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Case 1. a Gross appearance of the enucleated eye with a dome-shaped melanoma ( * ) straddling the equator and an associated complete retinal detachment. b Ischemic necrosis of the ciliary body demonstrated by smudgy, pale staining and clumping of the iris pigment epithelium. c 90% of the tumor exhibits ischemic necrosis with associated hemorrhage ( * ), and there is a microscopic rim of the intact tumor (arrow) at the periphery of the melanoma near the sclera. d The necrotic tumor consists of melanoma ghost cells with pale-staining cytoplasm and nuclei that occasionally exhibit basophilia. b , c HE. ×25. d HE. ×100.
Source publication
Background/aims:
The purpose of this study was to examine the clinical presentations and pathological features of spontaneously necrotic choroidal melanomas.
Methods:
The clinical and histological features of patients who underwent enucleation for uveal melanoma from 1989 to 2012 at Emory University and were found to have spontaneously necrotic...
Citations
... Pain at presentation is a feature strongly suggestive of spontaneous necrosis of choroidal melanoma. [6] Other suggestive findings that favor rapid growth and necrosis in our case included prior ultrasonographic evidence of a 7.0-mm-thick tumor that shrunk to 3.1 mm thickness, central excavation on fundoscopy and ultrasonography, and overlying retinoschisis at the site of central depression demonstrated by OCT. ...
... [1] This entity can masquerade as non-neoplastic scleritis and uveal effusion with pain and inflammation, often delaying the diagnosis. [1][2][3]5,6] A high index of suspicion is warranted for a thorough evaluation in the setting of scleritis with suspicious choroidal thickening. Ancillary investigations such as OCT, ultrasonography, and MRI can aid in arriving at an appropriate diagnosis in the presence of equivocal clinical features. ...
... This necrosis-related inflammation can lead to subsequent involvement of the underlying sclera and adjacent choroid, resulting in clinically significant scleritis and serous choroidal detachments, as was noted in our patients and resolved with treatment of the underlying tumor with concomitant corticosteroid administration in 2 patients. It has been hypothesized that tumor ischemia can result from watershed infarctions occurring between the anterior and posterior choroidal circulations in choroidal tumors centered around the equator [11]. While most hypotheses on the necrosis of choroidal tumors have been formulated in the setting of choroidal melanomas, a similar mechanism can be inferred to occur in the setting of choroidal metastasis undergoing rapid growth. ...
Purpose:
The aim of the study was to describe choroidal detachments and concurrent scleritis associated with necrotic choroidal metastasis or melanoma.
Methods:
We conducted a retrospective case series.
Results:
We report 4 patients with scleritis and choroidal detachment with an underlying malignant choroidal tumor. All patients underwent fine-needle aspiration biopsy for cytopathologic characterization of their choroidal tumor, and they all demonstrated evidence of tumor necrosis. Two patients were diagnosed with choroidal metastasis from lung and esophageal adenocarcinoma. Both patients ultimately expired from systemic metastasis. The remaining 2 patients were diagnosed with choroidal melanoma and were successfully treated with plaque radiotherapy.
Conclusion:
Choroidal detachment with concurrent scleritis can occur as a rare sequelae of tumor necrosis of an underlying choroidal malignancy.
... 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 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]. Moreover, in some case reports and small case series, severe ocular pain was almost always associated with necrotic uveal melanomas [11,58]. ...
... 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. ...
Simple Summary
Uveal melanomas may undergo necrosis, both spontaneously or following radiotherapy. Nowadays radiotherapy is the preferred treatment, whereas enucleation of the eye is used in selected cases. In order to differentiate the effects of radiotherapy from spontaneous degenerative changes in uveal melanomas, we compared the appearance of necrosis, both from a histopathological point of view and from the perspective of MR imaging, in two groups of patients with uveal melanoma: a group who had undergone previous proton beam radiotherapy (secondary enucleation); a control group who had undergone enucleation without any previous radiotherapy treatment (primary enucleation). Irradiated and nonirradiated uveal melanomas differ on the basis of the histological appearance, the MR imaging appearance and the distribution of necrosis. We hope that the findings we observed could be extended to all patients with uveal melanomas treated with radiotherapy, and may enhance the accuracy of radiologists in evaluating MR examinations after radiotherapy.
Abstract
Necrosis in uveal melanomas can be spontaneous or induced by radiotherapy. The purpose of our study was to compare the histopathologic and MRI findings of radiation-induced necrosis of a group of proton beam-irradiated uveal melanomas with those of spontaneous necrosis of a control group of patients undergoing primary enucleation. 11 uveal melanomas who had undergone proton beam radiotherapy, MRI and secondary enucleation, and a control group of 15 untreated uveal melanomas who had undergone MRI and primary enucleation were retrospectively identified. Within the irradiated and nonirradiated group, 7 and 6 eyes with histological evidence of necrosis respectively, were furtherly selected for the final analysis; the appearance of necrosis was assessed at histopathologic examination and MRI. Irradiated melanomas showed a higher degree of necrosis as compared with nonirradiated tumors. Irradiated and nonirradiated lesions differed based on the appearance and distribution of necrosis. Irradiated tumors showed large necrotic foci, sharply demarcated from the viable neoplastic tissue; nonirradiated tumors demonstrated small, distinct foci of necrosis. Radiation-induced necrosis, more pigmented than surrounding viable tumor, displayed high signal intensity on T1-weighted and low signal intensity on T2-weighted images. The hemorrhagic/coagulative necrosis, more prevalent in nonirradiated tumors (4 out of 6 vs. 1 out of 7 cases), appeared hyperintense on T2-weighted and hypointense on T1-weighted images. Our study boosts the capability to recognize radiation-induced alterations in uveal melanomas at MRI and may improve the accuracy of radiologists in the evaluation of follow-up MR examination after radiotherapy.
... This leads to ischemic necrosis of the tumor, release of cytokines and tumor swelling with further necrosis, anterior displacement of the lens iris diaphragm, and secondary glaucoma which potentiates further tumor necrosis. [4] In our case, localized scleritis is most likely due to rapidly growing large tumor which has outstripped its own blood supply. Alternatively, Brannan et al. suggested that unknown mechanism triggers tumor necrosis, release of cytotoxic products and the resulting vasculitis leads to infarction, swelling, and cellulitis of ocular and extraocular tissues. ...
We report a case of a patient who presented with symptoms of acute onset scleritis in the left eye but was found to have an underlying necrotic choroidal melanoma. Asymptomatic choroidal melanoma can undergo spontaneous necrosis and manifest as severe scleritis and should be considered as an important differential.
... Choroidal melanomas can rarely undergo spontaneous necrosis: this occurs in about 0.5-1% of all UMs [4]. We report a case of unusual presentation of a large choroidal melanoma of mixed cell type which showed atypical US findings. ...
... Spontaneous necrosis of choroidal melanomas is very rare, varying between 0.5 and 1% of all UMs [4]. The incidence of totally necrotic UMs has been reported at 3.6% and that of partially necrotic UMs as 5.7% in previous studies [7]. ...
... The incidence of totally necrotic UMs has been reported at 3.6% and that of partially necrotic UMs as 5.7% in previous studies [7]. These tumours are usually associated with significant haemorrhage and/or inflammation [4,8]. In 6 cases of spontaneous necrosis in UM examined by Thareja et al. [4], > 90% of the UM mass was necrotic in all cases, with only viable cells being present at the basal edges. ...
Purpose:
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 only 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.
... Regarding the pathophysiology underlying orbital inflammation in necrotic uveal melanoma, it has been hypothesized that as the tumor outgrows its vascular source, it can undergo necrosis as a result of hypoxia in the watershed area in the center of the tumor. 4,5 The process of necrosis in turn releases cytokines and other inflammatory mediators inciting inflammation and features mimicking orbital cellulitis. 4 In fact, an association between high levels of macrophages in uveal melanoma and a larger tumor size has been reported. ...
Purpose:
To report a case of necrotic uveal melanoma presenting as orbital cellulitis with an intraocular hemorrhage.
Observations:
A 61 year-old non-verbal male presented with a two-week history of right eyelid swelling and erythema unresponsive to antibiotics. In addition to these signs of orbital cellulitis, he presented with an opaque media precluding fundus visualization. He was later found to have a collar-button shaped mass consistent with uveal melanoma on B scan ultrasonography during an exam under anesthesia. The patient underwent enucleation with histopathology confirming a necrotic uveal melanoma.
Conclusion and importance:
This case demonstrates how necrotic uveal melanoma can present as orbital cellulitis and the importance of keeping the diagnosis on the differential.
... Frasier and Font [4] found that only 23% of uveal melanomas associated with inflammation were predominantly spindle cell type; most tumors were listed as having an epithelioid, mixed, or 'necrotic' cell type. The spontaneous necrosis of uveal melanomas is thought to develop due to the melano- 142 ma outgrowing its blood supply centrally in a watershed area [10] . In this case, the residual viable tumor persisted along prominent tumor vessels. ...
Background/aims:
To report a case and the unique histopathology of a necrotic uveal melanoma mimicking advanced Coats' disease in a young adult.
Method:
A 26-year-old male presented with a blind, painful eye, total exudative retinal detachment, and bulbous aneurysms consistent with Coats' disease. No masses were visualized on ultrasound or CT scan, and the patient underwent enucleation of the eye.
Results:
Histopathology of the involved eye confirmed a necrotic uveal melanoma with persistent spindle cells forming a collar around residual tumor vessels.
Conclusion:
Careful consideration is needed in approaching any patient with a blind, painful eye and opaque media, even in younger populations.
A 19-year-old girl presented with severe pain and redness in her right eye and also with nasal congestion and epistaxis since the past 5 months. The patient was systemically investigated and tested positive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR-3 ANCA) with raised erythrocyte sedimentation rate (ESR). The provisional diagnosis of granulomatosis with polyangiitis (GPA) was made, and she was started on systemic steroids and azathioprine after consultation with the rheumatologist. However on steroid taper, she developed severe reactivation of the scleritis and the corneal involvement was noted in the form of peripheral ulcerative keratitis. Instead of starting another course of high dose oral cortico-steroids or pulse cyclophosphomide, she was started on rituximab infusion (two doses), and oral methotrexate was added, leading to the successful remission of the disease.
