Intraocular schwannoma: case series of 28 patients and literature review.

PURPOSE: Intraocular schwannoma is a rare tumour, which is often misdiagnosed. We presented the demographics and clinical characteristics of patients with intraocular schwannoma. METHODS: Retrospective case series were collected between May 2005 and July 2021 in Beijing Tongren Hospital. RESULTS: A total of 28 patients were diagnosed with intraocular schwannoma on histopathological examination of surgical specimen. The median age was 39 years (range: 12-64). Fourteen patients were female and 14 were male. Among the all subjects, 21/28 patients (75.0%) presented as visual loss, and 3/28 patients (10.7%) had visual field loss. Intraocular schwannoma presented as nonpigmented mass in the ciliary body in 12/28 cases (42.9%), in the choroid in 9/28 cases (32.1%), and in ciliochoroid in 7/28 cases (25.0%). Intraocular schwannoma was often clinically misdiagnosed as uveal melanoma, which occurred in 16/28 patients (57.1%). Tumour excision with pars plana vitrectomy was performed for all included patients. Endoresection with lens removal was performed for tumours in the choroid, while transscleral resection was performed for tumours located in ciliary body or ciliochoroid. Increased light transmission was detected in 12/28 cases (42.9%). In the consecutive follow-up (median: 73 months, range: 7-193 months), no cases of recurrence or metastatic disease were detected. CONCLUSIONS: Intraocular schwannoma is a rare benign tumour. It usually presents as nonpigmented mass, which can easily be misdiagnosed as nonpigmented uveal melanoma.

Rhegmatogenous retinal detachment in choroidal melanoma: clinical features and surgical outcomes.

PURPOSE: To describe the clinical features, prognostic factors, safety and rate of success of surgery and visual outcomes in patients with rhegmatogenous retinal detachment (RRD) and choroidal melanoma (CM). METHODS: A retrospective, observational case-series of 21 patients with rhegmatogenous retinal detachment or combined tractional-rhegmatogenous retinal detachment in patients with choroidal melanoma over a period of 20 years. RESULTS: Nineteen patients were included in the final analysis. The mean elevation of CM was 4.0 mm and the mean largest diameter was 11.0 mm. RRD occurred after the CM treatment in 14 eyes at a mean interval of 44.2 months. The RRD was macula-on RRD in 6 eyes, there was posterior vitreous detachment (PVD) in 15 and PVR in 7 eyes. BCVA at presentation was 0.71 logMAR and final was 1.5 logMAR (p = 0.01). The primary surgical success rate was 59%. No intraocular or extraocular tumour dissemination occurred. Mean follow-up was 66 months. CONCLUSION: RRD in patients with CM is uncommon but requires multidisciplinary management. Anatomical results are favourable but visual outcomes are poor due to a combination of factors related to melanoma treatment, macular retinal detachment and PVR. Vitrectomy as a surgical intervention for RRD in treated CM appears to be safe in terms tumour dissemination.

An immunotherapeutic artificial vitreous body hydrogel to control choroidal melanoma and preserve vision after vitrectomy.

Choroidal melanoma, a common intraocular malignant tumor, relies on local radiotherapy and enucleation for treatment. However, cancer recurrence and visual impairment remain important challenges. Here, a therapeutic artificial vitreous body (AVB) hydrogel based on tetra-armed poly(ethylene glycol) was developed to control the recurrence of choroidal melanoma and preserve vision after vitrectomy. AVB loaded with melphalan (Mel) and anti-programmed cell death ligand-1 (αPDL1), was injected after surgical resection in the choroidal melanoma mouse model. Afterwards, the sequentially released Mel and αPDL1 from AVB could achieve a synergistic antitumor effect to inhibit tumor recurrence. AVB with similar physical properties to native vitreous body could maintain the normal structure and visual function of eye after vitrectomy, which has been evidenced by standard examinations of ophthalmology in the mouse model. Thus, the immunotherapeutic AVB may be a promising candidate as an infill biomaterial to assist surgical treatment of intraocular malignant tumors.

Multifocal choroidal melanoma: a clinicohistopathological correlation.

A woman in her late 50s presented with on-and-off redness and diminution of vision in her left eye for 6 months. Her best corrected visual acuity was 20/40 in the right eye and hand motion in the left eye. Anterior segment examination revealed a greyish-white lesion extending from 3 to 6 o'clock hours posterior to the iris and protruding into the anterior chamber. Left eye B-scan ultrasonography showed a multifocal choroidal lesion, a smaller one involving the posterior pole, and a larger lesion involving the complete nasal quadrant and anteriorly extending to the ciliary body and iris. Fine-needle aspiration biopsy performed from the anterior lesion showed a possible neoplastic aetiology of melanocytic origin of the cells. Finally, the patient underwent left eye enucleation with a ball implant. Histopathological examination of the enucleated eye confirmed the final diagnosis of multifocal choroidal melanoma involving the adjacent ciliary body and iris.

Choroidal metastasis from gastric cancer: A case report and review of the literature.

Choroidal metastatic tumours from gastric cancer (GC) are rare compared with breast and lung cancer. Here, we report a patient with GC who presented to our ophthalmology clinic with a one-week history of left eye visual disorder and pain. Fundoscopic and B-scan examinations suggested a choroidal metastatic tumour. Computed tomography (CT) and magnetic resonance imaging (MRI) scans confirmed our initial diagnosis. Histopathology and immunohistochemical findings showed the tumour most likely originated from the gastrointestinal tract. Although the patient was well after eye removal, he died two months after surgery. Metastasis of GC should be a consideration when a patient with a history of GC presents with eye pain, decreased vision, and/or high intraocular pressure.

Corneal Perforation as a Rare and Late Manifestation of Choroidal Melanoma.

PURPOSE: To report a case of corneal perforation as a rare and late manifestation of choroidal melanoma and to highlight the major histopathological findings of this unusual combined clinical presentation. METHODS: A 74-year-old male patient presented to our department due to corneal perforation of the right eye with the absence of light perception for 6 months. The intraocular pressure was hard on palpation. Because of the protracted finding and reduced visual prognosis, primary enucleation was performed. RESULTS: The histopathological examination revealed choroidal melanoma with epithelioid and spindle cell components at the posterior pole, which was positive for Melan-A, Human Melanoma Black 45 (HMB45), BAP1, and SOX10. The anterior segment showed complete anterior chamber hemorrhage and blood remnants in the trabecular meshwork. The cornea displayed diffuse blood staining with hemosiderin and hemosiderin-loaded macrophages and keratocytes. No inflammatory cells were present near the corneal perforation, which had a width of 3 mm. Intraocular heterotopic ossification was indicative of a long-standing condition. Postoperative cancer staging was normal. CONCLUSION: Corneal perforation should be considered as a very rare and late manifestation of advanced choroidal melanoma and may result from interaction between intraocular hemorrhage, elevated IOP, and its secondary signs such as corneal blood staining.

Outcomes of fractionated CyberKnife radiosurgery in patients with choroidal malignant melanoma.

OBJECTIVE: The aim of this study was to evaluate local tumor control and complication development rates of fractionated CyberKnife radiosurgery (CRS) in patients with choroidal melanoma. METHODS: A total of 29 patients with choroidal melanoma were treated with fractionated CRS at Ankara Oncology Research and Training Hospital, Department of Radiotherapy between May 2009 and December 2013. Patients were treated with CRS if the initial height of the choroidal melanoma was ≥ 6 mm, or juxtapapillary and/or juxtamacular tumors with a height of >2.5 mm. Ophthalmic examinations were performed at baseline and at months 3, 6, 9 and 15 after radiotherapy. Assessment of visual acuity and measurement of tumor base dimension and height using A-scan and B-scan echography were done at each visit. RESULTS: The mean age was 56 (27-75) years. Tumor was located on choroid in 23 and on ciliochoroid in 6 patients. 86.2% of all melanomas were classified as medium sized and 23.8% as large sized. A median total dose of 5000 cGy was applied. Median tumor height decreased from 7.5 mm at baseline to 4.4 mm at the last follow-up visit (p < 0.001). Median visual acuity decreased from 0.4 at baseline to hand motion (p < 0.001). One patient had been lost to the metastatic disease and one patient had been treated with enucleation due to recurrent tumor growth. CONCLUSION: CRS is an effective and reliable local treatment modality in uveal melanoma.

[Metastatic clear cell renal cell carcinoma: a potential mimicker of choroidal melanoma]. / Világossejtes vesesejtes karcinóma koroideális melanómát utánzó áttéte.

While metastases are the most common intraocular malignancies, ocular metastases of renal cell carcinoma are rare. The most frequent primary malignancy of the eye is uveal melanoma. The common ocular localization is the choroid in both cases. The clinical differentiation of choroidal metastasis from renal cell carcinoma and choroidal melanoma malignum is a diagnostic challenge for the ophthalmologist. We present two cases where renal cell carcinoma had metastasized to the choroid. Enucleation was performed in a 61- and a 71-year-old male patient with suspected advanced choroidal malignant melanoma following biomicroscopic and B-scan ultrasonography examination. Histopathological examination confirmed clear-cell renal cell carcinoma in both cases. The clinical and ultrasonographic appearance of clear-cell renal cell carcinoma metastasis may mimic choroidal malignant melanoma, and may only be suspected if a primary renal cell carcinoma is already established.

Robotic CyberKnife radiosurgery for small choroidal melanomas.

Plaque brachytherapy is the most common procedure for the treatment of small choroidal melanoma, especially in posteriorly located tumors. However, there is only little information on outcome after treatment with stereotactic radiosurgery, for example, CyberKnife radiosurgery. We reviewed patients with choroidal melanoma (maximum tumor height 4 mm) treated with CyberKnife radiosurgery. Demographic information, tumor dimension, complications, metastasis and overall survival during the whole follow-up were tracked and analyzed with a specific focus on local tumor control and potential risk factors. One hundred eighty-eight patients (102 female, 54.2%) with a median age of 63 years [interquartile range (IQR): 54-73 years] were analyzed over a median of 46 months (IQR: 24-62 months). Metastasis occurred in 14 patients (7.4%) in median of 18 months after treatment (IQR: 13-47 months) and survival was achieved in 178 patients (94.7%). Within the observation period, eye retention was observed in 166 patients (88.3%). Superior local control was achieved in patients who received a prescription dose of at least 21 Gy or more (91.6%; P = 0.04). Other potential risk factors incrementing local control were treatment planning with missing MRI in advance and too small target delineation during the planning process. Radiosurgery (CyberKnife) in a single, outpatient procedure is suitable for the treatment of small choroidal melanoma. We found local control rate after 3 years compared to the standard treatment with plaque brachytherapy. On the study side, 15 years of CyberKnife treatment allowed to identify risk factors that might increment local control and thus improve treatment regimens.

Very late orbital recurrence of choroidal melanoma four decades post enucleation.

Orbital recurrence of malignant choroidal melanoma is quite uncommon, occurring in about 3% of patients undergoing enucleation for large tumors. Orbital recurrences after more than 10 years from enucleation are even rarer. In literature, only few reports described orbital recurrence that occurred between 10 and 40 years after primary diagnosis. Herein we report a very late choroidal melanoma recurrence, 40 years post enucleation, of a 52 year-old female who had undergone left enucleation at the age of 12. She presented to our clinic for consultation in 2017, complaining of difficulty to contain the prosthesis, as well as, presence of small pigmented palpable nodules inferiorly in her anophthalmic socket. The patient was not aware of the medical condition which had lead to the enucleation. We requested her medical reports and detailed history through her family. We realized after reviewing her charts 40 years back, that her enucleation was due to malignant choroidal melanoma (CMM). Full screening was performed. After magnetic resonance imaging (MRI), that showed the presence of nodular masses in the anophthalmic socket, an excisional biopsy was performed. Histopathology confirmed the diagnosis of CMM (epithelioid and spindle cell type), supporting the hypothesis that residual melanoma cells may remain clinically dormant for long periods, even for decades. A literature review was performed in order to review similar cases and to understand and discuss multiple factors, which may explain this extremely delayed recurrence. To the best of our knowledge, this is the third case to be reported in the literature.

Venous-air embolism during vitrectomy for endoresection of choroidal melanoma: Case report.

INTRODUCTION: The purpose of this study is to report a case of venous-air embolism during a vitrectomy for endoresection of choroidal melanoma. CASE DESCRIPTION: A 31-year-old man went to the clinic because of photopsias and vision loss in his right eye. On fundoscopy of the right eye, a choroidal mass with an associated retinal detachment was found near the inferotemporal vascular arcade. Multimodal imaging was performed and diagnosis of choroidal melanoma was made. Metastatic workup ruled out systemic extension. The patient underwent pars plana vitrectomy for endoresection of the lesion. During the application of laser under air, he started complaining of chest pain and dyspnea. He presented signs of supraventricular tachycardia, tachypnea, hypotension and oxygen desaturation. He was managed with orotracheal intubation, bronchodilators and vasopressor support, and stabilization was achieved. He was discharged 2 days after with no sequalae. After 1-year of follow-up, the patient has a visual acuity of counting fingers and no signs of tumor recurrence or systemic extension. CONCLUSIONS: Although rare, vitreoretinal surgeons should be aware of this potentially fatal complication and take steps to prevent it.

Vitreoretinal Surgery in the Prevention and Treatment of Toxic Tumour Syndrome in Uveal Melanoma: A Systematic Review.

Toxic tumour syndrome (TTS) is a particularly aggressive form of secondary vasculopathy occurring after radiation therapy of uveal melanoma due to the persistence of the necrotic tumour mass inside the eye. The development of TTS confers a particularly unfavourable functional and anatomical ocular prognosis, ultimately requiring enucleation in most cases if untreated. Vitreoretinal (VR) surgery has been successfully applied for treatment and prevention of TTS using both resecting and non-resecting techniques. In this systematic review, we aim to define characteristics of uveal melanomas benefiting the most from secondary VR surgery and to outline the optimal type and timing of VR intervention in such cases. Analysis of the literature reveals that endoresection should be performed within 3 months after radiotherapy to tumours thicker than 7 mm and with a largest basal diameter between 8 mm and 15 mm with post-equatorial location, especially after proton beam treatment. Alternatively, endodrainage remains a valid therapeutic option in eyes with macula-off retinal detachment, tumour diameter larger than 15 mm or ciliary body involvement. VR surgery can be successful in the management of TTS following radiotherapy for uveal melanoma when timing and indication are appropriately evaluated.

Case Report: Two Genetically Distinct Choroidal Melanomas in the Same Eye Treated with Endolaser Therapy.

SIGNIFICANCE: This case report highlights the merits of using fine needle aspiration biopsy to obtain gene expression profiling of individual choroidal melanomas when more than one tumor arises in the same eye. It is also the first such case to document laser ablation therapy as the primary treatment. PURPOSE: This report describes a case of two primary choroidal melanomas with different genetic profiles in the same eye. CASE REPORT: An 80-year-old man presented to the office with a neoplasm of uncertain behavior in the left eye. The patient's visual acuity and IOP in the left eye, respectively, at the time of his first visit to the office were 20/25 and 8 mmHg. A dilated fundus examination revealed that there were two choroidal lesions in the left eye. The macular lesion was classified as type 1A, and the ciliochoroidal lesion was classified as type 1B. The patient underwent a vitrectomy of the left eye, followed by endolaser ablation of the tumors. The patient was also injected with bevacizumab. To date, the patient is free of known metastasis. Most recently, his visual acuity and IOP in the left eye were 20/30 and 14 mmHg, respectively. CONCLUSIONS: Although rare, multiple melanomas in the same eye may have differing genetic profiles, which may alter prognosis and management, depending on the class of tumor detected.

Ipsilateral orbital metastasis following enucleation of intraocular ciliochoroidal melanoma: a rare occurrence.

This case report demostrates an unusual occurence of orbital metastasis along with liver metastasis 5 years following enucleation for ciliochoroidal melanoma with no extraocular extension. It exemplifies that metastasis of the tumour can occur in the ipsilateral orbit after enucleation. Importance of close clinical follow-up after enucleation for choroidal melanoma, careful socket examination, and regular systemic metastatic workup is demonstrated. To the best of our knowledge, this is the first case report of uveal melanoma with ipsilateral orbital metastasis.

Evaluation of Choroidal Melanoma Vascularization by Color Doppler Flow Imaging: An Option for Follow-Up Tumor Control Assessment after CyberKnife®?

Background and Objectives: Thus far, tumor control for choroidal melanoma after teletherapeutic radiation is clinically difficult. In contrast to brachytherapy, the tumor height does not necessarily have to shrink as a result of teletherapy. Therefore, the objective of this study was to evaluate tumor vascularization determined by color Doppler flow imaging (CDFI) as a possible approach for monitoring the therapy response after teletherapy of choroidal melanoma. Materials and Methods: A single-center retrospective pilot study of 24 patients was conducted, all of whom had been diagnosed with choroidal neoplasm, treated and followed up. Besides tumor vascularization, the following parameters were collected: age, gender, tumor entity, location, radiation dose, knowledge of relapse, tumor height, radiation-related complications, occurrence of metastases, visual acuity in logMAR. Results: The level of choroidal melanoma vascularization markedly decreased in all included subjects after treatment with the CyberKnife® technology. Initially, the level of vascularization was 2.1 (SD: 0.76 for n = 10); post-therapeutically, it averaged 0.14 (SD: 0.4). Regarding the tumor apex, CDFI sonography also demonstrated a significant tumor regression (mean value pre-therapeutically: 8.35 mm-SD: 3.92 for n = 10; mean value post-therapeutically: 4.86 mm-SD: 3.21). The level of choroidal melanoma vascularization declined in the patient collective treated with ruthenium-106 brachytherapy. The pre-therapeutic level of vascularization of 2 (SD: 0 for n = 2) decreased significantly to a level of 0 (mean: 0-SD: 0). The tumor height determined by CDFI did not allow any valid statement regarding local tumor control. In contrast to these findings, the patient population of the control group without any radiation therapy did not show any alterations in vascularization. Conclusions: Our data suggest that the determination of the tumor vascularization level using CDFI might be a useful and supplementary course parameter in the follow-up care of choroidal melanoma to monitor the success of treatment. This especially applies to robot-assisted radiotherapy using CyberKnife®. Further studies are necessary to validate the first results of this assessment.

Unusual presentation of a choroidal melanoma.

A 49-year-old gentleman presented with a 1-day history of painful deterioration of vision in his right eye. He was lifting heavy weights just before this visual loss. On examination, his vision was perception of light in that eye, with a medically uncontrollable high intraocular pressure and hyphaema almost completely filling his anterior chamber. An ultrasound performed suggested a working diagnosis of a Valsalva related 'eight ball' bleed. After an anterior chamber washout, he had another episode of bleeding three days later. A pars plana vitrectomy was performed and the sample obtained was sent off to the laboratory, revealing a choroidal melanoma. Following enucleation, the histology confirmed a large necrotic spindle cell melanoma, with a haemorrhagic background. Fortunately, no liver metastasis was found. Since his diagnosis 3 years ago, he continues to be followed up in an artificial eye clinic and has regular liver scans under the care of his oncologist.

Transretinal biopsy via 23-gauge pars plana vitrectomy for retinal and choroidal tumors: cytopathological results, surgical complications, and patient outcomes.

PURPOSE: To evaluate the cytopathological results, surgical complications, and patient outcomes after transretinal biopsy (TRB) for diagnosis of retinal/choroidal tumors METHODS: Records of 40 cases who underwent TRB via 23-gauge (23 G) pars plana vitrectomy between March 2011 and March 2020 were reviewed. STUDY DESIGN: Retrospective. RESULTS: Twenty-six (65.0%) cases were women and 14 (35.0%) were men. The mean age at diagnosis was 57.2 (range: 18-83) years. The mean tumor base diameter was 12.0×9.8 mm and the mean tumor thickness was 4.9 mm. According to cytopathological examination, 29 (72.5%) cases had choroidal melanoma, 2 (5.0%) had non-small cell lung cancer metastasis, 1 (2.5%) had adenoma of retinal pigment epithelium, 1 (2.5%) had small cell lung cancer metastasis, 1 (2.5%) had invasive breast cancer metastasis, 1 (2.5%) had retinal astrocytic hamartoma, and 1 (2.5%) had pseudoneoplastic gliosis. Cytopathological examination of 4 (10.0%) cases revealed findings consistent with macular and extramacular degeneration. Postoperative complications were mild vitreous hemorrhage in 16 (40.0%) cases, gradually worsening cataract in 4 (11.8%), retinal detachment in 1 (2.5%), hyphema in 1 (2.5%), glaucoma in 1 (2.5%), and macular hole in 1 (2.5%). During the mean 11.1 (range: 1-55) months follow-up, 1 (3.4%) patient with choroidal melanoma developed liver metastasis. All patients were alive at the end of follow-up. CONCLUSIONS: TRB using 23 G pars plana vitrectomy can be used to make the cytopathologic diagnosis of retinal/choroidal tumors whenever the clinical diagnosis is not certain or in cases with known diagnosis to obtain information on cell type/cytogenetics. In our series, the most common diagnosis after cytopathologic examination was choroidal melanoma.

Unusual Tumours Hidden in Blind Eyes.

INTRODUCTION: This case report describes 3 cases of unsuspected neoplasms in previously blind eyes, with recent onset pain. Cases and observations: Case 1: Female with pain, redness in the non-seeing right eye (R/E) for two months. R/E had total cataract, low intraocular pressure and a well-defined globular mass lesion at the posterior pole, seen on ultrasound. Enucleation with an implant was done. Histopathology clinched the diagnosis of choroidal melanoma. Case 2: A 20-year male, developed pain, redness in left eye (L/E) for two months. L/E was blind since childhood, secondary to trauma. The patient underwent enucleation and detailed histopathological examination and immunohistochemistry supported a diagnosis of ependymoma with vascular malformation. Case 3: A 24-year male with pain, redness in L/E for six months, with decrease in size of eyeball. L/E had low vision since childhood. On examination, L/E was phthisical with diffuse conjunctival congestion, band-shaped keratopathy, cataract, and neovascularization of iris. Imaging revealed a small distorted globe with highly reflective mass along the posterior pole. Histopathology of the enucleated specimen confirmed the diagnosis of choroidal osteoma, with gliosis of the adjacent RPE. CONCLUSION: In the management of a painful blind eye, it is extremely important to rule out an intraocular malignancy, particularly in patients with recent onset of pain.

Ocular PEComas are frequently melanotic and TFE3-translocated: report of two cases including the first description of PRCC-TFE3 fusion in PEComa.

Ocular perivascular epithelioid cell tumor (PEComa) is exceedingly rare. We reported two examples involving the choroid and subconjunctival tissue, respectively, in patients aged 17 and 20 years. Both tumors comprised packets and sheets of large polygonal cells with moderately pleomorphic nuclei and prominent nucleoli, traversed by delicate fibrovascular septa. Melanin pigmentation was present in one case. The tumors showed HMB45 and TFE3 immunoreactivity. TFE3 gene translocation was confirmed by FISH break-apart probes. RNA seq revealed PRCC-TFE3 and NONO-TFE3 fusions, with the former representing the first description of PRCC-TFE3 in PEComa. Critical reappraisal of the reported cases showed that ocular PEComa frequently affected young patents with melanin pigmentation, frequent TFE3 protein expression, and/or TFE3 gene translocation. No recurrence or metastasis was reported after complete excision despite the presence of cytologic atypia.