Miniature Palpebral Plexiform Neurofibroma in Neurofibromatosis Type 2.

A 27-year-old woman with well-documented neurofibromatosis 2 developed a soft, painless, nodular lesion on the skin surface of the left upper eyelid over 2 years. Following excision, histopathology revealed a plexiform neurofibroma with intradermal nodules comprised of benign round and spindle cells that reacted diffusely with immunohistochemical stains SOX-10 and S100. A subset showed focal reactivity for neurofilament and CD34. A perineurium surrounded each nodule with cells staining positively for markers EMA (epithelial membrane antigen) and GLUT1 (glucose transporter 1). Plexiform neurofibromas are rare tumors that occur in 5%-15% of patients with neurofibromatosis 1. Cutaneous abnormalities in neurofibromatosis 2 have not been widely studied although reports have described schwannomas, plexiform schwannomas, and occasional neurofibromas. Plexiform neurofibromas in neurofibromatosis 2 have rarely been illustrated and the current case represents a unique bona fide eyelid example to date.

Apocrine Cystadenoma of the Eyelid, a Rare Neoplasm: Expanded Immunohistologic Profile.

An 83-year-old woman experienced the slow enlargement of a right lower eyelid mass. Histopathologic examination of the excised tissue showed a mucin-filled cystic tumor emanating from an apocrine bilayer that displayed bleb-like apocrine decapitation secretion. The outer flattened myoepithelial layer of the bilayer reacted with immunohistochemical stains for smooth muscle actin and calponin. In foci, the tumor exhibited a cribriform architecture with small pockets of mucin. Tumor cells were reactive for cytokeratin 7, Gross Cystic Disease Fluid Protein 15 (BRST-2), estrogen and progesterone receptors, androgen receptors, mammaglobin, epithelial membrane antigen, and GATA3. Ki67 showed a very low proliferation fraction. The lesion exemplifies the fourth instance of an eyelid apocrine cystadenoma in the literature.

Eccrine Cyst (Hidrocystoma) of the Inner Canthus: A Rare Entity With Immunohistologic Confirmation.

A small ruptured cyst was excised from the left inner canthus of a 68-year-old woman. Histopathology displayed a cyst lined by a double layer of cuboidal epithelium and filled with amorphous material. The lining cells were strongly positive for cytokeratins 5/6 and 14, with weak reactivity with cytokeratin 7. These findings were identical to those in a single previous report of an eccrine cyst of the eyelid, making this the second example of a bona fide eccrine hidrocystoma of the eyelid.

Pigmented Inflamed Seborrheic Keratosis of the Bulbar Conjunctiva.

A 57-year-old Black man presented with the recent onset of a pigmented temporal epibulbar lesion. As pigmentation of conjunctival epithelial lesions is correlated with complexion pigmentation, the lesion was presumed to represent a pigmented ocular surface squamous neoplasia (OSSN). Excisional biopsy, however, revealed a pigmented conjunctival seborrheic keratosis, a rare occurrence. The lesion lacked cytologic atypia. Intralesional processes of dendritic melanocytes were demonstrated by hematoxylin-eosin and Melan-A stains. Melanophages also contributed to clinical pigmentation. Subepithelial lymphocytic infiltration, elevated Ki67 proliferative rate, prominent mitotic activity, and subtle spongiosis afforded evidence of inflammation rather than malignancy in a lesion devoid of cytologic atypia.

Solitary Intratarsal Blue Nevus.

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.

Episcleral Apocrine Hidrocystoma Following Strabismus Surgery.

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.

Targeted Neoadjuvant Intra-arterial Chemotherapy in Lacrimal Gland Adenoid Cystic Carcinoma: A Histological Correlation Using Apoptotic Tumor Markers.

Neoadjuvant intra-arterial cytoreductive chemotherapy is used for the treatment of lacrimal gland adenoid cystic carcinomas (ACC) to improve outcomes in this condition with an otherwise dismal prognosis. We share our experience in the management of an advanced case of ACC using a novel, highly targeted intra-arterial cytoreductive chemotherapy delivery technique involving both the internal and external carotid circulation, with an attempt to correlate the effect histologically. Refinement of the chemotherapy delivery using the tumor's vascular anatomy and appropriate blood vessel selection may lead to future globe sparing procedures without compromising survival.

Conjunctival Exophytic Schneiderian-type Papillomas: A Rare Occurrence.

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.

Epibulbar Subconjunctival Apocrine Hidrocystoma.

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.

Osseous Metaplasia of the Eyelid in a Child.

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.

Myositis of an Extraocular Muscle, a Possible Drug Reaction: Histopathologic and Immunopathologic Analysis.

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.

Multicameral Steatocystoma Simplex of the Caruncle.

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.

Orbital and Eyelid Inflammation With "Muciphages" and Extravasated Mucin From an Ethmoido-orbital Mucocele.

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.

Pseudopemphigoid: Sarcoidosis presenting as cicatricial conjunctivitis with symblepharon.

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.

Ossifying Pilomatrixoma of the Eyelid.

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 ß-catenin, probably linking the tumor to a mutation in the ß-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.

Keratinous Cyst of the Caruncle Developing From a Sebaceous Gland Duct: Case Report With Immunohistochemical Analysis.

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.

Fibrous Dysplasia-like Lacrimal Sac Tumor Associated With Dacryocystitis.

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.

Idiopathic Conjunctival Calcinosis Associated With an Intradermal and Subepithelial Nevus of the Eyelid Margin.

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.