the most likely diagnosis was recorded for each patient before treatment. The conclusive diagnosis was based on a histologic examination or (only in case of acute pseudotumor) on a prompt and complete response to steroids. All lymphomas (seven cases) and pseudotumors (four cases) were correctly identified. Two cases of benign lymphoid hyperplasia were suspected to be pseudotumors. All epithelial tumors (six cases) were recognized, except for a pseudocystic necrotic carcinoma, which was suspected to be a dermoid. All dermoids (five cases) were identified. An intraosseous hemangioma was suspected to be an aneurysmal bone cyst, and a cholesterol granuloma was suspected to be a dermoid. CT and MRI, combined with clinical recognition of inflammatory signs, can provide sufficient information to guide the therapeutic approach to lacrimal fossa tumors. Identification of rare tumors and the differentiation of carcinomas from adenomas may result. General guidelines for treatment include: Excisional biopsy for rounded tumors with smooth margins (including cystic lesions) A steroid trial for recent wedge-shaped tumors Incisional biopsy for longstanding or steroid-resistant wedge-shaped tumors
Anatomia dell'orbita
Il capitolo consiste in un breve outline dell'anatomia dell'orbita propedeutico per i successivi capitoli del volum
Bilateral optic nerve compression caused by lymphomatous infiltration of all extraocular muscles
Orbital lymphoid tumors may arise within one or more extraocular muscles, although multiple and bilateral presentation is rare. Further more, bilateral optic nerve compression is an exceptional finding in orbital lymphoma. A 28-year-old man experienced bilateral proptosis and loss of vision, He had suffered from a stage IV non-Hodgkin lymphoma, not involving the orbit, which had been quiescent for 3 years. Ocular motility was impaired in all directions, Bilateral optic disc swelling was apparent. Orbital MRI revealed bilateral enlargement of all extraocular muscles and of lacrimal glands, causing apical optic nerve compression. Incisional biopsy confirmed the diagnosis of orbital small-cell lymphoma, Combination chemotherapy and oral prednisone administration resulted in a complete regression of orbital masses, Bilateral lymphomatous infiltration of all extraocular muscles with papilledema requires a differential diagnosis with other causes of bilateral papilledema and of extraocular muscle enlargement. A previous history of lymphoma, associated lacrimal gland involvement, and clinical features may help the diagnosis, Biopsy is required both for the differential diagnosis and for lymphoma grading
Clinical and radiological presentation of 95 orbital lymphoid tumors
Background: Although most orbital lymphoid tumors are characterized by a slow, painless onset and a mass that molds to orbital structures, different presentations may occur. Intensity on T2-weighted MRI is a possible means for differentiating lymphoid tumors (hyperintense) from pseudotumors (hypointense). In addition, it is generally assumed that 75% of orbital lymphomas are at stage I on presentation. Methods: The clinical, CT, and MRI presentation of 95 primary and secondary orbital lymphoid tumors (63 non-Hodgkin lymphomas and 32 lymphoid hyperplasias) and their histological grade and staging were reviewed. Immunohistochemistry and PCR were used to determine clonality. Results: No significant differences were found between non-Hodgkin lymphomas and lymphoid hyperplasias, except for the mean age of the patients (6.3 years older in non-Hodgkin lymphomas). In both groups the most common presentation was a mass with an indolent course; visual impairment was seen in 13%, conjunctival redness in 25%, pain in 12%, and acute orbital inflammation in 15% of the patients. In most cases, the CT showed one or more lobulated or rounded masses, molding to adjacent structures, or a wedge-shaped enlargement of the lacrimal gland. Intramuscular lymphomas were rare and always associated with extramuscular masses. On T2-weighted MRI, only 35% of lymphoid tumors were hyperintense. Only 49% of lymphomas were at stage I on presentation. Conclusion: An inflammatory presentation is not uncommon orbital lymphoid tumors. Shape, molding and multiple masses can help radiological diagnosis, whereas MRI T2 intensity is unreliable. Accurate staging can disclose systemic disease in more than 50% of cases of non-Hodgkin lymphoma
Optic-nerve Sheath Meningiomas - Clinical-features and Functional Prognosis
Seventeen cases of optic nerve sheath meningiomas were reviewed, in order to identify their clinical presentation, CT and MRI features, and to evaluate their visual prognosis in untreated cases and after surgery. The most common clinical features were represented by optic disk pallor and/or edema (16 cases), unilaterally reduced visual acuity (14 cases), afferent pupillary defect (12 cases), visual field alterations (nine cases out of 12), proptosis (12 cases), motility disturbances (eight cases), amaurosis fugax (five cases). On CT and MRI, eight tumors appeared fusiform, four excrescent, four tubular, one massive. The optic nerve could be recognized within the tumor in nine cases out of 16 on CT, and in eight out of nine on MRI. Contrast enhancement was observed in most cases. On T-2-weighted MRI, meningiomas proved to be hyperintense in four cases and isointense to fat in the remaining five. A histological diagnosis was obtained in eight cases: seven belonged to the meningotheliomatous type; the remaining to a transitional type. The visual prognosis in untreated patients was extremely variable and unpredictable on the basis of clinical, radiological or epidemiological factors. Of the six cases who had undergone neurosurgery, none experienced a visual improvement; two cases suffered a sudden visual deterioration. Optic nerve sheath meningiomas should be suspected when unilateral visual impairment is associated with optic disk alterations and proptosis. Contrast-CT is the first advised diagnostic step, followed by contrast-MRI if the diagnosis should be unclear
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