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A 42-year-old woman presented with a small pigmented lesion of the palpebral conjunctiva that had been present for a few months. Because of the possibility of melanoma, the lesion was resected. Microscopic examination displayed an intratarsal blue nevus at the level of the meibomian glands comprised of bland nonpigmented and pigmented cells that enveloped a sebaceous gland and its ducts. The cells were of admixed spindle and epithelioid configuration and were immunoreactive for Melan-A. The Ki67 proliferative marker was negative in these cells, contrasting with the epithelium of the overlying conjunctiva and the sebaceous ducts, and thereby militating against the diagnosis of melanoma. Clusters of melanophages were also present. Although an intratarsal blue nevus has been described as a component of a combined nevus, the current lesion demonstrates the occurrence of a sole tarsal blue nevus. Palpebral pigmented lesions should be customarily excised because many are melanomas.

Apocrine hidrocystomas are benign cystic tumors derived from apocrine sweat glands; they are most commonly located in the skin of the head and neck regions. Ophthalmic occurrences typically appear at the lash line and canthi of the eyelid, although rare instances have been described in the conjunctiva, caruncle, and orbit. The authors illustrate an exceptional instance of a mobile episcleral cyst in a 12-year-old girl that developed about 2 years following strabismus surgery. The cyst was located anterior to the insertion of the left medial rectus and was excised in response to the patient's complaints of irritation. Histopathology of the excised specimen displayed an empty cyst lined by a double layer of cuboidal epithelium with the inner layer exhibiting apical decapitation secretion. Confirmatory immunohistochemistry demonstrated reactivity of both layers with CK7 and the outer myoepithelial layer with D2-40. Postoperative and traumatic cysts formed after interruption of the bulbar conjunctiva are usually conjunctival cysts lined by stratified squamous nonkeratinizing epithelium.

Conjunctival papillomas are common tumors that exhibit an exophytic growth pattern, comprised of multiple filiform fronds of squamous epithelium that contain fibrovascular cores. The inverted (endophytic) variety of papilloma, often termed "Schneiderian," rarely occurs on the conjunctiva, with only 15 cases reported to date. Endophytic and exophytic papillomas are well described arising in the sinonasal Schneiderian epithelium where a low rate of malignant transformation may occur in the endophytic type; malignant transformation in exophytic sinonasal papillomas is exceedingly rare. The authors describe 2 cases of exophytic conjunctival papillomas with the morphology of a sinonasal or Schneiderian-type papilloma. Both were pink, sessile acquired growths in women in the sixth decade of life involving the inferior conjunctival fornix or nasal limbus. Nonkeratinizing squamous epithelium along with numerous goblet cells, intraepithelial mucinous cysts, and microabscesses were present. Immunohistochemistry showed reactivity for cytokeratin 7 and wild-type staining for p16 and p53, paralleling the findings in common conjunctival papillomas; both were also driven by low-risk human papillomavirus.

Apocrine hidrocystomas are benign cystic tumors derived from apocrine sweat glands; they are most commonly located in the skin of the head and neck regions. Ophthalmic occurrences characteristically appear at the lash line and canthi of the eyelid, although rare instances have been described in the conjunctiva, caruncle, and orbit. The authors describe an exceptional instance of a mobile epibulbar subconjunctival apocrine hidrocystoma in a 57-year-old woman without a history of previous ocular injury or surgery. Histopathology of the excised specimen displayed an empty cyst lined by a double layer of cuboidal epithelium with the inner layer exhibiting periodic acid-Schiff-positive apical decapitation secretion. Confirmatory immunohistochemistry included reactivity with cytokeratin-7, smooth muscle actin, D2-40, and CDGFP-15.

A yellow cyst of the caruncle in a 68-year-old man displayed the characteristic sebaceous glands and sebocytes of steatocystoma within the cyst wall, with a unique configuration of multiple branching compartments. The cyst lining was of trichilemmal character, lacking a keratohyalin granular layer, and replicated the immunohistochemical characteristics of a previously reported caruncular steatocystoma with the exception of a positive trichilemmal marker, calretinin, in the present case. Four previous cases of caruncular steatocystoma have been described, only one of which incorporated immunohistochemical analysis. Steatocystoma develops from a sebaceous gland duct, which displayed in this case multiple chambers subdividing what is usually a single round lumen.

Pilomatrixoma, an uncommon, usually benign cutaneous appendageal tumor, shows differentiation toward the hair follicle matrix cell. It undergoes various histopathologic stages, early on displaying epithelial and shadow cells along with granulomatous inflammation. In later stages, illustrated by this unusual case, epithelial cells disappear and are replaced by calcification and ossification. Immunohistochemistry in the current case showed transitional cell reactivity for β-catenin, probably linking the tumor to a mutation in the β-catenin gene CTNNB1. There was also transitional cell positivity for cyclin D1, a marker found in matrical cells of the human hair follicle. While pilomatrixoma occurs occasionally in the eyelid, the ossified eyelid variant in the current case is very rare, with only one preceding description in the literature.

A 58-year-old man presented with left-sided orbital inflammation, including chemosis and a lateral rectus abduction defect. Initially presumed to represent cellulitis, the condition responded poorly to oral and intravenous antibiotics. CT showed the epicenter of an infiltrate to involve the lateral rectus. The patient improved dramatically when oral prednisone was added. Lateral rectus biopsy displayed intramuscular polyclonal lymphoid infiltrates, rich with eosinophils. Complete resolution of the inflammatory process was confirmed by a follow-up CT. The presumptive diagnosis was idiopathic orbital myositis, an uncommon condition of unknown etiology. However, the patient had taken rosuvastatin, which has been rarely associated with diplopia and ophthalmoplegia, raising the question of whether this case was truly idiopathic.

Osteoma cutis is a term applied to bone formation in the dermis or subcutaneous tissue. It is deemed primary when occurring de novo without prior disease or secondary when associated with pre-existing inflammation, neoplasia or trauma. The authors describe a longstanding focal calcified and ossified lesion of the upper eyelid in a healthy 10-year-old girl along with a sterile corneal ulcer. The lesion most likely represented an ossified tarsal cyst or chalazion.

A yellow cyst of the caruncle in a 68-year-old man displayed the characteristic sebaceous glands and sebocytes of steatocystoma within the cyst wall, with a unique configuration of multiple branching compartments. The cyst lining was of trichilemmal character, lacking a keratohyalin granular layer, and replicated the immunohistochemical characteristics of a previously reported caruncular steatocystoma with the exception of a positive trichilemmal marker, calretinin, in the present case. Four previous cases of caruncular steatocystoma have been described, only one of which incorporated immunohistochemical analysis. Steatocystoma develops from a sebaceous gland duct, which displayed in this case multiple chambers subdividing what is usually a single round lumen.

Conjunctival involvement in sarcoidosis is commonly characterized by epibulbar nodules or follicular conjunctivitis. The authors describe an apparently healthy woman who developed extensive monocular cicatricial conjunctivitis with symblepharon. The array of conditions presenting with cicatricial conjunctivitis was considered, with mucous membrane pemphigoid leading the diagnostic possibilities. Conjunctival biopsy disclosed the non-infectious, non-caseating granulomas of sarcoidosis and a systemic evaluation disclosed pulmonary nodules and hilar lymphadenopathy. As the patient had no respiratory symptoms and an old history of hepatic steatosis, oral hydroxychloroquine and topical cyclosporin were chosen for therapy rather than systemic corticosteroids.

A 22-year-old woman presented with an acute compressive optic neuropathy due to a ruptured ethmoido-orbital mucocele. She underwent urgent orbital decompression and drainage of the mucocele via an endoscopic approach. Postoperatively, her course was complicated by an orbital compartment syndrome supervened, exhibiting severe eyelid edema caused by infiltration with mucin and mucin-containing macrophages ("muciphages"). Biopsy of the eyelid showed infiltration with "muciphages," macrophages laden with extravasated mucinous material. This is the first report that documents the clinical and histopathologic course of orbital inflammation following mucocele extravasation into the orbit and eyelids.

An enlarging white caruncular cyst in a 24-year-old woman proved to be a keratinous cyst lined by keratinizing squamous epithelium that lacked a keratohyalin granular layer (trichilemmal keratinization). The cyst most likely originated from a sebaceous gland duct. A panel of immunohistochemical stains was employed that compared and contrasted the cyst lining with the overlying caruncular conjunctival epithelium and further eliminated the likelihood of a conjunctival cyst. The most useful confirmatory stains were the positive trichilemmal marker calretinin, the positive sebaceous ductal marker cytokeratin 17 (suprabasal epithelium), and the negative conjunctival markers cytokeratins 7 and 19. Only one previous report of a very rare similar caruncular cyst is recorded in the literature without an extensive immunohistochemical analysis.

A 72-year-old woman who presented with right-sided epiphora and conjunctivitis underwent a probing and irrigation procedure with normal results. She improved with antibiotic-steroid drops. A swelling in the medial canthal region completely resolved. One year later, she returned with symptoms of dacryocystitis. An external dacryocystorhinostomy was performed. Characteristic dacryoliths were removed from the sac lumen, and biopsy of the sac wall showed spicules of lamellar bone within a fibrous stroma. Diagnosed as fibrous dysplasia of the lacrimal sac, this rare entity represents the second such case in the literature.The histopathology of an ossified lacrimal sac resembled fibrous dysplasia of bone and exemplifies the second case of this rare entity in the literature.

A cystic lesion of the eyelid margin in a 37-year-old man was diagnosed clinically as an apocrine hidrocystoma, a common lesion in that location. Histopathologic examination confirmed the presence of a hidrocystoma but also disclosed an extensive proliferation of bland epithelial cells arising from the inner layer of the cyst. The authors further characterized this unique occurrence on the spectrum of Moll gland eyelid tumors by immunohistochemical analysis.

Nasal glioma, encephalocele, and ectopic brain are rare congenital anomalies. The terminology applied to these entities has been historically confusing. In many cases, the terms overlap and may be employed synonymously although some authors emphasize their differences. The authors describe herein a child with an inner canthal mass of brain-like tissue that they interpret as nasal glioma, a variety of encephalocele that has lost its connection to the intracranial contents. This research was conducted in conformity with the Helsinki Declaration and Health Insurance Portability and Accountability Act regulations.

Two cases of limbal cysts lined by nonkeratinizing epithelium were studied with a panel of cytokeratins. One was a long-standing lesion in a 30-year-old man, whereas the other was excised from a 40-year-old man following pterygium surgery. Each cyst was immunostained with a panel of cytokeratins that were specific exclusively and separately for corneal and conjunctival epithelia. The epithelial lining of each cyst was CK12 positive for corneal epithelium and CK13 negative for conjunctival epithelium. It is hypothesized that a subset of corneoscleral cysts contain corneal epithelium, probably derived from a type of limbal stem cell differentiation.

A yellow cystic lesion of the caruncle in a 23-year-old woman proved to be a solitary steatocystoma, a rare occurrence in that location. While the histopathologic diagnosis was evident from clusters of sebaceous cells within the cyst wall, a panel of immunohistochemical stains further distinguished the lesion from a keratinous cyst. The most useful stains for differentiating the two conditions were carcinoembryonic antigen, epithelial membrane antigen, cytokeratins 17 and 19, and calretinin. Only three previous cases of caruncular steatocystoma simplex have been reported, none of which included immunohistochemical studies. The current findings support the origin of the cyst from the small duct that connects the unilobular sebaceous gland associated with vellus hairs to the follicular canal.

The authors describe a 4-year-old girl presenting with a 2-year history of a superomedial eyelid "bump" that appeared cystic on MRI. The clinical diagnosis was dermoid cyst, possibly of conjunctival origin. Following excision, histology showed a cyst that contained keratin and lanugo hairs in its lumen with sebaceous glands and chronic inflammation in its fibrous wall. An unanticipated finding was the presence of a trichilemmal (pilar) variety of epithelial lining that stained positively for calretinin, an immunostain that identifies trichilemmal epithelium. To the authors' knowledge this is the first case of a dermoid cyst with trichilemmal lining. This study was conducted in compliance with the rules and regulations of the Health Insurance Portability and Accountability Act and in conformity with the Oslo declaration.

A raised erythematous eyelid lesion that appeared in a 31-year-old man was diagnosed as an arteriovenous malformation (AVM), with confirmatory Doppler ultrasound demonstrating high arterial flow. Surgical excision, aided by electrocautery for extensive hemorrhage, resulted in an acceptable cosmetic result. Histopathology of the excised lesion showed collapsed capillary channels lined by endothelium. AVM is rarely encountered in the eyelid.

Conjunctival amyloid is usually of the immunoglobulin light chain (AL) variety. The authors report an exceptional case of bilateral limbal amyloid deposits that were identified by mass spectrometry as keratin-related specifically regarding to basal keratinocyte keratins 5 and 14. Cytokeratin-related amyloid has been described by immunohistochemical analysis of formalin-fixed cutaneous and mucous membrane lesions but not ocular tissues. The conjunctival lesion in the right eye contained intraepithelial dyskeratotic cells that extended to the surface, causing a lacy leukoplakia on clinical examination. The authors excluded the diagnosis of hereditary benign intraepithelial dyskeratosis in this patient. Collection of patient data and all protected patient health information was compliant with the Health Insurance Portability and Accountability Act.

The authors report 2 cases of apocrine cystadenoma of the eyelid, 1 of which was studied with immunohistochemical and other special stains. While a previous report describes the tumor in a palpebro-orbital location, no other detailed descriptions of a purely eyelid location are present in the literature. Apocrine cystadenoma occupies an unusual portion of the spectrum of Moll gland tumors of the eyelid. It has no definitive clinical characteristics and may be diagnosed pathologically by hematoxylin-eosin-stained sections.

A 37-year-old woman underwent excision of a congenital melanocytic nevus of the right lower eyelid margin for cosmetic reasons. She suffered from a severe dry eye syndrome which was partially alleviated with various topical eye drops. Histopathology of the excised tissue displayed flecks of calcium that were present within the immediate subepithelial conjunctival fibrous tissue but not the nevocytes or the cutaneous surface. Unlike calcinosis cutis, wherein calcium is deposited within the cutaneous dermis, calcification of the conjunctival substantia propria is rare and may be related to the dry eye state.

The authors describe an esthesioneuroblastoma (olfactory neuroblastoma) that occurred within the nasal cavity and brain in a 31-year-old man. Following excision, the tumor recurred in the left orbit and in mediastinal lymph nodes. Treatment included orbital excision and systemic chemotherapy. Histopathology showed a high-grade neuroepithelial tumor with positive immunohistochemical markers for neuroendocrine and epithelial components, an unusual combination raising issues concerning taxonomy.

A 74-year-old man presenting with proptosis and orbital inflammation was found on magnetic resonance imaging to have a unilateral intraconal mass. Biopsy revealed a high-grade malignant tumor that was interpreted as squamous cell carcinoma. Positron emission tomography-computed tomography imaging subsequently identified a primary lesion in the esophagus. Esophageal squamous cell carcinoma is a rare cause of orbital metastasis, with only 4 previously reported cases. The authors discuss an approach to orbital malignancies of obscure origin.

Endocrine mucin-producing sweat gland carcinoma, a rare, low-grade neoplasm with predilection for the eyelids, has been posited as a precursor to invasive mucinous adenocarcinoma. Endocrine mucin-producing sweat gland carcinoma and its concurrence with mucinous adenocarcinoma have received little attention in the ophthalmic literature. The combination of the 2 histologic patterns parallels endocrine ductal carcinoma in situ of the breast and its transition to Type B invasive mucinous carcinoma. The authors describe a 59-year-old man who developed a tumor of the right upper eyelid showing endocrine mucin-producing sweat gland carcinoma in the outer dermis and extensive mucinous carcinoma in the deeper tissue. Immunohistochemical analysis showed positivity for endocrine markers chromogranin, synaptophysin, CD56, estrogen, and progesterone in each histologic component of the tumor. This research was conducted in conformity with the Helsinki Declaration and HIPPA regulations.

A flesh-colored, supraciliary lesion of the left upper eyelid in a 47-year-old man was excised for cosmetic reasons. Histopathology and immunohistochemistry demonstrated CD34-positive benign spindle cells, factor XIIIa-positive dendritic cells, and CD163-positive histiocytes, all dispersed within a diffuse collagenous background. Prominent loose perivascular cuffs of fibroblastic cells and collagen surrounded small blood vessels. Interpreted as an angiofibroma, the histopathology bore resemblance to that of a single previously-reported forearm lesion termed a "dermal fibroma with a distinctive perivascular cell arrangement." The lesion represents the first eyelid example of an unusual variant of angiofibroma.

The authors describe an isolated, yellowish papular lesion of the upper eyelid in a 63-year-old man. Following excision, histopathologic analysis showed the features of a benign hypopigmented cellular blue nevus, the first and only case involving the eyelid skin.

The authors describe a rapidly enlarging, pedunculated brown tarsal lesion in a 34-year-old man with a history of chalazia. Following excision, histopathologic analysis showed the features of a necrotic pyogenic granuloma. This unique case expands the differential diagnosis of conjunctival malignant melanoma.

The authors describe a spindle cell lipoma that occurred in the anterior lamellae of the eyelid in a 47-year-old man. This benign tumor was reported previously only once within that location and poses histologic challenges when a “low fat” or “fat free” variant is present.

Purpose To describe a case series of ointment granuloma as a complication of sutureless transconjunctival blepharoplasty. Methods A retrospective review of the medical records of 8 patients with this complication was conducted, including the histopathology of excised tissues. Institutional review board oversight was waived as this was not an institutional study but a Health Insurance Portability and Accountability Act-compliant retrospective chart review from the private medical practice of one of authors' coauthors. It adhered to the principles set forth in the Declaration of Helsinki. Results Eight patients developed painless lower eyelid and anterior orbital masses following presumed successful blepharoplasty. Each had received intra- or immediate postoperative lubricating ointment. The mean time from surgery to appearance of the lesions was 50 days (range: 9 days-10 months). Three patients responded to intralesional injection of steroid with complete resolution. Five patients required surgical excision of the lesions without recurrence to date. Histopathological examination of the excised tissues revealed large, pleomorphic lipid-dropout pools bordered by attenuated histiocytes and giant cells. Conclusions The appearance of eyelid lumps or fullness in the early and mid-postoperative recovery should suggest ointment granuloma. If recognized early, management should commence with intralesional injection of steroids, although the definitive treatment is surgical excision. The authors recommend minimizing the access of topical ointments to the open inferior fornix, placing the medication sparingly onto the cornea at the close of surgery and instructing patients in avoiding overuse.

A 54-year-old African-American woman developed a pigmented papillary squamous cell carcinoma in the palpebral and bulbar conjunctiva of the right eye in areas that received no sun exposure. In situ hybridization performed on the tumor showed human papilloma virus 16. The left eye showed a pedunculated nonpigmented conjunctival dysplasia. The tumors were extirpated by cryosurgery and topical interferon alfa 2b in the right eye and simple surgical excision in the left eye.

Background: Surgical implantation of the intraocular sustained-release ganciclovir device is a safe and effective treatment for cytomegalovirus (CMV) retinitis. Previous histopathologic studies on eyes containing such implants have been limited by the necessity of removing the device before processing. Microtome sectioning of hard plastics within paraffin-embedded blocks is infeasible, and the anatomic relations of implant to eye are destroyed. Methods: The authors studied four eyes from three patients who had undergone implant insertion. Globes with implants in place were fixed in neutral 10% formalin, embedded in methylmethacrylate, sectioned on a special microtome, and stained with hematoxylin-eosin. Results: After methacrylate embedding, the precise anatomic relations of the implant to the neighboring uveoscleral coats were preserved. In two eyes, the suture tab of the implant protruded through the sclera, exiting subconjunctivally. In two eyes, the implant was totally intravitreal. In all patients, the device was supported by fibrous tissue which emanated from a surgical coloboma of the pars plana ciliaris. Focal granulomatous inflammation adjoined suture and implant materials but no other inflammation or deleterious effects on the ocular structures were noted. Conclusion: This report is the first to document the intraocular histopathology of the ganciclovir implant. The subconjunctival location, enhancing the potential for endophthalmitis, may be avoided by trimming of the suture tab close to the anchoring suture and not tying it too tightly. Methylmethacrylate embedding is a useful technique for preserving the microanatomy of intraocular implants.

A case of intraosseous orbital hemangioma is reported to alert surgeons to possible intraoperative hemorrhage during excision of such a lesion. A slowly enlarging mass was excised from the orbital rim of a 49-year-old woman. The clinical diagnosis was not suspected. In retrospect, roentgenographic findings included a focal honeycombed pattern of the zygomatic bone. Surgery was complicated by persistent low-volume bleeding. Histology showed endotheliallined blood-filled channels within the bone. Intraosseous orbital hemangioma is a rare, benign neoplasm that can often be diagnosed clinically by characteristic roentgenographic findings. Observation should be considered as a therapeutic alternative when the radiographic diagnosis is established and when ocular function is not compromised. [Ophthalmic Surg Lasers 2002;33:326-328]

A 24-year-old man developed abdominal carcinomatosis from signet cell carcinoma of the rectum. His only distal metastasis involved the superior orbit. Orbital pathology showed signet cells with a characteristic immunopathologic pattern. No hereditary syndrome was found. The authors identified only 5 cases in the literature describing colorectal adenocarcinoma metastatic to the orbit, with 2 showing histopathology. The authors believe that this rare case represents the first illustrating bona fide signet cell colorectal cancer involving the orbit.

This is the first known report of a relatively large postoperative pyogenic granuloma developing after a nonsutured transconjunctival blepharoplasty. Inflammation and separation or malapposition of the conjunctival wound edges probably permitted the lesion to proliferate in the inferior fornix. No foreign material could be implicated because no suture was used to close this incision. Additionally, Polydek suture material (braided polyester fiber) was associated with the complication of a suture tract and granuloma when used for a tarsal suspension procedure for ectropion repair in this patient.

Background: While molluscum contagiosum of the eyelid skin is commonly complicated by conjunctivitis, primary involvement of the conjunctiva or cornea by molluscum lesions is exceedingly rare. The authors studied a 34-year-old man with acquired immune deficiency syndrome (AIDS) in whom multiple molluscum lesions of the lids and a single nodule of the limbus developed. Methods: The nodular lesion was excised from the limbus and processed for histologic examination by light microscopy. Pertinent literature concerning epibulbar molluscum contagiosum was reviewed. Results: Histopathology of the excised lesion showed molluscum bodies within the acanthotic, hyperkeratotic conjunctival epithelium with surrounding chronic, nongranulomatous inflammation. Only 10 previous cases of conjunctival or corneal primary lesions have been reported, half of which showed associated cutaneous involvement. Lesions tended to be single, noninflamed, dome-shaped, and umbilicated, often with a yellowish central core. Patients were otherwise well and ranged in age from 3 to 55 years. Simple excision was effective in eradicating the lesions. Conclusion: Primary epibulbar molluscum contagiosum is rare. Although cutaneous molluscum is common in AIDS, this report is the first to document conjunctival molluscum in a patient with AIDS.