Eyelid Soft Tissue Chondroma: A Case Report.

Soft tissue chondromas are rare benign tumors that occur in extraosseous and extra-synovial locations. We report herein a rare presentation of eyelid soft tissue chondroma in a 45-year-old male presented with a 2-year history of a slowly enlarging subcutaneous firm mass on the left upper eyelid, and complete excision of the lesion followed by histopathological examination rendered the diagnosis of soft tissue chondroma.

Unilateral acquired blepharoptosis due to orbital metastatic disease as an initial presentation of an overlooked breast carcinoma - A case report.

INTRODUCTION AND IMPORTANCE: Orbital metastasis from breast cancer may be infrequently noted after the management of the primary lesion. It is rare in such cases to present with unilateral mechanical blepharoptosis without ophthalmoplegia. CASE PRESENTATION: We present a case of unilateral acquired blepharoptosis of the left upper eyelid without ophthalmoplegia or exophthalmos three years after the onset of a suspicious breast mass. The patient did not disclose a history of breast cancer nor any positive family history to the ophthalmologist and anesthesiologist. The radiology images revealed an ill-defined enhanced lesion at the superior medial aspect of the left orbit. The orbital biopsy of the lesion showed poorly differentiated carcinoma as per the initial histopathology report. Vigilant history-taking enabled us to get information about a previous breast lesion and to correlate this with specific histopathological findings. DISCUSSION: Management of orbital lesions might be challenging, and the approach should include detailed history and assessment. Biopsy and radio imaging are further needed to aid in providing the proper diagnosis. The clinicopathological correlation in our case has led to the final diagnosis of orbital metastatic breast cancer. CONCLUSION: Ophthalmologists should be aware of variable ocular presentations of malignancy and adopt a team approach to obtain a carefully detailed history from patients presenting with orbital diseases and communicate adequately with the ocular pathologists who are handling the biopsy. Long-term follow-up and enhancement of patients' awareness of possible late orbital metastasis are recommended in all patients with breast masses.

Modified Anterior Lamellar Recession for All Grades of Upper Eyelid Trachomatous Cicatricial Entropion.

Purpose: This study aims to assess the combination of anterior lamellar recession (ALR) with blepharoplasty, suprasternal fixation, and internal eyelash bulb extirpation of aberrant lashes posteriorly located in patients with any grade of upper eyelid trachomatous cicatricial entropion. Patients and Methods: We reviewed the clinical data of eighty-six patients (143 eyelids) including age, gender, systemic medical illnesses, and comprehensive ophthalmological assessment. Eyelid evaluation was recorded, including laterality, previous surgical technique used, possible trichiasis etiology, abnormality of the lid margin, tarsal plate consistency (shrinkage or loosening), skin fold overhanging, laxity of the pretarsal skin, margin reflex distance 1 (MRD1), lagophthalmos, and lid retraction. The success rate was assessed at 3, 6, 9, and 12 months postoperative. Results: The success rate was 97.2% in the third month, which decreased significantly to 92.3% in the 6th month and 90.2% in the 9th month (P = 0.01, and 0.001 respectively). In the 9th month, we had fourteen failed cases. All of them were submitted for a second intervention. Three underwent electrolysis, four cases underwent re-internal bulb extirpation, four cases underwent the same procedure, and three cases underwent epilation. The success of the failed cases after the second intervention was significantly associated with the type of reintervention (P = 0.03), in which all of them succeed except two cases that underwent epilation. Kaplan-Meier analysis showed that the mean recurrence time in our study was 6.8 months (95% CI = 5.8-7.7 months). Conclusion: This study showed the combination of ALR with blepharoplasty, suprasternal fixation, and cauterization or internal bulb extirpation of posteriorly located lashes procedure resulted in a high success rate in patients with any form of UCE with no increase in incidence or degree of lagophthalmos associated with UCE.

Histopathological changes of lymphatic malformation after bleomycin injection / Alterações histopatológicas da malformação linfática após injeção de bleomicina

ABSTRACT Lymphatic malformation is a rare orbital tumor that used to be treated surgically, with high complication rates, or recently with intralesional bleomycin injection. We report for the first time the histopathological changes of eyelid lymphatic malformation after water-soluble intralesional bleomycin injection in a 20-year-old woman who had unsuccessful orbital surgical debulking during childhood. The changes confirmed the assumption of fibrosis induced by intralesional bleomycin injection. The minimal bleeding during surgical intervention made it much easier than the usual lymphatic malformation bloody procedure, without postoperative recurrences and with favorable aesthetic outcomes.

RESUMO A malformação linfática é um tumor orbital raro que costumava ser tratado cirurgicamente, com alta taxa de complicações. Mais recentemente, passou a ser tratado com uma injeção intralesional de bleomicina. Este é o primeiro relato sobre as alterações histopatológucas da malformação linfática palpebral após uma injeção intralesional de bleomicina hidrossolúvel em uma mulher de 20 anos de idade que sofreu uma cirurgia malsucedida de debulking orbital durante a infância, confirmando a suposição de fibrose induzida por injeções intralesionais de bleomicina. O sangramento mínimo durante a intervenção cirúrgica tornou esta muito mais fácil que o procedimento sangrento habitual, sem recidivas pós-operatórias e com desfechos estéticos favoráveis.

Histopathological changes of lymphatic malformation after bleomycin injection.

Lymphatic malformation is a rare orbital tumor that used to be treated surgically, with high complication rates, or recently with intralesional bleomycin injection. We report for the first time the histopathological changes of eyelid lymphatic malformation after water-soluble intralesional bleomycin injection in a 20-year-old woman who had unsuccessful orbital surgical debulking during childhood. The changes confirmed the assumption of fibrosis induced by intralesional bleomycin injection. The minimal bleeding during surgical intervention made it much easier than the usual lymphatic malformation bloody procedure, without postoperative recurrences and with favorable aesthetic outcomes.

Demographics and histopathological characteristics of enucleated microphthalmic globes.

Microphthalmia is a rare ocular anomaly with a poorly understood etiology that is most likely related to heritable and/or environmental factors. Many papers have been published pertaining to the clinical manifestations and management of this condition; however, few reports have reported detailed histopathological findings, which are the focus of this study, in addition to highlighting the basic demographics in these cases. This was a retrospective, observational study of all consecutive enucleated microphthalmic globes (with or without cysts) at 2 tertiary eye hospitals in Riyadh, Saudi Arabia. Globes were classified into 2 groups: severe microphthalmos (axial length or mean diameter less than 10 mm in infancy or 12 mm after age 1 year) and mild microphthalmos based on larger measurements. Clinical and demographic data collected included sex, age at enucleation, eye involvement, nationality/region, consanguinity, family history of eye anomaly, pregnancy, systemic disease, or syndromes. For histopathological data, a descriptive analysis was mostly performed. For correlations of some of our qualitative data, Fisher's exact test was used. Eleven cases (6 mild and 5 severe microphthalmos) were initially identified with a female to male ratio of 4:7. Ten patients were Saudis, 7 of whom were from the central region. Consanguinity was found in 36% (4/11), and 3 of them had other ocular or systemic abnormalities (duodenal atresia, microcephaly, kidney agenesis, cryptophthalmos, and dysmorphic facial features). Histopathological data were available for 10 cases, half of which showed a coloboma and/or anterior segment anomaly. There was no significant correlation among gender, severity of microphthalmos or the presence of coloboma, although severe microphthalmic globes had a higher median of abnormal intraocular structures (9-interquartile range = 2 compared to 6-interquartile range = 1 in the mild group). Aphakia was found in half of the globes with associated anterior segment dysgenesis. We have concluded that microphthalmos is a visually disabling congenital anomaly that can be isolated or associated with other periocular or systemic anomalies, possibly in relation to consanguinity in our cases. Congenital aphakia was found in half of these cases and was mostly associated with absent Descemet's membrane and agenesis of anterior chamber angle structures, supporting previously suggested embryological concepts. These findings necessitate further wider genetic testing and proper premarital counseling in Saudi Arabia.

Paraneoplastic pemphigus associated with rapidly progressing endophthalmitis caused by Morganella Morganii: A case report.

INTRODUCTION: The ocular presentation of paraneoplastic pemphigus (PNP) has rarely been reported in the literature. In this report, we describe a 61-year-old male presenting with eruptive skin lesions associated with underlying non-Hodgkin's lymphoma who had rapid progressive corneal perforation with secondary endophthalmitis in the setting of PNP. CASE DESCRIPTION: A 61-year-old male presented to the emergency department complaining of skin eruption mimicking Stevens-Johnson syndrome, which was later found to be related to PNP. Initially, the patient complained of progressive ocular surface dryness in both eyes. Meanwhile, he developed mild pain in the right eye associated with blurry vision in both eyes and was managed with lubricants and topical antibiotics. A few days later, he was found to have corneal perforation with features suggestive of left endophthalmitis with possible early panophthalmitis. Intravenous antibiotic was administered, and primary evisceration of the left globe was performed. Histopathology revealed acute necrotizing keratitis and endophthalmitis. Vitreous analysis showed numerous gram-negative bacilli and a positive culture of Morganella morganii. The patient continued to be managed with frequent lubrications and punctual plugs in the fellow eye during the follow-up period. CONCLUSION: We describe the first case of endophthalmitis developing secondary to PNP-induced corneal melting and perforation. Anticipating unusual infectious sequelae in the setting of PNP might be warranted to actively detect and successfully manage dry eye disease before devastating complications develop.

Dacryops and clinical diagnostic challenges.

OBJECTIVE: The study aims to describe the clinical and histopathologic features of different types of dacryops and their clinical diagnostic challenges. METHODS: This is a retrospective cohort study of all surgically excised cases of dacryops in 2 tertiary eye hospitals in Riyadh, Saudi Arabia. RESULTS: The study included 58 dacryops specimens from 55 patients with an average age of 41.2 years (range, 4-78 years). The most common location was the upper lid (60.3%), whereas the least expected location was the caruncle (6.9%). The most common site of dacryops occurrence was in the accessory lacrimal gland (55.2%), the main lacrimal gland (32.8%), and then ectopic dacryops (12%). All patients presented with lid swelling alone except for 3 patients who experienced secondary mechanical ptosis. On physical examination, conjunctival scarring existed in 4 patients (6.9%). Preoperative diagnosis of dacryops was accurate in 44.8% of the cases. Dacryops of the main lacrimal gland was accurately diagnosed clinically in all cases compared with other locations, which was statistically significant (p < 0.001). The causes of inaccurate clinical diagnoses were hidrocystoma (26.9%), inclusion cyst (11.5%), and dermoid cyst (7.7%), whereas the remaining cases were diagnosed as cysts without a specific subtype (53.9%). Recurrence of the lesion was observed in 2 cases (3.5%). No clinical or histopathologic factors were associated with a risk of recurrence. CONCLUSION: Dacryops can represent a diagnostic challenge to ophthalmologists. Familiarity with clinical presentations and findings is required to diagnose dacryops outside the main lacrimal gland.

Superior oblique myositis mimics a subperiosteal abscess in a patient with sinusitis / Miosite oblíqua superior mimetiza abscesso subperiosteal em paciente com sinusite

ABSTRACT Isolated superior oblique myositis is a rare variant of idiopathic orbital myositis. We are reporting for the first time the case of a 19-year-old woman who had isolated superior oblique myositis with sinusitis that mimics a subperiosteal abscess. Despite the typical history of upper respiratory tract infection and laboratory test results and initial radiological findings suggestive of orbital cellulitis secondary to sinusitis, the initial response to systemic steroid with subsequent imaging changes and the relapse after cessation of steroid therapy helped us reach the diagnosis.

RESUMO A miosite oblíqua superior isolada é uma variante muito rara da miosite orbital idiopática. Trata-se do primeiro relato de uma mulher de 19 anos como um caso de miosite oblíqua superior isolada com sinusite que mimetiza abscesso subperiosteal. Apesar da história típica de infecção do trato respiratório superior, exames laboratoriais e achados radiológicos iniciais sugestivos de celulite orbital secundária à sinusite, a resposta inicial ao esteróide sistêmico com subsequentes alterações de imagem e recaída, após a cessação do esteróide, nos ajudou a alcançar o diagnóstico.

Primary mucinous adenocarcinoma of the eyelid: A case with initial clinical misdiagnosis requiring surgical re-excision of the tumor.

INTRODUCTION AND IMPORTANCE: Primary mucinous adenocarcinoma (PMA) of the skin is a rare condition that is usually seen in elderly patients, most commonly involves the periorbital region as a slow growing mass. Histopathological and immunohistochemical (IHC) stains are of paramount importance for the diagnosis of these lesions, which are usually misdiagnosed either as benign or metastatic mucinous adenocarcinomas. CASE PRESENTATION: We herein report a rare presentation of PMA in a 70-year-old male patient who presented with an upper eyelid residual lesion after being incompletely excised elsewhere as an epidermal cyst and was successfully managed by complete surgical excision with frozen section control of the margins and no evidence of recurrence. DISCUSSION: PMA is a rare sweat gland malignancy that involves the eyelid in 41.9% in the head and neck area and is a disease of the elderly with median age of 60 years and variable reported racial and gender predilection. Diagnosis of PMA is challenging both clinically and histopathologically, which was the case in our patient's initial incomplete excision with the presumed diagnosis of a benign epidermal cyst. Proper final tissue diagnosis and surgical management in our patient ensured his favorable outcome. CONCLUSION: Accurate diagnosis of PMA requires a high index of clinical suspicion and accurate histopathological diagnosis aided by proper IHC markers.

Superior oblique myositis mimics a subperiosteal abscess in a patient with sinusitis.

Isolated superior oblique myositis is a rare variant of idiopathic orbital myositis. We are reporting for the first time the case of a 19-year-old woman who had isolated superior oblique myositis with sinusitis that mimics a subperiosteal abscess. Despite the typical history of upper respiratory tract infection and laboratory test results and initial radiological findings suggestive of orbital cellulitis secondary to sinusitis, the initial response to systemic steroid with subsequent imaging changes and the relapse after cessation of steroid therapy helped us reach the diagnosis.

Massive Retinal Gliosis in an Infant Microphthalmic Globe: A Case Report.

BACKGROUND Massive retinal gliosis (MRG) is a rare benign intraocular tumor that results from the proliferation of well-differentiated glial cells in response to long-standing pathological processes, including glaucoma, trauma, chronic inflammation, vascular disorders, and congenital anomalies. This lesion is considered to be nonneoplastic and occurs ≥10 years after the predisposing insult. It usually affects children and can mimic other conditions, including uveal melanomas, vasoproliferative tumors of the retina, astrocytic hamartomas, and retinal hemangioblastomas. CASE REPORT We present a case of infant MRG with severe left eye microphthalmia. An 11-month-old boy was presented by his parents in the Oculoplastic Unit of a teaching university hospital with bilateral incomplete cryptophthalmos and small globes. An enucleation of the left globe was carried out to stimulate orbital bone growth and to improve the cosmetic outcome. The histopathological examination revealed a microphthalmic globe with sclerocornea and disorganized intraocular anterior segment structures. The retina was dysplastic with proliferating spindle-shaped glial cells showing fibrillar eosinophilic cytoplasm and filled most of the vitreous cavity. The glial origin of the cells was confirmed by the immunohistochemical markers (glial fibrillary acidic protein and synaptophysin), supporting the diagnosis of MRG. The optic nerve was markedly hypoplastic. CONCLUSIONS MRG is a rare intraocular tumor that is clinically difficult to diagnose. A definite diagnosis can be made only on the basis of a histopathological examination and immunohistochemical markers.

An incidental finding of intraocular choristoma in an enucleated microphthalmic globe: A histopathologic case report.

INTRODUCTION AND IMPORTANCE: Choristomas are benign growth of normal tissue in abnormal location and in the ophthalmic practice, they are more commonly found in the epibulbar region. Intraocular choristoma has been reported in different ocular structures but it is very rare especially in association with microphthalmos. CASE PRESENTATION: We present a 13-month-old child with bilateral microphthalmia with the left side being more significantly smaller than the right that required enucleation for introducing a larger silicone implant. The histopathological examination revealed an intraocular choristoma consisting of chondroid and adipose tissue with surrounding fibrosis. Other areas in the globe were also underdeveloped and dysplastic including the optic nerve, which was replaced by dense wavy collagen fibers and fibrovascular tissue. DISCUSSION: Even though choristomas are benign, they may be extensive interfering with visual development especially the ones involving the epibulbar area. Systemic disease can have choristomas as an ocular feature such as in Goldenhar-Gorlin syndrome. Choristomas inside the eye are rare and they commonly involve the uveal tissue and the optic nerve head mostly in the form of ectopic glandular tissue and choroidal osseous choristoma. Our case is unique in its intraocular retrolental location, composition of chondroid tissue and fat, in addition to the fact that it was found within a microphthalmic globe with other interesting histopathological findings. CONCLUSION: We report a case of an incidental finding of intraocular choristoma with associated microphthalmia, genetic testing may be useful for establishing a genetic etiology in such cases even in the absence of dysmorphic features.

A case of dyschromatosis symmetrica hereditaria with an associated eyelid hemangioma.

INTRODUCTION AND IMPORTANCE: Dyschromatosis symmetrica hereditaria (DSH) are rare autosomal dominant pigmentary genodermatosis characterized by reticular hyper- and hypopigmented skin macules on the dorsal aspect of the extremities and freckle-like spots on the face, sparing the palms and soles. Cutaneous hemangiomas were not reported in the literature with DSH. We describe for the first time to the best of our knowledge a case of DSH with histopathologically confirmed eyelid hemangioma. CASE PRESENTATION: A 25-year-old female was diagnosed with DSH in her childhood by a dermatologist then later developed cutaneous lupus erythematosus (CLE). Four years later she presented to our clinic with right lower eyelid painless mass. The histopathological examination showed inflamed epidermis overlying a mixed capillary and cavernous hemangioma. The patient had complete healing of the skin post-operatively with excellent cosmetic result. DISCUSSION: DSH is usually isolated, however, acral hypertrophy, psoriasis, dental anomalies, aortic valve sclerosis, dystonia and intracranial hemangiomas have been reported in association with the disease. The types of the hemangiomas reported were not specified with lack of tissue diagnosis. Our case is unique because of the late occurrence of this eyelid skin hemangioma, the concomitant CLE, the history of hyperthyroidism, and the positive family history of consanguinity. CONCLUSION: The pathogenesis of DSH is not well understood, however the previously reported intracranial hemangiomas and the currently reported skin vascular lesion would raise the role of inheritance and variable expression of such an association especially with concomitant CLE. This may warrant further studies on the etiology of DSH.

Presumed acute suppurative bacterial dacryoadenitis with concurrent severe acute respiratory syndrome coronavirus-2 infection.

Acute suppurative bacterial dacryoadenitis (ASBD) was not reported to occur following severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. We are presenting a unique case of concurrent presumed ASBD with SARS-CoV-2 infection. A 23-year-old previously healthy male presented with right upper eyelid swelling and pain over the lacrimal gland area for 3 days. Before his visit, the patient was tested positive for SARS-CoV-2 infection after experiencing mild flu-like symptoms, despite being vaccinated 2 months ago. He was found to have clinical and radiological features highly suggestive of ASBD with concurrent SARS-CoV-2 infection. He was admitted and initiated on systemic and topical antibiotic, followed by incision and drainage of an abscess collection. The patient showed dramatic clinical improvement with no recurrent signs of infection during the follow-up period. This acute dacryoadenitis is presumed to represent a secondary bacterial infection possibly aided by immune-related factors that may coexist in SARS-CoV-2-positive patients.

Surgical Observations of the Levator Aponeurosis Fibrotic Changes in Simple Congenital Ptosis Suggest Complex Pathogenesis.

PURPOSE: To shed light upon the possible role of the levator aponeurosis (LA) developmental fibrotic changes as an added etiology for simple congenital ptosis, which causes limitation of the levator function (LF). METHODS: This retrospective cohort study included patients with simple congenital ptosis who underwent skin approach LA resection as a primary intervention with an intraoperative photographic documentation of LA fibrotic changes. Preoperative demographics and clinical data were reviewed. The effect of LA fibrotic changes on the LF was assessed in different LA fibrotic changes with or without levator palpebrae superioris (LPS) muscle fatty infiltration. RESULTS: A total of 56 eyelids of 49 patients with a mean age (±SD) 6.7 (±3.2) years were enrolled in this study. The fibrotic changes of LA were observed as a sheet of fibrosis (19 eyelids) or fibrous bands (23 eyelids). Fatty infiltration of LPS was noticed in 28 eyelids, either with or without fibrotic changes of LA. Preoperative LF was diminished in LPS fatty infiltration compared with LA fibrotic sheets (P = 0.026). Postoperative LF improved significantly in both LA fibrotic sheets and LA fibrotic bands (9.4 ± 2.5 mm and 9.6 ± 2.8 mm, respectively) compared with LPS with fatty infiltration (6.4 ± 1.8 mm) (P = 0.004). CONCLUSIONS: Although our data are inconclusive due to lack of embryologic studies, the observed LA fibrotic changes may suggest a complex pathogenesis of simple congenital ptosis. The meticulous observation of the LA and the releasing of any adhesion or band to the surrounding structures could improve postoperative LF.

Late endoscopic evaluation of the ostium size after external dacryocystorhinostomy.

OBJECTIVE: To evaluate the effect of chronicity on the size of the ostium after external dacryocystorhinostomy (DCR) with intubation. METHODS: Design: A retrospective chart review of patients who underwent external DCR with intubation over 10 years from January 2003 at a tertiary hospital. All patients were recruited and examined with rigid nasal endoscope. RESULTS: A total number of 66 (85 eyes) patients were included. The mean age at the time of evaluation was 53.1 years with gender distribution of 54 females (81.8 %). The mean duration ±SD between the date of surgery and the date of evaluation was 33.2 ± 33.6 (6-118 months). Our study showed an overall anatomical and functional success of 98.8% and 95.3%, respectively. The mean size of the ostium (±SD) was 23.0 (±15.7) mm2 (ranging from 1 to 80.4 mm2). The size of the ostium was not a significant factor for failure (p = 0.907). No statistically significant correlation was found between the long-term duration after surgery and the size of the ostium (R: 0.025, p = 0.157). CONCLUSIONS: Nasal endoscopy after DCR is valuable in evaluating the ostium with no observed potential correlation between the long-term follow-up after surgery and the size of the ostium.

Sebaceous Gland Carcinoma with Misleading Clinical Appearance: A Case Report of an Eyelid Lesion.

BACKGROUND Sebaceous gland carcinoma (SGC) is a rare malignant lesion that occurs on the eyelids. It is known to mimic other benign or malignant lesions in clinical presentation, such as a chalazion, basal cell carcinoma, and squamous cell carcinoma. The histopathological diagnosis is the mainstay for diagnosis and is often challenging. CASE REPORT We describe a case of SGC in a 53-year-old woman who presented with a cauliflower-appearing lesion with pearly telangiectatic vessels and raised margins at the lower eyelid margin. Clinically, we suspected a diagnosis of basal cell carcinoma. Upon complete resection of the lesion, the final diagnosis was SGC based on the histopathological features and immunohistochemical staining characteristics of the tissue. CONCLUSIONS Due to the possibility of SGC presenting similarly to other lesions, it is essential for ophthalmologists to have a high index of suspicion in its diagnosis. The early and accurate diagnosis of such lesions is important for appropriate management to prevent metastasis or recurrence related to advanced tumors.

Clinical features differentiating intratarsal keratinous cyst from chalazion.

PURPOSE: To present the differentiating clinical findings between intratarsal keratinous cyst (IKC) and chalazion. METHOD: A retrospective review of medical records of all patients who presented between 2010 and 2018 at King Abdulaziz University Hospital with proven histopathological diagnosis of IKC was done. Complete ophthalmologic evaluation at presentation, surgical procedures performed, complications, histopathological findings, response to treatment and follow-up were recorded. RESULTS: Twelve patients were found to have IKC. All patients presented with an eyelid mass with no signs of local inflammation. All lesions were fixed to the tarsus with freely mobile overlying skin, which was found to be slightly pale compared to the surrounding skin in six patients. On palpation, IKC had well-defined boarders. Isolation with clear surgical plane for cyst excision was achieved in nine patients as they were superficially involving the tarsus. When IKC involved the deep part of the tarsus, bluish/ whitish nodules were seen upon eyelid eversion. Six patients were misdiagnosed and surgically treated as a chalazion elsewhere prior to presentation to us with recurrence. CONCLUSION: Differentiating IKC from chalazion can be challenging. Careful clinical evaluation helps reaching the right diagnosis and providing the correct treatment, which involves complete excision of IKC to prevent recurrence.

Primary extramedullary plasmacytoma of the eyelid conjunctiva - A case report and review of the literature.

Extramedullary plasmacytomas (EMPs) are uncommon plasma cell tumors that develop in soft tissue as isolated tumors without osseous involvement while secondary lesions are associated with systemic multiple myeloma (MM). Primary extramedullary lesions are most commonly found in upper respiratory tract, gastrointestinal tract and lymph nodes. They can be found either in patients with history of MM or preceding the manifestation of a systemic disease. Orbital manifestation of the lesion is rare but conjunctival involvement is very unusual. The reported cases in the English-written literature are only five cases. Herein, we report the sixth case of primary EMP in a middle-aged adult who presented with a lesion confined to the conjunctiva, unremarkable present and past medical history, and confirmed tissue diagnosis. In addition, a review and summary of the previously reported cases in the literature is presented. We aim to attract the attention of ophthalmic surgeons to consider plasmacytoma within the differential diagnosis of a conjunctival lesion.