Ennio Polito

Clinical and radiological presentation of 95 orbital lymphoid tumors.

Abstract• 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 in 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.

Endovascular treatment of a cavernous sinus dural arteriovenous fistula by transvenous embolisation through the superior ophthalmic vein via cannulation of a frontal vein

Abstract We describe a new approach for transvenous embolisation of cavernous sinus dural arteriovenous fistulae through the superior ophthalmic vein (SOV), i.e., via percutaneous cannulation of a frontal vein. Modern neurointerventional angiographic materials make it possible to reach the SOV in this way without puncturing it in the orbit or a surgical exposure. Orbital phlebography should still be in the repertoire of interventional neuroradiology units in large centres.

Orbital solitary fibrous tumor with aggressive behavior

AbstractBackground.Solitary fibrous tumor (SFT) is a rare spindle cell tumor that arises most often in the visceral pleura; however, a review of the literature shows at least 31 cases occurring in the orbit. Methods.A retrospective case series of three patients with orbital SFT: a 50-year-old man, observed in 1997, with an angioma-like lesion in the upper half of the orbit causing osteolysis of the orbital roof; a 24-year-old man, observed in 1992, with a superotemporal mass in the right orbit occupying the lacrimal gland region, firstly diagnosed as schwannoma, recurring 4 years after dacryoadenectomy; a 70-year-old man, with a retrobulbar mass diagnosed on a biopsy as hemangiopericytoma, recurring and infiltrating the orbital roof 4 years after surgery. Results.A review of the literature and presentation of three cases of SFT which showed infiltration of the orbital roof and/or recurrence. Conclusions.Our cases provide evidence of how orbital SFT can behave aggressively and mimic other orbital tumors, thus making mandatory the consideration of this relatively new entity in common clinical practice as well as careful follow-up. Their aggressive growth is unusual, described in only 6 of the 31 cases so far reported in the literature. Immunohistochemistry is of importance for the diagnosis, since CD34 immunoreactivity is peculiar to SFT.

Typical genomic imbalances in primary MALT lymphoma of the orbit

Primary orbital non‐Hodgkin lymphoma is a mucosa‐associated lymphoid tissue (MALT)‐type extranodal marginal zone lymphoma. Little information is available on its genome as conventional cytogenetics is limited by scarce biopsy material, while fluorescence in situ hybridization (FISH) explores only selected regions. Comparative genomic hybridization (CGH) performs full genomic analysis and is applicable to different sources of DNA, such as fresh and frozen cells, as well as paraffin‐embedded tissues. In this study, CGH was used to analyse primary MALT lymphoma of the orbit. Aneuploidy was identified in six of the ten cases studied. Gains (19) were more frequent than losses (5). The most frequent duplications involved chromosome 3 (common region at 3q24‐qter), as expected in marginal zone lymphoma, and chromosome 6 (common region at 6p21.1–21.3), which is typical of an orbital location. Other chromosome gains were found at 1p, 7, 8q, 9q, 12, 13, 17, 18, 19, 22, and X. Losses were located at 1q, 6q, 9q, 11q, and 13q. Two cases showed isolated duplications of chromosome 6p or 9q. Isolated imbalances were found only in tumours affecting the conjunctiva. Complex aneuploidies were observed in lymphoma of the retro‐orbital tissue. In summary, CGH in orbital MALT lymphoma provided new insights into typical genomic imbalances and underlying pathogenetic mechanisms. Copyright

MRI in Graves Orbitopathy: Recognition of Enlarged Muscles and Prediction of Steroid Response

The purpose of this study is to compare MRI to CT in the recognition of Graves orbitopathy and to compare MRI to clinical examination in the prediction of steroid response. Sixteen patients with dysthyroid orbitopathy (21 orbits) were examined by CT and MRI; muscle enlargement was measured by ultrasonography. Sensitivity in recognizing enlarged muscles was 85.4% for CT and 61.2% for MRI; CT recognized all affected orbits but 1, while MRI failed in 4 cases. Clinical inflammatory signs (p = 0.17) were more reliable predictors of steroid response than muscular T2 hyperintensity on MRI (p = 0.64). In a patient where histological examination documented edematous changes, MRI failed to reveal edema. MRI adds no morphologic information to CT; moreover, T2 intensity is less specific than clinical examination in documenting active disease and forecasting therapeutic outcome.

PROGNOSIS OF ORBITAL LYMPHOID HYPERPLASIA

Abstract• BackgroundOrbital 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.• MethodsThe 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.• ResultsNHL 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.• ConclusionBecause 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 Sjögren syndrome, an initially inflammatory lacrimal gland BLH, where a course of steroid is suggested before considering radiotherapy.

Imaging Techniques in the Diagnosis of Lacrimal Sac Diverticulum

Lacrimal sac diverticulum is a rare condition characterized by a cystic structure communicating with the sac. This abnormality may be directly demonstrated by dacryocystography only in a few cases; in the remaining cases, preoperative diagnosis may be arduous. We report 3 cases of lacrimal sac diverticulum. In all patients, symptoms were represented by a palpable mass in the lacrimal sac region, associated with permanent (case 1) or episodic (cases 2 and 3) epiphora. In the first case, CT-dacryocystography revealed an apparently solid mass causing inferior lacrimal obstruction, and ultrasonography disclosed a cystic space, not communicating with the sac. At surgery, a sac diverticulum was identified and excised. In the second patient, CT showed a homogeneous rounded mass, whereas a cystic character was revealed by T1-weighted MRI. Dacryocystography showed a lateral impression on the lateral wall of the sac. Ultrasonography revealed a cystic space communicating with the lacrimal sac. In the third case, ultrasonography demonstrated a diverticulum. CT is often unable to discriminate tumors from lacrimal cysts, both showing a parenchymal density. MRI can differentiate lacrimal cystic spaces from solid tumors by T1 intensity and by demonstration of their walls, but it is nonspecific for diverticula. Only dacryocystography and B-scan ultrasonography can reveal the narrow communication between the sac and the diverticulum. Observation is the recommended management for asymptomatic cases.

Inflammatory myofibroblastic tumor of the orbit.

The authors describe an unusual case of orbital inflammatory myofibroblastic tumor (IMT) in a 17-year-old patient who presented with a painful exophthalmos of the left eye. After complete surgical excision, the mass was diagnosed as an IMT based on morphological and immunohistochemical features. No tumor recurrence was evident during 28-month follow-up. The authors discuss histopathological and immunohistochemical characteristics and review the literature of orbital IMT.

Preoperative diagnosis of orbital cavernous hemangioma: a 99mTc-RBC SPECT study.

Purpose This study aimed to describe 99mTc-labeled RBC scintigraphy as a diagnostic method for orbital cavernous hemangiomas and to evaluate this diagnostic tool according to surgical outcomes. Methods Fifty-five patients with clinical and radiological (US, CT, and/or MRI) suspicion of unilateral cavernous hemangioma of the orbit underwent 99mTc-RBC SPECT study. Qualitative and semiquantitative evaluations were performed, and results were statistically analyzed. Results SPECT images showed focal uptake in the orbital mass in 36 of 55 patients. Nineteen patients had a negative scintigraphic pattern, with concordance of early and late absence of uptake of 99mTc-RBC. Our procedure showed 100% sensitivity and 88.9% specificity for the diagnosis of orbital cavernous hemangioma, with a positive predictive value of 90.9% and a negative predictive value of 100%. Conclusions 99mTc-RBC imaging is safe, easy to perform, and highly accurate in providing adequate clinical and surgical management. As a noninvasive and highly specific method for diagnosing orbital hemangioma, 99mTc-RBC scintigraphy can avoid more invasive imaging or biopsy.

In vivo confocal microscopy in a patient with conjunctival lymphoma

A case of primary conjunctival mucosa‐associated lymphoid tissue lymphoma studied by in vivo corneal confocal microscopy (IVCM) is described for the first time. Examination of the lower mediobulbar and forniceal conjunctiva of the left eye of a 37‐year‐old female patient showed a typical salmon‐pink patch. IVCM examination of the conjunctival lesion showed irregular, polygonal, conjunctival bulbar epithelial cells with blurred edges and without visible nucleus. Many small, roundish, hyper‐reflective cells were also seen. These cells were arranged diffusely or in nests in cyst‐like hypo‐reflective spaces. A few highly reflective cells were also visible among deep stromal collagen fibres. The IVCM picture recalled the histological profile of low‐grade mucosa‐associated lymphoid tissue lymphoma, characterized by sheets of neoplastic cells around reactive follicles. IVCM enabled non‐invasive evaluation of the eye surface at high magnification and with good contrast, and could be useful for early differential diagnosis of conjunctival lesions.