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Ocular Oncology and Pathology

Baudine C van Noort, Jan E E Keunen, Reinier O Schlingemann, Marina Marinkovic
Purpose: To report a patient with bilateral diffuse uveal melanocytic proliferation (BDUMP) due to a lung carcinoma, who survived 9.5 years with preservation of good visual acuity after cataract surgery and curative chemotherapy with neoadjuvant radiation therapy. Methods: Clinical review of a patient with BDUMP. Results: We report the case of a 64-year-old man presenting with bilateral visual deterioration. Ophthalmological examination showed bilateral cataract, a small elevated non-pigmented tumor in the iris and multiple elevated, diffuse round pigmented choroidal lesions...
January 2019: Ocular Oncology and Pathology
Lina Ma, Natalie Wolkow, Frederick A Jakobiec
An enucleated globe was submitted from an outside hospital to the Ophthalmic Pathology Laboratory for evaluation. There was a minimal amount of accompanying clinical history. Histopathologic examination revealed a mucinous adenocarcinoma of the choroid. The determination of the origin of the tumor proved to be challenging based on the lack of a definitive systemic diagnosis. Initial suspicions that the tumor may represent a breast carcinoma were disproved when immunohistochemical biomarkers for breast carcinoma were negative...
January 2019: Ocular Oncology and Pathology
Alexander D Blandford, Claudine Bellerive, Martin Tom, Shlomo Koyfman, David J Adelstein, Thomas P Plesec, Julian D Perry, Arun D Singh
Squamous cell carcinoma (SCC) of the orbit is almost uniformly the result of local invasion from a cutaneous primary, extension by perineural invasion, or the result of metastasis. This is owed to the lack of native squamous epithelium in the orbit. After review of the literature, to date, only 6 reports of 8 patients with primary orbital SCC exist. Of those cases, only 2 reported non-apical orbital SCC. There are 2 reports of orbital SCC after retina surgery with proposed transplanted conjunctival epithelium and subsequent malignant transformation of a conjunctival cyst...
January 2019: Ocular Oncology and Pathology
Sona N Shah, Kaitlin Kogachi, Zelia M Correa, Amy C Schefler, Mary E Aronow, Sonia A Callejo, Colleen M Cebulla, Shelley Day-Ghafoori, Jasmine H Francis, Sara Lally, Tara A McCannel, Katherine E Paton, Isabella T Phan, Renelle Pointdujour-Lim, Aparna Ramasubramanian, Pamela Rath, Carol L Shields, Alison H Skalet, Jill R Wells, Richard L Jennelle, Jesse L Berry
Background: The aim of this study was to determine the known radiation exposure, attitudes, and consequent risk modifications among female ocular oncologists in North America who routinely administer radioactive plaque brachytherapy treatment and are members of the International Society of Ocular Oncology. Methods: Nineteen female ocular oncologists completed an anonymous 17-question radiation exposure survey. Results: Eleven of the participants chose to routinely wear lead protection during surgery; 8 did not...
January 2019: Ocular Oncology and Pathology
Mansooreh Jamshidian-Tehrani, Bahram Eshraghi, Mohammad Zarei, Zohreh Nozarian, Seyed Mohsen Rafizadeh, Hadi Ghadimi
Hemangioendothelioma is rarely encountered in the orbit. We present a patient with a growing orbital mass for whom surgical excision was planned. Two previous attempts at removing the mass failed due to profuse bleeding. Endovascular embolization was performed before surgery to prevent massive hemorrhage. After embolization, retinal vascular accident (combined central retinal artery and vein occlusion) occurred. However, surgical excision (lateral orbitotomy and transcoronal craniotomy) was successful, and the vision improved postoperatively...
January 2019: Ocular Oncology and Pathology
Sruthi Arepalli, Aron Flagg, Arthi Venkat, Arun D Singh
Background/Aims: Sporadic Burkitt lymphoma is rarely associated with orbital involvement. Methods: We present a case of sporadic Burkitt lymphoma with extramedullary subperiosteal leukemic infiltrates of the orbit and facial bones. Results: Follow-up examination after chemotherapy and disease remission reveals resolution of the subperiosteal infiltrates. Conclusion: Despite an aggressive presentation, cure is common with appropriate, intensive treatment...
January 2019: Ocular Oncology and Pathology
Bertil Damato, Armin R Afshar, Lesley Everett, Anuradha Banerjee, Steven W Hetts
Background/Aims: Current retinoblastoma staging systems do not adequately describe the disease, especially in eyes with multiple tumors. The aims of this study were to develop methods for documenting individual tumors and to score disease burden over time. Methods: A coding system was devised to describe each tumor according to affected eye, meridian, anteroposterior location, activity, growth pattern, type of seed, and treatment. A scoring system for quantifying disease burden was developed, taking account of tumor number, size, spread, and secondary effects on the eye...
January 2019: Ocular Oncology and Pathology
Frederick A Jakobiec, Natalie Wolkow, Fouad R Zakka, Peter A D Rubin
An 87-year-old woman not known to have either a lymphoma or leukemia developed a left multinodular, fish-flesh superior epibulbar and forniceal mass. A biopsy disclosed a blastic tumor with scattered multinucleated immature megakaryoblasts. Immunophenotyping of bone marrow cells revealed strong positivity for CD7, CD31, CD43, CD45, CD61, and CD117; CD71, myeloperoxidase, and lysozyme were also positive in scattered cells. Forty percent of the neoplastic cells were Ki-67 positive. Cytogenetic studies indicated a trisomy 8 (associated with worse prognosis) and a t(12; 17) translocation...
January 2019: Ocular Oncology and Pathology
Nima Fatehi, Tara A McCannel, JoAnn Giaconi, Joseph Caprioli, Simon K Law, Kouros Nouri-Mahdavi
Aim: We report patient safety and intraocular pressure (IOP) control after placement of a glaucoma drainage device (GDD) in eyes with a history of treated malignant uveal melanoma. Methods: A retrospective review of the records of patients with uveal melanoma was performed. Outcomes were local tumor recurrence, rate of metastases, and to-nometric success, based on survival curves, defined as IOP < 21 mm Hg. Results: Eleven eyes with choroidal melanoma, 4 with iris melanoma, and 1 with ciliary body melanoma were followed for a median (interquartile range) of 2...
January 2019: Ocular Oncology and Pathology
Bertil E Damato, Gregory J Bever, Armin R Afshar, James L Rubenstein
Purpose/Background: The aim of this study was to report a patient with vitreoretinal lymphoma with clinical features providing hypothesis-generating insights into the pathophysiology of this disease. Methods: Clinical history and imaging studies (i.e., fundus photography, optical coherence tomography, fundus autofluorescence, and fluorescein angiography) were documented. Results: A 71-year-old woman presented with a 2-month history of blurred vision in the right eye and bilateral vitreous infiltrates unresponsive to topical and systemic steroids...
January 2019: Ocular Oncology and Pathology
Jose J Echegaray, Carlos A Medina, Charles V Biscotti, Thomas Plesec, Arun D Singh
We report two patients who developed a second distinct choroidal melanoma in the same eye following successful regression of their first choroidal melanoma after iodine-125 plaque brachytherapy. Neither patient demonstrated ocular melanocytosis, local tumor recurrence, or vitreous seeding. One patient had the second tumor arising from a previously documented choroidal nevus, and after undergoing enucleation, there was no detectable connection between the tumors on histopathologic examination. Germline BAP1 mutation was absent in both cases...
January 2019: Ocular Oncology and Pathology
Andrew W Stacey, Bronagh Clarke, Christos Moraitis, Ido Didi Fabian, Vicki Smith, Mandeep S Sagoo, M Ashwin Reddy
Purpose: The study aimed to assess the incidence of and risk factors leading to visual impairment and legal blindness in children with retinoblastoma. Procedures: This is a single-center, retrospective case series of all patients with bilateral retinoblastoma presenting from 2010 to 2014. Results: A total of 44 patients were included in the study. Visual impairment was present in 14 (38%) children, legal blindness was present in 7 (19%) children...
January 2019: Ocular Oncology and Pathology
Kelsey Roelofs, Matthew P Larocque, Albert Murtha, Ezekiel Weis
Background/Aims: To evaluate a treatment regimen for radiation papillopathy. Methods: This is a prospective noncomparative interventional case series of patients who developed radiation papillopathy after plaque brachytherapy for uveal melanoma. Treatment consisted of intravitreal bevacizumab (IVB) (1.25 mg in 0.05 mL) at the time of diagnosis, and 1 week later, intravitreal triamcinolone (IVK) (2.00 mg in 0.05 mL). One month later, patients again received both IVB and IVK...
November 2018: Ocular Oncology and Pathology
Atalie C Thompson, Chad M McCall, Alan D Proia
Purpose: To report a case of extranodal natural killer/T-cell lymphoma (ENKTCL), nasal type metastatic to the space beneath the retinal pigment epithelium (RPE) with coincident paraneoplastic lymphoma-associated retinopathy. Methods: Findings of clinical and histopathologic examination are presented with differential diagnoses and a literature review. Case Report: A 53-year-old man presented with bilateral blindness and had exudative retinal detachments overlying subretinal masses in both eyes...
November 2018: Ocular Oncology and Pathology
Diane T Siegel, Eszter Szalai, Jill R Wells, Hans E Grossniklaus
Purpose: The purpose of the study is to describe the clinical history and histopathologic findings of three cases of scleral thinning after lamellar scleral flap, including one case with confirmed extraocular tumor extension. Methods: The medical records and pathology specimens of three patients with scleral thinning after biopsy and plaque brachytherapy and lamellar scleral flap performed during a transscleral biopsy were reviewed. Results: The first two patients developed scleral thinning and visible pigmentation, but had tumors that were regressing in size on ultrasound...
November 2018: Ocular Oncology and Pathology
Ryan S Kim, Rishabh R Jain, David M Brown, Maria E Bretana, Eric N Kegley, Michael A Singer, Antonio V Aragon, Amy C Schefler
Objective: To determine whether increased choroidal thickness (CT) is associated with circumscribed choroidal hemangioma (CCH) and whether patients with CCH are predisposed to central serous chorioretinopathy (CSCR). Methods: We performed a retrospective consecutive observational study of 15 fellow eyes of 15 patients who had CCH. A- and B-scan ultrasonography, fluorescein angiography (FA), and optical coherence tomography (OCT) findings were reviewed to confirm the diagnosis of CCH...
November 2018: Ocular Oncology and Pathology
Lindsay A McGrath, Hardeep Singh Mudhar, Sachin M Salvi
Optic nerve haemangioblastomas remain exceedingly rare extrinsic tumours of the optic nerve, often associated with von Hippel-Lindau disease. The authors report a 25-year-old female with a slowly progressive unilateral optic nerve lesion, causing reduced vision and bilateral optic tract oedema. A diagnosis of optic nerve haemangioblastoma with piloid gliosis was made histologically after surgical resection. This is the first reported case of such dual pathology occurring in the optic nerve. The patient has been monitored without further adjuvant treatment, and has not had a recurrence to date, at 6 years of follow-up...
November 2018: Ocular Oncology and Pathology
Larissa Ioannidi, Konstantinos Seliniotakis, Georgios Bontzos, George Sourvinos, Viktor Haniotis, Irene Tsiapa, Thomas G Maris, Efstathios T Detorakis
Introduction: Imaging of small peripheral choroidal melanomas remains challenging in clinical and experimental settings, requiring wide-field ophthalmoscopy modalities. In this study, we applied surface-coil MRI to assess imaging feasibility for small peripherally located choroidal melanoma in a rabbit eye model. Methods: A female albino rabbit with a body weight of 2.8 kg was immunosuppressed with intramuscular cyclosporine 15 mg/kg daily for 3 days prior to inoculation of 14 million 92...
November 2018: Ocular Oncology and Pathology
Hardeep Singh Mudhar, Jeremy Prydal, Ian G Rennie
A 38-year-old female, otherwise fit and well, presented with a mass on her left medial bulbar conjunctiva that had been enlarging for several months. Examinations showed a fixed pinkish tumour, 9 mm in maximum extent, spanning from the plica to the medial limbus. The tumour was removed in toto. Histology revealed it to be a biphasic tumour composed of lobules and infiltrative cords within a sclerotic matrix. The cells were spindle-shaped to epithelioid, with nuclear atypia and occasional mitotic figures. The tumour was positive for smooth muscle actin, beta-catenin, and vimentin...
November 2018: Ocular Oncology and Pathology
Alisa J Prager, Larissa A Habib, Klaus J Busam, Brian P Marr
Multifocal uveal melanomas are extremely rare. In this case report, we describe a patient with 2 independent uveal melanomas in the same eye. A 52-year-old woman presented with a large choroidal melanoma and a smaller ciliary body mass, clinically thought to be a nevus, in her left eye. Enucleated specimen showed 2 primary lesions that were anatomically separate. Lesion 1 was a melanoma and lesion 2 was a melanoma arising centrally from a nevus. Both lesions harbored GNAQ mutations. This patient had no family history of uveal melanomas or signs of ocular melanocytosis and was negative for the BAP1 mutation...
November 2018: Ocular Oncology and Pathology
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