A Case Series of Choroidal and Orbital Neuroendocrine Tumors: Metastasis: Two Patients Treated With Peptide Radionuclide Therapy.

ABSTRACT: Neuroendocrine tumors (NETs) are rare cancers with heterogeneous histologies, response to treatments, and prognoses. Majority of these cancers originate in the gastrointestinal tract and metastasize to the liver. We report the cases of 5 patients with low-grade NET disease with rare metastases to the choroids. Two of the patients were treated with peptide receptor radionuclide therapy (lutetium 177 [ 177 Lu]). This is the first report confirming peptide radionuclide therapy safety in patients with low-grade NET with ocular metastases.

Multimodal evaluation of osteosarcoma choroidal metastasis.

BACKGROUND: Osteosarcoma (OS) is the most common primary bone carcinoma. Adulthood most frequent intraocular malignant tumor is choroidal metastasis; however, these are rarely related to sarcomas. There are only two OS-related choroidal metastasis cases reported in the literature, both prior to 1970. CASE PRESENTATION: A 20-year-old man with a history of tibial OS, right leg amputation, and lung and brain metastases, presented with decreased vision in his right eye (OD). Ophthalmic examination revealed a best-corrected visual acuity of hand movements and a large, posterior pole, nodular, subretinal mass, with associated fluid. B-scan revealed a heterogeneous lump, with medium/high reflectivity, and a height-to-base ratio (HBR) of 1-1.2, approximately. Computerized tomography (CT) scan showed a hyperdense and contrast-enhanced mass, while on magnetic resonance imaging (MRI) the lesion appeared T1-isointense and T2-hypointense. CONCLUSION: Choroidal OS metastasis can appear as a pink nodule with high HBR and intralesional hyperreflective deposits. Sudden visual changes in individuals with OS-related systemic metastatic disease should be monitored closely by ophthalmology and oncology jointly.

Advanced lung adenocarcinoma detected by choroidal metastasis in a patient with amyopathic dermatomyositis: A case report.

A 63-year-old Japanese man with amyopathic dermatomyositis treated with immunosuppressants became aware of distortion of his left visual field, and a metastatic choroidal tumor was suspected. His chest computed tomography (CT) showed a pulmonary nodule in the right upper lobe and mediastinal lymphadenopathy, and he was diagnosed with advanced lung adenocarcinoma with choroidal metastasis. Malignancies associated with dermatomyositis (DM) are often rapidly progressive and, in choroidal metastasis associated with lung cancer, a choroidal lesion is often diagnosed prior to lung cancer; therefore, CT performed at the time of diagnosis of choroidal metastasis may show lung cancer lesions. When ocular symptoms are observed in DM patients, metastatic malignancies should be suspected, and systemic examinations, such as positron emission tomography (PET)-CT, should also be performed.

Choroidal metastasis in pancreatic adenocarcinoma.

Pancreatic adenocarcinoma is the seventh largest cause of death from cancer with a death rate of 3.8%. The 5-year survival rate is only 5%. We report A case 28 year old male presented with a 3 week history of pain in upper part of abdomen and dyspepsia of similar duration. After 2 cycles of palliative chemotherapy he presented with diminution of vision in right eye and watering of eye. USG showed retinal detachment with vitreous hemorrhage in the right eye and left eye was normal. On fundoscopy choroidal metastasis was detected as an accidental finding in the right eye. Though Ca Pancreas usually presents in as locally advanced or metastatic disease choroidal metastasis are extremely rare. Despite disappearance of ocular metastasis he had a progressive disease and died of intraparenchymal hemorrhage. Reports of pancreatic cancer with metastasis to the choroid and optic nerve have been rare. There were few reports that demonstrated the significance of a choroidal lesion as the initial clinical sign of pancreatic cancer. This aggressive behavior of the lesion may be an important feature to determine the origin of the tumor. Cancer of the tail of the pancreas is often not detected in the early stages before metastasizing.

Choroidal metastasis with retinal detachment: A case report.

RATIONALE: Breast carcinoma is the most common primary source of choroidal metastasis (CM). In the present case, esophageal cancer was the primary tumour, brain metastasis occurred, and CM occurred later in the left eye with 2 retinal detachments, which is very rare. PATIENT CONCERNS: A 62-year-old man complained of a sudden decrease in visual acuity consisting of a small shadow in front of his left eye with a sensation of covered vision after 1 cycle of systemic chemotherapy and radiotherapy for resected esophageal cancer with brain metastasis. Fundus examination revealed exudative retinal detachment without retinal tears. CM with exudative retinal detachment was also considered. The patient refused further treatment. After the second cycle of chemotherapy, there were no significant changes in the retina and visual acuity improved. However, after craniocerebral surgery for brain metastasis, the visual acuity decreased again and showed 3 choroidal masses with macular involvement and retinal detachment but without retinal tears. DIAGNOSIS: The final diagnosis was CM with retinal detachment. INTERVENTIONS: The patient was advised to undergo enucleation of the left eye during the second retinal detachment, but he refused. OUTCOMES: Two months after the second retinal detachment, the patient died of systemic metastases. LESSONS: It is important to consider CM when the first retinal detachment and known cancer are diagnosed. At present, it is necessary to develop a standardised treatment plan as well as a multidisciplinary approach to early diagnosis, combined treatment, and timely intervention for such cases.

Sublingual Gland Carcinoma Revealed by Choroidal Metastasis.

A 65-year-old man presented with a 1-week history of left eye distortion. An elevated choroidal lesion covering 6 disc diameters was found in the posterior retina of the left eye. Systemic examination revealed sublingual gland carcinoma and multiple lung metastases, and the diagnosis was choroidal metastasis from sublingual gland carcinoma. Following chemotherapy and radiation therapy, the choroidal lesion shrunk and the patient's visual acuity improved. The patient died 23 months after his first visit. To the best of our knowledge, this is the first reported case of choroidal metastasis from sublingual gland carcinoma.

Clinical and ultrasonographic features of choroidal metastases based on primary cancer site: Long-term experience in a single center.

INTRODUCTION AND PURPOSE: Choroidal metastases (CM) are the most common intraocular malignancies. With longer survival rates for cancer patients, CM will be increasingly encountered. We evaluated clinical and ultrasonographic (US) characteristics of CM in order to identify diagnostic biomarkers that correlate with the primary tumor site. METHODS: The medical records of all patients with CM evaluated at the Ocular Oncology Unit between February 2010 and March 2020 were analyzed. RESULTS: 82 eyes of 70 patients were included. The primary cancer site was lung in 26 patients (37%), breast in 23 (33%), kidney in 9 (13%), gastrointestinal in 5 (7%), thyroid in 5 (7%), parathyroids and prostate respectively in 2 (3%). Fifty-five patients (78%) had other systemic metastases at the time of ocular diagnosis. Ten (14%) patients had no history of primary cancer. Bilateral CM were found in 20 patients (29%); fifty-six eyes (68%) had a single CM. The epicenter of CM was predominantly macula (43 eyes, 52%). The mean thickness was 4,1 mm (range 1,8-12,3). US structure was inhomogeneous in 67 eyes (82%). Reflectivity was mainly medium (39%) and medium-low (39%). In particular, CM from lung cancer showed lower reflectivity than those from the breast (p = 0,02). CM deriving from lung cancer were typically dome-shaped, whereas CM originating from breast were characteristically plateau shaped (p = 0,02). Seventy-four (91%) eyes presented fluid on optical coherence tomography. CONCLUSION: We significatively found that CM from lung cancer generally appear dome-shaped with medium-low internal reflectivity, whereas those from breast cancer typically present a plateau appearance and higher internal reflectivity. Though it is hard to identify the site of the primary tumor relying exclusively on clinical and US aspects, morphology and internal reflectivity can be considered as diagnostic biomarkers. Thus, the origin of the primary tumor can be suspected by integrating a constellation of findings.

Renal cell carcinoma choroidal metastasis recorded by smartphone with interface eyepiece adapter mounted on slit lamp: A case report.

RATIONALE: Ocular metastasis of renal cell carcinoma (RCC) is rare, and mainly located on the choroid. We report a choroidal metastasis from RCC, which was recorded by a smartphone with an interface eyepiece adapter mounted on a slit lamp. PATIENT CONCERNS: A 45-year-old female presented with 1-month history of painless occlusion of the vision field on the left eye, who had undergone right nephrectomy for RCC 19 months ago. DIAGNOSES: A smooth, hemispherical and brown protrusion was found behind the pupil nasally. An enhanced computed tomography scan of the orbit showed a slightly high-density hemispherical nodule involving the nasal portions of the left eyeball, the enhancement of the lesion was obvious and homogeneous. A metastatic choroidal space-occupying lesion from RCC was highly suspected according to the clinical and radiological findings. INTERVENTIONS: The patient was advised to undergo further treatment, such as radiotherapy. OUTCOMES: The images of choroid metastasis were recorded by the smartphone with the interface eyepiece adapter mounted on the slit lamp handily. CONCLUSIONS: The smartphone with an interface eyepiece adapter mounted on the slit lamp can be widely used to record the precious images in the clinic in a timely manner.

Choroidal neuroendocrine neoplasia in a dog.

OBJECTIVE: To report onset and progression of clinical signs of a neuroendocrine neoplasm (NEN) presumed metastatic to the choroid in a dog. ANIMALS STUDIED: A 7.5-year-old female spayed German shepherd dog mix referred for advanced imaging and evaluation of a subretinal mass in the right eye. PROCEDURES: Procedures performed included general physical and ophthalmic examinations; ocular, orbital, and abdominal ultrasonography; thoracic radiographs; cranial magnetic resonance imaging; serologic testing for infectious agents; analysis of hematologic as well as serum and urine biochemical parameters; echocardiography; electrocardiography; cytologic assessment of lymph nodes; and histopathology and immunohistochemistry of the enucleated globe. RESULTS: Examination and imaging identified a pigmented mass within and expanding the superior choroid. Following enucleation, a choroidal NEN with tumor emboli in scleral blood vessels was diagnosed by histopathologic assessment and confirmed by immunohistochemical labelling. Despite extensive and repeated diagnostic testing over many months, a putative primary site was not identified until 19 months after the initial ocular signs were noted. At that time, a heart-base mass and congestive heart failure were highly suggestive of a chemodectoma. CONCLUSION: This comprehensive report of a NEN presumed metastatic to the choroid in a dog suggests that ocular disease can be a very early and solitary sign of NEN in the dog.

Uveal Melanoma Metastasis to the Contralateral Eye Structures: A Retrospective Comparative Analysis of 13 Consecutive Patients.

PURPOSE: To report uveal melanoma (UM) metastasis to the contralateral ocular and periocular structures. DESIGN: Retrospective study. PARTICIPANTS: Thirteen patients with UM metastasis to the contralateral ocular and periocular structures were included. METHODS: Clinical records were reviewed retrospectively. MAIN OUTCOME MEASURES: The development and time to onset of contralateral ocular and periocular metastasis, systemic metastasis, and death. RESULTS: Of the 13 000 treated UM patients, 13 patients were diagnosed with UM metastasis to the contralateral ocular and periocular structures. Mean patient age at primary UM diagnosis was 60 years (median, 60 years; range, 37-87 years). The primary uveal melanoma was in the choroid (n = 11) or ciliary body (n = 2) and was treated with brachytherapy (n = 11), proton beam radiotherapy (n = 1), or enucleation (n = 1). Systemic metastasis developed in 11 patients (85%) at a mean of 66 months (median, 34 months; range, 12-216 months) after diagnosis of the primary UM. All 11 patients (100%) showed liver metastasis and 8 patients (62%) also showed extrahepatic metastasis. The sites of metastasis to the contralateral ocular or periocular structures included the choroid in 4 patients (31%), the orbit in 7 patients (54%), and the eyelid in 2 patients (15%). One patient with eyelid metastasis demonstrated concurrent conjunctival nodule. Mean time to diagnosis of contralateral ocular or periocular metastasis was 94 months (median, 48 months; range, 9-375 months). Contralateral choroidal metastasis was multifocal in 3 of 4 patients (75%). Of 7 patients with orbital metastasis, 5 showed extraocular muscle involvement with restricted ocular motility. Treatment for contralateral choroidal metastasis included brachytherapy (n = 2), transpupillary thermotherapy (n = 1), and observation (n = 1). Treatment for contralateral periocular (orbit or eyelid) metastasis was excision (n = 5), external beam radiotherapy (n = 2), and observation (n = 2). Of 13 patients, death was documented in 11 patients at a mean of 17 months (median, 9 months; range, 3-54 months) as a result of systemic UM metastasis (n = 10) or unrelated cause (n = 1). CONCLUSIONS: Metastasis resulting from UM to the contralateral ocular and periocular structures is rare and generally occurs in patients with disseminated metastasis. Orbital tissue is the most common site of involvement, and these patients have short life expectancy.

Incidental finding of regressed solitary unilateral choroidal metastasis from lung adenocarcinoma with EGFR exon-19 deletion treated with erlotinib.

A 49-year-old Asian Indian woman, with a previous history of biopsy proven stage IV primary lung adenocarcinoma with metastasis to liver, bones and central nervous system, presented with 1-month history of photopsia in right eye. She was on oral erlotinib since 6 months. Dilated fundus examination of right eye revealed a solitary dome-shaped brownish elevated lesion of approximately 1-disc diameter along the inferotemporal midperiphery with surrounding areas of hypopigmentation. Based on multimodal imaging, a diagnosis of resolved solitary unilateral choroidal metastasis from lung carcinoma in the right eye was made. In view of inactive and regressed choroidal metastasis, no intervention was mandated.

Multimodal Imaging in Choroidal Metastasis.

BACKGROUND: Choroidal metastasis represents the most common malignant intraocular tumours. OBJECTIVES: The objective of this study is to detect the structural and vascular features of choroidal metastasis by multimodal imaging. METHODS: Sixteen eyes of 16 patients with choroidal metastasis were enrolled in this prospective study. The multimodal imaging was performed in all patients: fluorescein angiography, indocyanine green angiography, enhanced depth imaging optical coherence tomography (EDI-OCT), OCT angiography (OCTA), and ultrasonography. RESULTS: The choroidal metastasis was located in the macula region in 9 eyes (57%) and in the extramacular region in 7 eyes (43%). EDI-OCT showed a mean thickness of 950 ± 246 µm, a smooth anterior tumour surface in 5 eyes (31%), and a lumpy bumpy appearance in 11 eyes (69%). The most frequent EDI-OCT features were represented by choriocapillaris thinning (100%), shaggy photoreceptors (82%), subretinal fluid with speckles (69%), subretinal lipofuscin pigment (6%), absence of drusen (100%), optical shadowing (94%), low-internal optical reflectivity (75%), and retinal pigment epithelium alterations (43%). OCTA revealed an absence of intratumoral vascular network in all cases. CONCLUSIONS: The multimodal imaging contributed to greater insights into the anatomical and vascular features of choroidal metastasis. It allows for the collection of useful information to establish an appropriate diagnosis and follow-up.

Clinical Spectrum of Uveal Metastasis in Korean Patients Based on Primary Tumor Origin.

PURPOSE: To describe the clinical features and prognosis of patients with uveal metastasis in Korea. DESIGN: Retrospective, observational case series. PARTICIPANTS: Patients diagnosed at 2 tertiary high-volume centers between November 2005 and November 2019. METHODS: Evaluation of multimodal imaging and electronic medical records. MAIN OUTCOME MEASURES: The clinical features and outcomes were assessed based on the primary cancer site. RESULTS: A total of 134 uveal metastases (128 choroidal, 3 iris, and 3 ciliary body tumors) were diagnosed in 95 eyes of 80 patients. Mean age at diagnosis was 56 years (median, 55 years; range, 24-86 years), with a minor preponderance of women (61%). Tumors were bilateral in 15 patients (19%) and the primary origin was established in 49 patients (61%) before ocular detection. The primary tumor originated in the lung (48%), breast (24%), gastrointestinal tract (10%), liver (3%), pancreas (3%), kidney (1%), cervix (1%), and nasopharynx (1%), with some remaining unknown (10%). The overall 5-year survival rate was 21%. Kaplan-Meier analysis revealed that the worst survival was found in pancreatic cancers (mean survival, 5.9 months; P = 0.045), and the best survival was found in gastrointestinal tract cancers (mean survival, 44.5 months). CONCLUSIONS: The primary tumor origins in Korean patients with uveal metastases differed from those reported in primarily population-based studies of White patients, with a higher prevalence of lung and gastrointestinal tract cancers.

Alectinib in the treatment of ocular metastases of ALK rearranged non small cell lung cancer: Description of 2 case reports.

RATIONALE: Choroidal metastasis is a rare metastatic location although the most common intraocular neoplasm. In general, choroidal metastases respond favorably to systemic therapy targeted toward the primary neoplasm. In patients with choroidal metastasis of ALK rearranged non small cell lung cancer (NSCLC), targeted therapy using Alk inhibitors gradually replaced radiotherapy as the best treatment. Alectinib is a second-generation ALK inhibitors. Here we describe 2 clinical cases of patients with choroidal metastasis of ALK rearranged NSCLC who received Alectinib as first-line therapy achieving disease control and quality of life improvement. PATIENTS CONCERNS: In case report 1, 62-year-old man presented with scintillated scotomas at the level of the right eye; in case report 2, 69-year-old man presented with respiratory distress, persistent cough resistant to medical therapy, pain, and blurred vision. DIAGNOSES: In case report 1, fundus and ultrasonographic examination showed circumscribed choroid thickening with dome-like appearance compatible with repetitive lesion. Computed tomographic/y (CT) showed multiple bilateral pulmonary nodular formations and adenocarcinoma of the lung was diagnosed by a transbronchial biopsy.In case report 2, CT showed a primary lesion of 36 × 27 mm in the middle lobe with bilateral lung metastases and lymphadenopathies. Multiple hepatic metastases and minor suspicious bone repetitions. A liver biopsy made a diagnosis of adenocarcinoma compatible with pulmonary primitiveness. An ocular fluoroangiography evidenced a left choroidal metastasis. INTERVENTIONS: Case report 1, 2, medical treatment with Alectinib 1200 mg/day was initiated. OUTCOMES: In case report 1, a few days after beginning the treatment, both systemic symptoms like respiratory distress and low vision were palliated. Reassessment by CT confirmed treatment response. In case report 2, clinically, visus disorders had already improved 2 weeks after beginning treatment. CT showed pulmonary, nodal, and hepatic response. Stability of bone metastases occurred after 2 months. In addition, ocular ultrasonography documented the regression of previously reported lesions confirmed treatment response. LESSONS: Alectinib works very well in intracranial metastases and is assumed to be so on the ocular ones as well, with benefit for the patient in quality of life.