Medial lacrimal sac excision versus conventional H-shaped lacrimal sac opening in external dacryocystorhinostomy: a comparative cohort study.

PURPOSE: The external dacryocystorhinostomy (Ex-DCR) is a reliable but surgically challenging procedure to overcome a nasolacrimal duct obstruction (NLDO). The aim of this study is to describe the outcomes of a modified technique of lacrimal sac opening and to compare it with the conventional Ex-DCR. METHODS: This comparative cohort study included adult patients undergoing Ex-DCR for primary acquired NLDO by or under supervision of one surgeon. Group 1 (conventional Ex-DCR including H-shaped incision, anastomosis of the anterior mucosal flap to nasal mucosal flap and resection of the posterior sac flap) was compared with Group 2 (modified Ex-DCR including excision of the medial lacrimal sac and anastomosis of remaining anterior sac flap to nasal mucosal flap). Outcome measures included the success rate (defined as complete symptom relief or patent irrigation after three months), reoperation rate, redo-free survival within five years, and occurrence of postoperative bleeding and postoperative infection. RESULTS: 138 patients were included. The success rates were 94.7% (54/57 cases) for Group 1 and 96.3% (78/81) for Group 2 (p-value = 0.658). The redo-rate was 5.3% (3/75) in Group 1 and 2.5% (2/81) in Group 2 (p = 0.331). Two-year redo-free survival probability estimates were 89.8% for Group 1 and 96.3% for Group 2, respectively. No complications occurred in Group 2, whereas in Group 1, one patient (1.8%) suffered from postoperative bleeding and one (1.8%) from postoperative infection. CONCLUSIONS: This study showed that our modified Ex-DCR technique is equally efficacious compared with the conventional Ex-DCR technique in adult patients with NLDO.

Adult orbital xanthogranuloma: long-term follow-up of treated cases.

BACKGROUND: Adult orbital xanthogranulomatous disease (AOXGD) is a group of rare disorders. Four subtypes are identified: adult-onset xanthogranuloma (AOX), adult-onset asthma and periocular xanthogranuloma (AAPOX), necrobiotic xanthogranuloma (NBX), and Erdheim-Chester disease (ECD). Therapy options vary and little is known about the long-term effect of the treatment. In this study, we will describe the clinical behaviour, effect of treatment, and long-term outcome in a consecutive series of patients with AOXGD. METHODS: This is a descriptive, retrospective study with a long follow-up term of 21 patients with histologically proven AOXGD, treated between 1989 and 2021 in the Rotterdam Eye Hospital and Erasmus MC University Medical Center. RESULTS: Twenty-one patients with histologically proven AOXGD were included. The follow-up ranged from 2-260 months (median of 67 months). Six of the nine patients with AOX were treated with surgery alone, with recurrence in two. Three received systemic therapy, with recurrence in one. All four patients with AAPOX received systemic treatment, the disease recurred in two. Two patients with NBX were treated with surgery alone, with recurrence in one. Four required additional therapy with recurrence in two. Both patients with ECD required systemic therapy. CONCLUSIONS: Recognition of AOXGD is important, in particular, because of the potential severe systemic locations in the different subtypes. Surgical excision might be a sufficient therapy for patients with AOX. Patients with AAPOX, NBX, and ECD warrant systemic therapy. Currently, there is no conclusive evidence for a superior treatment strategy, but further studies are necessary to investigate treatment options.

Radiation optic neuropathy and retinopathy in patients with presumed benign intraorbital tumours treated with fractionated stereotactic radiotherapy.

PURPOSE: To assess the long-term effects of fractionated stereotactic radiotherapy (fSRT) for the treatment of (presumed) benign intraorbital tumours on visual acuity, visual fields, globe and eyelid position, and complications including radiation retinopathy and deviations of retinal nerve fibre layer (RNFL) on OCT. METHODS: Multi-centre retrospective follow-up study of a consecutive series of 25 patients treated in the Rotterdam Orbital Center (collaboration between Erasmus Medical Center and Rotterdam Eye Hospital) between 2002 and 2018. Data on the dose of fSRT, visual acuity, Humphrey field analyser (HFA) perimetry, globe and eyelid position were obtained from the medical records. RESULTS: In this retrospective consecutive series of 25 patients with a median follow-up of 104 months (range 48-215 months), 80.0% of the patients had presumed optic nerve sheath meningioma and 20.0 % presumed cavernous haemangioma with signs suggestive of recent growth. In most patients, a better visual acuity and RNFL thickness were observed after stereotactic radiotherapy. Improvement of the visual field defects was observed after treatment, with a mean deviation of -14.98 dB (12.9 SD) before treatment versus -4.56 dB (10.8 SD) after treatment, respectively. Significant, but small changes in exophthalmometry values were observed with a mean of 14.92 mm (7.9 SD) versus 13.79 mm (7.3 SD) after treatment, respectively. Only 3 patients (15.0%) developed radiation retinopathy after stereotactic radiotherapy. All patients with radiation retinopathy had presumed optic nerve sheath meningioma. CONCLUSIONS: Based on our results, fSRT is an effective treatment modality for this subset of orbital tumours with few complications and good long-term visual and cosmetic outcomes. FSRT is non-invasive and safer than surgery for lesions in the posterior orbit or around the optic nerve.

Prognostic value of TERT promoter mutations in conjunctival melanomas in addition to clinicopathological features.

AIMS: To evaluate the prognostic value of clinical, histopathological and molecular features and to relate different treatment modalities to clinical outcome in conjunctival melanomas (CM). METHODS: Retrospective review of clinical, histopathological and BRAF V600E and telomerase reverse transcriptase (TERT) promoter mutation status and treatment modalities, correlated to recurrence and metastasis in 79 patients with CM, diagnosed between 1987 and 2015 in three tertiary referral centres in the Netherlands and Belgium. RESULTS: Out of 78 evaluable patients, recurrences occurred in 16 patients and metastasis in 12 patients (median follow-up time 35 months (0-260 months)). Tumour thickness >2 mm, pT status, the presence of epithelioid cells, ulceration and mitoses was significantly correlated with metastasis (p value 0.046, 0.01, 0.02, 0.001 and 0.003, respectively). Furthermore, CM frequently harbour BRAF V600E and TERT promoter mutations (29% and 43%, respectively). TERT promoter mutations were correlated to shorter metastasis-free survival (p value 0.002). No significant correlation was found for clinical parameters and metastatic disease. Palpebral, forniceal and caruncular melanomas were more prone to develop recurrences (p value: 0.03). Most CM were treated with excision with adjuvant therapy. CONCLUSION: In line with the recommendations in the Eighth Edition of the American Joint Committee on Cancer Staging for CM, the pathology report should include information about pT status, tumour thickness, presence of epithelioid cells, ulceration and mitoses. Furthermore, information about the presence of a TERT promoter mutation and BRAF V600E mutation is of interest for therapeutic decision making. The presence of a TERT promoter mutation is correlated to metastatic disease.

Accelerated growth of orbital schwannomas during pregnancy does not correlate with sex hormone- or growth factor receptor status.

Purpose: Until now, three cases of growth of an orbital schwannoma during pregnancy have been published. We aim to provide additional insight in the effect of pregnancy on orbital schwannomas. Methods: We present two additional cases of accelerated growth of orbital schwannomas during pregnancy and investigate receptor expression profiles for estrogen, progesterone, androgen, VEGF, EGF, FGF, PDGF-Rß and ki-67 in the two pregnant cases and six non-pregnant cases. Results: Case 1: A 26-year-old woman developed unilateral exophthalmos during pregnancy, with normal visual acuity and ocular motility. During a subsequent pregnancy, again the exophthalmos progressed. MRI showed a mass suggestive of schwannoma. After delivery, resection of the lesion was performed through an anterior approach. Pathology confirmed schwannoma. The expression profile was positive for estrogen- and FGF receptors and ki-67, but negative for progesterone-, androgen- and other growth factor receptors. Case 2: A 24-year-old woman presented with diplopia and unilateral pain during pregnancy. She had normal visual acuity, but a mild exophthalmos and elevation deficit. MRI revealed an extraconal mass suggestive of schwannoma. After delivery, resection was performed through an anterior approach. Pathology confirmed the diagnosis. The expression profile was positive for ki-67, but negative for sex hormone- and growth factor receptors. In the six non-pregnant cases the expression profiles varied, with only one subject showing a strong expression of estrogen-, progesterone- and androgen receptors. Conclusions: Orbital schwannomas can experience growth during pregnancy. The underlying mechanism remains unclear as hormone- and growth factor expression profiles show no correlation to the pregnant state.

Cellular angiofibroma of the orbit.

Cellular angiofibroma is a benign mesenchymal tumor most commonly located in the distal genital tract of both men and women. Although extragenital locations have been reported rarely, this is the first report of cellular angiofibroma of the orbit. A 58-year-old man presented with a mass in the left superomedial orbit since 2 years. Magnetic resonance imaging showed a well-demarcated lesion with a homogeneous intermediate signal intensity on both T1- and T2-weighted images, homogeneous contrast enhancement and high signal intensity on diffusion-weighted images. Complete excision was performed through a medial upper eyelid crease incision. Histopathology showed a vascular CD34-positive and STAT6-negative spindle cell tumor with monoallelic loss of FOXO1, indicating cellular angiofibroma.

Intracranial actinomycosis of odontogenic origin masquerading as auto-immune orbital myositis: a fatal case and review of the literature.

BACKGROUND: Actinomycetes can rarely cause intracranial infection and may cause a variety of complications. We describe a fatal case of intracranial and intra-orbital actinomycosis of odontogenic origin with a unique presentation and route of dissemination. Also, we provide a review of the current literature. CASE PRESENTATION: A 58-year-old man presented with diplopia and progressive pain behind his left eye. Six weeks earlier he had undergone a dental extraction, followed by clindamycin treatment for a presumed maxillary infection. The diplopia responded to steroids but recurred after cessation. The diplopia was thought to result from myositis of the left medial rectus muscle, possibly related to a defect in the lamina papyracea. During exploration there was no abnormal tissue for biopsy. The medial wall was reconstructed and the myositis responded again to steroids. Within weeks a myositis on the right side occurred, with CT evidence of muscle swelling. Several months later he presented with right hemiparesis and dysarthria. Despite treatment the patient deteriorated, developed extensive intracranial hemorrhage, and died. Autopsy showed bacterial aggregates suggestive of actinomycotic meningoencephalitis with septic thromboembolism. Retrospectively, imaging studies showed abnormalities in the left infratemporal fossa and skull base and bilateral cavernous sinus. CONCLUSIONS: In conclusion, intracranial actinomycosis is difficult to diagnose, with potentially fatal outcome. An accurate diagnosis can often only be established by means of histology and biopsy should be performed whenever feasible. This is the first report of actinomycotic orbital involvement of odontogenic origin, presenting initially as bilateral orbital myositis rather than as orbital abscess. Infection from the upper left jaw extended to the left infratemporal fossa, skull base and meninges and subsequently to the cavernous sinus and the orbits.

The tarsal plate manifestation of IgG4-related disease.

PURPOSE: To describe a clinical case of bilateral biopsy-proven IgG4-related disease confined to the tarsal plate. METHOD: Interventional case report. RESULTS: A 58-year-old woman presented with a tarsal swelling in the lateral part of the upper eyelids, with focal ulceration and mucus. Histology revealed fibrotic inflammation with increased IgG4-positive plasma cells fulfilling the criteria of IgG4-related disease (IgG4-RD). Serum IgG4 levels were increased, and pathological fluorodeoxyglucose uptake at positron emission tomography/CT scanning was restricted to the upper eyelids. After treatment with oral and topical prednisone, the tarsal lesions markedly regressed. CONCLUSIONS: Periorbital IgG4-RD may be confined to the tarsal plate. Treatment with systemic and topical steroids may induce significant regression.

The treatment outcomes in IgG4-related disease.

INTRODUCTION: IgG4-related disease (IgG4-RD) is an emerging systemic inflammatory disease involving nearly all organs eventually leading to fibrosis. Prompt and adequate treatment to prevent irreversible organ damage is therefore pivotal. To evaluate the treatment outcomes, we studied a well-defined cohort of patients with IgG4-RD. METHOD: 32 patients with histologically confirmed IgG4-RD diagnosed between 1999 and April 2017 were included and reviewed for demographic and clinical characteristics. The response to treatment with glucocorticoids, disease modifying antirheumatic drugs, rituximab and other therapeutic interventions were evaluated. RESULTS: Glucocorticoids as well as rituximab appeared successful therapeutic drugs leading to clinical remission (complete or partial remission) in all patients. Recurrences, however, were frequently seen (62% versus 100%, respectively). Diseases modifying antirheumatic drugs (DMARDs), including azathioprine, methotrexate and mycophenolate mofetil were effective in less than half of the cases. A minority of patients was treated with alternative treatments including hydroxychloroquine, thalidomide and infliximab which all appeared effective. Surgical intervention and radiotherapy in local disease seemed to induce clinical remission and were associated with low recurrence rates. CONCLUSION: Glucocorticoids and rituximab induce substantial responses as well as primary surgical intervention and radiotherapy, while the efficacy of DMARDs is limited. Based on the few data available, hydroxychloroquine, infliximab and thalidomide may be promising treatment options for second or third line strategies.

Chemotherapy-induced exacerbations of thyroid orbitopathy in a patient with B-cell lymphoma.

A 55-year-old woman with concurrent active thyroid orbitopathy and B-cell lymphoma developed acute exacerbation of thyroid orbitopathy after receiving Rituximab, cyclophosphamide, hydroxydaunorubicin, Prednisone (R-CHOP) chemotherapy, presenting with subtotal loss of vision and severe eyelid edema. Intravenous methylprednisolone was fully effective within several hours. Further exacerbations of her orbitopathy were seen following every subsequent chemotherapeutic treatment, but responded well to oral prednisone. This case shows that thyroid orbitopathy may severely and acutely progress after chemotherapy for concurrent B-cell lymphoma. Clinical awareness of this potential complication may prevent blindness in this rare subset of patients.

Eyelid Merkel cell carcinoma in a patient treated with golimumab.

PURPOSE: To describe a clinical case of biopsy-proven Merkel cell carcinoma of the eyelid following golimumab therapy for rheumatoid arthritis (RA). METHODS: Interventional case report. RESULTS: A 73-year-old woman with a history of chronic RA presented with a right upper eyelid mass. She had been treated with golimumab (tumor necrosis factor (TNF) inhibitors) injection therapy for the past 6 months. A biopsy showed findings suggestive of a Merkel cell carcinoma of the eyelid. CONCLUSIONS: Merkel cell carcinoma may be associated with anti-TNF treatment and should be included in the differential diagnosis of an eyelid tumor in patients treated with TNF inhibitors.

Infliximab for IgG4-Related Orbital Disease.

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition with unclear pathophysiology. It may occur as a single organ disorder, but multiorgan presentation is common and can mimic several conditions. The preferred therapy consists of steroids, but definite maintenance strategy remains unclear. The authors describe a case of a 61-year-old woman, initially diagnosed with idiopathic orbital inflammation refractory to multiple immunosuppressive agents. The disease was complicated with epilepsy, vision loss, and trismus. Treatment with various immunosuppressive agents was unsuccessful. Eventually the patient was effectively treated with infliximab. This is the second case of IgG4-RD treated with a TNF-blocker documented in literature and the first description to demonstrate its superiority over steroid sparing agents. Although speculative, TNF-blockers might exert their effect in IgG4-RD by interfering with the possible overexpressed TNF alpha due to fibrosis in this disease. Treatment with infliximab appears a good alternative for refractory IgG4-RD. However, further studies are required to define the value of infliximab in IgG4-RD.

Pure orbital blowout fractures reconstructed with autogenous bone grafts: functional and aesthetic outcomes.

The purpose of this study was to investigate the ophthalmic clinical findings following surgical reconstruction with autogenous bone grafts of pure blowout fractures. A retrospective review of 211 patients who underwent surgical repair of an orbital fracture between October 1996 and December 2013 was performed. Following data analysis, 60 patients who were followed up over a period of 1 year were included. A solitary floor fracture was present in 38 patients and a floor and a medial wall fracture in 22 patients. Comparing preoperative findings between these two groups, preoperative diplopia and enophthalmos were almost twice as frequent in the group with additional medial wall fractures: diplopia 8% and 14% and enophthalmos 18% and 55%, respectively. One year following surgery there was no diplopia present in either group. In the solitary floor fracture group, 3% still had enophthalmos. It can be concluded that at 1 year following the repair of pure orbital floor fractures using autogenous bone, good functional and aesthetic results can be obtained. In the group with both floor and medial wall fractures, no enophthalmos was found when both walls were reconstructed. When the medial wall was left unoperated, 29% of patients still suffered from enophthalmos after 1 year.

Patient survival in uveal melanoma is not affected by oncogenic mutations in GNAQ and GNA11.

BACKGROUND: Mutations in GNAQ and GNA11, encoding the oncogenic G-protein alpha subunit q and 11, respectively, occur frequently in the majority of uveal melanomas. METHODS: Exons 4 and 5 from GNAQ and GNA11 were amplified and sequenced from 92 ciliary body and choroidal melanomas. The mutation status was correlated with disease-free survival (DFS) and other parameters. RESULTS: None of the tumours harboured a GNAQ exon 4 mutation. A GNAQ mutation in exon 5 codon 209 was found in 46 out of 92 (50.0%) of the tumours. Only 1 out of 92 (1.1%) melanomas showed a mutation in GNA11 exon 4 codon 183, whereas 39 out of 92 (42.4%) harboured a mutation in exon 5 of GNA11 codon 209. Six tumours did not show any mutations in exons 4 and 5 of these genes. Univariate analyses showed no correlation between DFS and the mutation status. CONCLUSION: GNAQ and GNA11 mutations are, in equal matter, not associated with patient outcome.

PDGF enhances orbital fibroblast responses to TSHR stimulating autoantibodies in Graves' ophthalmopathy patients.

PURPOSE: Thyroid-stimulating hormone receptor (TSHR) stimulating autoantibodies are associated with Graves' ophthalmopathy (GO), the orbital manifestation of Graves' disease (GD). TSHR autoantibody levels and orbital TSHR expression levels correlate positively with GO disease activity. Platelet-derived growth factors (PDGF) are increased in GO and potently activate orbital fibroblast effector functions. We investigated the possible relationship between PDGF and TSHR expression on orbital fibroblasts and how that influences the immunopathological effects of TSHR autoantibodies on orbital fibroblast activity. METHODS: Orbital fibroblasts were stimulated with PDGF-AA, PDGF-AB, and PDGF-BB, and TSHR expression was determined by flow cytometry. Stimulatory effects of bovine TSH and GD immunoglobulins on orbital fibroblasts (with or without PDGF-BB preincubation) were determined by IL-6, IL-8, chemokine (C-C motif) ligand (CCL)-2, CCL5, CCL7, and hyaluronan ELISA. The TSHR blocking antibody K1-70 and the cAMP inhibitor H89 were used to determine involvement of TSHR signaling. RESULTS: PDGF-AB and PDGF-BB stimulation increased TSHR expression on orbital fibroblasts, whereas PDGF-AA did not. Furthermore, stimulation with bovine TSH and immunoglobulins from GD patients induced IL-6, IL-8, CCL2, and hyaluronan production by orbital fibroblasts, and PDGF-BB preincubation enhanced this response of orbital fibroblasts. Blocking studies with a TSHR blocking antibody and a cAMP inhibitor inhibited these effects, indicating the involvement of TSHR signaling and thus of TSHR stimulating autoantibodies herein. CONCLUSIONS: These findings indicate that PDGF-B containing PDGF isoforms amplify the immunopathological effects of TSHR-stimulating autoantibodies in GO patients by stimulating TSHR expression on orbital fibroblasts.

Orbit-infiltrating mast cells, monocytes, and macrophages produce PDGF isoforms that orchestrate orbital fibroblast activation in Graves' ophthalmopathy.

PURPOSE: Platelet-derived growth factors (PDGF) are regulators of fibroblast activity that may be involved in the pathophysiology of Graves' ophthalmopathy (GO). We unraveled the expression and origin of PDGF family members in GO orbital tissue and investigated the effect of PDGF isoforms on IL-6 and hyaluronan production and proliferation by orbital fibroblasts. METHODS: PDGF-A, PDGF-B, PDGF-C, PDGF-D, PDGF-Rα, and PDGF-Rß expression was determined by real-time quantitative PCR and PDGF-A and PDGF-B protein expression was determined by Western blot in orbital tissues. Orbital tissues were immunohistochemically stained for PDGF-A and PDGF-B expression, together with stainings for T cells, monocytes, B cells, macrophages, and mast cells. Effects of PDGF-AA, PDGF-AB, and PDGF-BB on orbital fibroblast proliferation and IL-6 and hyaluronan production were examined. Finally, effects of PDGF-BB- and PDGF-AA-neutralizing antibodies on IL-6 and hyaluronan production in GO whole orbital tissue cultures were tested. RESULTS: GO orbital tissue showed increased PDGF-A and PDGF-B mRNA and protein levels. Increased numbers of PDGF-A- and PDGF-B-positive monocytes, macrophages, and mast cells were present in GO orbital tissue. PDGF-BB stimulated proliferation and hyaluronan and IL-6 production by orbital fibroblasts the most, followed by PDGF-AB and PDGF-AA. Finally, in particular imatinib mesylate and PDGF-BB-neutralizing antibodies reduced IL-6 and hyaluronan production by whole orbital tissue cultures from GO patients. CONCLUSIONS: In GO, mast cells, monocytes, and macrophages may activate orbital fibroblasts via secretion of especially PDGF-AB and PDGF-BB. Preclinical studies with whole orbital tissue cultures show that blocking PDGF-B chain containing isoforms can be a promising treatment for GO.

Chromosomal aberrations in iris melanomas.

BACKGROUND: Uveal melanomas can develop in the choroid, ciliary body and iris. In choroidal and ciliary body melanomas, specific chromosomal changes correlate with metastatic disease. Iris melanomas have a better prognosis than choroidal melanomas, and it would be interesting to know if they share chromosomal changes. In addition, iris melanomas might harbour UV-induced mutations of tumour suppressor genes, such as PTEN and CDKN2A. METHODS: Twenty iris melanomas were analysed for chromosome 1p, 3, 6, 8, 9p and 10q abnormalities using fluorescence in situ hybridisation. These results were correlated to clinical follow-up data using statistical analyses. RESULTS: (Partial) loss of chromosome 3 was observed in nine iris melanomas, and gain of 8q was present in seven tumours. Loss of chromosome 9p was demonstrated in seven tumours, but no deletions of the PTEN region on chromosome 10 were found. Three patients died of metastatic disease, and one patient developed liver metastases, but is still alive. Univariate analysis indicated a lower disease-free survival for patients with diffuse growing melanomas (p=0.01), melanomas that lost a copy of chromosome 3 (p=0.03), or invading the ciliary body (p=0.01). In a multivariate analysis, none of the correlations were significant. CONCLUSION: Loss of chromosome 3 as well as loss of chromosomal region 9p21 (that entails tumour suppressor gene CDKN2A) plays a role in iris melanoma. A firm correlation with disease-free survival could not be established, possibly due to the small sample size.

[Orbital metastases of breast cancer. Radiologic features]. / Mammakarzinommetastasen der Orbita. Radiologische Eigenschaften.

BACKGROUND: We reviewed the radiologic features of 15 patients with orbital metastases originating from breast cancer. METHODS: This was a retrospective consecutive case series. Fifteen consecutive patients with orbital metastases originating from breast carcinoma were identified between March 1997 and September 2008. A retrospective chart review was carried out, and the radiologic findings were reviewed. RESULTS: The metastases were preseptal in 53%, intraconal in 60%, and both intraconal and extraconal in 33%. Lacrimal gland enlargement was noted in 33%, episcleral space involvement in 33%, bone involvement in 13%, and globe dystopia in 53%. The extraocular muscles were involved in 87%; in 60%, two or more muscles were involved. The medial and lateral rectus muscles were affected in 53% and 47%, respectively, and the inferior and superior rectus muscles in 33%. In 47% one or more radiologic features had not been noted by the radiologist, and in 20% the findings were misinterpreted as an"orbital pseudotumor." CONCLUSION: Orbital metastases originating from breast cancer may present heterogeneously. Orbital imaging most commonly shows unilateral and multifocal involvement of multiple extraocular muscles and intraconal and preseptal areas by an irregular lesion.

[Waldenstrom's macroglobulinaemia of the lacrimal gland in a patient with sarcoidosis]. / Makroglobulinämie Waldenström der Tränendrüse bei einer Patientin mit Sarkoidose.

We report a case of bilateral lacrimal gland involvement as the first sign of Waldenstrom's macroglobulinaemia in a patient with sarcoidosis. Histological analysis of an incisional biopsy revealed a lymphoplasmocytic lymphoma consistent with Waldenstrom's macroglobulinaemia. No noncaseating granulomas were encountered. Systemic treatment was initiated and resulted in complete resolution of the lesions. In a patient with a systemic disease, such as sarcoidosis, and lacrimal gland involvement, a biopsy of the lacrimal gland mass should be taken to make a correct diagnosis and start appropriate treatment.