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Background: Multiple myeloma (MM) is a malignant disorder of plasma cells that results in tumor cells replacing the bone marrow. In extramedullary MM (EMM), however, tumor cells proliferate outside the bone marrow. EMM may produce ophthalmoplegia through direct invasion of the superior orbital fissure, cavernous sinus, and/or sphenoidal sinus. Several mechanisms have been proposed including cranial nerve palsies, direct infiltration of bone, extraocular muscle metastasis, myelomatous meningitis, and parenchymal or paraneoplastic effects. Methods: We retrospectively reviewed the medical records of 7 patients at MD Anderson Cancer Center who suffered from ophthalmoplegia secondary to extramedullary MM between 2019 and 2021. We collected information regarding the symptoms, signs, radiographic and laboratory findings, management, complications, and prognosis of these patients throughout their disease course. Results: Skull base MRI revealed 4 patients with ophthalmoplegias secondary to superior orbital fissure invasion, 2 patients with ophthalmoplegias secondary to cavernous sinus invasion, and 1 patient with ophthalmoplegia secondary to sphenoid sinus invasion. Conclusions: This is a case series describing 7 patients with ophthalmoplegias secondary to EMM. Our article is unique because of the size of the included cohort, which is large when compared with most English language publications detailing such ophthalmoplegias. [ABSTRACT FROM AUTHOR]

Aims: To evaluate how tumour size, retinal location, and patient age affect the outcome of retinoblastoma foci treated with chemotherapy. Methods: Retrospective review of retinoblastoma foci treated with primary chemotherapy [...]

Purpose: Evaluate incidence of second primary malignancies (SPM) after non-acral cutaneous melanoma (NACM), acral lentiginous melanoma (ALM), mucosal melanoma (MM), and uveal melanoma (UM). Patients and Methods: First primary NACM, ALM, MM, and UM cases diagnosed 2000-2016 were extracted from SEER. Seer*Stat was used to calculate excess absolute risks (EAR) and standardized incidence ratios (SIR) of SPMs relative to a matched cohort from the general population. P-value of 0.05 determined significance. Results: Inclusion criteria was met by 109,385 patients with NACM, 2166 with ALM, 2498 with MM, and 6250 with UM. Increased incidence of malignancies occurred after NACM (SIR 1.51; 95%CI, 1.49-1.54), ALM (SIR 1.59; 95%CI, 1.40-1.81), MM (SIR 2.14; 95%CI, 1.85-2.45), and UM (SIR 1.24; 95%CI, 1.14-1.34) relative to the general population. Cutaneous melanoma occurred more frequently after NACM (SIR 9.54; 95%CI, 9.27-9.83), ALM (SIR 12.19; 95%CI, 9.70-15.14), MM (SIR 10.05; 95%CI, 7.18-13.68), and UM (SIR 2.91; 95%CI, 2.27-3.66). Patients with initial NACM (SIR 2.44; 95%CI, 1.64-3.51) and UM (SIR 44.34; 95%CI, 29.91-63.29) demonstrated increased incidence of eye and orbit melanoma. Renal malignancies occurred more frequently after NACM (SIR 1.24; 95%CI, 1.11-1.38), MM (SIR 3.54; 95%CI, 1.62-6.72) and UM (SIR 1.68; 95%CI, 1.09-2.48). Increased incidence of thyroid malignancies was observed after NACM (SIR 1.83; 95%CI, 1.61-2.06), ALM (SIR 3.74; 95%CI, 1.71-7.11), MM (SIR 4.40; 95%CI, 1.77-9.06), and UM (SIR 3.79; 95%CI, 2.52-5.47). Increased incidence of lymphoma was observed after NACM (SIR 1.20; 95%CI, 1.09-1.31) and ALM (SIR 2.06; 95%CI, 1.13-3.46). Conclusion: Patients with NACM, ALM, MM, and UM have increased incidence of SPMs compared to that expected from the general population. Each of these melanoma subtypes had increased occurrence of cutaneous melanoma and thyroid cancer; some, but not all, had increased occurrence of renal malignancies, eye and orbit melanoma, and lymphoma. [ABSTRACT FROM AUTHOR]

Retinoblastoma is the most common intraocular malignant neoplasm in children, with most cases initiated by a mutation of the tumor-suppressor gene RB1. (1-3) Despite extensive research efforts, retinoblastoma-associated mortality rate [...], Context.--Progression of retinoblastoma is associated with increased tumor angiogenesis. However, a clear relationship between the expression of angiogenic markers in specific regions of the tumor and tumor progression has not been established. This study investigates the association between angiogenic factors in retinoblastomas with choroidal and/or optic nerve invasion (high-risk/invasive retinoblastoma) and expression of Sox2, a stem cell marker. Objective.--To investigate the association between the expression of angiogenic factors and markers of tumor invasiveness, such as the stem cell marker Sox2, in retinoblastoma tissues. Design.--Immunohistochemistry was used to evaluate coexpression of the angiogenic growth factors vascular endothelial growth factor A (VEGF-A), VEGF receptor 2 (VEGFR-2), and endoglin (CD105); markers of glial differentiation (vimentin and glial fibrillary acidic protein); and a neural stem cell marker (Sox2). Expression was assessed in nonneoplastic and neoplastic ocular tissues collected from enucleated eyes of patients with retinoblastoma. During qualitative data interpretation, evaluating pathologists were masked to patient grouping. Results.--Expression of VEGF-A and VEGFR-2 in noninvasive (non-high-risk feature) retinoblastoma tumors was lower than in the invasive, or high-risk feature tumors. Moreover, our data indicate that the tumor cells, and not the surrounding stroma, secrete VEGF-A and that angiogenesis is mostly localized to the iris. Finally, our data showed that the expression of the neural stem cell marker Sox2 is associated with eyes with increased VEGF-A expression and tumor invasiveness. Conclusions.--Increased expression of angiogenic factors, with a concomitant increase in expression of the stem cell marker Sox2 observed in retinoblastoma tissues, may partially explain the aggressiveness of these tumors. The complex interaction of angiogenic and stem cell-related pathways in these tumors, especially in high-risk feature retinoblastoma, suggests that targeting tumor cells capable of secreting vasculogenic factors, as well as proangiogenic genes and signaling pathways, may be necessary for development of effective antimetastatic retinoblastoma drugs. (Arch Pathol Lab Med. 2015; 139:1531-1538; doi: 10.5858/arpa.2014-0262-OA)

Background: The aim of this review is to elucidate the type and frequency of ocular adverse events associated with selinexor with a goal to quantify the occurrence of these events in our investigator‐initiated trial. Methods: We retrospectively reviewed medical records of 174 patients treated with at least one dose of selinexor in combination with multiple standard chemotherapy or immunotherapy agents between July 2015 and July 2020 at a comprehensive cancer center in the U.S. All reported ocular adverse events were assessed. Results: A total of 174 patient medical records were reviewed. All patients received at least one dose of selinexor in combination with multiple standard chemotherapy or immunotherapy agents in our cohort of patients with advanced malignancies. A total of 34 (19.54%) patients experienced 37 ocular adverse events. The most frequently reported ocular symptom was blurred vision, which was reported in 22 (12.64%) patients. The most frequently reported treatment‐related adverse event was dry eye syndrome reported in 21 (12.1%) patients, and 19 (10.9%) of them were diagnosed with mild dry eye. The second most common treatment‐related adverse event was the progression of age‐related nuclear sclerosis (cataract) reported in 7 (4.0%) patients. None of the ocular adverse events required therapy discontinuation. Conclusion: Our findings highlight that ocular adverse events associated with oral selinexor were mild. The most frequently reported ocular treatment‐related adverse events were mild dry eye and progression of age‐related nuclear sclerosis. None of the ocular adverse events required therapy discontinuation. Implications for Practice: Patients receiving selinexor in combination with multiple standard chemotherapy or immunotherapy agents were reviewed, with a total of 34 patients experiencing 37 ocular adverse events. Findings highlight that ocular adverse events associated with oral selinexor were mild. The most frequently reported ocular treatment‐related adverse events were mild dry eye and progression of age‐related nuclear sclerosis. None of the ocular adverse events required therapy discontinuation. XPO1 inhibitors are associated with distinct adverse events. This article focuses on ocular adverse events in patients with advanced malignancies treated with selinexor. [ABSTRACT FROM AUTHOR]

A 23-year-old man with a history of metastatic melanoma developed painful vision loss to counting fingers with enhancement of optic nerve on contrast-enhanced magnetic resonance imaging (MRI) and received a diagnosis of optic neuritis from an outside hospital. Despite empiric corticosteroid therapy, the patient worsened and developed secondary central retinal vein occlusion with further deterioration of vision. Repeat MRI demonstrated optic nerve sheath (ONS) involvement suggestive of optic perineuritis (OPN) and an ONS biopsy confirmed a rare case of isolated metastatic melanoma. Our case highlights the clinical and radiographic features that can mimic OPN and delay diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Primary intraocular lymphoma (PIOL) is a rare malignancy with poor outcomes. Concerns regarding toxicity lead some clinicians to exclude orbital radiation therapy (RT). We aimed to quantify the ocular toxicity of RT in 11 PIOL patients treated with chemoimmunotherapy and bilateral orbital RT (median 36 Gy). A multidisciplinary team, including an ocular oncologist, followed patients for a median of 42 months after RT. Common adverse events included dermatitis (100%), conjunctivitis (82%), xerophthalmia (64%), and keratopathy (45%). All phakic eyes developed cataracts (100%); correction resulted in good vision recovery. New, visually significant retinopathy was observed in only one eye (<5%) and affected a patient with preexisting diabetes. This report suggests that severe, vision-threatening complications following orbital RT are uncommon. In the absence of comorbidities, orbital RT should not be withheld due to fear of vision-threatening toxicity. The risk of toxicity may be augmented by comorbidities, so an individualized approach is recommended. [ABSTRACT FROM PUBLISHER]

Melanomas of the choroid, ciliary body, and iris of the eye are collectively known as uveal melanomas. These cancers represent 5% of all melanoma diagnoses in the United States, and their age-adjusted risk is 5 per 1 million population. These less frequent melanomas are dissimilar to their more common cutaneous melanoma relative, with differing risk factors, primary treatment, anatomic spread, molecular changes, and responses to systemic therapy. Once uveal melanoma becomes metastatic, therapy options are limited and are often extrapolated from cutaneous melanoma therapies despite the routine exclusion of patients with uveal melanoma from clinical trials. Clinical trials directed at uveal melanoma have been completed or are in progress, and data from these well designed investigations will help guide future directions in this orphan disease. Cancer 2016;122:2299-2312. © 2016 American Cancer Society. [ABSTRACT FROM AUTHOR]