Orbital Solitary Fibrous Tumors: A 20-Year Cases Series Study in a Single Center.

PURPOSE: To investigate the clinical manifestations, imaging, pathology, and prognosis of orbital solitary fibrous tumors (OSFTs). In addition, the surgical incisions and the treatment outcomes were also evaluated. METHODS: A total of 89 patients with pathologically confirmed primary OSFTs were enrolled onto this study. Clinical and histopathological characteristics, imaging data, treatment modalities, and follow-up time, including tumor recurrence and death, were documented. The outcome measures included overall survival and disease-free survival time. RESULTS: Among 89 eligible cases, the median age of presentation was 39 years (range: 5-80 years) at the initial diagnosis. The most common presenting symptom was painless proptosis (54, 60.67%), then palpable mass (31, 34.83%), swelling (29, 32.58%), and impaired ocular motility (27, 30.34%). Tumor-related severe impaired vision was found in 11 patients (12.36%), including no-light-perception blindness (6, 6.74%), light-perception (2, 2.25%), and hand-movement (1, 1.12%). The preoperative imaging (computed tomography and magnetic resonance imaging) accurate diagnostic rate of OSFTs was 17.98% (16, 95% CI: 0.098-0.261), and misdiagnosis rate was 25.84% (23, 95% CI: 0.166-0.351). Grossly intact masses were excised for 27 patients (30.34%). Among the 89 patients, 33 (37.08%) were recurrences, and the median of these recurrent patients' interval between the first and the last operation was 7.33 years (range: 0.12-29.69 years). In 81 patients with complete follow-up data, the median course of the disease was 9.64 years (range: 1.55-33.65 years) from the onset OSFT. The overall survival rate of the 81 patients was 93.83% with a median course of 8.48 years (range: 0.38-30.4 years) from diagnosis of OSFT, and the disease-free survival rate of 81 patients was 91.36% with a median follow-up of 4.76 years (range: 0.08-19.22 years) after the last surgery. Of all the 81 patients, 5 patients (6.17%) developed local recurrence, and 3 patients (3.70%) died from tumor-related diseases, including pulmonary metastasis (2, 2.47%) and complications from intracranial lesions (1, 1.23%). Ten patients (11.24%) received postoperational radiation therapy, including 125I seeds implantation (5, 6.17%) and external beam radiotherapy (5, 6.17%), and remained no recurrence. CONCLUSIONS: In this series, OSFTs showed long courses and easy recurrence. Although it was very important to choose a proper surgical incision for intact resection of OSFTs at the initial surgery to avoiding recurrence, preoperative imaging is of very limited use since it is not able to identify OSFTs effectively. Postoperative radiotherapy may be beneficial to reduce the recurrence of OSFTs with malignant pathologic features.

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.

Clinical Analysis of Primary Malignant Lacrimal Sac Tumors: A Case Series Study With a Comparison to the Previously Published Literature.

PURPOSE: To report management of a series of patients with primary malignant lacrimal sac tumors and to compare these results to the previously published literature. MATERIALS AND METHODS: A total of 27 patients with pathologically confirmed primary malignant lacrimal sac lesions were enrolled into this study. Pathological classifications, clinical characteristics, various treatment modalities and follow-up time, including tumor recurrence, were documented. The outcome measures included overall survival, progression-free survival, and median survival time. RESULTS: Among 27 eligible cases, 33.33% (9/27) of the tumor was non-Hodgkin B-cell lymphoma, and 33.33% (9/27) was squamous cell carcinoma; both were the most common tumor in this series, followed by adenocarcinoma 18.52% (5/27), then melanoma 7.41% (2/27). Treatment modalities included surgery, radiotherapy, and/or chemotherapy, the overall survival rate of 27 patients was 70.37%, with a median follow-up of 45 months (range: 7 mo-16 y), 8 patients had died from metastatic disease, but 13 patients remained without evidence of recurrent tumor. The 5-year overall survival and progression-free survival for all cases were 73.33% and 66.67%, respectively. The median survival time for 5 deceased patients with interstitial brachytherapy was 98 months, and 5-year survival rate was 60%. CONCLUSIONS: In this series, among primary malignant lacrimal sac tumors, the proportion of lymphoma had increased when compared with the previously published literature, and multidisciplinary therapy may lead to a good prognosis in the majority of patients with the tumors and patients may benefit more from interstitial brachytherapy than external beam radiotherapy.

Similar therapeutic effects of 125I seed radiotherapy and γ-ray radiotherapy on lacrimal gland adenoid cystic carcinoma.

AIM: To evaluate the survival outcomes of patients with lacrimal gland adenoid cystic carcinoma who underwent eye-sparing surgery combined with 125I seed implantation radiotherapy or local external γ-ray radiotherapy. METHODS: In this retrospective comparative case series, the clinical records of 27 primary and 8 recurrent patients were reviewed. Univariate and multivariate analyses were used to identify risk factors associated with distant metastasis (DM), and the overall survival (OS) after the initial surgery was analyzed. RESULTS: The median follow-up after radiotherapy was 36mo (range 6-120mo). At the last follow-up after radiotherapy, 26 (74.3%) patients had no evidence of disease, 7 (20%) patients had DM, 2 (5.9%) patients died of DM, and 1 patient with DM was lost to follow-up. Univariate analyses showed that duration of symptoms, bone destruction, T stage classification, and wide excision surgery were risk factors influencing DM (P<0.05). The 5-year and 10-year OS rates after the initial surgery were 95.8% and 79.9%, respectively. The 5-year DM-free survival and disease-free survival rates after radiotherapy were 66.4% and 52.7%, respectively. CONCLUSION: 125I seed radiotherapy and local external γ-ray radiotherapy may have similar therapeutic effects in preventing DM. Patients with T1/T2 stage disease have a better prognosis than those with T3/T4 stage disease.

Clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma and intracranial segment of Retrobulbar optic nerve invasion.

BACKGROUND: To analyze the clinical characteristics, treatment and prognosis of children with unilateral retinoblastoma (RB) and intracranial segment of retrobulbar optic nerve invasion. METHODS: A total of 14 children with unilateral RB and intracranial segment of retrobulbar optic nerve invasion were enrolled in this retrospective study from January 2009 to December 2018. Clinical characteristics, treatment and prognosis were collected and analyzed. Survival curves were calculated by Kaplan-Meier method. RESULTS: Of 14 cases, there were 7 male and 7 female, ranging in age from 22.85 to 121.97 months (median, 41.03 months). Seventy-one percent of patients came from first-tier cities in China and effected in the left eye. Magnetic resonance imaging (MRI) results indicated that all patients presented with thickened and enhanced optic nerve and intracranial segment of optic nerve invasion. Nine patients received comprehensive therapeutic regimen (chemotherapy, eye enucleation, radiotherapy and intrathecal therapy). The patients were followed up to December 2019, with a median follow-up of 20.6 months. The median disease specific survival was 48.99 ± 8.62 months, and the overall survival (OS) rate was 64.3%. Radiotherapy and comprehensive therapeutic regimen had significant impact on survival time (all p < 0.05). CONCLUSIONS: The overall prognosis of unilateral RB patients with intracranial segment of retrobulbar optic nerve invasion was poor. Chemotherapy and surgical treatment were necessary, but more attention should be paid to radiotherapy and intrathecal therapy for improving prognosis.

Clinical and histopathological features of adenomas of the ciliary pigment epithelium.

PURPOSE: Adenomas of the ciliary pigment epithelium (CPE) are rare benign tumours which have mainly to be differentiated from malignant ciliary body melanomas. Here we report on a consecutive series of patients with CPE adenomas and describe their characteristics. METHODS: The retrospective hospital-based case series study included all patients who were consecutively operated for CPE adenomas. RESULTS: Of the 110 patients treated for ciliary body tumours, five patients (4.5%) had a CPE adenoma. Mean age was 59.0 ± 9.9 years (range: 46-72 years). Mean tumour apical thickness was 6.6 ± 1.7 mm. Tumour colour was mostly homogenously brown to black, and the tumour surface was smooth. The tumour masses pushed the iris tissue forward without infiltrating iris or anterior chamber angle. Sonography revealed an irregular echogram with sharp lesion borders and signs of blood flow in Color Doppler flow imaging. Ultrasonographic biomicroscopy demonstrated medium-low internal reflectivity and acoustic attenuation. In magnetic resonance imaging (MRI), the tumours as compared to brain were hyperintense on T1-weighted images and hypointense on T2-weighted images. Tumour tissue consisted of cords and nests of pigment epithelium cells separated by septa of vascularized fibrous connective tissue, leading to a pseudo-glandular appearance. The melanin granules in the cytoplasm were large and mostly spherical in shape. In four patients, the tumours were hyperpigmented. Tumour cells were large with round or oval nuclei and clearly detectable nucleoli. CONCLUSIONS: These clinical characteristics of CPE adenomas, such as homogenous dark brown colour, smooth surface, iris dislocation and anterior chamber angle narrowing but no iris infiltration, segmental cataract, pigment dispersion, and, as compared to brain tissue, hypointensity and, as compared to extraocular muscles or lacrimal gland, hyperintensity on T2-weighted MRI images, may be helpful for the differentiation from ciliary body malignant melanomas.

[The clinical characteristics and prognosis of 92 cases of delayed occurrence of retinoblastoma].

OBJECTIVE: To retrospectively summarize the clinical features, diagnosis and treatment of retinoblastoma (RB) patients in the children aged 5 years or above. METHODS: This was a case-series study. Total of 92 patients (107 eyes) with RB aged 5 years or above in Department of Pediatric,Beijing Tongren Hospital from September 2005 to May 2010 were included in the study. The diagnosis of RB was established using clinical examination and histopathology analysis.Statistical analysis in the characteristics of disease and initial symptoms was performed in 92 cases. Kaplan-Meier curve was employed to analyze the survival function. RESULTS: (1) The medium age of the patients was 6.25-year. Male accounted for 66.30% (61/92) and female was 33.69% (31/92) . According to the results of statistical scatter diagram, the age of the disease onset was ranged from 5.00 to 8.33 years. Percentile for age 5.77, 5.83 and 7.85 years were the 25th, 50th, 75th respectively. (2) In 92 cases, 83.69% (77/92) of the patients were unilateral RB and 16.31% (15/92) were bilateral RB. (3) Leukocoria was the most common manifestation in the diagnosis early RB, accounting for 55.43% (51/92) .Secondary symptoms were blurred vision, eye redness and photophobia, accounting for 23.91% (22/92). Other symptoms included strabismus, proptosis, glaucoma and the abnormal in fundus examination, accounting for 20.64% (19/92) .No family history of RB was found in all of cases studied. (4) Among 107 eyes with RB, 76 eyes were enucleated up to February 2013. The rate of enucleation was 71.02%. (5) By February 2013, the average following-up time was 39 months, during the time 1 case was lost, 6 cases were dead, the overall survival rate was 93.46% (85/92) . The result of Kaplan-Meier curve showed that the estimates of 5-year expected average total survival time was (72.61 ± 1.89) months(95%confidence interval was 68.9-76.3 months). CONCLUSIONS: Unilateral RB are the most common cases in the age of 5 years or above, and the most common symptom is Leukocoria.Overall survival rate of older RB patient is high with comprehensive treatment.

Multidisciplinary collaborative therapy for 30 children with orbital rhabdomyosarcoma.

OBJECTIVE: To explore clinical experience and propose new ideas for treating children diagnosed with orbital rhabdomyosarcoma (RMS). METHODS: We retrospectively analyzed the clinical data for30 patients (16 males and 14 females, with a median age of 6.2 years) with primary orbital RMS who were enrolled in the Department of Eye Oncology and Pediatrics of our hospital from November 2004 to December 2012. International Rhabdomyosarcoma Organization Staging Standards indicated that among the 30 patients, 4 cases were in phase II, 20 were in phase III, and 6 were in phase IV. All patients underwent a multidisciplinary collaborative model of comprehensive treatment (surgery, chemotherapy, external radiotherapy, 125I radioactive particle implantation, and autologous peripheral blood stem-cell transplantation). RESULTS: Follow-up was conducted until March 2013, with a median follow-up time of 47.2 months (5 to 95 months), and 7 deaths occurred. The 2-year estimated survival rate reached 86.1%, the ≥3-year estimated survival rate was 77%, and the 5-year estimated survival rate was 70.6%. CONCLUSIONS: The multidisciplinary collaborative model can be a safe and effective approach to the comprehensive treatment of children with orbital RMS. It has clinical significance in improving the tumor remission rate.

[Transnasal endoscopic removal of orbital apex cavernous hemangioma].

OBJECTIVE: To explore the strategy and indications of endoscopic transnasal resection of orbital apex cavernous hemangiomas (OACH). METHODS: Eleven patients aged from 30 to 62 years-old diagnosed as OACH bypostoperative histopathology were reviewed retrospectively. Four males and 7 females were included. Both ophthalmological examination and rhinologic evaluation were adopted preoperatively. The surgeries were carried out under general anesthesia endoscopically by the same senior surgeon. After ethmoidectomy, the orbital lamina papyracea was opened, and the orbital fat and muscles were pushed back into the orbit by using the brain cotton, and then the orbital tumor was removed. The patients were kept follow-up both in ophthalmologic and rhinologic departments. RESULTS: As suggested by preoperative imagings, 4 lesions located in the extraconal space (nasal side), 6 between the optic nerve and the internal rectus muscle of the intraconal space, and 1 outside the optic nerve in the intraconal space. Total resection was achieved in 9 cases, and orbital decompressions were done in 2 cases. Meanwhile, orbital wall reconstruction was done in 7 cases. The follow up ranged from 6 to 47 months. Seven patients achieved visual acuity improvement and no deteriorations were found in other 4 patients. Defects of vision field in 3 patients disappeared after 2 weeks. No operative or postoperative complications occurred. CONCLUSIONS: The OACH located in the nasal side of extraconal space and between the optic nerve and the internal rectus muscle of the intraconal space can be accessed endoscopically by intranasal approach. Using the brain cotton to push the orbital fat and muscles back into the orbit and an experienced endoscopic surgeon are important to access a successful intranasal endoscopic removal of orbital apex tumor.

Retinoblastoma in a young adult mimicking Coats' disease.

Retinoblastoma is the most common childhood primary intraocular malignancy, with the majority of cases being diagnosed before 5 years of age. Retinoblastoma in adults is extremely rare. Here, we report the case of a 20-year-old man who presented with a 3 year history of blurred vision in the right eye. Imaging did not reveal the typical presentation of retinoblastoma. After considering Coats' disease, a diagnosis of late-presenting retinoblastoma was made through cytological analysis. Diagnosis of retinoblastoma should be considered in the presence of uncertain mass lesions in the fundus of an adult.

Epithelial tumors of the lacrimal gland in the Chinese: a clinicopathologic study of 298 patients.

OBJECTIVE: To examine the clinicopathologic characteristics and clinical features of epithelial tumors of lacrimal gland in China. METHODS: The retrospective case series study included all 298 patients of epithelial lacrimal gland tumors which had been collected in the ophthalmic pathologic laboratory of the Tongren Hospital Beijing in the study period from 1961 to 2005. RESULTS: Pleomorphic adenomas were the most common tumors (n = 213 (72%)), followed by adenoid cystic carcinoma (n = 58 (20%)), pleomorphic adenocarcinoma (n = 13 (4%)), primary adenocarcinoma (n = 8 (3%)) and other tumors (n = 6 (2%)). The most frequent symptoms and clinical signs were restrictions of the lid or eye motility (121 patients; 41%), any other kind of lid involvement (71 patients; 24%), swelling (57 patients; 19%), pain (57 patients; 8%), and decrease in vision (15 patients; 5%). A restriction of the eye motility was noted for 61 patients (21%), due to an involvement of the extraocular muscles with the tumor and due to a tumor-induced displacement of the globe. Apart from the duration of symptoms, the tumor types did not vary significantly in age, gender, and laterality. CONCLUSIONS: Based on the archives of the Beijing Tongren ophthalmo-pathological laboratory, the most common epithelial tumors of the lacrimal gland in mainland China were pleomorphic adenomas, followed by adenoid cystic carcinoma. The spectrum and clinical signs of epithelial lacrimal gland tumors did not differ markedly between Chinese patients and Caucasian patients.

Sphenoid wing meningioma en plaque: report of 37 cases.

BACKGROUND: Sphenoid wing meningioma en plaque is a special morphological subgroup of intracranial meningiomas, defined by a carpet-like, soft tissue component that infiltrates the dura and invades the sphenoid wing and orbit associated with a significant hyperostosis. This report summarized our experiences in 37 patients with sphenoid wing meningioma en plaque who had been treated with transcranio-orbital approach surgery. METHODS: A retrospective study was made on clinical manifestations, neuroradiological features, and operative techniques in 37 patients undergoing transcranio-orbital approach from Sep. 1998 to Apr. 2009. Patients ages: 16 years to 67 years, 45.5 years in average; sex: 15 males, 22 females. Chief complaints were progressive proptosis and visual acuity deficits. All patients were operated on using a fronto-temporal approach with orbital decompression. The extent of tumor resection and postoperative complications were investigated. RESULTS: Simpson grade II resection was achieved in 9 patients, Simpson grade III in 22 patients and Simpson grade IV in 6 patients. Pathological examination showed 27 (73%) patients were meningothelial meningiomas. After surgery, proptosis improved in all patients, visual acuity improved in 18 patients (69%). Temporary ophthalmoplegia was found in 8 patients, cerebrospinal fluid leak was found in 1 patient. Duration of follow up was from 3 months to 9 years, tumor recurred in 7 patients, and 5 patients underwent second surgery, including two trans-nasal endoscopic surgeries to resect sphenoid sinus-involved tumor. There were no operation-related deaths or other significant complications. CONCLUSIONS: Sphenoid wing meningioma en plaque, mainly meningothelial meningiomas, are more likely to produce adjacent hyperostosis and have characteristic radiological appearances. All the hyperostosis bone of the great wing of sphenoid bone should be removed to prevent recurrence. Extensive tumor removal with bony decompression at the orbital apex can produce satisfactory cosmetic and functional outcome. Close co-operation between the neurosurgeons and the ophthalmologists is important.

Malignant neurilemoma with xeroderma pigmentosum.

UNLABELLED: Xeroderma pigmentosum is a rare autosomal recessive disease characterised by hypersensitivity to sunlight, and is associated with a high incidence of skin cancer. We report a case of xeroderma pigmentosum with malignant neurilemoma in a 46-year-old woman which is unique due to its presentation, which was confirmed histopathologically. TRIAL REGISTRATION NUMBER: 31095.

[Effects of placement of orbital implant on orbital osteocytes].

OBJECTIVE: To investigate the influence of enucleation with or without orbital implant on the occurrence of apoptosis of orbital osteocytes and to explore the mechanism of orbital implant in the prevention and treatment of orbital malformations. METHODS: It was an experimental research. Twenty-one age and weight-matched New Zealand rabbits were divided into three groups: enucleation group, enucleation with implant group and the control group. At one-month-old, the left eyes of the rabbits were enucleated in the enucleation group; an orbital implant was inserted after enucleation in the implant group. The left orbits of rabbits in the control group were served as the controls. At two-month-old, all rabbits were sacrificed, apoptosis of osteocytes of the zygomatic bones was observed by photomicroscopy, electron microscopy and TUNEL staining technology. The ratios of apoptosis osteocytes were calculated and analyzed in these three groups. RESULTS: Classical apoptosis of osteocytes was found with photomicroscopy and electron microscopy. The distribution of apoptosis of osteocytes was irregular in bone sections. The ratio of apoptosis cells in the enucleation group was significantly different from that in the other two groups (P<0.01). There was no significant difference in ratios of apoptosis cells between the implant group and the controls (P>0.05). CONCLUSIONS: Apoptosis of osteocytes participates in the normal development of bony orbit. The results of this study indicate that apoptosis of osteocytes plays a role in the development of orbital malformation after enucleation and orbital implant can prevent the orbital malformation after enucleation.

[Clinical study of lacrimal gland tumor involving anterior and middle cranial fossae].

OBJECTIVE: To investigate the clinical manifestations of lacrimal gland tumor involving the anterior and middle cranial fossae and the effect of transcranial-orbital approach in treatment of such tumor. METHODS: A retrospective study was conducted on the clinical data of 23 cases lacrimal gland tumor involving the anterior and middle cranial fossae confirmed by radiological examination, including 11 cases of adenoid cystic carcinoma, 6 cases of pleomorphic adenocarcinoma (malignant mixed tumor), 2 cases of adenocarcinoma, 1 case of squamous cell carcinoma, 1 case of ductal carcinoma, 1 case of mucoepidermoid carcinoma, and 1 case of benign mixed tumor, 15 males and 8 females, aged 42.5 (2 - 76), with a case history of 43 months (2 months to 27 years), with the chief complaints of progressive proptosis, disgenesia of the eye ball, and orbit pain, all undergoing transcranial-orbital operation from August 1998 to February. 2006. Follow up was conducted for 1 month to 7 years. RESULTS: Postoperatively, ophthalmoplegia was found in 4 cases, and blindness in 1 case. There was no operative death or other significant complication. Recurrence of tumor occurred in 4 cases, and 1 case died from distant metastasis of adenocarcinoma. CONCLUSIONS: Malignant lacrimal gland tumors, mainly adenoid cystic carcinomas, incline to involve the anterior and middle cranial fossae. Adequate orbital apex decompression and exposure of the tumor can result from suitable transcranial-orbital approach. However, complete surgical excision is difficult, and the tumor has a tendency to recur post-operatively. Suitable treatment strategy should by combination of operation with irradiation or chemotherapy. Prognosis is poor.

[Resection of optic nerve sheath meningioma via transcranio-orbital approach].

OBJECTIVE: To investigate the clinical effect of early stage operation for optic nerve sheath meningioma. METHODS: Retrospective study The clinical data of 21 cases of optic nerve sheath meningioma, 6 males and 25 females, aged 39 (12-60), with the chief complaints of loss of vision in 13 eyes (including 6 blind eyes) and proptosis in 8 eyes who underwent resection of the tumors via transcranio-orbital approach were analyzed retrospectively. Radiology showed 15 tumors growing along the optic nerve and enclosing it (flat type), 5 of which showed intracranial invasion; and other 6 of which had the tumors located on one side of optic nerve with spherical shape (nodular type) without intracranial invasion. The patients were followed up for 2-6 years. RESULTS: Total resection was accomplished in 15 cases, and partial removal in 6 cases. Function of oculomotor nerve was preserved in 17 cases, and function of abducent nerve was preserved in 18 cases, visual function was successfully preserved in the 2 cases of small tumor. Tumor recurred in two cases. CONCLUSION: Resection of optic nerve sheath meningioma via transcranio-orbital approach has the advantage of totally removing the tumor and preserving the function of the oculomotor and abducent nerves, preventing tumor from intracranial invasion. Early stage operation is recommended. Visual function may be preserved in the cases of small tumors.

[Clinical and pathological features of 112 cases with ocular adnexal lymphoproliferative lesions].

OBJECTIVE: To investigate the histopathological classification and clinical features of ocular adnexal lymphoproliferative lesions. METHODS: The clinical, histomorphological and immuno-histochemical features of 112 cases of ocular adnexal lymphoproliferative lesions (116 paraffin specimens) were studied retrospectively. The lesions were classified according to the World Health Organization classification of tumors of haematopoietic and lymphoid tissues (2001). RESULTS: This group of patients' ages averaged at 49. The mean duration between the onset of the symptoms and the time of presentation was 22 months. Sixteen patients (14.3%) had bilateral lesions. Proptosis or local orbital mass was presented in 69 cases (61.6%). Reactive lymphoid hyperplasia was diagnosed in 11 cases (9.8%) and atypical lymphoid hyperplasia in 10 cases (8.9%). The rest 91 cases (81.3%) were diagnosed as lymphoma, among which 74 cases (81.3%) were extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-EMZL). CONCLUSIONS: MALT-EMZL is the most frequent type of ocular lymphoproliferative lesions in ocular adnexa. The ocular adnexal lymphoproliferative lesions show an indolent course clinically, tending to affect the old. Bilateral case is not uncommon.

[Clinical and pathological features of 273 cases of lacrimal epithelial tumors].

OBJECTIVE: A retrospective study of histopathological and clinical aspects of 261 cases of lacrimal tumors (273 paraffin specimens) from Ophthalmic Pathology Laboratory, Beijing Tongren Hospital (Dec. 1961 to Jun. 2002) was performed to investigate the pathological classification and clinical features of these tumors. METHODS: Clinical features were analyzed from 261 patients of lacrimal tumors, including patient's history, age, gender, laterality, visual acuity, ultrasound B scan, X-ray, CT, MRI, pre- and post-operation condition, follow-up data and others. These data were compared with the pathological features. RESULTS: Pleomorphic adenoma was the most common tumor in epithelial tumors of lacrimal gland, followed by adenoid cystic carcinoma, pleomorphic adenocarcinoma and adenocarcinoma. Recurrence was observed in 12 cases. The recurrence rate is 4.6%. Four cases was dead, the mortality was 1.5%. CONCLUSION: Pleomorphic adenoma shows a low mortality and a relatively high recurrence rate. The main treatment for pleomorphic adenoma is surgical treatment. It is important to have a correct clinical diagnosis, to select the proper surgical method, drug treatment and postoperative management based on the pathological diagnosis, which can result in a decrease of recurrence rate.

[Clinical and pathologic analysis of orbital pseudotumor].

OBJECTIVE: To investigate the relationship between the histopathology and the imaging analysis of orbital pseudotumor to judge the histopathological types of these tumors before treatment according to imaging analysis, in order to search for different therapeutic approaches for different pathologic types and to predicate prognosis. METHODS: Fifty-six cases (58 eyes) of orbital pseudotumor that had been operated in Tongren hospital were studied. The clinical manifestations of these patients were reviewed. Changes in the imaging analysis and histopathologic examination were observed and their relationship was studied. Methods of treatment and their prognosis were also analyzed. RESULTS: Historically, the lymphocytic infiltrated type of orbital pseudotumor composed of large amounts of chronic inflammatory cells, only a small amount of collagen was found, so the imaging features of the lymphocytic infiltrated type presented as low or none reflecting areas with a clear posterior demarcation on B scan, and the signals were low or equal on T(1)WI and high on T(2)WI, which could be enhanced by contrast. Oppositely, the fibrotic type was composed of large amounts of fibrotic tissue, and few chronic inflammatory cells were found, so that only the anterior border of the lesion could be identified and the posterior demarcation was blur on the B scan, and the signals were low or equal on T(1)WI and low on T(2)WI, which could not be enhanced or could be enhanced only slightly by changing the contrast. The mixed type presented as an admixture of chronic inflammatory cells and fibric hyperplasia, and the imaging of the mixed type was midway between the lymphocytic infiltrated type and the fibrotic type. Forty-two patients of lymphocytic and mixed types which were identified by histopathology and imaging analysis received a local and systemic corticosteroid therapy. The effect was unstable, and recurrence occurred easily. Therefore, operations were performed on these patients, thirteen of them were treated with low-dose radiotherapy (2 000 - 3 000 cGy) two weeks after operation. Fifteen cases recurred after the operation and 4 cases recurred after the radiotherapy. The fibrotic type of orbital pseudotumor was identified in 12 cases in which no effect could be observed after conservative therapy and operations were performed subsequently. Two cases recurred and were operated again. Exenteration was performed in 2 cases of 56 cases. The cure rate of the composite treatment in the present series was 68.5%. CONCLUSIONS: Different types of orbital pseudotumor present different imaging exhibition, and imaging features are closely related with histopathological changes. Lesions can be localized by CT scan, and also can be localized by B scan and MRI. While it is difficult to obtain a cure result in the orbital pseudotumor, the cure rate can be improved by composite treatment.