Chalazion-mimicked eyelid angiosarcoma in a young Asian with good prognosis: a case report.

BACKGROUND: Angiosarcoma is an extremely rare malignant tumor. So far, only about 42 cases of angiosarcoma involving the eyelids have been reported. Eyelid angiosarcoma occurs more frequently in elderly Caucasian males and is prone to misdiagnosis. We present a case report in a young Asian male patient with eyelid angiosarcoma that was misdiagnosed as a chalazion. CASE PRESENTATION: A 46-year-old South Korean male with no underlying disease had a right lower lid mass. The lesion was initially misdiagnosed as a chalazion at a local clinic, but a diagnosis of eyelid angiosarcoma was made after the first biopsy trial. PET-CT was performed to ensure that there was no metastasis in the whole body. Surgical excision with enough surgical margin was used alone for treatment and reconstruction was performed with a tarsoconjunctival advancement flap (modified Hughes procedure), which helped ensure good cosmesis. No recurrence was observed 4 years and 5 months after the surgery. CONCLUSIONS: The current study presents the first case of chalazion-mimicked eyelid angiosarcoma in a young Asian male aged under 50 years. This case shows that even if a benign eyelid disease is suspected in a young patient, an incisional biopsy must be performed to confirm whether the lesion is malignant. Since the prognosis is good for the case of eyelid angiosarcoma, if there is no clear evidence of distal metastasis, surgical resection should be performed with an enough safety margin.

The Eyelid Angiosarcoma: A Systematic Review of Characteristics and Clinical Course.

A systematic search for eyelid angiosarcoma was performed from inception to December 2020 in Medline, EMBASE, and the Cochrane databases. Forty two eyelid angiosarcoma cases in 32 articles were analyzed. Eyelid angiosarcomas showed an incidence peak in the eighth decade of life, and was reported more frequently in Caucasian males. Eyelid angiosarcomas were associated with a mortality rate of 26.2%, a recurrence rate of 14.3%, and a cure rate of 45.2%. Four years event-free survival (EFS) rate was 36.0%, with median EFS of 36 months. Eyelid angiosarcomas with bilateral involvement or metastasis showed higher mortality and recurrence rates than unilateral eyelid invasion cases. In the prognosis analysis according to treatment modalities, the mortality and recurrence rates were the lowest in patients who underwent surgical excision. The 4-year EFS probability in a group with surgical excision was 60.6%, but in a group without surgical excision it was 30.3%. A total of 45.2% of the cases was misdiagnosed and 21.4% of the cases could not be correctly diagnosed with the first biopsy trial. The prognosis for eyelid angiosarcomas was better than that of angiosarcomas invading the face and scalp. Surgical excision was the most important treatment modality; thus, should be considered as the first treatment of choice.

Long-Term Clinical Outcomes in Treatment-Naïve Patients With Orbital Adnexal Mucosa-Associated Lymphoid Tissue Lymphoma: A Single-Center Study.

Ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (OAML) is the most common type of ocular lymphoma with a higher prevalence in Asia than in Western countries. OAML represents 1%-2% of all non-Hodgkin's lymphoma, 5%-15% of extranodal lymphomas, and approximately 55% of orbital malignancies. "Watch and wait" after biopsy or surgical resection, radiation therapy, and systemic treatment, including antibiotics administration and chemotherapy with various combinations of regimens can be considered for OAML treatment. Radiotherapy is adapted for limited-stage disease with excellent clinical outcomes of 85-100% complete remission and relatively superior local control efficacy and treatment duration. In contrast, chemotherapy has rarely been tested as frontline therapy. Nonetheless, several studies have reported a favorable response and long duration of progression-free survival using chemotherapy adaptations. When the disease involves both eyes or spreads beyond the conjunctiva, the risk of recurrence increases and limited-stage OAML has a recurrence rate of approximately 25% following radiotherapy only. Therefore, although recent consensus in the literature is that patients with limited-stage OAML recommended treating with radiation, physicians may choose the treatment modality not only by its efficiency but also by its adverse events profile and patients' well-being. Herein, we present a large single-center study on OAML that included 292 patients who were followed up for up to 237 months. We collected and analyzed real-world data focusing on treatment outcomes and the role of radiotherapy as frontline therapy, and aimed to compare outcomes and complication profiles of chemotherapy, especially in limited-stage OAML, to identify an optimal treatment strategy.

Clinical Features and Outcomes of Patients with Orbital Schwannoma.

PURPOSE: To report the clinical features, treatment, and outcome of 11 patients with orbital schwannoma in Korean patients. METHODS: The medical records of 11 orbital schwannoma patients treated between April 2007 and April 2021 were retrospectively reviewed. The demographic data, clinical characteristics, radiological features, and outcomes were reviewed. RESULTS: The mean age at the time of diagnosis was 49.00 ± 14.45 years. The most common initial symptom was ocular protrusion (n = 7), and other symptoms were decreased visual acuity ( n = 5), restriction of eye movement ( n = 4), swelling ( n = 3), and pain ( n = 2). Locations of tumors were superomedial; followed by the orbital apex and inferolateral. The most common shape seen in our patients was beads like multilobulated appearance; followed by a round, oval, fusiform, and dumbbell shape. MRI of T1-weighted revealed isointense or hypointense, whereas the T2-weighted indicated hyper or isointense lesion. Five patients had optic neuropathy at presentation, and 1 of them showed improved vision after surgery. Complete or incomplete excision was performed for all. Surgical complications include decreased vision and paraesthesia. There has been no recurrence to date. CONCLUSIONS: Orbital schwannoma is a rare disease and it is difficult to distinguish it from other orbital tumors because the initial symptoms are nonspecific. Differential diagnosis by combining the shape, location, and contrast enhancement findings seen on computed tomography and magnetic resonance imaging can be helpful in surgical treatment. Complete excision gives the best results without recurrence, but if the patient is in an inaccessible location, only surgery to reduce the volume can satisfy the patient without recurrence.

Relapse in patients with limited-stage ocular adnexal lymphoma treated by chemoimmunotherapy: Extended follow-up of a phase 2 study.

BACKGROUND: Approximately 50% of limited-stage ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) patients with adverse prognostic factors relapse after radiotherapy. Chemoimmunotherapy has been proposed as an alternative frontline therapy. However, only a few studies have reported its long-term treatment outcome. METHODS: In 2011, we commenced a phase 2 trial to investigate the efficacy of rituximab, cyclophosphamide, doxorubicin, and prednisolone (R-CVP) in bilateral and non-conjunctival limited-stage OAML patients. Results of the clinical trial showed a response rate of 100% and a 4-year progression-free survival of 90.3% without significant toxicity. We extended the study period to December 2020 to determine the long-term efficacy of R-CVP chemoimmunotherapy. RESULTS: At a median observation period of 66.0 months, eight of 33 study patients had relapsed. The cumulative incidence of relapse was 18.9% at 5 years and 44.7% at 8 years. The majority of relapses developed more than 4 years after treatment. Local relapse was more prevalent than distant relapse. The relapse risk of orbital and lacrimal diseases was likely to be higher than that of conjunctival and eyelid diseases (HR: 2.5, 95% CI: 0.498-12.500, p = 0.25). CONCLUSION: Although the response rate was remarkable for chemoimmunotherapy, the risk of late relapse was considerable. Based on our findings, clinical trials for limited-stage OAML patients should have a long-term observation period. To minimize radiation toxicity and reduce the risk of delayed relapse (local relapse and distant relapse), a future study with sequential or combination treatment of local low-dose radiation and systemic chemoimmunotherapy can be considered.

Long-term Efficacy of Dacryoendoscopy-guided Recanalization and Silicone Tube Intubation.

PURPOSE: To investigate the long-term efficacy of dacryoendoscopy-guided recanalization and silicone tube intubation in patients with obstruction in the lacrimal drainage system and to identify factors related to surgical outcome. METHODS: We retrospectively reviewed the medical records of patients with primary nasolacrimal duct obstruction and canalicular obstruction who underwent dacryoendoscopy-guided recanalization and silicone tube intubation between August 2014 and March 2016. Factors related to surgical outcome were examined and compared between the success group (eyes with complete response and partial response) and the failure group. Kaplan-Meier survival analysis and multivariable logistic regression analysis were used to analyze the success rate according to the factors found to have statistical significance. RESULTS: The study included 74 eyes of 51 patients. The mean age of the patients was 60.3 ± 10.0 years (range, 34-80 years). The success group consisted of 66 eyes (89.2%) (complete response, 56 eyes, 75.7%; partial response, 10 eyes, 13.5%) and the failure group consisted of eight eyes (10.8%). The median follow-up period was 58 months (range, 6.5-72 months), and the overall success rate was 89.2%. Compared to the eyes with preoperative lacrimal irrigation test of partial passage, the eyes with no passage were associated with a lower success rate (95.9% vs. 76.0%, p = 0.01). Postoperative inflammation was also associated with a lower success rate (96.6% vs. 60.0%, p < 0.001). CONCLUSIONS: Dacryoendoscopy-guided recanalization and silicone tube intubation is effective and can be considered a first choice of treatment for eyes which show partial passage in the lacrimal irrigation test. The management of postoperative inflammation is essential to ensure surgical success.

Association between age-related cataract and blepharoptosis in Korean adults: a population-based study.

Cataract and blepharoptosis are both commonly encountered ophthalmic problems in older adults. Since they share similar risk factors, it is plausible that there may be an association between the two conditions. We examined data from the Korean National Health and Nutrition Examination Survey (KNHANES) 2010-2012 to determine if there is an association between age-related cataract and blepharoptosis. Multivariable adjusted logistic regression analysis was conducted to examine the odds ratio (OR) and 95% confidence interval (CI) for association of each specific type of cataract with presence of blepharoptosis. Of the 10,387 eligible participants, 4782 (46.0%) had cataract and 1419 (15.8%) had blepharoptosis. There were more participants with blepharoptosis in the cataract group, compared with those in the no cataract group. After adjusting for potential confounders, participants with blepharoptosis had a higher risk of total cataract (OR: 1.557, 95% CI 1.201-2.019) and nuclear subtype cataract (OR: 1.305, 95% CI 1.050-1.620). Blepharoptosis was associated with significantly higher odds of cataract in obese participants when compared with non-obese participants (p for interaction = 0.0236). Our study revealed a positive association between age-related cataract and blepharoptosis; it suggests that thorough ophthalmic assessment is needed when assessing patients who are planning cataract or blepharoptosis surgery.

Lack of Association between Chlamydophila psittaci and Ocular Adnexal MALT Lymphoma in Korean Patients-Is the Geographic or Genetic Difference Significant?

Clamydophila psittaci (C. psittaci) has been proposed to be an etiologic factor in extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in the ocular adnexa. However, the pathogenetical significance of the infection has not been fully elucidated. Many previous studies have shown controversial results regarding C. psittaci detection rates in said patients, ranging from 0 to 87%. We investigated the presence of C. psittaci in a single institutional cohort (n = 150) of ocular adnexal MALT lymphoma (OAML) patients in Korea. We tried to exclude the methodological biases derived from the different primer sets in polymerase chain reaction-based studies. For that reason, we applied five sets of primers, including four previously reported and one newly designed primer set. There was no case of C. psittaci-positive OAML in repeated trials validated with appropriate positive and negative controls. All 150 cases showed negative results with five primer sets. These results suggest that the pathogenetic role of C. psittaci in ocular adnexal MALT lymphoma might have been overestimated to date, at least in the Korean population. Therefore, the molecular diagnosis of C. psittaci is considered a very low priority.

Analysis of Clinical Characteristics and Prognosis of Patients With Periocular Malignant Melanoma in Korea.

OBJECTIVE: To report the clinical features of 20 patients with malignant melanoma and to evaluate the survival and prognosis of patients with malignant melanoma in Korea. METHODS AND ANALYSIS: The medical records of 20 patients with malignant melanoma treated between March 2004 and March 2020 were reviewed retrospectively. The demographic data, clinical characteristics, and outcomes were also reviewed. Outcome measures included local recurrence, metastasis, and tumor-related mortality. Prognostic factors associated with recurrence, metastasis, and survival were analyzed using a Cox proportional hazards model. RESULTS: Among the 20 patients with periocular malignant melanoma, 4 (20%) showed local recurrence during follow-up (6.61 ±â€Š6.36 years). The 1-, 5-, and 10-year recurrence rates were 5%, 10.3%, and 24.1%, respectively. 10 (50%) patients developed distant metastasis, mainly involving lung, brain, parotid gland and spine. The 1-, 5-, and 10-year rates of metastasis were 5%, 10.9%, and 35.7%, respectively. Significantly fewer metastases were detected following initial extensive surgical excision (P = 0.04). 8 (40%) patients died of malignant melanoma. The main risk factor for mortality was tumor thickness (HR: 3.88, P < 0.01). Based on Kaplan-Meier survival estimates, the 1-, 5-, and 10-year tumor-related survival rates were 75.8%, 55.6%, and 55.6%, respectively. CONCLUSION: Tumor thickness is a significant prognostic factor affecting the survival rate. It is important to reduce the metastatic rate via extensive resection without leaving any residual tumor in the margin during surgery.