68ORBIT, EYELIDS, AND OCULAR ADNEXA

Hand-Schu¨ller-Christian disease: lytic skull lesions, diabetes insipidus, age 20–40.

Letterer-Siwe disease: young, sick patients with systemic, visceral lesions (gastrointestinal system most often involved, and orbit rarely involved). High mortality; worst prognosis of the histiocytoses.

LYMPHOMA Painless proptosis, slow growth, solid but diffusely infiltrative mass; may mold but not erode bone. Orbit is the most common extranodal site for lymphoma.

CT shows ‘‘pregnant pancake’’ appearing mass. Echography shows low internal reflectivity. Consider systemic work-up with whole-body CT and bone marrow biopsy.

Biopsy; send in formalin for light microscopy, and send fresh tissue for flow cytometry. Pathology shows Dutcher bodies (intranuclear) and Russell’s bodies (cytoplasmic inclusions); vast majority are monoclonal (>60% B cells).

Treat with radiation therapy 1500–2000 cGy and chemotherapy (not concurrent with radiation therapy), but usually do not excise. Follow CBC and serum protein electrophoresis (SPEP) every 6 months for 5 years.

Most important prognostic factor is extent of disease at time of presentation: 86% with stage 1E (involvement of single extralymphatic site) have benign indolent course. No prognostic significance whether unilateral or bilateral involvement. If eyelid is involved, there is a 67% risk of systemic lymphoma (because of lymphatics), 35% risk for orbital lymphoma; if conjunctiva are involved, there is a 20% risk.

Neoplastic, Orbital: Mesenchymal Tumors

GENERAL Diverse group of tumors that comprise 11% of orbital lesions. Ewing’s sarcoma makes up 29% of orbital mesenchymal tumors, rhabdomyosarcoma 29%, fibrous dysplasia 26%, fibrous histiocytoma 11%, and fibrosarcoma 2%.

EWING’S SARCOMA Primitive tumor usually arising from the long bones and pelvis with peak incidence 10–20 years old. Orbit is secondarily involved.

FIBROSARCOMA OR OSTEOSARCOMA Slowly growing orbital mass usually from sinuses; associated with Gardner’s syndrome (colon polyps).

FIBROUS DYSPLASIA Relatively common slow growing bony mass from abnormal bone maturation, typically in childhood. May cause proptosis, globe ptosis, compressive optic atrophy and pain. CT shows ‘‘ground glass’’ appearance. Debulk, but difficult to excise completely.

Albright’s syndrome: ossifying fibroma (aggressive fibrous dysplasia), with sharply demarcated sclerotic rim; F > M. Associated with precocious pubertys; cafe´ au lait spots, strabismus.

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FIBROUS HISTIOCYTOMA, DERMATOFIBROMA Relatively common orbital mesenchymal tumor usually in young adults. May cause congestive proptosis (like a hemangiopericytoma). Generally benign (2% are malignant, usually defined as five mitotic figures per high-powered field), well circumscribed with storiform pattern of spindle and histiocyte cells on pathology and vimentin (þ) and S100 ( ). Excise completely, as they have a tendency to recur, with a greater chance of malignant transformation.

RHABDOMYOSARCOMA Most common malignant orbital tumor in children (average age: 7 years old), with male predominance. Characterized by new onset rapid proptosis, conjunctival injection, edema, usually no pain or decreased vision, and palpable lid mass (25%); may have incidental history of trauma that confuses the diagnosis. A-scan shows low internal reflectivity (dense, compact tumor cells). Schedule prompt biopsy if suspicious.

Pathology shows ‘‘bubble gum and raisins’’ appearance of dark cells with pink connective tissue, which is the tumor trying to make smooth muscle. May be desmin þ, muscle specific actin (MSA) (þ). Phosphotungstic acid-hematoxylin (PTAH) or Masson trichrome stains may show cross-striations (best seen with pleomorphic variant; in the embryonal variant, striations are usually seen only with electron microscopy).

Four variants:

Embryonal (75%): most common, usually superonasal orbit; may demonstrate cross-striations on pathology.

Botryoid: involves mucosal surfaces usually from the sinuses into conjunctiva and eyelids.

Alveolar (15%): most aggressive, usually involves inferior orbit (alveolar ‘‘near the lungs’’); second most rare.

Pleomorphic: most rare and most benign.

Treat with debulking surgery, then radiation therapy 5000 cGy over 6 weeks (may cause hypoplastic orbit, radiation retinopathy) and chemotherapy to eliminate microscopic cellular metastatic cancer (typically VAC: vincristine, actinomycin D, cyclophosphamide). Historically, all cases were exenterated. Group 1: local disease; group 2: regional disease; group 3: distant metastatic cancer.

Neoplastic, Orbital: Metastatic and Invasive Tumors

GENERAL Metastatic and invasive tumors account for 9% of orbital lesions, 25% of orbital tumors; usually hematogenous spread. In children, cancer usually metastasizes to the orbit; in adults, it often spreads to the uvea. Female patients are more likely to have a breast or lung primary cancer; males patients, usually lung or prostate. Most common lesions are breast CA, lung CA, sarcoma, gastrointestinal tract, and prostate CA, and

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70 ORBIT, EYELIDS, AND OCULAR ADNEXA

extension from eyelid, intraocular, brain (sphenoid wing meningioma), and sinus tumors.

EYELID TUMOR EXTENSION Incidence rates are BCC (67%), sebaceous cell carcinoma (22%), melanoma (7%), and SCC (4%); is less common due to lower incidence but especially prone to extend to orbit via perineural spread. Usually presents with a ‘‘lumpy-bumpy’’ muscle or nerve appearance on imaging (multifocal).

INTRAOCULAR TUMOR EXTENSION Via emissary vessel or scleral extension; two thirds of cases are melanoma, one third retinoblastoma (in Africa, RB is leading cause of childhood orbital malignancy).

METASTATIC BREAST CANCER Scirrhous variant may cause enophthalmos.

METASTATIC MALIGNANT MELANOMA If metastatic, usually to EOM; extrascleral extension from choroid in 7% of choroidal MM (early exenteration gives a 97% 5-year survival; late surgery 62%; if no exenteration, 0% survival).

METASTATIC NEUROBLASTOMA Most common metastatic tumor to the orbit in children; usually from adrenals (50%), retroperitoneal (25%), mediastinum, and neck. Overall 20% have ocular involvement. Bilateral in 60%; may have abrupt ecchymotic proptosis (tumor erosion through blood vessels). May present as paraneoplastic opsoclonus-myoclonus-ataxia syndrome; if from thorax, may have Horner’s syndrome. Mean age 2 years old (90% are <5 years old); prognosis is age-dependent and poor in general but considerably better if <1 year old (72% survival). Treat with emergent radiation therapy and chemotherapy.

METASTATIC PROSTATE CA Osteoclastic bony mass with a predilection for the superior orbit.

NASOPHARYNGEAL CA Higher incidence in males (2–3x) and Asian patients. Typically causes nasal obstruction, rhinorrhea, epistaxis, otitis media, proptosis, and ipsilateral dry eye.

ORBITAL GRANULOCYTIC SARCOMA, CHLOROMA Orbital tumor that precedes or occurs simultaneously with leukemia. Average age 7 years old, M > F. Characterized by rapid growth; grossly has a green color.

SINUS TUMOR EXTENSION 72% are maxillary sinus SCC with proptosis, vision loss, and eyelid edema; 17% are from ethmoid sinus.

Neoplastic, Orbital: Neurogenic Tumors

GENERAL 24% of orbital neoplasms.

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MALIGNANT GLIOMA Rare tumor arising from CNS astrocytes; in the orbit is usually a class IV astrocytoma (glioblastoma multiforme). More common in older patients; one third of cases are unilateral, two thirds are bilateral. Characterized by vision loss; half have disk edema. One hundred percent in chiasm and 50% in optic tracts at diagnosis; poor prognosis.

MENINGIOMA Tumor arising from the meninges (in the orbit usually from the sphenoid bone). 60% female, average age 40 years old (for bilateral tumors, the average age is 12 years old); only 6% are bilateral. Has BCClike activity (slow growing, local extension, rare metastasis). Of orbital meningiomas, two thirds are from brain extension, and one third are primary from the ON. Pathology demonstrates psammoma bodies (from Greek psammos, ‘‘sand’’).

ON sheath meningioma: accounts for only 10% of all meningiomas, but is the most common primary meningioma of the orbit. Typically presents with unilateral painless vision loss, optic atrophy, axial proptosis, and optociliary shunt vessels in a middle-aged female. Most are tumors of the meningoepithelial cells from arachnoid villi and benign. May infiltrate fat and muscle and show ‘‘tram tracking’’ on CT. Most are isolated; however, there is a slight association with NF, especially in children, where it is more aggressive. Observe if clinically stable; rarely biopsy. May also treat with radiation therapy, chemotherapy, RU-486, or excise if progressive growth (be prepared to excise up to the chiasm).

Sphenoid-wing meningioma: more common than sheath meningioma; invades orbit from intracranial space and encroaches on ON or chiasm with decreased VA, proptosis, and optociliary shunt vessels. CT usually shows hyperostotic sphenoid bone but may be lytic. Debulk; cannot completely excise. Increased risk with NF 1 and NF 2.

ON GLIOMA, JUVENILE PILOCYTIC ASTROCYTOMA (JPA) Accounts for two thirds of neurogenic orbital tumors; benign tumor but with potentially serious consequences. Ninety percent occur within the first decade (mean age 8.5 years). Presents with painless axial proptosis, vision loss, ON atrophy (two thirds of cases), ON edema (one third of cases), optociliary shunt vessels. Fusiform pattern on CT; MRI shows tumor that is isoor hypointense to the ON with T1 weighting and hyperintense to ON with T2.

Pathology: should not see histology because treatment is usually observation, but will show arachnoid hyperplasia, central liquefaction necrosis. Rosenthal fibers are present as for all astrocytomas and have ‘‘hairlike’’ appearance (thus the name pilocytic). If associated with NF 1 (30%), the pathology of ON glioma is different and has component of meningioma with arachnoid or meningoepithelial hyperplasia.

Treatment: observe if stable. When it begins to grow toward chiasm (39% progress), surgical excision is curative; may need chemotherapy and radiation therapy.

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