To Freeze or Not to Freeze? Recommendations for Intraoperative Examination and Gross Prosection of Thyroid Glands.

The use of intraoperative consultation for indeterminate thyroid lesions is not advocated but is still requested by some surgeons. Obscured cytomorphology and nonrepresentative sampling limit the specificity of intraoperative assessment. Formalin fixation of thyroid glands before sectioning also minimizes artifacts introduced by fresh sectioning. Inking of thyroid may vary based on institutional preferences and information desired by clinical teams. Sectioning may occur in the conventional transverse method or the modified transverse vertical method to more thoroughly evaluate the lesion's periphery. Gross examination of thyroid lesions should always consider possible high-grade features, such as necrosis or extrathyroidal extension.

Characteristics of Pelvic Cytology in Patients with Gynecologic Malignancies: Comparison of Pre and Post-Neoadjuvant Specimens.

INTRODUCTION: The presence of tumor cells in pelvic cytology (PC) specimens can portend a worse outcome for patients undergoing gynecologic surgery. Primary debulking surgery (PDS) was a mainstay for most of these tumors; however, recent advances have triaged selected patients to neoadjuvant chemotherapy (NACT) with interval debulking surgery (IDS). Reduction in tumor cellularity and histologic alterations has been noted in these cases; however, similar cytologic characterization has not been performed. MATERIALS AND METHODS: PC was searched to find those in NACT patients. Additional PDS were included as controls. Cases were scored for cellularity of malignant cells and background components were described, and when available, pretreatment and posttreatment specimens from the same patients were compared. RESULTS: In all, 19 specimens from 16 patients were found, 6 (32%) of which were paired PTS and IDS from the same patient. Only 6/19 (32%) were from IDS, the remainder PTS. A majority (15/19; 79%) of specimens were malignant; all negative cases were PTS. Few (4/16; 24%) were endometrial primaries; the remainder were pelvic high-grade serous carcinoma. No difference in tumor cell morphology or inflammatory component was noted between the 2 groups, though in 3/3 paired specimens from PDS and IDS, the cellularity of malignant cells decreased in the IDS specimens. DISCUSSION/CONCLUSION: No identifiable trend was noted regarding cellularity of specimens in the pre compared to the post-neoadjuvant setting. A trend toward reduced cellularity was noted in individual patients, but no alteration in background cells or tumor morphology was noted.

Paracicatricial melanocytes as a sign of melanoma.

Intradermal melanocytes in the setting of melanoma represent a diagnostic challenge to dermatopathologists as their presence may represent superficially invasive melanoma vs benign nevus cells or reactive dermal melanocytes. Previous dermatologic literature suggests that the absence of cytologic atypia in intradermal melanocytes and their presence in nonmelanocytic neoplasms lends to their characterization as reactive, benign, melanocytic proliferation. A 67-year-old female presented for evaluation of a 10-mm irregularly pigmented dark brown macule on the left cheek. Initial shave biopsy showed transected malignant melanoma measuring at least 0.6 mm in thickness. Multiple reexcision specimens demonstrated residual melanoma with banal appearing intradermal epithelioid melanocytes within and surrounding the scar. The melanocytes tracked into the skin graft, which had previously been free from involvement. Positron emission tomography-computed tomography (PET CT) and lymph node biopsies did not show evidence of metastatic melanoma. Ten months after her diagnosis and following five surgical excisions, the patient was diagnosed with metastatic melanoma to the brain and succumbed to intracranial hemorrhage. We present a case in which paracicatricial melanoma may simulate benign paracicatricial melanocytic hyperplasia. These findings have significant therapeutic and prognostic implications for the practicing dermatologist and dermatopathologist.

Myeloid Sarcoma with Megakaryoblastic Differentiation Arising in the Conjunctiva.

An 87-year-old woman not known to have either a lymphoma or leukemia developed a left multinodular, fish-flesh superior epibulbar and forniceal mass. A biopsy disclosed a blastic tumor with scattered multinucleated immature megakaryoblasts. Immunophenotyping of bone marrow cells revealed strong positivity for CD7, CD31, CD43, CD45, CD61, and CD117; CD71, myeloperoxidase, and lysozyme were also positive in scattered cells. Forty percent of the neoplastic cells were Ki-67 positive. Cytogenetic studies indicated a trisomy 8 (associated with worse prognosis) and a t(12; 17) translocation. Desmin, smooth muscle actin, pancytokeratin, CAM 5.2, adipophilin, tryptase, S100, SOX10, MART1, and E-cadherin were negative, ruling out a nonhematopoietic tumor. The conjunctival lesion was diagnosed as a myeloid sarcoma with megakaryoblastic differentiation, a rare variant. It probably arose from a myelodysplastic syndrome. This is the first case of its type to develop in the conjunctiva.

A Case Report of Basal Cell Carcinoma in a Non-Sun-Exposed Area: A Rare Presentation Mimicking Recurrent Perianal Abscess.

Basal cell carcinoma (BCC), a common malignancy, arises most often in sun-exposed areas but does rarely occur in non-sun-exposed sites. Prior tissue injury, especially sharp trauma and chronic inflammation, increases the risk of BCC. We describe a 66-year-old male patient with recurrent perianal abscesses who was found to have a large pigmented basal cell carcinoma. The mass was excised without recurrence at two-year follow-up. Perianal BCC is commonly larger at the time of diagnosis than tumors in sun-exposed sites, likely related to delay in diagnosis. Increased size can lead to increased surgical complexity and more pronounced effects on nearby structures. Early detection is important for optimal patient outcomes. In selected patients presenting with a perianal mass, basal cell carcinoma should be included on the differential diagnosis.

Clinicopathologic and Magnetic Resonance Imaging Analysis of a Multifocal Orbital Lymphoid Tumor.

OBJECTIVE: To distinguish between a multifocal orbital lymphoid tumor and a major simulator represented by a diffuse lymphaticovenous malformation. METHODS: We performed a comparison of clinical and radiographic (magnetic resonance imaging [MRI]) findings of these two disparate entities and demonstrated how a misdiagnosis can be prevented. RESULTS: Orbital lymphoid tumors develop in adults at around 60 years of age, whereas extensive lymphaticovenous malformations are generally detected in the first decade. Despite these differences, this is the first description of clinical confusion between them. MRI with gadolinium injection in the current lymphoid tumor displayed a low signal on T2-weighted images, rapid and uniform enhancement, and reduced diffusion. Lymphaticovenous malformations are heterogeneous, display poor or only focal perfusion, and fail to exhibit diminished diffusion. Newer techniques such as diffusion-weighted imaging and dynamic contrast-enhanced imaging may be able to provide additional differential diagnostic information. The final pathologic diagnosis was an extranodal marginal zone lymphoma. CONCLUSIONS: Despite the obvious distinctions between orbital lymphoid tumors and lymphaticovenous malformations, several clinical radiologic specialists misdiagnosed the present orbital lesion as a vascular lesion. A combined clinicoradiographic analysis should obviate such errors and facilitate the correct diagnosis in the future.

Early Neuroblastic and Astrocytic Differentiation Demonstrated Immunohistochemically in a Small Intraocular Medulloepithelioma.

PURPOSE: To investigate the source of fibrous astrocytes and neuroblasts in a small ciliary body medulloepithelioma appearing as a leukocoria in a 3-week-old baby girl. METHODS: Histopathologic and immunohistochemical studies included Alcian blue, periodic acid-Schiff, and antisera for the detection of S100 protein, CD99, glial fibrillary acidic protein (GFAP), CRX, NeuN, neurofilaments, synaptophysin, desmin, and myogenin. RESULTS: A small, nonteratoid ciliary body medulloepithelioma with collections of Alcian blue+ mucoplysaccharides was present in the enucleated globe. The retinal mass displayed multilaminar dysplastic rosettes that were CRX+, NeuN-, and synaptophysin-. Intraretinal neurofilaments and scattered NeuN+ neurocytes were also identified. At the base of the retinal mass ribbons and pseudopapillae of CRX+, NeuN- medullary epithelium were found. The latter developed from an S100+ and weakly CD99+ monolayer of premedullary epithelium. GFAP+ fibrous astrocytes and NeuN- neuroblasts streamed from the medullary epithelium. CONCLUSIONS: A multilaminar medullary epithelium and a precursor monolayer of premedullary epithelium were both identified. Neuroblasts and fibrous astrocytes were determined to arise separately from the medullary epithelium. The early stage of tumorigenesis afforded by a small tumor provided the opportunity to discover morphologic and immunohistochemical evidence for these differentiations.

Ellipsoid Smooth Muscle Tumor of the Lower Eyelid: An Exploration of Its Possible Origin.

Ocular adnexal smooth muscle masses/neoplasms are extremely rare. Such lesions are comparatively more common in the conjunctiva than in the orbit and are most unusual in the eyelid. A 58-year-old woman slowly developed over 4 months a firm, movable sausage-shaped lesion in the deep lateral half of the right lower eyelid. The lesion ran parallel to and above the orbital rim. At surgery, the lesion was located between the orbicularis muscle and the inferior orbital septum. The term ellipsoid is used descriptively and does not imply any particular biologic behavior. Immunohistochemical evaluation revealed smooth muscle actin and desmin positivity. Due to the ubiquity of small blood vessels and the absence of smooth muscle bundles in the potential space between the orbicularis striated muscle and the inferior orbital septum, venular smooth muscle emerges as a highly likely source for the lesion.

A Lacrimal Gland Choristoma of the Lacrimal Sac.

Choristomatous lacrimal gland tissue has been detected in many different sites of the ocular adnexa, but has never before been convincingly described in the submucosa of the lacrimal sac. A 77-year-old woman with epiphora had a biopsy of the sac wall preformed during a dacryocystorhinostomy that contained such a lacrimal choristoma. Zymogen granules were found in the cytoplasm of the secretory cells with the periodic acid-Schiff reaction. No mucus-producing cells, as found in normal sac submucosal glands, were detected using the Alcian blue, mucicarmine, and Gomori methenamine silver histochemical stains. Gross cystic fluid protein-15 positivity was demonstrated immunohistochemically. The clinical implications of this choristoma are explored.

Bilateral upper and lower eyelid margin swelling and madarosis due to lymphoma.

Over a 2-year period, swellings of all 4 eyelid margins developed in a 32-year-old woman and was accompanied by complete loss of eyelashes. An inflammatory dermatologic condition was considered the most likely cause. A full-thickness right lower eyelid biopsy revealed a multinodular lymphoid tumor at the eyelid margin which immunophenotypically and genetically was diagnosed as an extranodal marginal zone lymphoma. The mode of presentation of the disease was considered to be most unusual, as was its B cell lineage, since the majority of primary cutaneous lymphomas are of T-cell origin. Systemic workup demonstrated bilateral involvement of the external auditory canals.

Limbal Cysts: A Subset Exhibiting Cornea-Specific Cytokeratins.

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.

Exophytic Osteochondroma of the Brow.

Most bony and cartilaginous lesions of the orbit and periorbital compartments are benign, grow endophytically, and are composed of dense lamellar bone (eburnated or ivory osteomas). An 87-year-old woman had a well-circumscribed, firm, round, and exophytic lesion of the brow region for at least 15 years. Excisional surgery disclosed an osteocartilaginous lesion with an enveloping pseudocapsule (periosteum/perichondrium) and a narrow stalk connecting it to the frontal bone. The periphery of the lesion displayed lamellar bone which appeared to be replacing a central cartilaginous zone. The adjacent deep preaponeurotic fat displayed nodules of collagen with myxoid change and occasional CD34 spindle cells suggestive of a spindle cell lipoma. Because of the osteochondroma's deep location in the preaponeurotic fat, the lesion differs from an osteoma cutis found in the dermis which fails to exhibit a cartilaginous component or a periosteum. Other clinically simulating lesions are described.

Conjunctival Squamous Cell Neoplasia Associated With Ocular Cicatricial Pemphigoid.

The purpose of this study was to describe a possible causal relationship between ocular cicatricial pemphigoid (OCP) and ocular surface squamous neoplasia. Two middle-aged female patients with already diagnosed OCP were studied in regard to the subsequent onset of conjunctival squamous neoplasia. Their clinical histories, ocular examinations, clinical photographs, and results of biopsies and any ancillary immunofluorescent laboratory evaluation studies were carefully reviewed. One patient had a preinvasive squamous dysplasia and the other an invasive squamous cell carcinoma, both in the unequivocal setting of OCP with bilateral conjunctivitis, symblephara, and forniceal foreshortening. The patients had been receiving intensive immunotherapy consisting of some combination of corticosteroids, rituximab, and interferon alpha. Both patients had a positive immunofluorescent study demonstrating immunoreactants at the level of the epithelial basement membrane. Each patient had 2 earlier negative immunofluorescent studies before a third was positive. While rare, there is 1 previous report of an association between OCP and conjunctival squamous neoplasia. The current report provides more data supporting the proposal that this conjunction is more than a random event. Repeat immunofluorescent studies after an initial negative result in a patient with strong clinical signs of OCP are imperative due to the frequency of false negative studies in the context of clinically persuasive disease.

Dacryoadenitis as the Initial Presentation of a Natural Killer T-Cell Lymphoma.

Primary orbital natural killer T-cell lymphoma (NKTCL) is a rare condition with only a few published cases in the literature. Over 1 month, an 81-year-old man developed progressive left periocular inflammation unresponsive to treatment. Clinical examination and imaging studies demonstrated a left lacrimal gland enlargement. Bilateral anterior uveitis and erythematous nontender cutaneous lesions were also found. Biopsies of the skin and lacrimal gland on the back revealed histopathologic and immunohistochemical findings confirming Epstein-Barr virus-positive NKTCL. Metastatic work up disclosed multifocal involvement in the pancreas, stomach, and chest wall. Palliative treatment consisting of nonanthracycline-based chemotherapy and radiation was instituted, but the patient died 5 months after the onset of symptoms. This is the first example of acute dacryoadenitis, and the second of bilateral anterior uveitis, in the setting of NKTCL. Absence of naso-sinus involvement in the current case is rare in NKTCL. Despite treatment, the prognosis remains dismal. Orbital specialists should include NKTCL in the differential diagnosis of lacrimal gland/orbital masses and perform an incisional biopsy if the clinical scenario so dictates.

Dermatofibroma of the Eyelid: Immunohistochemical Diagnosis.

A 66-year-old man developed a painless 2 mm to 3 mm recurrent nodule at the left upper eyelid margin. Excision disclosed a spindle cell lesion without frank atypia or mitotic activity growing in a twisted fascicular pattern often referred to as storiform. All the surgical margins were involved with tumor. Immunohistochemistry demonstrated that many of the constituent spindle and dendritic tumor cells were CD34, factor XIIIa, and CD 163, the latter 2 being biomarkers for monocytic lineage. The lesion was diagnosed as a dermatofibroma rather than a fibrous histiocytoma, a term that should be reserved for more aggressive lesions of deeper fascial planes. Facial dermatofibromas are rarer and more likely than those of the extremities to recur and therefore deserve wider local excision at first surgery with careful and frequent clinical follow ups. Eyelid dermatofibroma has probably often been misdiagnosed as another tumor in the past. Immunohistochemistry can supply valuable biomarker criteria for diagnosis.

Eyelid Eccrine Cyst: An Exceptional Lesion Among Dominant Apocrine Cysts.

A 71-year-old woman developed a small bluish lesion beneath the cilia of the left lower eyelid. Excision and microscopic examination disclosed a cyst with an intimately associated eccrine sweat gland. Immunohistochemistry demonstrated that the cyst's epithelium was strongly CK5/6, CK14, CK7 weakly positive, and gross cystic disease fluid protein-15 and smooth muscle actin negative. This is the first immunohistochemically proven eccrine cyst of the eyelid skin. Apocrine cysts develop only at the eyelid margin where the glands of Moll are located. They immunostain positively for cytoplasmic gross cystic disease fluid protein-15 in the adlumenal cells and smooth muscle actin in an outer myoepithelial (abluminal) layer.

Unsuspected Conjunctival Orbital Dermoid Cyst: Aids in Diagnosis.

A 25-year-old man with Type 1 diabetes mellitus experienced rapid visual decline and was scheduled for right cataract surgery. At the time of administering an inferotemporal retrobulbar block, a white discharge appeared spontaneously on the surface of the globe. Superotemporally a cyst was found and its contents were subtotally evacuated. Microscopically, eosinophilic, acellular material with chatter artifact and small vacuoles was detected and initially thought to represent a lens choristoma. This material stained moderately with the periodic acid Schiff method and was focally Congo red positive without apple green birefringence; it also stained blue with the Masson trichrome method. Acid-fast staining disclosed the presence of rare vellous hairs. Adherent cells were not epidermal cells (CK5/6) but conjunctival epithelial cells (CK7); CD68-positive histiocytes were also identified. The lesion was diagnosed as a disrupted orbital dermoid cyst of conjunctival origin.

Dermatofibroma of the eyelid with monster cells.

Dermatofibromas are most frequently encountered in women on the lower extremities, often after minor trauma. A recurrent lesion of the right lower eyelid developed in a 64-year-old woman. It harbored "monster cells" that were large, with either multiple nuclei or a single, large, convoluted, and hyperchromatic nucleus. The presence of these cells does not signify a malignant transformation. The background cells were either histiocytoid (many were adipophilin positive), spindled cells, or dendritiform cells without mitoses. Factor XIIIa, CD68, and CD163 immunostaining was positive, and a subpopulation of CD1a+ Langerhans cells was intermixed. Facial and eyelid dermatofibromas are more likely to recur and deserve wider, tumor-free surgical margins. Their microscopic differential diagnosis includes a cellular scar, peripheral nerve tumor, atypical fibrous xanthoma, and dermatofibrosarcoma protuberans.

ATTENUATION OF THE GANGLION CELL LAYER IN A PREMATURE INFANT REVEALED WITH HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: To report on subclinical retinal abnormalities shown through handheld spectral domain optical coherence tomography on a premature infant. METHODS: Case report. RESULTS: The initial and follow-up exams on a premature infant revealed severely attenuated ganglion cell and nerve fiber layers. There was cystoid macular edema in both eyes at the initial visits, which resolved by the 1-year follow-up. DISCUSSION: Optical coherence tomography can reveal significant retinal abnormalities in premature infants which are not detectable through funduscopic exam. Documenting such findings may be useful for the comprehensive management of vision problems in children with a history of premature birth.