Clinical, morphologic, immunophenotypic, and molecular genetic analysis of bilateral ocular adnexal lymphoid neoplasms in 17 patients

We investigated the clinical, morphologic, immunologic, and molecular genetic characteristics of the lymphoid tumors occurring in 17 patients with bilateral ocular adnexal lymphoid neoplasia. We found no obvious differences in the age, sex, clinical appearance, or ophthalmic findings between patients with unilateral and those with bilateral ocular adnexal lymphoid neoplasms. Five orbital and two conjunctival lymphoid neoplasms removed from five patients were polyclonal pseudolymphomas, while nine orbital, eight conjunctival, and two eyelid lymphoid neoplasms removed from 12 patients were monoclonal B cell non-Hodgkin's lymphomas. Each pair of simultaneously bilateral lesions occurring in a single individual was morphologically and immunologically identical and exhibited identical immunoglobulin gene DNA rearrangement patterns. None of the five patients with polyclonal pseudolymphomas developed nonocular non-Hodgkin's lymphoma, but one developed a contralateral conjunctival monoclonal B cell non-Hodgkin's lymphoma. Six of the 12 patients with ocular adnexal monoclonal B cell non-Hodgkin's lymphomas developed nonocular non-Hodgkin's lymphoma. The incidence of previous or subsequent systemic nonocular non-Hodgkin's lymphoma in patients with bilateral ocular adnexal lymphoid neoplasms is comparable to that of patients with unilateral disease. In both patient groups, the development of nonocular non-Hodgkin's lymphoma is most commonly associated with ocular adnexal lymphoid neoplasms displaying follicular or diffuse small cleaved cell (poorly differentiated lymphocytic) morphologic characteristics.     

The differentiation of idiopathic inflammatory pseudotumor from lymphoid tumors of orbit: Analysis of 319 cases

purpose To analyze the clinical, morphologic, immunophenotypic, and molecular genetic differences between idiopathic inflammatory pseudotumor and lymphoid tumors of the orbit. methods 209 patients with IOIP and 110 patients with lymphoid tumors seen between January 1, 1978 and December 31, 1999 in Zhongshan Ophthalmic Center, Sun Yat-sen University were evaluated retrospectively. results More patients with lymphoid tumors had palpable mass than patients with idiopathic orbital inflammatory pseudotumor (IOIP) (P < 0.0001), with the percentage of 90% and 65%, respectively, whereas more patients with IOIP had swollen eyelid, eyelid or conjunctival congestion, pain, retinal folds or hamorrhage, and optic nerve atrophy than patients with lymphoid tumors, with the percentage of 55% and 40% (P = 0.014), 42% and 24% (P = 0.001), 24% and 1% (P < 0.0001), 14% and 4% (P = 0.004), 7% and 2% (P = 0.043), respectively. Ultrasound and computed tomography/magnetic resonance image (CT/MRI) scan usually could not differentiate IOIP from lymphoma. Nearly one third of patients with IOIP could not be easily differentiated pathologically from lymphoid tumor. Immunophenotypic, and molecular genetic analyses can differentiate IOIP from lymphoid tumor based on polyclonal or monoclonal proliferation of lymphocytes with the IOIP being polyclonal and lymphoma monoclonal. conclusions Idiopathic orbital inflammatory pseudotumor and lymphoid tumor showed distinguishing clinical, morphologic, immunophenotypic, and molecular genetic characteristics.     

Incidence of benign and malignant lesions of eyelid and conjunctival tumors

PURPOSE: To examine the incidence of benign and malignant eyelid lesions and conjunctival tumors. SUBJECTS AND METHODS: One-hundred-and twenty-eight cases (131 eyes) which were treated during the period from January 1990 to February 2004 were histopathologically diagnosed for eyelid or conjunctival tumors (87 cases of eyelid tumors and 41 cases of conjunctival tumors) in retrospective evaluations. The incidence of benign or malignant lesions, the pathological classification, age, sex, and clinical diagnostic accuracy were all investigated. RESULTS: Sixty-four (73%) of the tumors were found to be benign eyelid tumors. The common benign eyelid tumors were 14 nevocellular nevi, 9 seborrheic keratosis, 7 epidermoid cysts, and 6 papillomas. Twenty-four (27%) eyelid tumors were malignant. These included 9 basal cell carcinomas, 9 sebaceous gland carcinomas, 4 malignant lymphomas, and 2 metastatic tumors. Thirty-four (79%) conjunctival tumors were benign, and the common benign conjunctival tumors were 9 nevocellular nevi and 7 papillomas. Nine (21%) conjunctival tumors were malignant, comprising 7 malignant lymphomas and 2 squamous cell carcinomas. The mean ages of malignant eyelid and conjunctival tumor patients were significantly older than those of benign tumor patients. Clinical accuracy in predicting basal cell carcinoma and sebaceous gland carcinoma was 11.1% and 44.4%, respectively. CONCLUSIONS : Approximately 70 approximately 80% of all eyelid and conjunctival tumors are benign. Clinicians should suspect that the lesions are malignant when seeing elderly patients with eyelid or conjunctival tumors. Excised eyelid lesions should be submitted for histopathologic confirmation because there are some cases where clinical diagnosis does not match pathological diagnosis.     

Lymphoproliferative lesions of the lacrimal gland: clinicopathological, immunohistochemical and molecular genetic analysis

BACKGROUND: Lacrimal gland lymphoproliferative disorders are usually classified as orbital adnexal tumours. Because the lacrimal gland is the only orbital structure with native lymphocytes, we examined cases with primary involvement of the gland. METHODS: The 14 cases were selected from a review of all cases in the surgical pathology files of the Ottawa Hospital between 1992 and 2003. The lesions were categorized according to the latest World Health Organization classification of tumours of lymphoid tissues. We conducted a clinical, histopathological, immunohistochemical, immunophenotypic and molecular genetic analysis of the cases. RESULTS: The 8 female and 6 male patients, aged 20 to 88 (mean 60) years, were followed for an average of 4 years (range 11 months to 13 years). All presented with supratemporal orbital swelling. The 5 primary lymphomas, of mucosa-associated lymphoid tissue (MALT), were confined to the lacrimal gland (stage IE); 1 tumour transformed to diffuse large B-cell lymphoma, necessitating chemotherapy, and the other 4 were treated with radiation. One of the 5 patients had previously had Sj?gren's syndrome. The 6 secondary lymphomas (4 follicular) presented either concurrently with systemic lymphoma or up to 12 years afterwards and were treated in a variety of ways; all the patients had an orbital relapse. At the last follow-up assessment, 6 of the patients with lymphoma had no evidence of disease, 3 were alive with disease, 2 had died (1 of lymphoma, the other with no evidence of disease), and the status of 1 patient was not known. Of the 3 patients with reactive proliferations, 2 had reactive lymphoid hyperplasia (associated with Sj?gren's syndrome in 1), and 1 had Rosai-Dorfman disease. All 9 lymphomas that underwent molecular genetic analysis were of B-cell lineage, and 8 had a monoclonal rearrangement in the immunoglobulin heavy-chain gene (IgH); the 9th lymphoma showed an oligoclonal rearrangement. One lymphoma showed the t(14;18) translocation, typical of follicular lymphoma; no lymphoma showed the t(11;18) translocation, commonly found in MALT lymphoma (but only 2 cases were studied). Molecular genetic analysis was performed in 2 of the cases of reactive lymphoid hyperplasia: monoclonal IgH rearrangement was detected in 1 case (the patient with Sj?gren's syndrome), oligoclonal rearrangement in the other. INTERPRETATION: Lacrimal gland lymphomas are B-cell tumours that develop in older adults. Primary tumours, a hIgH proportion of which have MALT characteristics, have a favourable prognosis. Molecular genetic studies may be useful when morphologic and immunophenotypic studies give equivocal results.     

A prevalence study of IgG4-related ophthalmic disease in Japan

Purpose

Immunoglobulin (Ig)G4-related ophthalmic disease belongs to a category of ocular adnexal lymphoproliferative disorders, the most frequent group of orbital tumors and simulating lesions. The aim of this study was to elucidate the number of IgG4-related diseases of orbital lymphoproliferative disorders and correlate ages and sex of such patients from 18 centers in Japan.

Methods

One thousand and fourteen patients with orbital lymphoproliferative disorders were enrolled in this study. All had pathologically diagnosed lymphoproliferative disorders with surgical samples of ocular adnexal tissue. Patients with conjunctival lesions and intraocular lymphoma were excluded.

Results

Of the 1,014 cases of orbital lymphoproliferative disorders 404 (39.8 %) had extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, 156 (15.4 %) had other malignant lymphomas, 191 (18.8 %) had non-IgG4 orbital inflammation, 219 (21.6 %) had IgG4-related orbital inflammation, and 44 (4.3 %) had IgG4-positive MALT lymphoma. Median age of the IgG4-related orbital inflammation group was 62 years, which is significantly lower than that of the MALT lymphoma group (median 66 years) and higher than the non-IgG4 orbital inflammation group (median 57 years). The male/female ratio was 105/114 in the IgG4-related orbital inflammation group.

Conclusions

Nearly a quarter of orbital lymphoproliferative disorders in Japan are related to IgG4.     

Long-term follow-up results of observation or radiation for conjunctival malignant lymphoma

PURPOSE: To elucidate the long-term prognosis of biopsy-proven malignant lymphoma of conjunctival origin after a treatment option of observation or radiation. DESIGN: Prospective noncomparative interventional case series. PARTICIPANTS: Thirteen patients with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) of conjunctival origin. METHODS: In the 11-year period from 1992 to 2002, 13 consecutive patients (7 female and 6 male) with ages ranging from 32 to 68 years (mean, 57) who showed malignant lymphoma of conjunctival origin were enrolled in the study. Histopathological and immunohistochemical studies of biopsy specimens of the conjunctival lesions proved extranodal marginal zone B-cell lymphoma of MALT in all patients. At the initial visit, magnetic resonance imaging (MRI) showed no orbital tumors, and gallium scintigraphy demonstrated no systemic involvement. The patients chose observation or radiation after the biopsy. MAIN OUTCOME MEASURES: Clinical disappearance of the conjunctival lymphoma lesions. RESULTS: Five patients chose 30-Gy radiation to the remaining conjunctival lesions after the biopsy, whereas the other 8 patients were simply observed without administration of any medication. During the follow-up, ranging from 1 to 11 years (mean, 5.4), 7 of these 8 patients without radiation showed spontaneous regression of the conjunctival lesions 1 to 5 years after the biopsy. Yearly MRI of the orbit and gallium scintigraphy disclosed no lesions in any of the patients. CONCLUSIONS: A large fraction of patients with MALT lymphoma of conjunctival origin had spontaneous regression after the biopsy. Follow-up without radiation is an option for patients with MALT lymphoma of conjunctival origin after the pathologic diagnosis by biopsy.     

Clinicopathologic and immunogenetic analysis of mucosa-associated lymphoid tissue lymphomas arising in conjunctiva.

PURPOSE: To identify mucosa-associated lymphoid tissue (MALT) type lymphoma in conjunctival infiltrates. METHODS: Clinical, histopathologic, immunophenotypic, and immunogenotypic studies were performed on 14 patients with conjunctival lymphoid infiltrates. Surgical biopsy specimens were subjected to histopathologic, immunohistochemical, and gene rearrangement analysis. RESULTS: Thirteen of the 14 patients (92.9%) met the diagnostic criteria for MALT lymphoma, and the remaining patient showed morphologic features of diffuse, small lymphocytic lymphoma. Genotypic analysis confirmed immunoglobulin heavy chain gene rearrangement in all of the 12 patients on whom the analysis was performed. Two patients with bilateral lesions exhibited identical immunoglobulin rearrangement patterns in each pair of lesions. All patients were alive at the last follow-up (mean: 39.9 months). Nine of the 14 patients were alive without disease, 4 had localized recurrences, and 1 had a residual tumor. CONCLUSIONS: These findings indicate that conjunctival lymphoid infiltrates usually have the features of MALT lymphoma with genotypic B lymphocytic monoclonality and a favorable prognosis.     

Characterization of lymphoproliferative lesions of the conjunctiva: immunohistochemical and molecular genetic studies

BACKGROUND: Conjunctival lymphoproliferative lesions have not been selected for independent analysis with newer immunohistochemical and molecular genetic techniques to highlight their unique profile. METHODS: Retrospective case series examined biopsies from 16 consecutive patients with conjunctival lymphoproliferative lesions. The histopathologic, immunohistochemical, and molecular genetic features were characterized, as well as the frequency of tumour type, prognostic implications, clinical features, and treatments offered. RESULTS: The diagnosis was lymphoma in 12 cases, atypical lymphoid hyperplasia (ALH) in 1 case, and reactive lymphoid hyperplasia (RLH) in 3 cases. The primary lymphomas consisted of 4 mucosa-associated lymphoid tissue lymphomas (MALTL), 1 follicular lymphoma (FL), 2 diffuse large B-cell lymphomas (DLBCLs), 1 lymphoplasmacytic lymphoma, and 1 T-cell lymphoma. Primary lymphomas were treated with radiation (n = 7), surgery (n = 1), and topical chemotherapy (n = 1). Complete remission was achieved in 8 of 9 primary lymphomas. Two cases of recurrence to the other conjunctiva were treated with radiation and both remained disease free. Secondary lymphomas included 2 DLBCL and 1 MALTL. Complete remission was seen in 2 patients after radiation plus chemotherapy, while the patient treated with chemotherapy alone was lost to follow-up. The 1 case of ALH presented bilaterally and achieved complete remission after topical chemotherapy treatments. The 3 RLH cases were surgically managed and 2 of the 3 recurred and were subsequently excised. Eleven lymphomas were of B-cell lineage by immunophenotyping. Molecular genetic studies of immunoglobulin heavy chain (IgH) gene rearrangement by polymerase chain reaction (PCR) showed clonal bands in 6 of 12 lymphomas, 1 of 3 RLH (polyclonal by immunophenotyping) and 1 ALH. BCL2-IgH [t(14;18)] rearrangement was seen in 8 of 12 cases (1 FL, 3 DLBCLs, 4 MALTLs) by real-time quantitative PCR. INTERPRETATION: Conjunctival lymphomas are predominantly B-cell type with a high prevalence of MALTL. An unexpected finding was the BCL2-IgH rearrangement seen in 4 of 5 MALTL cases in our series. The significance of this remains unclear.     

High incidence of autoimmune disease in Japanese patients with ocular adnexal reactive lymphoid hyperplasia

PURPOSE: To determine the incidence of autoimmune disease in Japanese patients with ocular adnexal lymphoid proliferations. DESIGN: Case series study. METHODS: The authors investigated the incidence of autoimmune disease in 88 patients with primary ocular adnexal lymphoid proliferations. Southern blot analysis was used to determine the presence of B-cell clonality in reactive lymphoid hyperplasia (RLH) associated with autoimmune disease. RESULTS: Histopathologic analysis indicated that 15 (17%) patients had RLH, 62 (70%) patients had mucosa-associated lymphoid tissue lymphoma (MALToma), and 11 (13%) patients had primary lymphomas. The incidence of autoimmune disease was seven (47%) of 15 patients with RLH, two (3%) of 62 patients with MALToma, and zero (0%) of 11 patients with primary lymphoma. B-cell clonality was detected in one (14%) of seven RLH patients with autoimmune disease. CONCLUSIONS: Ocular adnexal RLH with or without B-cell clonality is highly associated with autoimmune disease.     

Lymphoproliferative disease of the orbit

PURPOSE OF REVIEW: Malignant lymphoma of the ocular adnexa has been reported on for many years, but many steps forward have been recently made. This paper highlights the staging strategies and treatment options based on a review of the most updated and relevant bibliography. RECENT FINDINGS: A relevant improvement in the management of ocular adnexal lymphoma is represented by PET, which improves the diagnosis and the staging of the disease. Acquisitions have been made in the treatment: low-dose radiotherapy is confirmed for primary orbital mucosa-associated lymphoid tissue lymphoma; oral chlorambucil is proposed as an alternative; immunotherapy is proposed for the treatment of systemic disease. Interestingly, the role of Chlamydia psittaci as the possible cause of mucosa-associated lymphoid tissue lymphomas and the efficacy of doxycycline for the treatment of ocular adnexal lymphomas have been investigated with promising results. A large series of natural killer/T-cell lymphoma has been described and its lethality despite aggressive conventional chemotherapy has been confirmed. SUMMARY: Orbital lymphoma is the most common malignant tumor of the orbit and its incidence is increasing proportionally with the rise of the average survival rate of the general population. The combined efforts of orbital surgeons, hematologists, oncologists and radiotherapists have lately produced a mass of new information that can effectively improve the management of orbital lymphoma.     

Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma

OBJECTIVE: To investigate the clinical features of conjunctival lymphoid tumors and factors predictive of systemic lymphoma. DESIGN: Retrospective, observational case series. SETTING: Clinical practice of ocular oncology. PARTICIPANTS: The participants included 117 consecutive patients with lymphoid tumors of the conjunctiva treated at the Oncology Service of Wills Eye Hospital between 1974 and 1999. MAIN OUTCOME MEASURE: The main outcome measure was the development of systemic lymphoma. Cox proportional regression models were used to calculate the risk of eventual systemic lymphoma. Kaplan-Meier survival estimates were used to analyze the development of systemic lymphoma as a function of time. RESULTS: There were 55 males (47%) and 62 females (53%); 110 patients (94%) were white and 7 patients (6%) were African AMERICAN: The mean age at ocular presentation was 61 years, and the conjunctival lymphoid infiltrate was unilateral in 72 patients (62%) and bilateral in 45 patients (38%). In 8 patients (7%), initial unilateral conjunctival disease evolved into bilateral involvement over a mean of 32 months. Additional sites of ocular involvement were found in 27 patients (23%) and included lymphoid tumor in the eyelid in 3 cases, orbit in 18, choroid in 5, and vitreous in 1. Systemic lymphoma was known to exist before ocular diagnosis in 16 patients; (14%; for a mean of 51 months) and was found subsequent to ocular diagnosis in 20 patients (17; at a mean of 21 months). Therefore, of 117 patients with conjunctival lymphoid infiltration, 36 (31%) had or eventually developed systemic lymphoma, and 81 (69%) did not manifest systemic lymphoma during the mean follow-up of 38 months. Of the 64 patients with unilateral conjunctival involvement, 11 (17%) manifested systemic lymphoma, and of the 53 patients with bilateral involvement, 25 (47%) manifested systemic lymphoma. By univariate analysis, the clinical factors at date first seen predictive of the presence or development of systemic lymphoma included location of the tumor at an extralimbal site (fornix or midbulbar conjunctiva; P = 0.02) and increasing number of conjunctival tumors (P = 0.02). Using Kaplan-Meier life table analysis of those 101 patients who had conjunctival lymphoid tumor(s) and no evident systemic lymphoma at presentation, systemic lymphoma was eventually discovered in 7% of patients at 1 year, 12% at 2 years, 15% at 5 years, and 28% at 10 years. Overall, only one patient (<1%) died of systemic lymphoma, at 28 months after the diagnosis of the ocular disease. CONCLUSIONS: Lymphoid tumors of the conjunctiva are associated with systemic lymphoma in 31% of patients. Systemic lymphoma is found more often in those patients with forniceal or midbulbar conjunctival involvement and in those with multiple conjunctival tumors. Long-term systemic follow-up is advised, because related systemic lymphoma can manifest many years later.     

T-cell lymphoblastic lymphoma involving the ocular adnexa: report of two cases and review of the current literature

The majority of ocular adnexal lymphomas are B-cell in origin. We report two cases of T-cell lymphoblastic lymphoma (T-LBL) involving the ocular adnexa. One patient presented with a painless pink conjunctival lesion and inferior orbital fullness. The second patient presented with a painless orbital mass. The diagnoses were confirmed by histopathology and immunohistochemistry. Both patients had extensive multifocal lesions during staging. Prompt intensified chemotherapy regimens were initiated. T-LBL is an aggressive disease with poor prognosis. This report emphasizes the importance of timely diagnosis by the ophthalmologist with co-management and treatment with an oncologist.     

Clinical and radiological presentation of 95 orbital lymphoid tumors

Background: Although most orbital lymphoid tumors are characterized by a slow, painless onset and a mass that molds to orbital structures, different presentations may occur. Intensity on T2-weighted MRI is a possible means for differentiating lymphoid tumors (hyperintense) from pseudotumors (hypointense). In addition, it is generally assumed that 75% of orbital lymphomas are at stage I on presentation. Methods: The clinical, CT, and MRI presentation of 95 primary and secondary orbital lymphoid tumors (63 non-Hodgkin lymphomas and 32 lymphoid hyperplasias) and their histological grade and staging were reviewed. Immunohistochemistry and PCR were used to determine clonality. Results: No significant differences were found between non-Hodgkin lymphomas and lymphoid hyperplasias, except for the mean age of the patients (6.3 years older in non-Hodgkin lymphomas). In both groups the most common presentation was a mass with an indolent course; visual impairment was seen in 13%, conjunctival redness in 25%, pain in 12%, and acute orbital inflammation in 15% of the patients. In most cases, the CT showed one or more lobulated or rounded masses, molding to adjacent structures, or a wedge-shaped enlargement of the lacrimal gland. Intramuscular lymphomas were rare and always associated with extramuscular masses. On T2-weighted MRI, only 35% of lymphoid tumors were hyperintense. Only 49% of lymphomas were at stage I on presentation. Conclusion: An inflammatory presentation is not uncommon in orbital lymphoid tumors. Shape, molding and multiple masses can help radiological diagnosis, whereas MRI T2 intensity is unreliable. Accurate staging can disclose systemic disease in more than 50% of cases of non-Hodgkin lymphoma.     

Radiotherapy for localized orbital mucosa-associated lymphoid tissue lymphoma

Fourteen patients with localized orbital mucosa-associated lymphoid tissue lymphoma diagnosed between 1998 and 2005 were reviewed. Five patients were males and 9 were females, with a mean age of 58 years. In 8 patients, the disease arose from the conjunctiva, and in 6 patients, it originated from the retrobulbar space. Patients were treated with radiotherapy alone at a dose range from 30 to 54 Gy. All patients with conjunctival lymphoma achieved complete remission (CR). Four patients with retrobulbar lymphoma obtained CR, and unconfirmed CR (CRu) was observed in 2 cases. Orbital extraconal lymphoma disappeared. However, intraconal cord-like tumor and mass involving the medial extraocular muscle remained with the absence of regrowth over the long term. There have been very few reports discussing the histology of residual mass after radiotherapy. Residual mass was suggested to be reactive lymphoid hyperplasia. As a dose of more than 40 Gy induced dry eye syndrome or cataract, the dose must not exceed 40 Gy in order to achieve safe treatment of orbital mucosa-associated lymphoid tissue lymphoma.     

Lymphomas involving the eye and the ocular adnexa

PURPOSE OF REVIEW: To describe recent advances in the understanding of the pathogenesis of the most common malignant lymphomas that occur as primary and secondary tumors in ocular tissues. RECENT FINDINGS: Advances have been made in the understanding of the genetic alterations in mucosa-associated lymphoid tissue lymphomas, including various chromosomal translocations, such as the most recently described t(3;14)(p14.1;q32) involving the FOXP1 gene. Further, the development of ocular adnexal mucosa-associated lymphoid tissue lymphomas has been associated with Chlamydia psittaci in some geographic areas. Subdivision of diffuse large B-cell lymphoma into clinically prognostic groups had been achieved on the basis of gene expression profiles using complementary DNA microarrays. Tumor-infiltrating cells, such as macrophages, have been demonstrated to be of prognostic significance in follicular lymphoma. SUMMARY: Understanding of the ocular adnexal and intraocular lymphomas has advanced with progress in lymphoma classification systems, namely the World Health Organization lymphoma classification. This knowledge is being fine tuned with advances in technology, such as complementary DNA microarrays. The clinical significance of this scientific progress has yet to be determined.     

112例眼附属器淋巴增生性病变临床组织病理学初步分析

目的初步探讨眼附属器淋巴增生性病变的发病情况、临床特征以及组织病理学特点和分型。方法收集112例经组织病理学诊断为眼附属器淋巴增生性病变患者的116份石蜡切片标本,分析归纳临床相关资料,进一步分析组织病理学特点,并根据2001年世界卫生组织淋巴及血液组织肿瘤分类方案进行重新分类诊断。结果患者年龄8～80岁,平均49岁。病程20d至22年,平均22个月。双眼患病者16例（14．3％）。临床常见表现为眼球突出、局限性肿块（69例,占61．6％）。组织病理学检查显示反应性淋巴细胞增生11例（9．8％）,非典型淋巴细胞增生10例（8．9％）,淋巴瘤91例（81．3％）。其中,黏膜相关组织型边缘带B细胞淋巴瘤74例（占淋巴瘤患者81．3％）。结论眼附属器淋巴增生性病变以黏膜相关组织边缘带B细胞淋巴瘤最为多见,好发于中老年人,临床表现为慢性迁延性病程,双眼发病并不少见。（中华眼科杂志,2005,41：871-876）     

Ocular adnexal monoclonal lymphoid tumors with a favorable prognosis

Fourteen patients with well- or intermediately differentiated monoclonal B-lymphocytic tumors of the conjunctiva or orbit had a favorable prognosis with follow-ups of 4 to 9 years (mean and median, 7.5 years). The lesions were, for the most part, diffuse proliferations of small lymphocytes, either with round or minimally indented nuclear outlines. Mitotic activity was sparse to nonexistent; occasionally there were scattered small abortive or residual germinal centers, and some lesions exhibited lymphoplasmacytoid features and dispersed multinucleated giant cells (polykaryocytes). None of the six patients with conjunctival lesions had extraocular manifestations. An identical tumor of the submandibular gland developed in one of eight patients with orbital lesions and another patient had multiple extranodal involvements of the oropharynx, liver, and both kidneys, but after chemotherapy the patient has survived for 8 years from orbital presentation and is currently in remission. The authors believe that these low-grade tumors share many biologic resemblances to extranodal lymphoepithelial tumors of other organs (lung, gut, parotid, thyroid), which as a group have been aggregated together as mucosa-associated lymphoid tumors (MALT) and which can often remain localized to their sites of origin.     

Clinicopathologic characteristics of orbital lymphoid hyperplasia

The clinical characteristics and microscopic features of the biopsy specimens of 66 patients with orbital lymphoid tumors were analyzed with respect to clinical outcome (disease remaining localized to the orbit or developing associated extraorbital lesions). The patients' average age was 63 years, and their tumors had a marked tendency to arise in the anterosuperior orbit. No clinical or radiographic findings helped to distinguish the cases of localized disease from disseminated disease. The lesions were divided microscopically into three categories: reactive lymphoid hyperplasia (16), atypical lymphoid hyperplasia (38), and malignant lymphoma (12). Extraorbital lesions developed in 25%, 50%, and 75% of cases, respectively. The five-year mortality rates were also significantly different for each type of lesion: 6%, 19%, and 58%, respectively.     

Bilateral conjunctival mucosa-associated lymphoid tissue lymphoma misdiagnosed as allergic conjunctivitis

PURPOSE: This report aimed to discuss two nearly identical cases of primary conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma mimicking allergic conjunctivitis. METHODS: Two patients were referred for intractability to conventional treatment of initial diagnoses of allergic conjunctivitis. On ocular examination, normally pigmented, giant papilla-like lesions were found bilaterally in the upper conjunctiva. Excisional biopsies were performed. RESULTS: Histopathologic and immunohistochemical examination of the conjunctival biopsies indicated MALT lymphoma in both patients. The patients subsequently received radiation therapy and achieved complete remission with no evidence of recurrence during follow-up periods of 13 and 11 months. CONCLUSION: Conjunctival low-grade lymphoma may share similar clinical features with allergic conjunctivitis. Ophthalmologists should be concerned that primary conjunctival low-grade malignant lymphoma can be misdiagnosed as allergic conjunctivitis     

Mucosa-associated lymphoid tissue lymphoma with intraocular involvement

PURPOSE: To report the clinicopathologic features of a patient with mucosa-associated lymphoid tissue (MALT) lymphoma of the conjunctiva and associated intraocular involvement. METHODS: This study is a retrospective clinicopathologic correlative case report summarizing the clinical, radiologic, and histopathologic findings of a patient with conjunctival MALT lymphoma and associated intraocular involvement. RESULTS: Ophthalmic examination and fluorescein angiography demonstrated progressive conjunctival infiltration bilaterally, marked uveal effusions in the left eye, and cellular white infiltrates of the choroid in the right eye. MRI of the orbit revealed a diffusely infiltrating intra- and extraocular lesion extending around the globe and optic nerve in the left eye without evidence of intracranial extension. Conjunctival biopsy showed low-grade tumor cells, consistent with the diagnosis of MALT lymphoma. The patient was successfully treated with external beam radiation with marked clinical improvement. CONCLUSION: Conjunctival MALT lymphomas, typically indolent and localized tumors, may extend into the orbit and invade local tissues such as the choroid with devastating consequences. A conjunctival biopsy can provide an easy diagnosis of this treatable entity.