Bone destruction of orbital wall in idiopathic orbital inflammatory pseudotumor: does it always imply malignancy?

AIM: To assess the clinical presentations and outcomes of idiopathic orbital inflammatory pseudotumor (IOIP) patients with orbital wall bone destruction (OWBD) and to propose an expanded classification system that includes bone destruction. METHODS: The study retrospectively reviewed clinical presentations, imaging findings, treatment modalities, and outcomes of six patients diagnosed histopathologically with IOIP and OWBD at the Beijing Tongren Hospital, Capital Medical University between October 2018 and June 2021. RESULTS: Over two years, 6 (10%) of 60 IOIP patients at our hospital exhibited OWBD, but this may overrepresent severe cases. The cohort consisted of three men and three women, aged 17 to 60y (mean 35.5±16.1y). Presenting symptoms included proptosis, eyelid swelling, decreased visual acuity with pain, and palpable mass. Imaging revealed multiple anatomical structures involved with the medial wall being the most common site of bone destruction. Histopathological examination showed classic type in five patients and sclerosing type in one patient. All patients underwent surgical resection followed by methylprednisolone treatment. Follow-up (mean 30.3±3.1mo) indicated three patients had no recurrence, while others had varying degrees of symptom persistence or recurrence. CONCLUSION: IOIP with bone destruction is a rare but significant subtype that mimics malignancy, leading to potential diagnostic and therapeutic challenges. Our findings suggest that complete surgical resection combined with adjunctive glucocorticoid therapy can yield favorable outcomes. However, larger-scale studies are needed to further optimize therapeutic approaches.

Guiding function of positron emission tomography-computed tomography examination in the diagnosis and treatment of ocular adnexal mucosa associated lymphoid tissue lymphoma.

AIM: To explore the role of positron emission tomography-computed tomography (PET-CT) examination in the diagnosis and treatment of ocular adnexal mucosa associated lymphoid tissue lymphoma (OAML). METHODS: The general clinical data, postoperative PET-CT results, treatment regimens, and the prognosis of 21 histopathologically confirmed OAML patients between October 2017 and September 2021 were collected. Among the 21 patients, five patients underwent surgical treatment alone, 13 patients underwent surgical treatment combined with radiotherapy, and three patients underwent surgical treatment combined with chemotherapy. RESULTS: The follow-up period ranged from 8 to 79mo, with four cases of recurrence and no deaths. Through PET-CT examination, two patients exhibited both local ocular metabolic elevation and systemic metastasis, and one of these patients had cervical lymph node metastasis, while the other had submandibular and parotid gland metastasis. Nine patients showed only local ocular metabolic elevation, while 10 patients had no abnormal metabolic activity locally. CONCLUSION: PET-CT examination plays a crucial role in detecting residual lesions and recurrence following tumor resection, aiding in precise disease staging, and facilitating the development of personalized treatment plans, ultimately improving patient prognosis.

Treatment of lacrimal gland adenoid cystic carcinoma: a systematic review and Meta-analysis.

AIM: To evaluate lacrimal gland adenoid cystic carcinoma (LGACC) of prognosis in patients who underwent different treatment regimens. METHODS: We searched PubMed, EMBASE, and the Cochrane Library for studies done on the treatment of LGACC, between January 1987 and April 2022. A Meta-analysis was conducted to pool the 5-year overall survival rate (OR), and the 5-year recurrence rate (RR) and 5-year metastasis rate (MR) were assessed. RESULTS: The 30 studies involved 585 patients were included in the Meta-analysis. The pooled 5-year OR with surgery alone was 50%, the 5-year RR was 63%, and the 5-year MR was 34%. The pooled 5-year OR with surgery and adjuvant radiotherapy combined was 67% (95%CI 61%,73%), the 5-year RR was 41%, and the 5-year MR was 35%. The pooled 5-year OR with surgery and adjuvant chemoradiotherapy combined was 72% (95%CI 59%, 84%), the 5-year RR was 48%, and the 5-year MR was 36%. The pooled 5-year OR with surgery, intra-arterial cytoreductive chemotherapy, and adjuvant chemoradiotherapy combined was 78% (95%CI 68%, 89%), the 5-year RR was 15%, and the 5-year MR was 27%. CONCLUSION: Comprehensive treatment is more effective than surgery alone. Surgery combined with intra-arterial chemotherapy and adjuvant chemoradiotherapy seems to add value to the therapeutic effect of comprehensive treatment of LGACC but further high-quality research is required to validate this.

Progress of Cancer Stem Cells in Retinoblastoma.

The theory of cancer stem cells is a breakthrough discovery that offers exciting possibilities for comprehending the biological behavior of tumors. More and more evidence suggests that retinoblastoma cancer stem cells promote tumor growth and are likely to be the origin of tumor formation, drug resistance, recurrence, and metastasis. At present, some progress has been made in the verification, biological behavior, and drug resistance mechanism of retinoblastoma cancer stem cells. This article aims to review the relevant research and explore future development direction.

Orbital liposarcoma: a retrospective, single-center study of thirteen patients.

AIM: To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma. METHODS: The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital, from 2006 to 2021 were collected and analyzed. The data includes age, gender, affected orbital side, course of disease, status of disease (primary or recurrent), clinical manifestations, preoperative visual acuity, operative treatment, the relations between liposarcoma and surrounding tissue, longest diameter of liposarcoma, histological subtype, immunohistochemical indicators, follow-up treatment and prognosis. RESULTS: The initial symptoms are diverse. Proptosis is the most frequent chief complaint and the others included vision loss, epiphora, diplopia, and eyelid palpable mass. Results of imaging examination [computed tomography (CT) or magnetic resonance imaging (MRI)] showed orbital mass. In terms of treatment, 10 patients received tumor resection, and the mean longest diameter of the tumor was 3.39±1.36 cm. The other 3 patients had optic nerve invaded, so they received orbital exenteration. Pathological examination results confirmed the diagnose of liposarcoma for 13 patients. Six patients displayed as myxoid type, and three patients in each type of dedifferentiated and well-differentiated type. One patient was verified as pleomorphic, which was a rare type of liposarcoma. All of the patients showed Vimentin positive, and most showed CD34 and S-100 positive. Besides, four patients showed smooth muscle actin positive. All thirteen patients were alive. CONCLUSION: Orbital liposarcoma is a rare disease and it has no specific clinical manifestation. The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit. It is recommended to perform pathological examination to achieve early detection and early treatment.

Different serum levels of IgG and complements and recurrence rates in IgG4-positive and negative lacrimal gland benign lymphoepithelial lesion.

AIM: To analyze the differences in immune indicators and prognosis between IgG4-positive and negative lacrimal gland benign lymphoepithelial lesion (LGBLEL). METHODS: This was a single-center retrospective clinical study including 105 cases of IgG4-positive LGBLEL and 41 cases of IgG4-negative LGBLEL. Basic information, related indicators of peripheral venous blood samples using immunoscattering turbidimetry, treatment (partial surgical excision and glucocorticoid therapy) and prognosis (recurrence and death) were collected. Survival curves for recurrence were created using the Kaplan-Meier analysis. Univariate analysis and multivariate regression analysis were used to analyze prognostic factors. RESULTS: The mean age was 50.10±14.23y and 44.76±11.43y (P=0.033) in IgG4-positive and negative group respectively. The serum C3 and C4 was lower in IgG4-positive group (P=0.005, P=0.002), while the serum IgG and IgG2 was higher in IgG4-positive group (P=0.000 and P=0.008). Twenty-one cases had recurrence in IgG4-positive group and 3 cases recurrence in IgG4-negative group. The 5-year recurrence-free cumulative percentages of IgG4-positive group was 81.85%, and 83.46% in the IgG-negative group (P=0.216). The history of preoperative glucocorticoid therapy, serum C4, IgG1 and IgG2 were the factors affecting recurrence in IgG4-positive group, while serum C4, and IgG1 were the factors affecting recurrence of LGBLEL. CONCLUSION: Serum C4 and IgG1 are the factors affecting recurrence of LGBLEL, while the IgG4 does not affect recurrence of LGBLEL.

Clinical analysis of 2790 children with retinoblastoma: a single-center experience in China.

BACKGROUND: In this study, we aimed to analyze the clinical characteristics and prognosis of children with retinoblastoma (RB) in a single center in China with a large sample collection spanning 17 years. METHODS: The clinical data of 2790 children with RB treated in Beijing Tongren Hospital from 2005 to 2021 were collected, and a retrospective analysis was conducted. RESULTS: The median age of the participants was 28.3 months. There were 3624 affected eyes, 12.4% of which were in groups A-C, 67.1% in groups D-E and 16.2% were not specified. The primary symptom observed in most cases was a white pupil, accounting for 66.5%, followed by strabismus (12.8%). The median follow-up time was 59.7 months. The enucleation rate was 71.3% (703/986) in a single left eye and 72.5% (702/968) in a single right eye. The overall survival (OS) rate was 95.8% (2444/2552) because 237 patients dropped out, and 109 died. Kaplan‒Meier survival analysis showed that the median survival time (MST) was 125.92 months [95% confidence interval (CI) = 124.83-127.01]. Cox multivariate survival analysis showed that trilateral RB (P = 0.017), metastasis site (P = 0.001), and combined distant tissue metastasis (P = 0.001) were independent prognostic factors for RB. The OS of 44 cases of familial RB was 93.2% (41/44), with an MST of 80.62 months (95% CI = 67.70-93.54). CONCLUSIONS: The timing of eye protection treatment and enucleation should be comprehensively judged to avoid worsening prognosis due to operation time delay. More importantly, the promotion and popularization of diagnosis and treatment technologies are necessary to further improve RB prognosis.

Analysis of clinical and pathological features of ciliary body medulloepithelioma.

AIM: To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma. METHODS: The clinical and pathological data of 11 patients (11 eyes) who were diagnosed with ciliary body medulloepithelioma at Beijing Tongren Hospital, Capital Medical University, from 2007 to 2021 were retrospectively analyzed. RESULTS: The initial symptoms of 11 patients included vision loss (6 eyes), atrophia bulbi (1 eye), proptosis (2 eyes), and leukocoria (2 eyes). Most patients suffered with corneal opacity, anterior chamber flare and hyphema. Iris neovascularization and synechia, complicated cataract, and secondary glaucoma occurred in several cases. Three patients even had lens subluxation and retinal detachment. B-scan ultrasonography showed vitreous opacity and a medium-high uneven echo mass in the eyeball. Ultrasound biomicroscopy examination showed a spherical or hemispherical ciliary body mass with uneven internal echoes and irregular cystic spaces. All of the 11 patients were diagnosed with malignant ciliary body medulloepithelioma by pathological evidence. In this study, 6 patients had enucleation (2 patients had systemic chemotherapy after surgery), and the other 5 patients had local tumor resection (1 patient had plaque radiotherapy after surgery). CONCLUSION: Ciliary body medulloepithelioma is a rare intraocular tumor and may be easily confused with retinoblastoma. Analyzing the clinical and pathological features of ciliary body medulloepithelioma is useful to further understand ciliary body medulloepithelioma, and can make an accurate diagnosis and better treatment.