Orbital solitary fibrous tumors: A multi-centered histopathological and immunohistochemical analysis with radiological description

BACKGROUND: Solitary fibrous tumors (SFT), formerly called hemangiopericytoma, are rare tumors derived from mesenchymal cells originally described in the pleura, but these tumors may affect extraserosal tissues including the lacrimal gland and orbit. OBJECTIVE: Conduct a multi-centered clinical, radiological and histopathological analysis of 17 orbital SFT cases. DESIGN: A retrospective case series. SETTING: Three eye centers in two countries. PATIENTS AND METHODS: The data collected from the charts of 17 adult patients presenting with tissue diagnosis of orbital hemangiopericytoma or SFT from January 2003 to December 2018 included demographics, clinical imaging and histopathological information including immunohistochemical (IHC) characteristics. MAIN OUTCOME MEASURES: The demographic characteristics, clinical presentation, and histopathological patterns or variants of SFT were analyzed. SAMPLE SIZE: 17 adult patients. RESULTS: Mean age was 45 years (range 23-80 years). Male to female ratio was 3:1. The right eye was affected in 12 (70.5%) patients. Commonest presentation was proptosis in 13/17 (76% of patients). Other symptoms were impaired motility (29%) and ptosis (11%). Lesions mostly affected the medial orbit (35%), then orbital apex in 11%. The histopathological classic pattern-less variant was the commonest. One case with aggressive behavior, multiple recurrences and atypical features was encountered. Immunohistochemical (IHC) markers used included CD34 expression in all cases, Bcl-2 expression in 10/11, CD99 in 9/9 and Vimentin in 4/4. STAT6 was used in 2 cases. CONCLUSIONS: SFTs are rare tumors affecting the orbit in both genders equally in their mid-forties, but showed male predominance in our analysis with a predominant classic histopathological pattern. Tissue diagnosis is essential and requires IHC studies for confirmation. LIMITATIONS: Sample size is relatively small owing to the rarity of this tumor in the orbit. CONFLICT OF INTEREST: None

Axial Globe Position Measurement: A Prospective Multicenter Study by the International Thyroid Eye Disease Society

Identify a reproducible measure of axial globe position (AGP) for multicenter studies on patients with thyroid eye disease (TED)

Efficacy and Safety of Immunosuppressive Agents for Thyroid Eye Disease

Purpose: To determine the reliability of 3 scales for assessing soft tissue inflammatory and congestive signs associated with thyroid eye disease. Methods: This was a multicentered prospective observational study, recruiting 55 adults with thyroid eye disease from 9 international centers. Six thyroid eye disease soft tissue features were measured; each sign graded using 3 scales (presence/absence [0-1], 3-point scale [0-2], and percentage [0-100]). Each eye was graded twice by 2 independent raters. Accuracy (fraction of agreement) was calculated between the 2 trials for each rater (intrarater reliability) and between raters for all trials (interrater reliability) to determine the most sensitive scale for each feature that maintained a threshold of agreement greater than 0.70. Trial, intrarater reliability, and interrater reliability were determined by accuracy measurement of agreement for each inflammatory/congestive feature. Results: Fifty-five patients had 218 assessments for 6 thyroid eye disease metrics. The intrarater reliability for each feature was consistently better than the interrater reliabilities. Using an agreement of 0.70 or better, for the interrater tests, conjunctival and eyelid edema could be reliably measured using the 0-1 or 0-2 scale while conjunctival and eyelid redness could only be reliably measured with the binary 0-1 scale. Caruncular edema and superior conjunctival redness could not be measured reliably between 2 raters with any scale. The percentage scale had poor agreement unless slippage intervals of >20% were allowed on either side of the measurements. Conclusions: Of the specific periocular soft tissue inflammatory features measured between raters in the Clinical Activity Score and Vision, Inflammation, Strabismus, Appearance scales, edema of the eyelids and conjunctiva could reliably be measured by both 0-1 and 0-2 scales, erythema of the eyelid and bulbar conjunctiva could reliably be measured only by the 0-1 scale, and the other parameters of superior bulbar erythema and caruncular edema were not reliably measured by any scal

REPLY: Risk Factors for Orbital Exenteration in Periocular Basal Cell Carcinoma

We reported 1 that we were not able to achieve negative tumor margins on half of the patients who underwent exenteration because in those 12 patients tumor infiltration had already reached the bone. In fact, as mentioned in the text, the CT scan had already reported evidence of bone invasion in 8 patients. We consider clear margin only those cases having evidence of negative margin on soft tissues of the exenterated orbit, since the evaluation of clear margin on bone is doubtful, particularly if the medial bone and the lacrimal bone and the nasolacrimal duct are involved because the removal of the bone structure to obtain negative margin in radical manner could be life threatening considering the adjacent structures as the frontal lobe. Sixteen of our exenterated patients had medial canthus and lower eyelid involvement. Eventually, we did not perform routinely frozen section for exenteration procedure since the goal of this operation is always to remove the whole orbital content. We use frozen section intraoperative margin control when we deal with a lesion of the superficial periorbital region for which an orbital invasion is suspeced. Infact this technique does not allow intraoperative control of the bone. As mentioned in the text, in these twelve patients were offered an additional therapy, accepted by 7 and consisting in local radiation therapy. Moreover, 4 patients because of relapse had further surgical excision and other 4 additional radiotherapy. We are conscious that often the interpretation of data from a retrospective research may have some limitations. Nevertheless, we would like to emphasize that the main objective of this study is to evaluate the percentage of patients with basal cell carcinoma who need exenteration and which are possible risk factors

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

Epithelioid myoepithelioma of lacrimal gland.

A rare case of myoepithelioma of the lacrimal gland composed exclusively of epithelioid cells is described.A 57-year-old female had been complaining for 1 month of progressive swelling of the temporal region of the left orbit. Computed tomography scan revealed a lesion involving the left lacrimal gland. The lesion was entirely removed en-bloc along with the lacrimal gland. Grossly, the tumour was a well-circumscribed 3.5×25×1.5 cm mass. An extensive sampling was performed. The surgical specimen was routinely formalin fixed and paraffin embedded. Sections 5-m thick were obtained and stained with haematoxylin-eosin. Immunostaining was performed on 5-m serial sections. Histologically, the lesion showed a solid pattern of growth and was composed exclusively of cells with epithelioid (plasmacytoid) features. Atypical myoepithelial cells, abnormal mitotic figures, necrosis and invasive growth throughout the tumour were absent. Myoepithelioma of the lacrimal gland is extremely rare, and thus far only seven cases have been reported. Of these cases, two were considered malignant and one potentially malignant . All but one showed spindle to cuboidal cells arranged in a solid or trabecular pattern . Myoepithelial cells are generally immunoreactive for S-100, vimentin, cytokeratin, desmin, m-actin and sm-actin. There is a considerable variation in the immunoreactivity for these antigens in our case, as in the seven previously reported cases. These differences may be dependent on the degree of differentiation .The size of the tumour prompted us to recommend a careful clinical follow-up. The patient had an uneventful post-operative course, and there has been no evidence of relapses over 24 months of follow-up