Response to Miles Kiernan Re: a rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland

Our goal is to contribute to the medical literature with our case of CEPA and hope to better help fellow clinicians diagnose and treat these rare tumors

Etiologies of Proptosis: A review

Proptosis, the protrusion of the eyeball from the orbit, results from a wide variety of pathologies that can be vision- or life-threatening. Clinical history, associated physical exam findings, and imaging features are all crucial in establishing the underlying etiology. The differential diagnosis is broad, and includes infectious, inflammatory, vascular, and neoplastic entities that range from benign and indolent, to malignant and aggressive. While treatment varies significantly based on the disease process, all are aimed at preserving vision, salvaging the globe, preventing disfigurement, and reducing mortality. Both internists and general ophthalmologists should be familiar with the causes of proptosis in order to initiate the work-up for, and appropriately triage, affected patients

A rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland

Carcinoma ex pleomorphic adenoma (CEPA) of the lacrimal gland is a rare malignant tumor that arises from a pre-existing pleomorphic adenoma. Lacrimal gland CEPA with mucoepidermoid histological subtype is exceedingly rare. Diagnosis can be aided by radiographic findings, though the gold standard is histopathological analysis following excisional biopsy. Management options include complete surgical excision with or without adjuvant radiation therapy based on tumor grade and invasiveness. We present a 76-year-old woman with 6 months of diplopia and unilateral proptosis. Her initial exam was remarkable for hypoglobus, proptosis, and limited elevation of the right eye. Computed tomography (CT) scan demonstrated a superior, well-circumscribed, extraconal orbital mass. An excisional biopsy was performed, and histopathological findings were consistent with mucoepidermoid carcinoma ex pleomorphic adenoma with positive margins in the tumor capsule. The patient received radiation therapy and remains markedly improved with no disease recurrence at 5 months post-operatively

Orbital Involvement in Multiple Myeloma

PURPOSETo characterize clinical and radiographic features, management, and outcomes of patients with orbital involvement of multiple myeloma (MM). METHODSA retrospective chart review identified patients with MM and orbital involvement confirmed by histopathology at a single institution between 1995 and 2021. A comprehensive literature review was performed via PubMed to identify all previously reported cases of orbital MM. RESULTSRetrospective review identified 7 patients (43% male, mean age 68.7 years). Presenting symptoms included proptosis and diplopia. Orbital lesions were primarily located laterally (42.8%) with associated extraocular muscle (57.1%) or lacrimal gland (42.9%) involvement. Five patients (71.4%) had a previous diagnosis of systemic MM. Six patients received chemoradiation (85.7%). All patients had improvement of orbital disease with 2 patients deceased due to disease at follow-up (mean 8.9 months). Literature review identified 111 cases (46.8% male, mean age 58.6 years). 48.6% presented with orbital disease as the first manifestation of systemic MM. Lesions were most commonly located superolaterally (20.2%) with extraocular muscle infiltration (25.2%), lacrimal gland involvement (7.2%), and orbital bony destruction (39.6%). Treatments included chemoradiation, chemotherapy, or radiation alone. Approximately half (51.4%) of patients experienced improvement in orbital disease following treatment, and 48.6% were deceased at follow-up (mean 20.1 months). CONCLUSIONSThis study provides a new retrospective study and updated comprehensive literature review regarding orbital MM. Given its poor prognosis, characterization of orbital MM is essential for early diagnosis. Orbital MM is often unilateral, located superolaterally, and may represent the first manifestation of systemic disease. Treatment includes chemotherapy and radiation, which may improve orbital disease; however, the overall prognosis remains poor

A comparison of proptosis reduction with teprotumumab versus surgical decompression based on fat-to-muscle ratio in thyroid eye disease

PurposeTo explore if orbital fat-to-muscle ratio (FMR) is predictive of whether surgical decompression or teprotumumab leads to greater proptosis reduction in thyroid eye disease (TED).MethodsA single-center retrospective cohort study comparing surgical decompression with teprotumumab according to FMR. All TED patients completing an 8-dose course of teprotumumab between January 2020 and September 2022 and all patients undergoing bony orbital decompression from January 2017 to December 2019 were included. Subjects were excluded if they were <18 years, received both surgical decompression and teprotumumab, or lacked orbital imaging. The primary exposure variable was teprotumumab or surgical decompression. The secondary exposure variable was baseline FMR. The primary outcome measure was change in proptosis (mm).ResultsThirty-eight patients, mean age 53.5 years (±11.4), were included in the teprotumumab group and 160 patients, mean age 48 years (±11.1), in the surgical group. Average proptosis reduction after teprotumumab and surgical decompression was 3 mm (±1.44) and 5 mm (±1.75), respectively. The FMR was stratified at the median of 1.80. In subjects with FMR < 1.80, teprotumumab showed equivalent proptosis reduction compared to surgical decompression, -0.33 mm (SE 1.32) p = .802. In subjects with FMR ≥ 1.80, surgical decompression led to significantly more proptosis reduction than teprotumumab, 3.01 mm (SE 0.54), p < .001.ConclusionsBaseline FMR can be used to counsel patients as to proptosis reduction with teprotumumab versus surgery. Subjects with low FMR obtain comparable proptosis reduction with teprotumumab or surgery, whereas high FMR is associated with more significant proptosis reduction following surgery over teprotumumab

Associations Between Thyroid Eye Disease and Glaucoma Among Those Enrolled in the National Institutes of Health All of Us Research Program

PurposeTo assess the association between thyroid eye disease (TED) and glaucoma.MethodsPatients 18 years of age and over enrolled in the NIH All of Us Research Program, a nationwide cohort, were extracted. Those with conditions relating to TED were identified and compared with 2020 US Census-matched controls without a diagnosis of TED in a 1:4 ratio. The authors used Pearson's χ 2 tests to study demographics by TED status, and logistic regression to generate odds ratios and 95% confidence intervals to evaluate the association between TED and glaucoma (any type, including glaucoma suspect), using those without TED as the reference group. Multivariable models were adjusted for age, gender, race/ethnicity, eye doctor visits, and smoking status.ResultsA total of 393 cases of TED and 1,572 US Census-matched controls were identified. The median age of the cohort was 63 years (interquartile range: 48-73 years). Age, gender, and race/ethnicity varied by TED status ( p < 0.001). Overall, 114 (29.0%) of TED cases had a diagnosis of glaucoma, compared with 94 (6.0%) of non-TED controls. On bivariate logistic regression models, those diagnosed with TED were significantly more likely to be diagnosed with glaucoma compared with controls (odds ratio: 6.42; 95% confidence interval: 4.76-8.70; p < 0.001). This trend persisted on multivariable logistic regression controlling for confounding factors (odds ratio: 3.37; 95% confidence interval: 1.85-6.20 p < 0.001).ConclusionsIndividuals with TED were significantly more likely to be diagnosed with glaucoma. Clinicians caring for patients with TED should be aware of this elevated risk and arrange glaucoma evaluation, accordingly