Abstract
Purpose
To describe two cases of medium-sized uveal melanoma presenting with hemorrhagic choroidal detachments.
Observations
The first case is a 39-year-old man who presented with choroidal hemorrhage and angle closure glaucoma. The second case is a 42-year-old man who presented with choroidal hemorrhage and posterior scleritis. Vitrectomy with transvitreous fine needle aspiration biopsy was ultimately required to diagnose malignant uveal melanoma in each case.
Conclusions and importance
Intraocular hemorrhage is a rare presenting sign of uveal melanoma. When it does occur, it is typically associated with large tumors. Hemorrhagic choroidal detachments are particularly rare in uveal melanoma, and can limit the diagnostic utility of clinical exam, B-scan ultrasonography, and magnetic resonance imaging. Although it is uncommon, it is important to maintain a high index of suspicion for choroidal melanoma in any patient with unexplained choroidal hemorrhage.
1
Introduction
Intraocular hemorrhage is a rare presenting sign of uveal melanoma, present in less than 3 % of new cases. When it does occur, the hemorrhage usually within the retina or the vitreous and is typically associated with large uveal melanomas. The presence of suprachoroidal hemorrhage at the time of presentation can make melanoma detection challenging due to limitations on clinical exam and imaging modalities such as B-scan ultrasonography and magnetic resonance imaging (MRI), which may not reliably differentiate between choroidal hemorrhage and a choroidal mass. Here we describe two cases of medium-sized uveal melanoma, stage IIA by American Joint Commission on Cancer 8th edition, that presented with acute choroidal hemorrhage: one with choroidal hemorrhage and angle closure glaucoma, and the other with choroidal hemorrhage and posterior scleritis. After initial conservative management, vitrectomy was ultimately needed to remove associated non-clearing vitreous hemorrhage, visualize the underlying pathology, and sample the choroidal mass with a fine needle aspiration biopsy (FNAB). In each case, the FNAB obtained at the time of vitrectomy demonstrated malignant melanoma.
2
Findings
Case 1: A 39-year-old man presented with sudden vision loss and pain in the right eye. Visual acuity (VA) on presentation was light perception and the intraocular pressure (IOP) was elevated to 38 mmHg in the affected eye. Slit lamp exam was notable for a shallow anterior chamber, and a dilated fundus exam demonstrated large hemorrhagic choroidal detachments obscuring view to the posterior pole. A B-scan ultrasound was performed, which demonstrated large hemorrhagic choroidal detachments as well as an indistinct, dome-shaped mass in the temporal periphery that measured 7.7mm in height and 14.2mm in largest base diameter with heterogenous internal reflectivity ( Fig. 1 A). No intrinsic vascularity was noted on ultrasound of the lesion. MRI of the orbits demonstrated choroidal hemorrhage with an underlying mass. The mass was hyperintense on pre-gadolinium T1-weighted images ( Fig. 1 C) and did not show significant enhancement on post-contrast images ( Fig. 1 D). There was enhancement of the structures adjacent to the lesion in the lateral orbit. Computed tomography scans of the abdomen and chest were obtained to rule out choroidal metastasis from an unknown primary and were unremarkable.
Initial management included topical and oral IOP-reducing agents. Serial exams demonstrated improvement in the choroidal hemorrhage and progressive decrease in the size of the choroidal mass. At two months after presentation, his VA remained light perception, and IOP was 15 mmHg on topical IOP-lowering drops. The choroidal hemorrhage persisted, but had decreased in size. The choroidal mass height had decreased to 4mm ( Fig. 1 B). However, recurrent vitreous hemorrhage precluded adequate examination of the lesion so the decision was made to proceed with pars plana vitrectomy and transvitreous fine needle aspiration biopsy of the choroidal lesion. Intraoperative examination of the retina demonstrated widespread retinal atrophy, pigment migration, and increased pigmentation throughout the fundus. Needle biopsy was obtained with a 25-gauge needle through pars plana valved cannulas. Cytology demonstrated highly atypical melanocytic cell proliferation, consistent with a choroidal melanoma ( Fig. 1 E and F).
The patient elected to pursue enucleation. Surgical pathology demonstrated a choroidal melanoma with a basal diameter of 13mm and a maximum height of 4mm (pT2a, stage IIA). The neoplasm breached Bruch’s membrane and showed patchy but extensive growth in the suprachoroidal plane, resembling pagetoid spread ( Fig. 1 G). Bruch’s membrane was otherwise well preserved outside of the focal disruption overlying the tumor. The remaining globe contained a broad region of chronic suprachoroidal hemorrhage with rare suprachoroidal nests of melanoma cells seen at the periphery of the hemorrhage ( Fig. 1 H). No definite intravascular melanoma or thrombus formation was identified, though many choroidal vessels appeared congested and dilated. The iridocorneal angle was uninvolved by neoplasm, and there was no evidence of significant peripheral anterior synechiae formation.
Case 2: A 42-year-old man presented with three days of vision loss in his superior visual field and severe pain in the right eye. Best corrected visual acuity on presentation was 20/70 and IOP was 11 mmHg. His exam was notable for mild eyelid edema with reactive ptosis, conjunctival chemosis, diffuse non-blanching scleral injection, mild vitreous hemorrhage, and inferior subretinal and choroidal hemorrhage ( Fig. 2 A). B-scan ultrasound showed massive choroidal thickening with a faintly visible dome-shaped mass underlying the irregularly shaped choroidal hemorrhage. The mass showed a homogenous hypoechoic signal and measured 5.1mm in height and 11mm in largest base, and no intrinsic vascularity was detected ( Fig. 2 C). An MRI of the orbits was obtained. Pre-gadolinium T1-weighted imaging demonstrated hyperintensity of the mass and choroidal hemorrhage ( Fig. 2 E). Post-gadolinium T1-weighted images demonstrated enhancement of the lateral orbit, lacrimal gland, and lateral sclera. There was questionable heterogeneous enhancement of the choroidal lesion ( Fig. 2 F). The patient was managed with multiple interventions including oral steroids, non-steroidal anti-inflammatory drugs, and antibiotics for a diagnosis of posterior scleritis of unknown etiology. His pain improved, and serial B-scan ultrasound exams demonstrated progressive decrease in size of both the choroidal hemorrhage and the choroidal lesion, which measured 3.1mm in height and 9.0mm in largest base three weeks after his initial presentation (cT1a, stage IIA, Fig. 2 D). Initial CT scans of his abdomen and chest were unremarkable. One month after presentation he developed worsening vitreous hemorrhage. A B-scan ultrasound at that time showed recurrent choroidal hemorrhages and a new total retinal detachment with subretinal hemorrhage. He underwent external drainage of choroidal hemorrhage from the superonasal quadrant, vitrectomy, transvitreous biopsy of the inferotemporal choroidal lesion with a 25-gauge needle, membrane peel, fluid air exchange, endolaser, and gas tamponade. Cytology showed highly atypical melanocytic cell proliferation, consistent with malignant melanoma ( Fig. 2 G and H). The patient wished to pursue proton beam irradiation therapy. However, prior to radiation and one month after his initial surgery, he developed a recurrent total open-funnel retinal detachment. He underwent cataract extraction with intraocular lens placement, vitrectomy, membrane peel, 360-degree retinectomy, and fluid air exchange with silicone oil tamponade. Tantalum ring markers were placed at the time of his repeat retinal detachment repair to guide his radiation therapy. Three months post operatively, his tumor had regressed after proton beam irradiation, and his vision was 20/300 ( Fig. 2 B).
