Spontaneous Necrosis of a Large Choroidal Melanoma: Unusual Presentation in a 49-Year-Old Male

Abstract

Spontaneous intraocular haemorrhage can occur due to underlying malignancies, such as choroidal melanoma.• Choroidal melanomas usually demonstrate a solid dome-or mushroom-shaped low-to-medium reflective lesion with a regular internal acoustic structure on ultrasound (US), often with subretinal fluid at the margins. Colour Doppler US frequently shows pulsatile blood flow at the base of the melanoma. None of these features were observed in the case presented. • Rapid growth can occur in monosomy 3 and/or BAP1 mutant melanoma, leading to tumour necrosis, haemorrhage and secondary glaucoma. Novel Insights• A massive vitreous haemorrhage can obscure the underlying diagnosis of a large choroidal melanoma.• Atypical large choroidal melanomas can occur without any of the above-mentioned characteristics on US.• Rapid expansive growth of a choroidal melanoma does not necessarily equate a "high risk" uveal melanoma but can occur also in spindle cell melanomas with disomy 3 and in the absence of somatic BAP1 mutations. AbstractPurpose: To demonstrate a case of massive vitreous haemorrhage obscuring the underlying diagnosis of a large mixed-cell choroidal melanoma which had undergone spontaneous necrosis. Case Report: A 49-year-old man in good general health suddenly lost vision in his right eye due to an extensive vitreous haemorrhage 1 day after a workout at the gym. He reported good vision prior to that without any symptoms of flashes, floaters, or shadows. He was referred to the vitreoretinal department of a tertiary eye hospital, where he presented with a drop in vision to light perception Spontaneous Necrosis of a Large Choroidal Melanomaonly in the right phakic eye. Pars plana vitrectomy was performed in the right eye, which revealed intraoperatively massive retinal ischemia and choroidal haemorrhage, but no obvious tumour mass that could have been biopsied. The vitrectomy cassette specimen was sent for histopathology, where "ghost-like" melanoma cells were identified. The eye was subsequently enucleated, revealing an extensively necrotic and haemorrhagic choroidal melanoma of mixed cell type with only small viable tumour foci at the base and almost complete lysis of the detached retina. Conclusion: Some uveal melanomas (UMs) undergo spontaneous necrosis due to rapid growth, with the centre of the tumour outstripping its established blood supply in the "watershed area" of the eye, and becoming hypoxic with associated necrosis of intraocular structures. Such UMs are often associated with haemorrhage and/or inflammation and usually cause significant destruction of ocular tissues, resulting in enucleation as the only treatment option.

Histopathologic and MR Imaging Appearance of Spontaneous and Radiation-Induced Necrosis in Uveal Melanomas: Initial Results

“…In this regard, we would like to emphasize that the pattern of massive spontaneous necrosis described in the aforementioned previous reports [ 52 , 53 ], tends to be mainly a hemorrhagic/coagulative-type necrosis, that can be distinguished from the equally extensive radiation-induced necrosis, but not of hemorrhagic-type, typically seen in irradiated tumors.…”

“…Spontaneous focal necrosis of uveal melanoma is indeed a quite common event [ 50 ], being reported in 5.7–33% of the cases in different series [ 38 , 39 , 51 ]; nevertheless, spontaneous massive tumor necrosis (degree of necrosis ≥ 80%) is rare, ranging between 0.5% and 3.6% [ 51 , 52 ]. In patients with uveal melanoma a wide range of symptoms and clinical signs can be associated with tumor necrosis: pain, poor vision, ocular and orbital inflammation, glaucoma, retinal detachment, vitreous hemorrhage, hyphema, exophthalmos [ 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 ]. In particular, according to Thareja et al, acute pain is the most common clinical finding, being present in all patients of their study [ 53 ].…”

“…In early reports, some authors [ 50 , 59 ] postulated that the mass effect of the neoplasm would determine acute angle closure and secondary glaucoma, with a resultant increase in intraocular pressure above systemic blood pressure; this, in turn, leads to vascular compromise and ischemic necrosis of the melanoma and ocular tissues. More recently, other authors [ 52 , 53 ] focused their attention on vascular impairment as the primum movens initiating the necrotic process. In uveal melanomas characterized by particularly rapid growth, the vascular supply may become insufficient, leading to tumor necrosis.…”

“…These authors have hypothesized that when uveal melanomas outgrow their own blood supply an ischemic necrosis occurs in the central portion of the tumor with release of proinflammatory cytokines resulting in perivascular inflammation, oedema and further necrosis. In this setting, anterior displacement of the iris diaphragm often occurs, with secondary angle-closure glaucoma and increase in intraocular pressure, furtherly enhancing tumor necrosis that may broaden involving also the iris and ciliary body [ 52 , 53 ]. Then again, the association of necrosis in uveal melanomas, with intraocular inflammation (scleritis, episcleritis, uveitis, and panophthalmitis), secondary angle-closure glaucoma, and increase in intraocular pressure has long been known [ 11 , 51 , 55 , 60 ].…”

Necrosis of uveal melanoma post-COVID-19 vaccination

“…Uveal melanoma (UM) is the most common primary intraocular malignancy among Caucasian adults (five to eight per million), but less common among Asians, presenting at least a decade earlier. [ 1 2 ] Spontaneously necrotic UMs are well known, albeit extremely rare (3%–6% of UMs). [ 3 ]…”