Bilateral choroidal metastasis from adenoid cystic carcinoma of the submandibular salivary gland.

PURPOSE: A case of bilateral choroidal metastasis from an adenoid cystic carcinoma of the submandibular gland is described. CASE DESCRIPTION: A 45-years-old woman with a history of metastatic adenoid cystic carcinoma presented with visual impairment in both eyes. Fundus images showed bilateral creamy-white choroidal masses. Optical coherence tomography revealed subretinal fluid with high reflective speckles and a "lumpy bumpy" anterior contour of the lesions. Fluorescein angiography showed a hypofluorescent pattern of the lesions in early arterial phases, and progressive late hyperfluorescence. A diagnosis of bilateral choroidal metastasis from adenoid cystic carcinoma was made. The patient was advised to underwent palliative chemotherapy, but she expired a few weeks after the diagnosis. CONCLUSION: Salivary gland carcinoma rarely metastasizes to the choroid, with few cases described in literature. In patients with a history of salivary glands tumor the possibility of choroidal metastatization should always be considered.

Multimodal imaging of optic disc melanocytoma.

PURPOSE: To describe the findings of four patients (four eyes) with optic disc melanocytoma (ODM), using multimodal imaging. METHODS: Retrospective case series. RESULTS: On ocular ultrasonography ODMs appeared as hyperechogenic lesions with moderate-to-high internal reflectivity. On blue-light fundus autofluorescence, ODMs showed total hypoautofluorescence, while, on infrared reflectance images appeared as bright and well-marginated lesions. MultiColor composite images showed reddish-brown lesions with well-defined margins. Swept-source optical coherence tomography revealed elevated lesions covering the optic disc with an irregular hyperreflective surface, dishomogeneous internal structure with hyperreflective dots, and posterior shadowing. In all cases optical coherence tomography angiography detected intratumoral blood vessels which were not detectable with fluorescein angiography. CONCLUSION: Multimodal imaging in ODM might be useful both at presentation, increasing the diagnostic accuracy, and at follow-up, providing helpful details, that can help to rule out the possibility of malignant transformation and other ocular complications.

Treatment of periocular advanced basal cell carcinoma with Hedgehog pathway inhibitors: A single-center study and a new dedicated therapeutic protocol.

The management of difficult-to-treat periocular basal cell carcinoma (BCC) becomes very challenging in cases of delayed diagnosis, leading to the development of locally advanced BCC. The aim of this study was to evaluate the outcomes of Hedgehog pathway inhibitors (vismodegib and sonidegib) treatment in patients affected by periocular locally advanced BCC. We focused on the common adverse events and their correlation with the administration schedule, to determine a management protocol specific for the periocular area. This observational prospective study included a single-center case series with patients who were histologically confirmed to have periocular or orbital locally advanced BCC, treated with Hedgehog pathway inhibitors. All patients benefitted in terms of regression or stabilization of the neoplasm. In the first months of treatment, the HPIs were well tolerated, and the first important side effects appeared after about 5 months of continuous use of the drug. These data could lead to a new type of therapeutic scheme where neoadjuvant therapy could be followed by pulse therapy as an adjuvant to surgery.

Clinical outcomes and secondary glaucoma after gamma-knife radiosurgery and Ruthenium-106 brachytherapy for uveal melanoma: a single institution experience.

We retrospectively analyzed data from records of 48 patients (48 eyes) treated with gamma-knife (n = 18) or Ruthenium-106 brachytherapy (n = 30) for uveal melanoma, in our Ocular Oncology Unit between December 2013 and September 2019, with the aim to evaluate treatment outcomes, and incidence and risk factors for secondary glaucoma. Patients demographics and tumor characteristics at diagnosis were recorded. Follow-up data were collected regarding local tumor control, treatment complications, enucleation need, metastases occurrence and survival status. The median follow-up period was 33.7 months in the gamma-knife group and 26.2 months in the brachytherapy group. The mean tumor thickness, the largest basal diameter and the tumor volume were significantly higher in the gamma-knife group than in the brachytherapy group. The local tumor control rate was 100% in the brachytherapy group and 77.8% in the gamma-knife group. In the gamma-knife group, six patients were enucleated, no patient treated with brachytherapy underwent enucleation. The overall survival rate was 96.7% in the brachytherapy group and 94.44% in the gamma-knife group. Secondary glaucoma occurred in 10 patients after gamma-knife and in one patient after brachytherapy: it should be emphasized that larger lesions were treated with gamma-knife, whereas smaller tumors were selected for brachytherapy. We found a significative correlation of tumor thickness (P value = 0.043) and volume (P value = 0.040) with secondary glaucoma occurrence after gamma-knife treatment. Moreover, secondary glaucoma significantly correlated with radiation retinopathy in the gamma-knife group (P value = 0.009). This study shows preliminary clinical results that could be useful for further studies with more patients and longer follow-up.

Extensive conjunctival melanoma successfully treated with surgical resection and pre- and postoperative topical mitomycin C.

PURPOSE: To report a case of a large conjunctival melanoma (CM) successfully treated with surgical resection and pre- and postoperative topical mitomycin C (MMC). METHODS: This is a single observational case report. RESULTS: A 58-year-old man was referred to us for a large pigmented conjunctival lesion of the right eye. Slit lamp examination revealed an extensive pigmented lesion diffusely involving the temporal bulbar conjunctiva, with multifocal intensely dark areas and an elevated limbal component extending on the corneal surface. The remaining bulbar and forniceal conjunctiva was not involved. The lesion was clinically diagnosed as CM. Clinical examination and head and neck ultrasonography did not show regional lymphadenopathy. The patient was treated with neoadjuvant topical MMC 0.04% four times a day for 3 weeks. At the end of MMC therapy we observed a reduction in pigmentation and thickness of the lesion. Subsequently, the patient underwent surgical excision of the lesion with "no touch technique," double freeze-thaw cryotherapy of the margins and reconstruction of the tissue defect with amniotic membrane graft. Histopathologic examination of the specimen confirmed the presence of CM, surrounded by primary acquired melanosis with atypia. The excision margins were positive for tumor involvement, so we decided to perform an adjuvant treatment with other four cycles of topical MMC. CONCLUSIONS: The patient was closely followed-up after the treatment, without any local or distant recurrence at 32 months.

Combined brachytherapy and vitreoretinal surgery for a large retinal capillary hemangioma with exudative retinal detachment.

INTRODUCTION: We report about a large retinal capillary hemangioma (RCH) with exudative retinal detachment and a macular fold, treated with Ruthenium-106 brachytherapy (Ru-106 BT) and scleral buckling surgery, followed by pars plana vitrectomy (PPV), for the removal of macular tractions. CASE DESCRIPTION: A 17-year-old boy was referred to our Ocular Oncology Unit for a large RCH in the left eye. BCVA was hand motion. The RCH measured 4.9 × 6.85 mm in basal diameters and 4.0 mm in thickness and was located in the mid-peripheral temporal retina. It was surrounded by extensive subretinal exudation, forming an exudative retinal detachment, with a retinal fold that extended from the lesion to the optic disc. We performed Ru-106 BT and at the moment of the plaque removal we placed a radial buckle with the aim to unbend the retinal fold. At 3-months follow-up the exudation decreased, we achieved the opening of the peripheral side of the retinal fold, but the macula was still detached. We decided to perform a lens sparing PPV, macular peeling and air tamponade, to remove the vitreoretinal tractions ab interno and to try to complete the opening of the macular fold. After 1-month BCVA was counting fingers, the retina appeared attached, also in the macular area, but the retinal fold remained partially close in the macular side. After 6 months the tumor was inactivated, the macula remained attached, unfortunately, the macular fold remained partially close. CONCLUSION: Ru-106 BT and scleral buckling concurrent approach can be an effective treatment modality in selected cases of large RCHs, followed by PPV to remove eventual vitreo-retinal tractions.

Ocular oncology service during the COVID-19 outbreak in Florence (Italy): Practical considerations for the management of patients.

The Coronavirus disease 2019 (COVID-19) outbreak has imposed the adoption of strategies to limit the risk of contagion for cancer patients without compromising their healthcare. As well as cancers of other sites, the treatment of certain ocular and periocular malignancies is considered non-deferrable and should proceed despite the pandemic. Delays in treatment of these patients may result in negative outcomes. Herein, we provide some practical considerations deriving from our experience at the Ocular Oncology Unit of Careggi University Hospital (Florence, Italy).

Multimodal imaging of a retinal cavernous hemangioma.

PURPOSE: We describe the characteristics of an isolated retinal cavernous hemangioma in a young female patient using multimodal imaging. CASE DESCRIPTION: Fundus examination of the right eye showed a large vascular mass, composed by clusters of dark red and dilated saccular angiomatous formations with superficial whitish fibroglial tissue and hemorrhages, located in the inferonasal peripheral retina, along the vascular arcade. On green-light fundus autofluorescence the lesion appeared hypoautofluorescent, with moderately hyperautofluorescent areas. On multiColor imaging it showed mainly a green pseudocolor, with knobby surface and well-defined irregular margins. Fundus fluorescein angiography showed early hypofluorescence within the tumor mass and late incomplete staining, without leakage. Ocular ultrasonography documented an elevated well-defined hyperechogenic lesion, with high internal reflectivity, without choroidal excavation, retinal detachment, or acoustic shadowing. Swept source optical coherence tomography showed multilobulated cavernous formations, varying in size, containing for the greatest part hyperreflective material, within an interconnecting fibrous scaffold and with an overlying partially adherent epiretinal membrane. Optical coherence tomography angiography documented a few highly reflective, tortuous and abnormal branching veins extending into the tumor mass, with a root-like appearance, and surrounding nodular changes, corresponding to the angiomatous formations. CONCLUSIONS: Different imaging modalities can be used in combination for a better evaluation of the pathological features of this rare vascular tumor.

Large choroidal metastasis with exudative retinal detachment as presenting manifestation of small cell lung cancer: A case report.

A 64-year-old man, diagnosed with a retinal detachment of his left eye, was sent to our hospital to evaluate vitreoretinal surgery. Left eye best-corrected visual acuity was hand motion. Fundus examination showed a voluminous slightly pigmented choroidal neoformation with secondary retinal detachment. Ultrasonography revealed a large hyperechogenic choroidal mass with retinal detachment, initial choroidal excavation, and low-to-medium internal reflectivity. The clinical-instrumental aspects of the lesion suggested a primary malignancy. The patient underwent chest radiography, which showed a large mass located in the right lung. Contrast-enhanced computed tomography of chest and abdomen showed a voluminous lung lesion, another gross lesion of the liver, and other abdominal localizations. The patient underwent biopsies of the pulmonary and hepatic lesions and both samples showed an epithelial malignancy with neuroendocrine differentiation, compatible with metastatic small cell lung cancer. The patient's clinical condition declined within one month from presentation. In the interim, a contrast-enhanced brain computed tomography documented the presence of cerebellar metastases. The patient was admitted to the oncology department and started chemotherapy and supportive care, but unfortunately he died during the course of the treatment, 5 months after his initial presentation. This case is peculiar both for the unusual presentation of small cell lung cancer and for the morphological appearance of the choroidal lesion that suggested a primary tumor.

A rare case of non-small cell lung cancer choroidal metastasis responding to ALK tyrosine kinase inhibitors.

Choroidal localization from non-small cell lung cancer is rare and when it occurs may cause visual alterations. Targeted therapy against actionable gene mutations represents the standard of care in advanced non-small cell lung cancer. We report the case of a 53-year-old woman affected by metastatic anaplastic lymphoma kinase (ALK)-rearranged non-small cell lung cancer who received ALK tyrosine kinase inhibitors, from January 2017. The patient had a complete response of choroidal metastasis after therapy with ALK tyrosine kinase inhibitors. She recovered a complete visus and actually she still continue therapy with alectinib. The patient had a complete recovery of visus in addiction to a long response on treatment.

Adenocarcinoma Arising in the Conjunctiva With Periparotid Lymph Node Metastases.

PURPOSE: To report a rare case of adenocarcinoma (ADC) arising in the conjunctiva which locally recurred and metastasized to the periparotid lymph nodes. METHODS: This is a single observational case report. RESULTS: A 79-year-old male patient was referred to us for a suspected recurrence of conjunctival carcinoma of the right eye. At presentation, we observed an elevated conjunctival lesion with corneal involvement. He was treated with neoadjuvant mitomycin C 0.04% eye drops, followed by surgical excision of the lesion, cryotherapy of the excision margins, and reconstruction with amniotic membrane graft. The tumor was histologically diagnosed as ADC. The negative systemic evaluation and the immunoprofile led us to believe the primitive nature of the tumor. The excision margins were positive, and the patient was lost to follow up for 7 months, and when he came back, a new local recurrence was diagnosed. Then, he received rescue treatment with mitomycin C 0.04% eye drops with complete regression of the lesion. No local recurrence was observed until the 14-month follow-up visit, during which the patient complained of swelling in the right parotid region. Subsequently, he underwent total parotidectomy with neck dissection. Metastasis was found in 3 periparotid lymph nodes. The patient did not receive further treatments, and no recurrences were observed over the following 20 months. CONCLUSIONS: ADC arising in the conjunctiva is a very rare occurrence. Additional observation is required for the management of this rare conjunctival tumor.

Primary Orbital Synovial Sarcoma Mimicking a Periocular Cyst.

Synovial sarcoma (SS) is a high-grade soft-tissue sarcoma occurring predominantly in older children and young adults. Only approximately 7% occur in the head and neck region, with SS representing less than 0.1% of all head and neck cancers. Orbital location is exceedingly rare with only 8 cases reported so far in the literature. It is noted for its propensity for late local recurrences and metastases. Histologically, SS is monophasic, biphasic, or poorly differentiated and harbors a specific chromosomal translocation t(X;18)(p11.2;q11.2) in >95% of cases. In this article, we describe a case of monophasic SS primarily arising in the left supero-nasal orbital region in a 24-year-old woman, clinically mistaken for a periocular cyst. The case is peculiar for its highly unusual location and for its clinical deceptively benign appearance.

Intra-arterial chemotherapy for retinoblastoma in 70 eyes: outcomes based on the international classification of retinoblastoma.

OBJECTIVE: To analyze our 5-year experience of intra-arterial chemotherapy (IAC) for retinoblastoma as primary or secondary therapy. DESIGN: Retrospective interventional case series. PARTICIPANTS: A total of 70 eyes of 67 patients. INTERVENTION: Ophthalmic artery chemotherapy infusion under fluoroscopic guidance was performed using melphalan (3, 5, or 7.5 mg) in every case, with additional topotecan (1 mg) and/or carboplatin (30 or 50 mg) as necessary. MAIN OUTCOME MEASURES: Tumor control and treatment complications. RESULTS: The mean patient age at IAC was 30 months. The treatment was primary in 36 eyes and secondary in 34 eyes. Those primary therapy eyes were classified according to the International Classification of Retinoblastoma (ICRB) as group A (n = 0), B (n = 1), C (n = 4), D (n = 17), or E (n = 14). The secondary therapy eyes had failed previous intravenous chemotherapy (n = 34) in every case. Each eye received a mean of 3 IAC sessions per eye (median, 3; range, 1-7 sessions). After IAC with a mean follow-up of 19 months, globe salvage was achieved in 72% of primary-treated cases and in 62% of secondary-treated cases. Specifically, primary therapy achieved globe salvage for group B (100%), group C (100%), group D (94%), and group E (36%). Of all 70 eyes, complete regression was achieved for solid tumor in 48 of 51 eyes (94%), subretinal seeds in 40 of 42 eyes (95%), and vitreous seeds in 34 of 39 eyes (87%). After each catheterization (n = 198), the main complications included transient eyelid edema (5%), blepharoptosis (5%), and forehead hyperemia (2%). More lasting complications included vitreous hemorrhage (2%), branch retinal artery obstruction (1%), ophthalmic artery spasm with reperfusion (2%), ophthalmic artery obstruction (2%), partial choroidal ischemia (2%), and optic neuropathy (<1%). Over the past 3 years, the combined incidence of ophthalmic, retinal, and choroidal vascular ischemia was reduced to 1%. There was no patient with stroke, seizure, neurologic impairment, limb ischemia, secondary leukemia, metastasis, or death. CONCLUSIONS: Five-year experience with IAC indicates that this technique is remarkably effective for the management of retinoblastoma as both a primary and a secondary treatment.

Evaluation of macular thickness after uncomplicated cataract surgery using optical coherence tomography.

PURPOSE: To evaluate central macular thickness (CMT) after cataract surgery in selected groups of patients. 
 METHODS: The study comprised 4 groups-patients with epiretinal membrane, patients with high myopia, patients with diabetes without retinopathy, and healthy subjects-who underwent phacoemulsification and intraocular lens implantation. Central macular thickness was measured with spectral domain optical coherence tomography (OCT) using the 3D macular cube scan. The OCT evaluation was performed preoperatively and 1, 6, 15, 30, 60, 90, and 360 days after surgery. Visual acuity was measured preoperatively and after 6 and 360 days after surgery.
 RESULTS: The study included 258 patients, 164 women and 94 men, with a mean age of 74 (SD 7.6) years. A statistically significant increase in CMT was observed from day 30 in patients with epiretinal membrane (p = 0.010) and diabetic patients (p = 0.026), reaching its maximum thickness at day 60 (p = 0.001 and p = 0.001), while it was observed only on day 360 in healthy subjects (p = 0.018) and those with high myopia (p = 0.003). The correlation between CMT and visual acuity was statistically significant only in the diabetic group (r = 0.61, p<0.01).
 CONCLUSIONS: Following cataract surgery, CMT changes according to characteristic patterns in the different groups studied. These changes did not prevent an optimal recovery of visual function.

Pneumatic retinopexy for retinal detachment associated with choroidal coloboma.

PURPOSE: To report a case of retinal detachment in an eye with choroidal coloboma treated with pneumatic retinopexy and laser. METHODS: Case report. RESULTS: A 34-year-old woman who had had poor vision in her left eye since early childhood and high myopia in her right eye complained a sudden deterioration of vision in her left eye for 3 days. Fundus examination of the left eye showed a large choroidal coloboma, extending to the disc margin from 5-8 o'clock inferiorly and above the inferotemporal arcade, excluding the fovea and the parafovea. Superotemporal bullous detachment of the retina was also observed, including the macula. The patient underwent a pneumatic retinopexy with SF(6) gas (0.5 cc) injection into the vitreous cavity. The following day laser was applied around the margin of the choroidal coloboma. Three months later, visual acuity was 20/200 and fundus examination disclosed a completely attached retina. CONCLUSIONS: This case demonstrates the feasibility of using pneumatic retinopexy in selected cases.