Cavernous Hemangioma of the Bony Orbit

Purpose: To study the clinical features, diagnosis and management of intraosseous cavernous hemangioma of the orbit. Methods: Five cases of intraosseous cavernous hemangioma seen in our hospital from Jan 1, 1986 to Dec 31, 2000 were reviewed. Results: Among all five cases, two were male and three were female. The mean age was 47.6 years old, ranging from 39.0 to 55.0 years. The left orbit was affected in 4 cases and the right one in 1 case. The bony involvement occurred in frontal bone (two cases), zygomatic bone (two cases) and sphenoid bone (one case). A painless, slowly enlarging hard bony mass fixed to the bone with no pulsations was the main clinical sign. The x-ray and CT appearance of intraosseous cavernous hemangioma of the orbit were characteristic and usually diagnostic. The differential diagnosis of it included fibrous dysplasia, eosinophilic granuloma, multiple myeloma and metastatic carcinoma. Treatment is local removal of the bone containing the tumor. Conclusions: Intraosseous cavernous hemangioma is a rare tumor of the orbit and usually has good surgical result. Eye Science 2005;21:147-151.     

Preservation and Observation of Anterior Ciliary Vessels in Rectus Muscles during Strabismus Surgery

Purpose :To observe the form and number of the anterior ciliary vessels (ACV) in rectus muscles. To train technician in preservation and obsevation of ACV, so preservation can be done in caes with a risk of anterior segment ischemia (ASI). Methods:Curved foreign knife, iris hook, plastic rubber band and standard operating loupes (3x. ) or microscope were used in the surgery on 34 cases of comitant strabismus and 18 cases of paralytic strabismus.Results:The ACVs per muscle in medial, lateral, superior, inferior rectus were 3. 08,3. 26,3. 50 and 3. 50 respectively in 89 muscles of 52 surgical strabismus cases. All ACVs in 16 rectus muscles and 90 out of 220 ACVs in 73 rectus muscles were too small or too short to be dissected. The success rate of ACV preservation was 91. 5% (119/130). 105 out of 130 vessels were saved using loupe magnification and 14 pit pf 130 vessels were saved under operating microscope. Conclusions :The number of ACV in rectus muscles are more than 2 in our observation cases. The ACV     

Mantle Cell Lymphoma in a Lacrimal Gland in a Female and a Review of the Literature

Purpose： To report a rare case of Mantle cell lymphoma in lacrimal gland and review of the literature Case report： We report a case of a 59-year-old female who presented with an upper eyelid mass in the right eye for 3 months, without pain and irrigation. A computerized tomog- raphy （CT） scan showed a mass in the bilateral lacrimal gland region, more significant in right eye. The patient underwent a lacrimal gland mass excision surgery and diagnosis of mantle cell lymphoma by histopathology. Immunochemistry for CD20, CD79a, CD5, and CyclinD1 was positive. She was recommended to the Shantou cancer hospital for chemothera- pY.     

眶区皮样囊肿的CT表现

目的 分析眶区皮样囊肿及表皮样囊肿的CT表现,揭示该病的特殊性CT征,作为诊断依据.方法 复习经手术及病理组织学证实的皮样及表皮样囊肿CT片共55例,分析其所在位置、范围、形状、边界、密度、CT值及继发改变.结果 55例病变均被CT发现,53例提示病理组织学诊断,2例眶缘有瘘管者缺乏特征性CT表现.病变位于眶内者44例:内上象限2例、外上象限36例、内下象限3例、外下象限3例;按所在间隙分析,中央间隙(肌锥内)1例、周围间隙9例、骨膜下间隙24例、眶骨壁内10例.眶缘者5例均位于眶上缘.颞窝者6例均位于颞肌下.囊肿密度不均,CT值-158.67Hu～+62.00Hu.典型的骨壁改变45例.结论 CT能够在术前对皮样囊肿作准确定性、定位诊断,对制定手术方案有很大帮助.

Abstract：

Objective To study the findings of the orbit dermoid and epidermoid cyst on CT, then to reveal the CT characteristics of the disease for diagnosis. Methods To reviewing the CT image of 55 cases, all had proved by histopathology or operation. And to analyze its position, extent, shape, boundary, density, CT value and subsequent changes. Results CT could discover all the lesions. Fifty-three CT findings indicated its histopathology diagnosis and 2 patients with orbit margin fistula lacked CT characteristics. There were 44 cases in the orbit, 2 in the upper inner quadrant, 36 in the upper outer quadrant, 3 in the lower inner quadrant, 3 in the lower outer quadrant, 1 in the muscle cone, 9 in the space between muscle cone and periosteum, 24 in the subperiosteal space, 10 in the bone wall. There were 5 cases in the margo supraorbitalis, 6 under the temporalis.The density of the cysts was heterogeneity, and the CT value was from -158.67Hu to +62.00 Hu. Forty-five cases with typical boll wall changes were found. Conclusions Orbital CT scanning is the effective way in the preoperative diagnosis of orbital dermoid and epidermoid cysts for determining its nature and location. It is helpful to formulating the procedures of operation.     

A Macular Hole in a Female Adult with Bilateral Coats Disease

Purpose:To report a case of a macular hole in a female adult with bilateral Coats disease.Methods:The fundus photograph, fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) were performed in two eyes, and observed theirs characters.Results:Fundus photograph showed massive yellowish-white exudation in the temporal midperiphery of both eyes, but the degree was slighter in the right eye. There was 1/3 DD macular hole in left eye. FFA revealed general dilatation of capillaries, multiple aneurysms, and tortuous and closure of vessels in superotemporal midperiphery in both eyes, but the degree was slighter in the right eye. There was a one-third DD round transmitted fluorescence according the macula hole. ICGA revealed that hyperfluorescence of aneurysms in superotemporal midperiphery, the images of aneurysms were more clearly than in FFA. There was a 2/3 DD hypoflurescence of macula in the late phase of angiography.Conclusions: FFA and ICGA have respective advantage in revealing vas     

三维眶底重建钛网在复合性眼眶骨折中的应用

目的 探讨应用三维眶底重建钛网治疗复合性眼眶骨折修复眶底缺损的手术方法和临床效果.方法 回顾性系列病例研究.回顾性分析13例(13只眼)行三维眶底重建钛网植入眼眶骨折修复眶底重建手术的复合性眼眶骨折患者的临床资料.其中眶颧颌骨折6例,鼻眶筛骨折5例,眼眶四壁多发性骨折2例.术中联合行颧骨、上颌骨骨折、鼻骨以及额骨骨折修复,8例眼球重度凹陷的患者同时行高密度聚乙烯眶内充填.手术前后测量眼球突出度、眼球运动以及眼眶CT扫描(水平位、冠状位以及三维重建).术后随访3～6个月.结果 术前患眼眼球凹陷平均(3.9±1.7)mm,眼球下移(3.1±2.6)mm.13例患者中,12例存在眼球运动障碍,其中Ⅰ级有4例,Ⅱ级有7例,Ⅲ级有2例.术后除3例患者残存眼球凹陷1.0 mm,其余10例患者眼球凹陷矫正;8例患者眼球下移完全矫正,3例残留眼球下移1.0 mm,2例患者眼球上移1.0 mm;术后3个月后8例患者眼球运动受限完全矫正,其余5例眼球运动障碍减轻,其中残留Ⅱ级运动障碍2例,Ⅰ级运动障碍3例;随访过程中未发现三维眶底重建钛网移位、排斥、感染及其他不良反应.结论 三维眶底重建钛网具有良好的生物相容性,采用三维眶底重建钛网治疗复合性眼眶骨折,可有效恢复眼眶容积,矫正眼球内陷和下移,改善眼球运动.

Abstract：

Objective To evaluate the outcomes of the clinical application of three-dimensionally preformed titanium mesh plates for posttraumatic complex orbital bone fracture reconstruction. Methods Thirteen patients (13 eyes) manifested with complex orbital fractures (including orbital-zygomatic- maxillary bone fracture in 6 patients,nasal-orbital-ethmoid bone fracture in 5 patients and complex multiple periorbital bone fracture in other 2 patients ) underwent the reconstructive surgery by using three-dimensionally preformed titanium mesh plates. Eight cases underwent high density porous polyethylene implantation simultaneously. Evaluation of enophthalmos,eyeball movement,and orbit CT scan were taken pre- and post-operatively. Results The average enophthalmos was (3.9 ± 1.7 ) mm and the position of the affected eyeball was( 3. 1 ± 2. 6)mm bellow the contralateral side preoperatively. Twelve patients suffered from eyeball movement restriction,including grade Ⅰ in 4 cases,grade Ⅱ in 7 cases and grade Ⅲ in 2 cases. During the 3 to 6 months post operative follow-up,enophthalmos was completely corrected in 10 patients while the other 3 patients had residual 1.0 mm enophthalmos. The affected eyeballs were repositioned in 8 cases and 3 cases had residual 1 mm lower position and 2 cases with a 1 mm higher positioned eyeball. Eye movement was not restricted in 8 patients,whereas 3 patients had grade Ⅰ movement restriction and 2 patients had grade Ⅱ movement restriction. There were no rejection,infection and other complications during follow-up.Conclusions Three-dimensionally preformed titanium mesh plates can be used to reconstruct posttraumatic complex orbital bone fractures in order to improve the orbital volume and to correct enophthalmos effectively.     

Auricular cartilage versus donor sclera as a wrapping of hydroxyapatite orbital implants

AIM: To retrospectively compare postoperative outcomes after primary enucleation and placement of a hydroxyapatite(HA) implant without wrapping, wrapped with auricular cartilage or donor sclera. METHODS: Medical records of patients presented as intraocular tumor or severe ocular injury were identified from the electronic medical record system. Cases underwent enucleation and HA orbital implantation were enrolled in this study and were divided into 3 groups according to the wrapping material of HA implant. Cases with autogenous cartilage caps were enrolled in group A(n=11), with donor sclera caps in group B(n=12), and without any wrapping material in group C(n=9). Follow-ups were set at 1, 2 wk, 1, 3, 6, and 12 mo after surgery.RESULTS: Altogether 32 cases finished the followup and were enrolled in this study. Three cases(27.27%) in group A, 4 cases(33.33%) in group B, and 4 cases(44.44%) in group C developed one complication each after surgery. In group A, no HA exposure occurred, but conjunctival inclusion cyst occurred in one and severe conjunctive chemosis in two cases. In group B, one HA exposure occurred, conjunctive inclusion cysts occurred in one, severe conjunctive chemosis occurred in one, and conjunctival granuloma occurred in one case. In group C, one HA exposure occurred, severe conjunctive chemosis occurred in two cases, and conjunctival granuloma occurred in one case. The case of exposure of none-wrapped implant was noted in the first 6 mo after placement of the orbital implant. The case of exposure of donor sclerawrapped implant was noted at the 12 mo after placement of the orbital implant. Both exposure cases were treated successfully with conservative treatment.CONCLUSION: With low incidence of implant exposure and mild complications, auricular cartilage can be a good choice of alternative wrapping material of orbit implant with satisfied outcome.     

The effects of simultaneous operation on dissociated vertical deviation with horizontal and torsional strabismus

AIM: To investigate the therapeutic effects of simultaneous horizontal and vertical operations on dissociated vertical deviation(DVD) associated with other deviations.METHODS: Forty-five cases of DVD with horizontal and torsional strabismus underwent combined operation were collected retrospectively. All clinical records were analyzed. All patients were followed up for 6 to 24 mo. Wilcoxon signed-ranks test was performed to evaluate the changes of vertical and horizontal deviation. χ~2 test was used to evaluate the changes of binocular visual function. RESULTS: Forty-five cases included 36 patients with intermittent exotropia and binocular inferior oblique overaction(IOOA), 5 patients with concomitant esotropia and binocular IOOA, 4 patients with intermittent exotropia and monocular superior oblique palsy. The superior rectus recession(SRR) combined with horizontal rectus recession and the myectomy of inferior oblique or anterior transposition were operated simultaneously to correct all types of strabismus. There were 43 cases who achieved normal eye position in vertical direction, while 2 cases were with undercorrection of 5~Δ to 6~Δ. In patients with horizontal strabismus, 2 cases of exotropia were with overcorrection of 6~Δ to 8~Δ, 1 case of esotropia was with undercorrection of 6~Δ, and 1 case of monocular superior oblique palsy with compensatory head posture was not significantly improved. The binocular visual function of most patients recovered after operation. The difference of the binocular visual function and eye position were significant compared with that before operation(P<0.05).CONCLUSION: The simultaneous operation on DVD with horizontal and torsional strabismus is successful.     

Mycobacterium Chelonei Keratitis：Report of a Case and Review of the Literature

Background: Mycobacterium (M) chelonei keratitis is a rare opportunistic eye infection that can cause significant morbidity when not being treated properly. The first case was documented by Gangadharam et al in 1978 and since then, a total of 49 cases were reported in the literature. One alarming fact is that more than 50% of cases were found in the Chinese population and mostly reported in recent years. The key to successful management of M. chelonei keratitis is early diagnosis by high index of suspicion. In order to alert ophthalmologists of this condition, we report a typical case of M. chelonei keratitis and review the literature of all the reported cases with special reference to its risk factors, treatments and outcome.Methods: The cases reported in the literature and a case of our own were reviewed and analyzed.Results: Our case was a 42-year-old gentleman who developed M. chlonei keratitis following pterygium surgery. He had typical clinical features of irregular infiltrates with radiating proj     

Gene Rearrangement Analysis of Orbital Lymphoid Infiltrating Disorders

Purpose: To determine whether the use of polymerase chain reaction for B-cell gene rearrangement in patients with orbital lymphoid infiltrate disorders could be useful in the diagnosis of lymphoma, especially, in differentiating benign lesion from malignant one. Methods: In addition to clinical, pathological, and immunohistochemical evaluations, 48 cases of orbital lymphoid infiltrate disorders were examined for immunoglobulin heavy (IgH) gene rearrangement by means of PCR to amplify the FR3 region with formalin-fixed and paraffin-embedded tissues.Results: Gene rearrangement in the third frame-work of the IgH region was detected in specimens obtained from 15 cases of malignant lymphoma, 4 of reactive lymphoid hyperplasia and 3 of orbital pseudotumor. All of these patients showed a discrete band (100bp) which reflected monoclonal proliferation of B lymphocytes. 5 cases of malignant lymphoma, 6 of reactive lymphoid hyperplasia and 15 of orbital pseudotumor did not show a discrete band on PCR.Conclusions:     

Orbital progressive transformation of germinal centers as part of the spectrum of IgG4-related ophthalmic disease: Clinicopathologic features of three cases

Progressive transformation of germinal centers (PTGC) is a form of follicular hyperplasia recently associated with immunoglobulin G4-related disease (IgG4-RD), but the ophthalmic manifestations of this combination are poorly described. In this retrospective case series, we present three cases of IgG4-related orbital disease (IgG4-ROD) showing varying degrees of PTGC involving the orbit and lacrimal gland. Three adult women presented with ill-defined lacrimal gland enlargement. Histologic sections showed variable fibrosis and large, irregular lymphoid follicles with prominent mantle zones penetrating the germinal centers, highlighted by Bcl-2 and/or IgD immunostains. The interfollicular areas contained a mixture of plasma cells, scattered histiocytes and eosinophils. Mixed T and B-cells were present, and no signs of monoclonality were identified. All cases showed more than 100 IgG4 positive cells per high power field. Epstein-Barr virus in situ hybridization performed in one case was negative. The serum IgG4 level was tested in one case and showed elevation above the normal range. After 2–10?months of follow-up, the patients showed either near-complete resolution or no remaining signs of ophthalmic disease. Increasing awareness of these PTGC in extra-nodal locations, including the orbit, may provide a better understanding of the histologic spectrum of this disease.     

Orbital Lipofibromatosis in a Child: A Case Report

Purpose: Lipofibromatosis is a rare, benign, soft tissue tumor that typically involves extremities particularly in children. We report a young girl with lipofibromatosis involving the superotemporal quadrant of the left orbit.

Case Report: An 8-year-old girl presented with a history of a gradually expanding mass in the superotemporal quadrant of the left orbit over several years. Orbital imaging showed a soft tissue mass superolateral to the globe with possible infiltration into the lacrimal gland and lateral rectus muscle. The lesion was excised preserving adjacent structures. Histopathological examination revealed that the lesion consisted of adipose tissue with fibroblastic elements, being consistent with a diagnosis of lipofibromatosis. No recurrence of the lesion was seen in 18 months follow-up.

Comment: Orbital involvement of lipofibromatosis as reported here is indeed a very rare entity. Diagnosis can be confirmed by histopathological analysis. As the lesion has a tendency to infiltrate into surrounding structures, surgical excision without hampering important structures is a treatment of choice.     

泪腺病变的CT分析

目的探讨不同泪腺病变的CT表现,提高泪腺病变的诊断水平。方法20例23眼泪腺病变患者,比较分析其CT表现特点。结果10例11眼泪腺炎性假瘤表现为泪腺增大,与周围结构如眼环分界不清,部分边缘锐利,其中1例伴有外直肌及上直肌增厚。1例1眼泪腺淋巴细胞反应性增生表现为右侧泪腺体积增大,边界清楚。5例5眼泪腺混合瘤,主要表现为泪腺区类圆形软组织肿块,密度均匀。2例4眼良性淋巴上皮性病变表现为对称性双侧泪腺弥漫性肿大,与周围组织分界清楚。2例2眼泪腺癌表现为眼眶外上方软组织肿块影,侵犯邻近组织结构并破坏眶壁骨质。结论根据CT表现特点,多数泪腺病变可明确诊断。     

Eosinophilic granuloma (Kimura’s disease) of the orbit: a case report

· Background: Eosinophilic granuloma of the soft tissue, Kimura’s disease, is a benign slow-growing tumor that is manifested clinically by one or more inflammatory nodules involving mainly the face and scalp, but rarely the eye. · Case report: The patient was a 32-year-old male with swelling of the left lower eyelid, marked peripheral blood eosinophilia and increased serum immunogloblin E. MRI revealed swelling of all rectus muscles of the left eye, but no tumor mass. Corticosteroid treatment reduced the swelling of the eyelid, but it recurred after corticosteroid was discontinued. Eight years later the patient returned with a complaint of increased swelling of the left lower eyelid. An elastic, nontender, soft tumor mass was palpable subcutaneously in the left lower eyelid extending into the orbit. MRI revealed a tumor mass in the left orbital space. The parotid gland was also swollen and palpable. Both tumors were resected surgically, and histopathological study revealed prominent proliferation of lymphoid follicles with germinal centers showing interfollicular infiltration by eosinophils. The pathological findings in the parotid gland were similar. The diagnosis was Kimura’s disease. · Conclusion: This patient is unique in that he had no tumor at the first examination, only swelling of the rectus muscles, and a tumor mass appeared many years later. Unilateral swelling of the rectus muscles may be one of the first signs of Kimura’s disease. Not only tumor but also swelling of the rectus muscles limited ocular movement. Received: 30 June 1998 Revised version received: 5 October 1998 Accepted: 6 October 1998     

Orbital Richter Syndrome

Abstract

We report two patients with previously diagnosed chronic lymphocytic leukemia who developed Richter syndrome in the orbit as the sole extranodal site. The medical history, clinical findings, orbital imaging and histopathological features of the patients were reviewed. Treatment protocols and the outcomes were also assessed. The first patient developed Richter syndrome at the age of 64 years, 3 years after the diagnosis of chronic lymphocytic leukemia. The tumor was located at the inferotemporal quadrant of the orbit. The second patient was 59 years old when Richter syndrome arose in the lacrimal gland, 4 years after the diagnosis of chronic lymphocytic leukemia. Incisional biopsy from the orbital tumors were performed. Histopathological findings included diffuse CD20, CD 23, CD5, bcl2, bcl6 positive lymphocytic infiltration. Both patients were treated with chemotherapy and rituximab. During 3 years of follow-up, there was no orbital or systemic recurrence of the disease. Richter syndrome may develop in the orbital soft tissue and the lacrimal gland, and the orbital disease appears to have a better prognosis compared to patients with systemic involvement.     

Angiolymphoid hyperplasia with eosinophilia of the lacrimal gland: a case report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare clinicopathologic entity that has been the subject of considerable confusion and debate. ALHE shares both clinical and histopathologic features with Kimura disease (KD). Because of this overlap, ALHE and KD have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing ALHE and KD as two separate nosological entities, based on their clinical, laboratory, and especially histopathologic findings. ALHE commonly affects women in the third and fourth decades, with head and neck involvement. ALHE has been described rarely in the orbit, eyelids, and lacrimal gland. We report a case of ALHE involving the left lacrimal gland in a 40-year-old Mexican woman. Because ALHE can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, it should be considered in the differential diagnosis of orbital lesions occurring in adults.     

Kimura disease of the orbit and ocular adnexa.

Kimura disease (KD) is a distinct clinicopathologic entity that has been the subject of considerable confusion and debate. Although common in Asia, KD rarely occurs in non-Asian patients. Kimura disease shares both clinical and histopathologic features with angiolymphoid hyperplasia with eosinophilia (ALHE). Because of this overlap and the rarity of KD in Europe and the United States, KD and ALHE have been used synonymously in the Western medical literature, as they were thought to represent variations of the same disease. Some pathologic reports have called for distinguishing KD and ALHE as two separate entities, based on their histologic features. Kimura disease occurs most commonly in the head and neck region and has been described in the orbit, eyelids, and lacrimal gland more frequently than ALHE. Because both diseases can cause proptosis, lid swelling, ocular dysmotility, or a palpable mass, they should be considered in the differential diagnosis of orbital lesions occurring in adults. We report two cases of KD involving the orbit and ocular adnexa, and review additional cases reported in the literature. The ophthalmic literature does not clearly reflect the current understanding that KD and ALHE are best considered two separate clinicopathologic entities.     

Orbital solitary fibrous tumor with aggressive behavior

BACKGROUND: Solitary fibrous tumor (SFT) is a rare spindle cell tumor that arises most often in the visceral pleura; however, a review of the literature shows at least 31 cases occurring in the orbit. METHODS: A retrospective case series of three patients with orbital SFT: a 50-year-old man, observed in 1997, with an angioma-like lesion in the upper half of the orbit causing osteolysis of the orbital roof; a 24-year-old man, observed in 1992, with a superotemporal mass in the right orbit occupying the lacrimal gland region, firstly diagnosed as schwannoma, recurring 4 years after dacryoadenectomy; a 70-year-old man, with a retrobulbar mass diagnosed on a biopsy as hemangiopericytoma, recurring and infiltrating the orbital roof 4 years after surgery. RESULTS: A review of the literature and presentation of three cases of SFT which showed infiltration of the orbital roof and/or recurrence. CONCLUSIONS: Our cases provide evidence of how orbital SFT can behave aggressively and mimic other orbital tumors, thus making mandatory the consideration of this relatively new entity in common clinical practice as well as careful follow-up. Their aggressive growth is unusual, described in only 6 of the 31 cases so far reported in the literature. Immunohistochemistry is of importance for the diagnosis, since CD34 immunoreactivity is peculiar to SFT.     

Breast Cancer Metastasis Presenting as Conjunctival Chemosis

A 72-year-old woman presented with a painful right eye. A few weeks before, she had noticed a red, swollen area in the conjunctiva of the same eye. On slit lamp examination, it appeared as chemosis and vascular injection; artificial tears were prescribed. A month later, a firm mass developed on the superotemporal orbital rim, in the area of the lacrimal gland. A CT scan revealed infiltrative structures in both the left and right orbit, with contrast staining in the right lacrimal gland and near the left optic nerve. A biopsy was taken of the conjunctival swelling as well as of the lacrimal gland. Both tissues showed infiltration with lobular breast carcinoma metastases.Key Words: Breast cancer, Orbital metastasis, Conjunctival chemosis     

Monoclonal antibodies in the diagnosis of lymphoproliferative diseases of the orbit and orbital adnexae

AIMS: To describe the value of monoclonal antibodies in the differential diagnosis of orbital lymphoproliferative disorders. METHODS: A total of 200 sequential cases of malignant lymphoma diagnosed at St Bartholomew's Hospital as part of the ocular lymphoma service at Moorfields Eye Hospital and the Institute of Ophthalmology have been examined. Cases were acquired between January 1998 and June 2005. Each case had detailed immunophenotypic analysis using a panel of monoclonal antibodies and was classified using the WHO classification of lymphoma. These cases are discussed in relation to earlier studies of orbital lymphoma reported by our group. RESULTS: Lymphomas fell into three main categories. Extranodal marginal zone lymphoma was the largest group with 151 cases, arising within the orbital soft tissue, conjunctiva and lacrimal gland. Cases arising in the conjunctiva and lacrimal gland showed a higher female predominance than those arising within the deeper soft tissue. A small number of cases were associated with organ specific autoimmunity, including thyroid eye disease complicating Graves' disease. Follicular lymphoma and diffuse large B-cell lymphoma formed the next two groups, occurring with equal frequency. Many of the follicular lymphomas had evidence of disseminated disease on completion of staging. A miscellaneous group of T-cell and B-cell lymphomas formed a minority of cases during the study period. CONCLUSION: Extranodal marginal zone lymphoma is the most frequent type of primary orbital and orbital adnexal lymphoma. Its major differential diagnosis is with orbital lymphoid hyperplasia, chronic dacryoadenitis, and follicular conjunctivitis. Systemic types of lymphoma may present within the orbit or involve the orbit secondarily.