Diego Strianese

Epithelial lacrimal gland tumors: pathologic classification and current understanding.

OBJECTIVE To apply the updated epithelial salivary gland classification scheme to a large cohort of lacrimal gland tumors so as to provide an updated lacrimal gland tumor classification scheme. METHODS A retrospective multicenter cohort study of 118 cases of epithelial neoplasia was undertaken. Main outcome measures included pathologic analysis, subtyping, and survival. RESULTS Of 118 cases, 17 (14%) were reclassified using the proposed expanded classification scheme based on the current World Health Organization classification of salivary gland tumors. The most frequent neoplasms were pleomorphic adenoma and adenoid cystic carcinoma, of which we highlight more unusual histologic features. Three tumors were found to be unclassifiable with the updated scheme, with 2 having histologically malignant features. Deficiencies and variations in pathologic assessment were noted. Variation in the histologic findings of pleomorphic adenoma and assessment of the extent of invasion of carcinoma ex pleomorphic adenoma were highlighted. CONCLUSIONS The use of the more histologically diverse classification of salivary gland tumors can be successfully applied to the epithelial lacrimal gland neoplasms. This expanded classification system led to reclassifying 14% of cases. Currently, there are no consistent pathologic standards for processing and evaluating these lesions.

An analysis of 2,480 space-occupying lesions of the orbit from 1976 to 2011.

Purpose: To evaluate the frequencies of orbital space-occupying lesions seen at single orbital unit in a period of 35 years. Methods: In this retrospective case series, the authors reviewed the medical records of 2,480 consecutive patients referred to the authors’ Orbital Unit for evaluation of an orbital mass between 1976 and 2011. The final diagnosis in each case was established by a combination of history, ocular findings, diagnostic imaging, and histopathologic analysis, when available. The number and percentage of benign and malignant tumors were determinated, also according to the age of the patients and the tumor location within the orbit. This study adhered to the principles of the Declaration of Helsinki. Results: Of the 2,480 lesions, 1,697 (68%) were benign and 783 (32%) were malignant. The most frequent benign tumors were dermoid cyst (14%) and cavernous hemangioma (9%). The most common malignant tumors were non-Hodgkin lymphoma (12%), basal cell carcinoma (3%), and orbital metastases (3%). In patients younger than 60 years, benign tumors are more frequent, whereas in patients older than 60 years, the frequency of malignant tumors increased. Regarding the distribution in the orbit, the most common tumors were dermoid cyst (206 cases) in the upper-outer quadrant, mucocele (155 cases) in the upper-inner quadrant, basal cell epithelioma (35 cases) in the lower-inner quadrant, cavernous hemangioma (68 cases) in the lower-outer quadrant, and meningioma (90 cases) in central space. Most of the tumors were located in the upper-outer quadrant. In the lower-inner quadrant, malignant tumors were more frequent than benign tumors. Conclusions: The authors’ findings highlight the distinctive nature of the orbital oncology experience at an ocular oncology department.

Orbital venous-lymphatic malformations (lymphangiomas) mimicking cavernous hemangiomas

PURPOSE To illustrate that orbital venous-lymphatic malformations (lymphangiomas) may rarely simulate cavernous hemangiomas. METHODS Retrospective case review. RESULTS Five patients were identified from a series of 85 patients with venous-lymphatic malformations. The age range was 21 to 69 years, and all cases presented with a history of slowly progressive or long-standing proptosis. Computerized tomography revealed relatively homogeneous intraconal masses that were well defined anteriorly. Two of the cases had expansion of the orbit, and one had focal calcification. The three who had magnetic resonance imaging showed heterogeneous contrast enhancement. The preoperative diagnosis in every case was cavernous hemangioma, and intraoperatively the lesions resembled cavernous hemangiomas. However, posterior dissection was difficult in all patients because of dense adhesions and, in one case, led to a central retinal artery occlusion. The histology was characteristic of orbital venous-lymphatic malformations in all five cases. CONCLUSIONS Deep orbital venous-lymphatic malformations presenting in adulthood may be rarely confused with cavernous hemangiomas. In doubtful cases, significant intralesional heterogeneity, best seen on magnetic resonance imaging, and focal calcification may help distinguish the two entities. This differentiation is important, because dissection of venous-lymphatic malformations is fraught with more complications than surgical excision of a cavernous hemangioma.

Primary Optic Nerve Sheath Meningioma in Children

Primary optic nerve sheath meningioma represents a proliferation of meningothelial cap cells of the arachnoid villi within the optic nerve sheath. Patients younger than 20 years of age make up less than 5% of all cases of pediatric primary optic nerve meningiomas. Histopathologically, the most common subtypes in children are transitional (54%) and meningotheliomatous (38%). This tumor has been called aggressive in the pediatric population, with surgical excision recommended. However, the tumor may spread intraorbitally, intracranially, or intraocularly after subtotal surgical resection. Recent studies examined the use of fractionated, stereotactic radiation in children with this tumor; however, follow-up is limited. Neurofibromatosis type 2 is concomitantly diagnosed in 28% of patients with pediatric primary optic nerve sheath meningioma. There have been no known deaths attributed primarily to this tumor.

Radiation therapy in primary orbital lymphoma: a single institution retrospective analysis

BackgroundPrimary orbital lymphoma is a rare disease that accounts for 10% of all orbital tumors. Radiotherapy on the orbital cavity is the treatment of choice for this unusual presentation of localized non-Hodgkins lymphoma (NHL). The aim of this study is to retrospectively evaluate the effectiveness and the toxicity of radiation treatment in patients with primary orbital lymphoma.MethodsForty-seven consecutive patients having primary orbital lymphoma treated in our department between May 1983 and September 2006 were investigated in a retrospective study. Either 60Co γ rays or 6 MV X rays were used to deliver daily fractions of 1.8 or 2.0 Gy, 5 times/week, with total doses ranging from 34.2 to 50 Gy. Forty-three patients had stage IE, three had stage II and one stage IV disease. Thirty-eight patients had marginal zone B-cell lymphoma, 5 diffuse large B cell lymphoma, 3 mantle cell lymphoma and 1 Burkitt lymphoma. Local control (LC), disease free survival (DFS), overall survival (OS) and late side effects were evaluated in all patients.ResultsWith a median follow up of 45 months, LC was obtained in 100% of patients. The estimated 5- and 7-year DFS rates were 75.8% and 55.3%, and the 5- and 7-year OS rates were 88.7% and 79.9% respectively. Acute toxicity was minimal. Late toxicity such as cataract, keratitis, retinopathy and xerophthalmia occurred respectively in 12 (25.5%), 5 (10.6%), 1 (2.1%), and 9 (19.1%) patients.ConclusionRadiotherapy is an effective and at the same time well tolerated treatment for primary orbital lymphoma.

Primary Orbital Liposarcoma

PURPOSE To describe 6 new cases of primary orbital liposarcoma and provide a review of the relevant literature. DESIGN Noncomparative consecutive case series and literature review. PARTICIPANTS Six patients with primary orbital liposarcoma. METHODS Review of patient charts, imaging, and histopathology; literature review. MAIN OUTCOME MEASURES Patient demographics; clinical presentations; results of radiologic imaging; histopathology; surgical techniques used and their complications; other treatment modalities; outcomes and recurrences. RESULTS Six cases of primary orbital liposarcoma were identified, 5 of which were primary presentations and 1 of which was a recurrence. In 4 cases, exenteration was deferred, resulting in recurrence of disease in all 4. All cases were exenterated, and 2 cases had local recurrence despite exenteration. Two cases were associated with the Li-Fraumeni syndrome and other malignancies. Literature review identified 34 other cases of primary orbital liposarcoma, which, partly because of its rarity, is frequently initially misdiagnosed. The most common subtype is myxoid (56.8%); other types are pleomorphic (10.8%) and well differentiated (29.7%). Well-differentiated tumors have the best prognosis. Non-exenterating surgery was associated with recurrence, although recurrence post-exenteration also occurred. Although radiotherapy has an established role in the treatment of nonorbital liposarcoma, the role of both radiotherapy and chemotherapy in the management of primary orbital liposarcoma is still unclear. CONCLUSIONS Orbital liposarcoma remains a diagnostic and surgical challenge. Exenteration remains the treatment of choice, but clinicians must also be aware that liposarcoma may herald the diagnosis of the Li-Fraumeni familial cancer syndrome.

Lateral orbitotomy for removal of sphenoid wing meningiomas invading the orbit.

OBJECTIVE This study defines the indications, results, and limits of lateral orbitotomy coupled with resection of the sphenoid wing for removing lateral sphenoid wing meningiomas with intraorbital extension. METHODS Eighteen patients with lateral sphenoid wing meningiomas and tumor extension into the lateral or superolateral compartments of the orbital cavity were treated by microsurgical lateral orbitotomy and resection of the sphenoid wing without craniotomy. The approach consisted of a linear skin incision along the upper eyelid crease extending to 2 cm from the canthal angle and resection of the lateral orbital rim, lateral orbital wall, and infiltrated sphenoid wing. RESULTS A complete resection (Simpson I), including the infiltrated bone, dura, and periorbita, was obtained in 13 patients (72%); in the other 5 cases (28%), the tumor mass and most infiltrated dura were removed, but the entity of dural resection up to the normal tissue could not be exactly defined (Simpson II). Follow-up ranged from 5 to 17 years (mean 9.7 years). CONCLUSION A select group of lateral sphenoid wing meningiomas with tumor extension in the lateral or superolateral compartments of the orbital cavity may be successfully approached and removed through a lateral orbitotomy with resection of the sphenoid wing and without craniotomy. Cases with tumor extension to the anterior clinoid process and superior orbital fissure and those with extension medial to the axis of the optic nerve require a transcranial approach.

Expression of apoptosis-related markers in malignant epithelial tumours of the lacrimal gland and their relation to clinical outcome

Objective: To investigate the correlation between the expression of apoptosis-related markers and prognosis in malignant epithelial tumours of the lacrimal gland. Materials and Methods: Series of cases. Participants: Twenty one cases with malignant epithelial tumours of the lacrimal gland. Histological diagnosis was re-examined and blocks selected were evaluated for the following parameters: incidence of apoptosis with TUNEL assay, expression of p53 and Bcl-2 using monoclonal antibody. Predictors factors for survival, local recurrence and cumulative probability of death were statistically evaluated. Results: Re-eximination of the 21 specimens was as follow: 11 adenoid cystic carcinomas, 4 mucoepidermoid carcinomas, 3 squamous cell carcinomas and 3 adenocarcinomas. Eleven of the 21 patients (53%) died during the follow-up period (4–192 months; mean 71). Bcl-2 staining >6% was significantly correlated with the death of patients. A statistically significant positive relationship for TUNEL and p53, and an inverse correlation for Bcl-2 staining, was demonstrated with overall survival. Conclusion: The correlation with survival of apoptotic index, p53 and Bcl-2 expression suggest the more tumour cells go in apoptosis, upregulating p53 and down-regulating Bcl-2, the better the survival of patients. This study establishes a role of apoptosis-regulatory proteins in the pathogenesis of malignant epithelial lacrimal gland tumours, and supports the hypothesis that evaluation of the expression of apoptosis-related markers in these tumours may provide a prognostic tool.

Traumatic displacement of the globe into the ethmoid sinus

PURPOSE To describe displacement of the globe into the ethmoid sinus after an orbital trauma. METHOD Case report. A 58-year-old man sustained trauma of the left eye and orbit, which resulted in displacement of the globe into the ethmoid sinus. One day after injury, surgery was performed to restore the intact globe into position within the orbit. RESULTS After operation, the globe held in its anatomical site, and 10 months after surgery the visual acuity was 20/100, slight pallor of the optic disk was present, and there was no limitation of the horizontal and vertical ductions. CONCLUSIONS To our knowledge, this is the first case of traumatic displacement of the globe into the ethmoid sinus with satisfactory restoration of normal globe position and preservation of vision.

Unilateral proptosis in thyroid eye disease with subsequent contralateral involvement: retrospective follow-up study

BackgroundThe purpose of this retrospective follow-up study is to evaluate the prevalence of patients with thyroid eye disease presenting with apparent unilateral proptosis and determine the occurrence of exophthalmos in contralateral non-proptotic eye over the time. Associated features with this event were evaluated.MethodsA cohort of 655 consecutive patients affected by thyroid eye disease with a minimum follow-up of 10 years was reviewed. Exophthalmos was assessed by using both Hertel exophthalmometer and computed tomography (CT). The influence of age, gender, hormonal status and of different therapies such as corticosteroids, radiotherapy and surgical decompression on this disease progression was evaluated.ResultsA total of 89 patients (13.5%) (95% confidence interval [CI] 15%-10%) had clinical evidence of unilateral exophthalmos at the first visit. Among these, 13 patients (14%) (95% CI 22%-7%) developed subsequent contralateral exophthalmos. The increase of protrusion ranged from 2 to 7 mm (mean of 4.2). The time of onset varied from 6 months to 7 years (mean time: 29 months). Smoking status, young age and surgical decompression are significantly associated with development of contralateral proptosis (p< .05).ConclusionsAsymmetric thyroid eye disease with the appearance of unilateral exophthalmos at the initial examination is a fairly frequent event, while subsequent contralateral proptosis occurs less commonly. However, physicians should be aware that young patients, particularly if smokers, undergoing orbital decompression in one eye may need further surgery on contralateral side over time.