Orbit-The International Journal on Orbital Disorders-Oculoplastic and Lacrimal Surgery最新文献

A 28-year-old male presented to the emergency room suffering an ocular burn injury from a welding rod. Given the mechanism of injury, severe delayed injury of the ocular adnexa occurred, requiring enucleation, partial exenteration of the superior orbit, and extensive reconstruction. Histopathology of the affected tissue was analyzed. This is the first report that details the clinical course of a patient with delayed high amperage and low voltage electrical burn injury.

Solitary fibrous tumors (SFTs) are rare neoplasms of mesenchymal origin. While the mean age of presentation is 58 years old, we report the case of the youngest documented patient with an SFT of the orbit. A 13-month-old child was evaluated for eyelid asymmetry and then referred to the oculoplastic service. On examination, a soft tissue mass of the right inferomedial orbit was observed. MRI demonstrated a well-circumscribed, extraocular lesion in the inferomedial right orbit, potentially fibrous in nature. Excision was performed without complications. Pathologic examination demonstrated fibrous tissue proliferation with a staghorn vascular pattern, as well as benign fibrous cells with tapering nuclei and abundant pericellular reticulin. Immunohistochemistry (IHC) demonstrated that the cells stain diffusely positive for CD34 and vimentin. With the MRI findings, pathology, and IHC, the diagnosis was confirmed to be SFT. SFTs of the orbit, although rare, may occur in the pediatric population.

Congenital opticmeningoceles were recently described as intraorbital optic nerve cystic lesions, in which neural tissue is found to be part of the lining of the lesion. We describe the case of an eleven-month-old patient with a right opticmeningocele, followed through seven years. He presented with proptosis and hypotropia since birth and imaging exams disclosed an intraconal cystic lesion. A needle puncture revealed a fluid characteristic of cerebrospinal fluid. Surgical drainage performed through a lid crease approach resulted in recurrence of the proptosis. Surgical excision of the capsule finally was definitive, and pathology revealed an epithelial-lined capsule. Eight months after the capsule excision, a strabismus surgery was performed to improve the right hypotropia. Although visual prognosis of opticmeningoceles is poor, a good cosmetic result can be obtained with a multidisciplinary approach involving orbit and strabismus surgeons. The complete capsule excision is believed to be the preferable surgical option for permanently treating the proptosis once needle puncture and cyst drainage are associated with recurrence. The orbital access for approaching the lesion depends on the location of the cyst and should provide wide exposure to avoid new surgical procedures in young patients.

We describe a case of primary ductal adenocarcinoma of the lacrimal gland with novel histopathological characteristics corresponding to a biphasic growth course and provide a comprehensive genomic profile of this malignancy. A 39-year-old male with a history of slowly progressive unilateral proptosis and hypoglobus presented after 1 month of hyperacute exacerbation. Orbital imaging revealed a superior mass with osseous erosion. The patient underwent orbital exploration and excisional biopsy via lateral orbitotomy. Histopathology demonstrated high-grade adenocarcinoma with a well-differentiated glandular component alongside a poorly differentiated sarcomatoid region. The glandular section was immunopositive for Her-2, CK7, GATA3, and androgen receptor. Tumor recurrence necessitated en-bloc exenteration with dural resection alongside adjuvant radiotherapy and chemotherapy. This represents the first report of sarcomatoid differentiation in primary ductal adenocarcinoma of the lacrimal gland, which may incite hyperacute progression. Conversely, GATA3 immunopositivity may correlate with indolent growth. Genomic variants such as SEMA3C represent potential therapeutic targets for this condition.

A 68-year-old man with a history of remote sinus surgery presented with several months of progressive diplopia, proptosis, and epiphora. He was found to have a large, cystic mass in the lateral extraconal space of the right orbit, which was removed. Pathology demonstrated an epidermoid cyst with recurrence at post-operative month 3. Follow-up resection was performed with the insertion of a modified Jones tube into the maxillary sinus to serve as a persistent outlet and prevent future recurrence. At post-operative month 18 after tube insertion, he remains without disease recurrence. The authors describe this novel technique as a method to prevent recurrence in orbital cyst management.

Traditionally, for patients who are poor candidates for surgery and/or radiotherapy, palliative chemotherapy is often offered but with significant toxic side effects. However, recent advancements in our understanding of tumor biology and molecular genetics have brought new understanding to the molecular pathways of certain tumors and cancers. This has ushered in a new era of precision medicine specific to a tumor or cancer treatment pathway (targeted therapy) or directed to host-tumor responses (immunotherapy). This article will focus on recent updates in the application of available targeted and immunotherapy for managing orbital and periorbital tumors and tumor-like conditions, which include cutaneous basal cell carcinoma, cutaneous squamous cell carcinoma, cutaneous melanoma, Merkel cell carcinoma, sebaceous gland carcinoma, solitary fibrous tumor, dermatofibrosarcoma protuberans, orbital meningioma, neurofibromatosis, Langerhans cell histiocytosis, ocular adnexal lymphoma, orbital lymphatic malformation, and adenoid cystic carcinoma.

Atypical fibroxanthoma (AFX) is a rare neoplasm, with a limited number of cases reported in the periocular region. In this case report, we detail a 63-year-old woman who presented with a polypoid, exophytic lesion on her right upper eyelid that had been progressing for a year. The lesion was meticulously excised with security margins and reconstructed using a glabellar flap. Following a thorough microscopic and immunohistochemical analysis, AFX was diagnosed. Despite its sometimes clinical and histological benign appearance, AFX is classified as a malignant neoplasm; however, it carries an excellent prognosis with low metastasis and recurrence rates. Complete excision with safety margins is essential and an adequate post-operative surveillance is recommended. Owing to its rarity, ophthalmologists should remain vigilant and include AFX in their differential diagnosis, as the tumor's benign appearance may lead to misdiagnosis of this malignant entity.

We present a patient who presented with an orbital mass lesion which was a metastatic lesion from a porocarcinoma of the scalp with progressive deterioration of the patient.A 78-year-old male presented with functional decline and a rapidly growing scalp lesion of 3 months duration. In addition to the scalp lesion, Computed Tomography showed an incidental finding of a left lateral orbital wall tumour. Fine-needle aspiration of the two lesions revealed malignant cells with similar morphologies. Punch biopsy of the scalp lesion showed histological features suggestive of a porocarcinoma. Patient underwent palliative radiotherapy and immunotherapy and subsequently succumbed to the disease.

Mucormycosis is a serious fungal infection caused by fungi in the order of Mucorales. Orbital mucormycosis occurs more frequently in rhino-orbital, sino-orbital, and rhino-orbito-cerebral forms of the disease, while isolated orbital mucormycosis is much less common. Herein, we present four cases of immunocompetent children who developed primary cutaneous mucormycosis, which subsequently invaded and progressed to orbital mucormycosis following direct traumatic injury caused by pecking from Acridotheres tristis (Common Myna). Given the low prevalence of orbital mucormycosis in healthy children, an unknown source of infection and delayed diagnosis followed by late therapeutic interventions could result in life-threatening conditions and serious sequelae.