Cutaneous melanoma metastatic to the orbit: review of 15 cases.

PURPOSE: To describe the clinical features, management, and outcomes of 15 patients with cutaneous melanoma metastatic to the orbit. The authors review emerging treatments for metastatic melanoma and their ocular implications. METHODS: Retrospective chart review of 15 patients with orbital metastasis from cutaneous melanoma. RESULTS: At presentation of the orbital metastasis, systemic metastatic cutaneous melanoma was present in 13 (87%) patients. The mean interval from diagnosis of cutaneous melanoma to orbital metastasis was 40 months (median, 37 months; range, 0-117 months). The most common presenting signs were dysmotility (63%), proptosis (56%), and blepharoptosis (19%). Four patients (25%) presented with pain. Metastasis involved extraocular muscle in 6 orbits (35%), intraconal space in 4 (24%), extraconal space in 7 (41%), and lacrimal sac in 1 (6%). The tumor was unifocal in all cases, unilateral in 13 patients (87%), and bilateral in 2 (13%). The mean tumor basal dimension was 20 × 20 mm and mean thickness was 16 mm. Treatments included complete surgical excision in 1 patient (6%), external beam radiotherapy (EBRT) in 7 (47%), systemic chemotherapy in 8 (53%), and immunotherapy in 5 (33%). Orbital tumor control was achieved in 2 orbits (18%) following focal therapy alone (excision or EBRT), 4 (36%) following systemic therapy alone (chemotherapy or immunotherapy), and 3 (27%) following combination focal plus systemic therapy. Three patients required exenteration. Survival rates at 1 year/2 years were 100%/0% following focal therapy, 50%/25% following systemic therapy, and 100%/66% following combination therapy. CONCLUSIONS: Cutaneous melanoma metastatic to the orbit tends to involve muscle (35%) or intraconal soft tissue (24%) as a painless (75%), circumscribed (87%) mass. Treatment with systemic chemotherapy and/or immunotherapy resulted in orbital tumor control in 80% of cases. Overall survival was 25.1 months.

Massive macronodular juvenile xanthogranuloma of the eyelid in a newborn.

A newborn baby with a lump on his right upper eyelid that was unresponsive to warm compresses and oral antibiotics presented at 3 weeks of age with a yellow mass measuring 20 mm in diameter at the base. Preliminary diagnosis was benign choristomatous mass; and warm compresses were continued. The mass continued to enlarge and 5 weeks later was 35 mm, with a tight, atrophic overlying epidermis and greater pupillary occlusion. Concern for possible malignancy prompted surgical resection and reconstruction with a supraclavicular graft. Histopathology disclosed that the eyelid tissue was nearly completely replaced by a highly cellular histiocytic neoplasm with prominent eosinophilic, often foamy cytoplasm, and nuclear pleomorphism. Poorly formed Touton giant cells were found. The mass showed positive immunoreactivity, with histiocytic markers CD163 and factor XIII, and was negative for cytokeratin markers, smooth muscle actin, and desmin. These features were compatible with JXG.

Calcified scleral choristoma in organoid nevus syndrome simulating retinoblastoma.

A child referred for management of retinoblastoma who alternatively had a calcified scleral choristoma as part of previously undiagnosed organoid nevus syndrome is described. A 31-month-old male infant with scalp alopecia was referred for retinoblastoma management after a calcified mass in his left eye was found. Ophthalmic examination revealed the mass was of choroidal or scleral origin, underlying the retina. The amelanotic circumpapillary mass extended superonasally in a geographic configuration and measured 14×12 mm. There was no subretinal fluid, hemorrhage, feeder vessels, or tumor seeding. Ocular ultrasonography confirmed a homogeneous calcified intraocular mass 3.1 mm in thickness. Enhanced depth imaging optical coherence tomography revealed that the lesion was located within the sclera compressing the overlying choroidal tissue. Further evaluation disclosed cutaneous aplasia cutis congenita with nevus sebaceous of Jadassohn. Magnetic resonance imaging disclosed an arachnoid cyst of the brain. Later, optical coherence tomography revealed the mass to be in the deep choroid or within the sclera. This constellation of ocular, cutaneous, and neurological features were suggestive of organoid nevus syndrome. At the 2-year follow-up, the findings were stable. The calcified choristoma of organoid nevus syndrome, located within the sclera in this case, has distinctive clinical features that differentiate this benign tumor from retinoblastoma.

Fabrication and mechanical evaluation of anatomically-inspired quasilaminate hydrogel structures with layer-specific formulations.

A major tissue engineering challenge is the creation of multilaminate scaffolds with layer-specific mechanical properties representative of native tissues, such as heart valve leaflets, blood vessels, and cartilage. For this purpose, poly(ethylene glycol) diacrylate (PEGDA) hydrogels are attractive materials due to their tunable mechanical and biological properties. This study explored the fabrication of trilayer hydrogel quasilaminates. A novel sandwich method was devised to create quasilaminates with layers of varying stiffnesses. The trilayer structure was comprised of two "stiff" outer layers and one "soft" inner layer. Tensile testing of bilayer quasilaminates demonstrated that these scaffolds do not fail at the interface. Flexural testing showed that the bending modulus of acellular quasilaminates fell between the bending moduli of the "stiff" and "soft" hydrogel layers. The bending modulus and swelling of trilayer scaffolds with the same formulations were not significantly different than single layer gels of the same formulation. The encapsulation of cells and the addition of phenol red within the hydrogel layers decreased bending modulus of the trilayer scaffolds. The data presented demonstrates that this fabrication method can make quasilaminates with robust interfaces, integrating layers of different mechanical properties and biofunctionalization, and thus forming the foundation for a multilaminate scaffold that more accurately represents native tissue.