Carboplatin and Pembrolizumab Chemoimmunotherapy Achieves Remission in Recurrent, Metastatic Sebaceous Carcinoma.

A 72-year-old Caucasian woman who had recurrent sebaceous carcinoma of the right orbit with bilateral cervical lymph node involvement 24 months after orbital exenteration was treated with carboplatin (area under the curve of 5) and pembrolizumab (2 mg/kg) for 6 cycles, followed by maintenance pembrolizumab. She obtained a complete pathological remission and remains free of local, regional, and systemic disease at 15 months.

Granulomatous dacryoadenitis in regional enteritis (crohn disease).

PURPOSE: To evaluate the clinical and immunopathologic features of 2 patients with bilateral dacryoadenitis associated with regional enteritis. DESIGN: Retrospective, clinicopathologic study. METHODS: Clinical records, photographs, and imaging studies were reviewed and microscopic sections of lacrimal gland biopsy samples were critically re-evaluated. The microscopic slides were stained with hematoxylin and eosin, special stains for organisms, and a range of immunohistochemical biomarkers, including CD3, CD4, CD5, CD8, CD20, CD68, CD138, CD1a, and immunoglobulins Ig G, IgG4, and IgA. RESULTS: Both patients were young women with a well-established diagnosis of regional enteritis. Histopathologic examination of biopsy samples disclosed moderate intraparenchymal fibrosis and lymphoplasmacytic infiltrates without lymphoid follicles. Small to medium intraparenchymal, noncaseating granulomas lacking multinucleated giant cells and, in 1 patient, CD68-positive and CD1a-negative palisading granulomas in widened interlobular fibrous septa were detected. Vasculitis and IgG4 plasma cells were not observed. Additional immunohistochemical studies revealed that CD8 T lymphocytes (suppressor or cytotoxic subset) predominated over CD4-positive T lymphocytes (helper cells) surrounding the necrobiotic foci and were intermixed with the CD68-positive histiocytes in the absence of CD20 B lymphocytes. Special stains for organisms demonstrated negative results. CONCLUSIONS: Dacryoadenitis is the rarest form of ocular adnexal involvement in regional enteritis, which affects the orbit far more frequently than ulcerative colitis. It is a granulomatous process with the possibility of palisading necrobiotic foci. In contrast, ulcerative colitis causes an interstitial lymphocytic and nongranulomatous myositis. Sarcoidosis, Wegener granulomatosis, and pseudorheumatoid nodules must be ruled out. Treatment options entail a wide variety of agents with selection based on empirical considerations and tailored to the patient's symptoms.

Postoperative maxillary sinusitis mimicking tumor recurrence on positron emission tomography.

A significant overlap of standard uptake values occurs in a variety of pathological processes, potentially causing misdiagnosis for tumor recurrence. The authors present a case of acute maxillary sinusitis masquerading as local recurrence of malignancy.

Optic disc contractility in morning glory disc anomaly.

Morning glory disc anomaly is a sporadic congenital abnormality of the optic disc that is often unilateral. These eyes possess heterotropic smooth muscle and can undergo spontaneous contraction with transient vision loss. The authors report an unusual case of contractility of a morning glory disc anomaly induced by ocular massage.

A pregnant pause.

A 23-year-old pregnant woman presented with a rapidly progressive unilateral optic neuropathy. The evaluation was complicated by her being pregnant and the issues surrounding the evaluation and management of the pregnant patient with a neuro-ophthalmic finding is discussed. Eventually an orbital apex lesion was found and proved to be an orbital schwannoma. Rapid growth of an orbital schwannoma should be included in the differential diagnosis of progressive visual loss in a pregnant patient.

Expanding the phenotype of a neurofibromatosis type 1-like syndrome: a patient with a SPRED1 mutation and orbital manifestations.

A 4-year-old child with no medical history presented for evaluation of a small, palpable nodule near the left inferolateral rim. The lesion had a bluish hue and had been slowly enlarging over the course of several months. MRI of the orbits revealed a heterogenous and infiltrative preseptal and extraconal mass which enhanced with gadolinium, and sphenoid wing dysplasia on the left. A complete ophthalmic and physical examination failed to reveal any other stigmata of neurofibromatosis type 1 (NF1) or neurofibromatosis type 2 (NF2). An incisional biopsy of the palpable mass revealed a plexiform neurofibroma. Molecular sequencing of the NF1 and NF2 genes did not reveal causative mutations. Further investigation revealed a loss of function mutation in SPRED1 on chromosome 15. Although loss of function mutations in the SPRED1 gene are known to cause several dermatologic changes associated with the NF1-like phenotype, to our knowledge, this is the first description of a SPRED1 gene mutation resulting in ophthalmic abnormalities.

Orbital burkitt lymphoma in immunocompetent patients: a report of 3 cases and a review of the literature.

PURPOSE: To describe a series of 3 immunocompetent patients with the sporadic form of orbital Burkitt lymphoma and review the outcomes of such patients reported in the scientific literature. METHODS: Retrospective review of medical records and the literature. Cases of orbital Burkitt lymphoma in immunocompromised and African patients were excluded from the review. Measured parameters included gender, age, ocular signs, imaging results, the range of systemic involvement, and treatment. RESULTS: Current cases and review of the scientific literature resulted in 16 immunocompetent patients with sporadic orbital Burkitt lymphoma. The median age at presentation was 12 years. Most common ocular signs at presentation were proptosis (13/16), external ophthalmoplegia (9/16), and eyelid edema (7/16). Optic neuropathy was noted in 5 of 16 patients. Concomitant paranasal sinus involvement was present in 8 of 16 patients. Fourteen (88%) of the patients had systemic involvement, of which the most common locations were central nervous system (6/16), lymphatics (6/16), bone marrow (6/16), and liver (4/16). Survival data were available for 13 patients. Seven patients (54%) died within 12 months of presentation. CONCLUSIONS: Sporadic orbital Burkitt lymphoma occurs in immunocompetent individuals with a wide age range. Fifty percent presented with adjacent paranasal sinus involvement. Concurrent or eventual systemic involvement is common. The large number of patients with central nervous system involvement in our review of the literature supports the role of lumbar puncture and neuroimaging in the workup of all patients with orbital Burkitt lymphoma. Prognosis remains guarded, with significant mortality within 1 year of presentation.

Ewing sarcoma presenting as a subconjunctival mass.

A 14-year-old previously healthy boy presented for evaluation of a subconjunctival mass that had been present for 2 to 3 years, but had recently started to enlarge. Imaging demonstrated an anterior orbital lesion that enhanced with gadolinium. An excisional biopsy was performed and revealed highly malignant-appearing small, blue, round tumor cells. Immuohistochemical stains were strongly reactive with CD99 (MIC2 and O13), CD56, and retained INI-1 reactivity. The EWS-FLI1 chimeric fusion gene transcript was also detected by reverse transcription-polymerase chain reaction analysis, confirming Ewing sarcoma. Full-body CT, bone scan, and bone marrow biopsy were all negative, proving this to be a premetastatic, primary orbital tumor arising from soft tissue.

"Sutureless" repair of orbital floor and rim fractures.

PURPOSE: To report on the surgical outcomes of patients undergoing orbital fracture repair without periosteal or conjunctival closure. DESIGN: Retrospective interventional case series. PARTICIPANTS: Eighty-two patients (85 eyes) who underwent transconjunctival repair of isolated floor (n = 38) or complex orbital fracture (n = 47) without conjunctival closure. METHODS: A transconjunctival incision below the tarsus provides preseptal access to the inferior orbital rim, after which the periosteum is incised for exploration and repair of orbital floor fractures. Forced ductions are performed after release of entrapped tissue and placement of an orbital floor implant, and the conjunctiva is reapproximated with forceps and draped into the inferior fornix. MAIN OUTCOME MEASURES: Incidence of postoperative complications, including orbital implant exposure, infection, and migration. RESULTS: Patients were followed for an average of 318 days postoperatively (range 82-978 days). One patient experienced a pyogenic granuloma at the conjunctival incision that resolved with steroid drops. Other complications included lateral canthal dystopia or pyogenic granuloma at the lateral canthotomy site, when used. There were no cases of postoperative implant exposure, infection, or migration. CONCLUSIONS: Forgoing closure of the periorbita and conjunctiva after transconjunctival orbital floor and rim fracture repair is associated with a low incidence of postoperative complications. This technique is applicable in the repair of both isolated floor fractures and complex orbital fractures. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any material discussed in this article.

Preliminary study of positron emission tomography in the detection and management of orbital malignancy.

PURPOSE: To determine the clinical efficacy of positron emission tomography (PET) in the treatment of patients with orbital malignancy. METHODS: Prospective, consecutive case series to assess the ability of PET to (1) identify lesions previously demonstrated on CT or MRI; (2) characterize the metabolic activity of these lesions; and (3) determine the presence of metabolically active metastases elsewhere in the body. RESULTS: Eighteen patients with suspected orbital malignancies, ages 32 to 78 years, underwent PET in addition to CT and/or MRI. Sixteen of 18 also underwent orbital biopsy. Histopathologic diagnosis included lymphoma (55%), carcinoma (22%), melanoma (11%), sarcoma (5.5%), and lymphoid hyperplasia (5.5%). All orbital lesions were seen on conventional imaging. Overall, 61% of orbital lesions were demonstrated on PET, including 55% of tumors that were lymphoproliferative and 100% (n = 5) of other malignancies. PET was useful in ruling out tumor recurrence after exenteration in 2 patients. CONCLUSIONS: In this small clinical series, PET proved effective in demonstrating nonlymphoproliferative orbital malignancy. This series is too small to study any correlation between histopathologic aggressiveness and PET findings in orbital lymphoma.