Myoepithelioma of the Lower Eyelid.

A 59-year-old woman presented with a 4-year history of a progressively growing, subcutaneous, painless, left lower eyelid mass with associated swelling and discomfort. Excisional biopsy established the diagnosis of a myoepithelioma, a tumor which, to the authors' knowledge, has not been previously reported in the lower eyelid.

Orbital Lymphangioma Confined to an Extraocular Muscle.

A 48-year-old woman presented with a 1-year history of an enlarging, nontender right lateral intrapalpebral mass overlying the insertion of the right lateral rectus muscle, associated with a 3-month history of worsening right orbital pain and retrobulbar headaches. MR imaging revealed an isointense right orbital mass contained entirely within the right lateral rectus muscle and indistinguishable from it. Transconjunctival biopsy established the diagnosis of lymphangioma. To the authors' knowledge, this case represents the first reported orbital lymphangioma contained entirely within an extraocular muscle.

Lacrimal Gland Ductal Cyst Complicated by Abscess Formation.

A 52-year-old woman with a 7-month history of a left lateral upper eyelid nodule presented with an acute, 1-day history of left upper eyelid swelling and erythema associated with chemosis, epiphora, and pain. Magnetic resonance (MR) imaging revealed a well-circumscribed cystic mass indistinguishable from the left lacrimal gland containing a fluid-fluid level. Surgery confirmed an abscess within the lacrimal gland; histopathologic examination revealed a dilated lacrimal duct with an inflammatory exudate within the lumen. This report details one of the few reported cases of an acute lacrimal gland ductal cyst abscess and, to the authors' knowledge, the first with MR images.

Intravascular papillary endothelial hyperplasia of the periocular region.

Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion, typically found in the skin, consisting of a papillary proliferation of endothelial cells intimately related to a thrombus; it appears to be a rare variant of an organizing process. IPEH very rarely develops in the periocular region, with fewer than 20 such cases currently reported in the English language literature. We describe one of the largest ever case series of this uncommon lesion occurring in the periocular region and discuss its clinical features, imaging characteristics, and surgical management.

Orbital cellulitis, sinusitis and intracranial abnormalities in two adolescents with COVID-19.

We review two cases of adolescents with orbital cellulitis, sinusitis and SARS- CoV-2 infection presenting to emergency departments within a 24 hour period. SARS-CoV-2 samples obtained within 24 hours were positive, supporting prior infection despite relatively limited early symptoms of COVID-19. Unusual clinical and radiographic characteristics included hemorrhagic abscess with blood of varying age in the first, intracranial epidural abscess in the second, radiographic signal consistent with hemorrhagic or thrombotic phenomena, retro-maxillary antral fat changes, and meningeal enhancement or extension in both cases. Radiographic findings thereby mimic fungal infection, although final cultures and ancillary investigation for allergic and invasive fungal disease have remained negative. These cases highlight two unusual orbital presentations of cellulitis occurring in the context of SARS-CoV-2 co-infection.

Derepression of hTERT gene expression promotes escape from oncogene-induced cellular senescence.

Oncogene-induced senescence (OIS) is a critical tumor-suppressing mechanism that restrains cancer progression at premalignant stages, in part by causing telomere dysfunction. Currently it is unknown whether this proliferative arrest presents a stable and therefore irreversible barrier to cancer progression. Here we demonstrate that cells frequently escape OIS induced by oncogenic H-Ras and B-Raf, after a prolonged period in the senescence arrested state. Cells that had escaped senescence displayed high oncogene expression levels, retained functional DNA damage responses, and acquired chromatin changes that promoted c-Myc-dependent expression of the human telomerase reverse transcriptase gene (hTERT). Telomerase was able to resolve existing telomeric DNA damage response foci and suppressed formation of new ones that were generated as a consequence of DNA replication stress and oncogenic signals. Inhibition of MAP kinase signaling, suppressing c-Myc expression, or inhibiting telomerase activity, caused telomere dysfunction and proliferative defects in cells that had escaped senescence, whereas ectopic expression of hTERT facilitated OIS escape. In human early neoplastic skin and breast tissue, hTERT expression was detected in cells that displayed features of senescence, suggesting that reactivation of telomerase expression in senescent cells is an early event during cancer progression in humans. Together, our data demonstrate that cells arrested in OIS retain the potential to escape senescence by mechanisms that involve derepression of hTERT expression.

Chronic, Symptomatic Orbital Inflammation Resulting From Retained Bone Wax.

A 58-year-old man presented with a 9-month history of a chronically draining surgical wound and low-grade periorbital inflammation following a right lateral orbitotomy. Imaging of the right orbit revealed a peculiar lesion in the right lateral orbit that was hypointense on both T1- and T2-weighted MRI with peripheral enhancement. Exploratory orbitotomy and biopsy established the diagnosis of a chronic foreign body inflammatory reaction to bone wax; symptoms resolved following evacuation of the retained foreign material.A 58-year-old man developed a chronic, symptomatic, inflammatory reaction to bone wax following a lateral orbitotomy; only once previously has symptomatic orbital inflammation following orbital surgery as a result of bone wax been reported.

Clinical and Magnetic Resonance Imaging Characteristics of Postfenestration Optic Nerve Sheath Pseudomeningoceles.

PURPOSE: The clinical significance of postoperative pseudomeningocele formation following optic nerve sheath fenestration (ONSF) has not been fully characterized. A literature review identifies 9 previously published cases the authors believe demonstrate pseudomeningocele formation and approximately 19 other similar findings that were either transient or less defined blebs. This study was undertaken to more clearly define the clinical, radiographic, and histopathologic features associated with this entity. METHODS: Sixteen-year, single-center, retrospective chart review of all ONSF cases performed by 2 surgeons. Clinical data, intracranial pressure, radiographic imaging, and histopathology of clinically detected pseudomeningoceles after ONSF were reviewed. RESULTS: Eighty-six eyes in 57 patients underwent ONSF (28 unilateral, 12 bilateral sequential, 17 bilateral simultaneous). Forty-nine of 57 patients had elevated intracranial pressure preoperatively (41 idiopathic intracranial hypertension, 4 venous thrombosis, 2 meningitis, 1 arteriovenous malformation, and 1 sarcoid). In 32 patients undergoing postoperative imaging, 4 eyes (4.7%) in 4 patients developed well-defined pseudomeningoceles, of which 3 were symptomatic and 2 required surgical revision. Each pseudomeningocele developed in the setting of elevated preoperative intracranial pressure (350, 360, 430, 500 mm H20). Magnetic resonance imaging and/or computed tomography revealed sharply demarcated fluid-filled sacs adjacent to the optic nerve. The contents of these sacs were hypointense on T1-weighted imaging, hyperintense on T2-weighting, variably enhanced with contrast, and hypointense on fluid attenuated inversion recovery, and were thus consistent with cerebrospinal fluid. Histopathologic analysis of one of these outpouchings demonstrated an acellular, fibrocollagenized lining consistent with pseudomeningocele. Three eyes in 3 additional patients had less well-defined findings on imaging interpreted as bleb-like or cyst-like change. CONCLUSIONS: Pseudomeningoceles following ONSF may be asymptomatic or may cause symptomatic orbital mass effect and rarely visual loss, amendable to surgical excision. Post-ONSF pseudomeningoceles are identified on computed tomography or magnetic resonance imaging to occur at the locations of fenestration sites and contain cerebrospinal fluid communicating with the subdural space that may act as a "filtration" bleb in some cases. Imaging findings may represent a spectrum spanning intraorbital cerebrospinal fluid leakage, partial walling off of bleb, or fully developed cysts. Resection of optic nerve pseudomeningoceles is considered in symptomatic cysts or eyes with papilledema that fails to improve.

Orbital Hobnail Hemangioma.

A 64-year-old woman presented with a 1 year history of progressive left proptosis and "blurry vision." Imaging studies revealed a left superior orbital mass distorting the optic nerve and superior rectus muscle. Excisional biopsy established the diagnosis of hobnail hemangioma. This report describes the first case of an orbital hobnail hemangioma, a lesion that more commonly presents in the dermis.

Successful Treatment of Extensive Squamous Cell Carcinoma of the Conjunctiva and Upper Eyelid Skin With Topical Therapy Alone.

This study reports a case of extensive periocular squamous cell carcinoma of the conjunctiva, extending into the superior fornix, over the upper eyelid margin, and involving the superficial layers of the eyelid skin up to the eyebrow, that was successfully treated solely with topical therapy.

Oncogene-induced telomere dysfunction enforces cellular senescence in human cancer precursor lesions.

In normal human somatic cells, telomere dysfunction causes cellular senescence, a stable proliferative arrest with tumour suppressing properties. Whether telomere dysfunction-induced senescence (TDIS) suppresses cancer growth in humans, however, is unknown. Here, we demonstrate that multiple and distinct human cancer precursor lesions, but not corresponding malignant cancers, are comprised of cells that display hallmarks of TDIS. Furthermore, we demonstrate that oncogenic signalling, frequently associated with initiating cancer growth in humans, dramatically affected telomere structure and function by causing telomeric replication stress, rapid and stochastic telomere attrition, and consequently telomere dysfunction in cells that lack hTERT activity. DNA replication stress induced by drugs also resulted in telomere dysfunction and cellular senescence in normal human cells, demonstrating that telomeric repeats indeed are hypersensitive to DNA replication stress. Our data reveal that TDIS, accelerated by oncogene-induced DNA replication stress, is a biological response of cells in human cancer precursor lesions and provide strong evidence that TDIS is a critical tumour suppressing mechanism in humans.

Molecular heterogeneity of head and neck squamous cell carcinoma defined by next-generation sequencing.

Head and neck squamous cell carcinoma (HNSCC) can be divided into two different clinical entities based on their association with high-risk subtypes of human papilloma virus (HPV16 and HPV18). Dissimilarities in prognosis and molecular profiles have attracted much attention in recent years, in part because of increasing rates of HPV infection in HNSCC; however, the underlying mechanisms and detailed genetic profiles that set these tumors apart are still elusive. To elucidate oncogenic pathways in HNSCC with and without HPV infection, we used targeted next-generation sequencing to interrogate single-nucleotide polymorphisms (SNPs) in 50 cancer-related genes. We detected SNPs in 25 of these genes from HNSCC tissue specimens with and without HPV infection. In 5 of the 25 genes, variant patterns were similar regardless of HPV infection status. A greater number of sequence variants in genes from the tyrosine kinase receptors and their associated pathways were preferentially present in HPV(+) specimens. SNPs in genes related to tumor-suppressor functions were more prevalent in HPV(-) HNSCC specimens. The observations may help to elucidate mechanisms involved in the molecular pathogenesis of two clinically diverse subclasses of HNSCC. Over-representation of SNPs in either HPV(+) or HPV(-) HNSCC is another indicator of potentially actionable sequence variants for targeted therapy.

Pediatric orbital hemangiopericytoma associated with trisomy 15.

We report a case of an orbital hemangiopericytoma (HPC) in a female infant diagnosed at 7 weeks of age. Cytogenetic analysis of the tumor revealed trisomy 15. To the authors' knowledge, this is only the second reported case of this chromosomal aberration being associated with HPC, both cases occurring in infants.

Giant Orbital Hydrocystoma in Children: Case Series and Review of the Literature.

OBJECTIVE: To describe the clinical features, ancillary diagnostic studies, and treatment outcomes in a cohort of pediatric patients with giant orbital hydrocystomas. DESIGN: Retrospective case series. PARTICIPANTS: Pediatric patients with giant orbital hydrocystomas treated in the practice of one surgeon (PDL). METHODS: A retrospective review of the clinical charts of pediatric patients with orbital hydrocystoma was performed and diagnostic information collected. Results were reviewed and compared with reported clinical data in the literature. MAIN OUTCOME MEASURES: Clinical presentation and histopathological findings of pediatric orbital hydrocystomas. RESULTS: Three pediatric cases of giant orbital hydrocystoma were encountered, each with an unusual feature, including deep orbital location, occurrence following trauma, and eccrine pathology. CONCLUSION: Giant orbital hydrocystomas may present in the pediatric population. Ophthalmologists should be cognizant of this entity when evaluating a child with a large, cystic orbital mass.

Angiosarcoma arising from the frontal sinus.

Primary sinonasal angiosarcomas are very rare tumors. They typically occur in the nasal cavity of middle-aged patients. They are classically highly aggressive. Primary treatment is surgical excision when feasible. We describe a unique case of angiosarcoma in a young woman arising from the frontal sinus with distant metastasis. This case represents the first report of angiosarcoma arising from the frontal sinus in the English literature. The traditional treatment options for the advanced nature of her disease and overall poor prognosis are discussed. We further review the literature and discuss alternative treatments options. Newer chemotherapeutic regiments on the horizon show promise in helping to control this disease.

Periocular sebaceous cell carcinoma following chemical burn: a unique case of a Marjolin ulcer.

This study reports a unique case of a Marjolin ulcer in a patient who presented with periocular sebaceous cell carcinoma 16 years after a lye chemical burn. A 68-year-old woman presented with a long history of irregular and tender right upper and lower eyelid margins, periocular erythema, madarosis, trichiasis, ptosis, and chemosis, occurring many years after chemical burn to the area. After eyelid biopsy established the diagnosis of sebaceous cell carcinoma, subsequent map biopsies of the conjunctival surface revealed extensive sebaceous cell carcinoma with pagetoid spread, necessitating anterior exenteration for definitive treatment. This report describes the first case in which a sebaceous cell carcinoma has arisen in the area of a chemical burn. Sebaceous cell carcinomas should be included in the spectrum of Marjolin ulcers, and ophthalmologists should be aware of this entity when evaluating patients with a history of a burn to the eyelids.

Fibrosing mediastinitis: an unusual cause of pulmonary symptoms.

Fibrosing mediastinitis (FM), also known as granulomatous or sclerosing mediastinitis, is an uncommon but serious cause of chest symptoms. Due to an infectious or inflammatory challenge, production of collagen occurs in the confined space of the mediastinum. Collagen formation leads to compression of vital structures, resulting in cough, chest pain and dyspnea. The majority of cases of FM occur as a result of prior exposure to Histoplasma capsulatum. The following is a case of a previously healthy young woman who presented with a 3-month history of cough, chest pain and trouble breathing, and was subsequently found to have fibrosing mediastinitis. Fibrosing mediastinitis should be considered in the differential diagnosis of cough, chest pain and dyspnea, primarily when findings such as increased venous pressure are present on physical exam and hilar abnormalities are seen on chest radiograph. Clinical presentation, diagnosis and management of fibrosing mediastinitis are discussed.