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Purpose: To review the clinical radiographic and histopathologic findings associated with orbital glial heterotopia., Methods: A literature search in PubMed and Scopus was performed to include all articles published in English between 1980 and January 1, 2019. A case series including 29 case reports of 29 patients, as well as the authors' 2 cases, were considered in the literature review., Results: The majority of the cases had onset of symptoms (86%) and age at presentation (71%) before 5 years of age. The most common presenting symptoms and signs were swelling (45%), strabismus (32%), and proptosis (26%). The most common lesion locations described were inferolateral (19%) or primarily posterior orbital or apical (19%). The most common findings associated with orbital glial heterotopia were microphthalmia (10%) and anophthalmia (6%); however, the majority did not have systemic abnormalities (71%). The most common imaging modality was CT scan (71%). Diagnosis was made with histologic analysis in all cases, and confirmed after subtotal resection (35%), total resection (39%), or incisional biopsy (26%). The majority of the cases report no growth on repeat imaging, with only 3 reports of recurrence., Conclusions: Glial heterotopia in the orbit is a rare clinical entity most commonly presenting in children. We present 2 cases of orbital glial heterotopia in adults, with a literature on these lesions in both the pediatric and adult populations. Surgeons and pathologists should be aware of this atypical presentation in adulthood. Biopsy is required for diagnosis but is not without risk. Prognosis is generally favorable.Orbital glial heterotopia, commonly considered a rare congenital lesion generally presenting in children, may first become symptomatic in adulthood. Biopsy is required for diagnosis, with symptoms and prognosis dependent on location and growth of the lesion.

A rare variant of adenoid cystic carcinoma is the dedifferentiated sarcomatoid form, which has previously been reported in the hard and soft palate, maxillary sinus, submandibular glands, and nasal cavity. The authors report the first case of a dedifferentiated sarcomatoid adenoid cystic carcinoma occurring in the lacrimal gland, that of a 52-year-old man. The patient presented with a 4-month history of diplopia, decreased vision, and right upper eyelid swelling. Radiographic imaging showed a soft tissue mass in the extraconal compartment superolateral to the right eye. The patient subsequently underwent surgical debulking. Histologic examination of the tissue revealed classic cribiform adenoid cystic carcinoma and a sarcomatous component consisting of malignant spindle cells and fusiform cells arranged in whorls. Dedifferentiation is a well-established phenomenon in salivary gland tumors that is associated with aggressive behavior and poor prognosis; however, the exact nature of such dedifferentiated neoplasms remains unclear.

A 70-year-old woman presented with a slow-growing right lower eyelid lesion. Histopathologic and immunohistochemical evaluation of an excisional biopsy revealed a neuroendocrine neoplasm, consistent with a typical carcinoid tumor. Systemic investigation for occult visceral primary tumor was negative. Re-excision of the lesion for free surgical margins was performed. Eight months later, the patient remains well with no evidence of other primary malignancy or metastatic disease.

Purpose: To describe 6 patients with hybrid cysts of the eyelid that demonstrated follicular and apocrine differentiation., Methods: Clinicopathologic series of 6 cases obtained from review of 1,099 cases of hidrocystoma diagnosed at the New York Eye and Ear Infirmary from 1990 to 2006., Results: Six patients, aged 38 years to 85 years (mean 66.5 years), was examined for cystic eyelid lesions. Histopathologic evaluation revealed cystic structures, lined by a combination of apocrine, infundibular (epidermoid), and trichilemmal-type epithelia. Luminal contents consisted of variable amounts of lamellated and compact keratin, and serous material. Contiguity with a hair follicle was demonstrated in 4 of 6 lesions. Immunohistochemical evaluation showed intense immunoreactivity of the entire cyst lining in all lesions for high molecular weight cytokeratin and variable immunoreactivity for low molecular weight cytokeratin. All cystic structures displayed immunoreactivity for carcinoembryonic antigen. Weak focal immunoreactivity for S-100 protein and gross cystic disease fluid protein-15 was observed in most lesions., Conclusions: Hybrid cysts can show apocrine, trichilemmal, and infundibular differentiation, suggestive of their origin at the junction of keratinizing squamous and glandular epithelia of the hair follicle. Although, to our knowledge, only 1 such lesion has been described in the eyelid skin, it is likely that hybrid cysts of the eyelid are not an infrequent finding.