To investigate the clinical features and prognosis of patients with orbital inflammatory myofibroblastic tumor (IMT).

This retrospective study collected clinical data from 22 patients diagnosed with orbital IMT based on histopathological examination. The patients were followed up to assess their prognosis. Clinical data from patients, including age, gender, course of disease, past medical history, primary symptoms, ophthalmologic examination findings, general condition, as well as imaging, laboratory, histopathological, and immunohistochemical results from digital records were collected. Orbital magnetic resonance imaging (MRI) and(or) computed tomography (CT) scans were performed to assess bone destruction of the mass, invasion of surrounding tissues, and any inflammatory changes in periorbital areas.

The mean age of patients with orbital IMT was 28.24±3.30y, with a male-to-female ratio of 1.2:1. Main clinical manifestations were proptosis, blurred vision, palpable mass, and pain. Bone destruction and surrounding tissue invasion occurred in 72.73% and 54.55% of cases, respectively. Inflammatory changes in the periorbital site were observed in 77.27% of the patients. Hematoxylin and eosin staining showed proliferation of fibroblasts and myofibroblasts, accompanied by infiltration of lymphocytes and plasma cells. Immunohistochemical staining revealed that smooth muscle actin (SMA) and vimentin were positive in 100% of cases, while anaplastic lymphoma kinase (ALK) showed positivity in 47.37%. The recurrence rate of orbital IMT was 27.27%, and sarcomatous degeneration could occur. There were no significant correlations between recurrence and factors such as age, gender, laterality, duration of the disease, periorbital tissue invasion, bone destruction, periorbital inflammation, tumor size, fever, leukocytosis, or treatment (P>0.05). However, lymphadenopathy and a Ki-67 index of 10% or higher may be risk factors for recurrence (P=0.046; P=0.023).

Orbital IMT is a locally invasive disease that may recur or lead to sarcomatoid degeneration, primarily affecting young and middle-aged patients. The presence of lymphadenopathy and a Ki-67 index of 10% or higher may signify a poor prognosis.

To analyze the general profile, clinical symptoms, pathological features, and prognostic characteristics of extranodal Rosai-Dorfman disease (RDD) with ocular involvement.

This was a retrospective series of case study. Clinical data from 35 cases who had extranodal RDD with ocular involvement were collected for analysis, including 5 cases diagnosed at our hospital and 30 reported in the literature which searched via PubMed, the Chinese National Knowledge Infrastructure (CNKI), the Chinese Science and Technology Periodical Database (VIP), and WanFang Data database from database creation to April 2023. Lesion location, clinical presentation, pathological presentation, treatment modality, follow-up time, and prognosis were recorded.

Lesions of five cases were located in the orbit, eyelid, lacrimal gland, or conjunctiva. The main presenting features were proptosis, eyelid swelling, and conjunctival hyperemia with decreased vision. Four patients underwent surgical resection, one received surgery and adjunctive immunosuppression, and none experienced recurrence during follow-up. A total of 30 cases were retrieved from the literature. The mean age was 41.4y, and 66.7% were male. The lacrimal gland and conjunctiva/subconjunctiva were the most commonly affected sites (each 20.0%). Most patients received surgical management (50.0%) or immunosuppressive therapy (20.0%). Only one recurrence (3.3%) was reported during follow-up.

When symptoms like ocular protrusion and visual acuity loss occur, RDD should be considered in the differential diagnosis. The diagnosis of RDD primarily depends on pathological histology, which serves as the key basis for confirmation. Although RDD generally has a favorable prognosis, long-term follow-up of patients is still essential to closely monitor for potential recurrence.

To examine carcinoma ex pleomorphic adenoma (CXPA) and its development to provide information for its clinical assessment and prognosis.

The clinical data of 26 patients with pathologically diagnosed CXPA were included for analysis. The patients’ medical histories and data (e.g., gender, age, eye laterality, clinical manifestations, pathologic and immunohistochemical indices, treatments, and prognosis) were recorded.

The average age of the 26 patients was 59.6±15.7y. There was no significant difference in the gender distribution. The incidence of CXPA bone destruction was approximately 57.7%, and the incidence of optic nerve involvement and extraocular muscle involvement was approximately 15.4% and 19.2%, respectively. The most common pathological type was adenocarcinoma (34.6%), followed by ductal carcinoma (26.9%). Five patients had recurrence or metastasis (19.2%). The 5-year recurrence-free survival rate was 59.0%. There were no significant differences in survival rates among patients with different pathologic types and stages (P>0.05). Bone destruction, nerve invasion, and peripheral tissue invasion did not significantly affect survival rate (P>0.05). Surgery combined with ¹²⁵Ⅰ radiation therapy leads to a better survival prognosis (P<0.05).

CXPA has a variety of pathologic classifications, with characteristics of bone destruction and peripheral tissue invasion. Surgery combined with ¹²⁵Ⅰ endoradiotherapy is a preferable treatment option. However, long-term follow-up and close observation for recurrence or metastasis should be performed.

To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma.

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.

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).

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.

To analyze the differences in immune indicators and prognosis between IgG4-positive and negative lacrimal gland benign lymphoepithelial lesion (LGBLEL).

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.

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.

Serum C4 and IgG1 are the factors affecting recurrence of LGBLEL, while the IgG4 does not affect recurrence of LGBLEL.

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).

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.

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.

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.

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.

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.

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.

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.