General Approach and Algorithm for Managing Emergent Neoplasms of the Orbit and Adnexa

Lora Rabin Dagi Glass

Lora Dagi Glass completed her ophthalmology residency at the Columbia University Medical Center, followed by a fellowship in oculoplastic surgery at the Massachusetts Eye and Ear Infirmary. Her research has focused on orbital processes ranging from thyroid eye disease to optic nerve glioma and orbital melanoma.



Seanna Grob

MD, MAS received a BA in Molecular and Cellular Biology from the University of California, Berkeley and a MD and MAS from the University of California, San Diego. She completed her ophthalmology residency at Massachusetts Eye and Ear/Harvard Medical School. She is currently the Director of the Ocular Trauma Service at Massachusetts Eye and Ear and Brigham and Women’s Hospital. She has been accepted into a 2-year American Society of Ophthalmic Plastic and Reconstructive Surgery-approved fellowship and will start in 2017.



Suzanne K. Freitag

Suzanne K Freitag, MD, received a BA from Duke University and MD from Jefferson Medical College in Philadelphia, PA. She completed ophthalmology residency at Wills Eye Hospital followed by a neuroophthalmology fellowship at the Wilmer Eye Institute at Johns Hopkins. She then completed a 2-year American Society of Ophthalmic Plastic and Reconstructive Surgery-approved fellowship in Boston. She is the Director of the Ophthalmic Plastic Surgery Service at Massachusetts Eye and Ear Infirmary and an Assistant Professor of Ophthalmology at Harvard Medical School.


Malignancies of the orbit can be classified into primary, secondary, or metastatic in nature. Primary orbital malignancies arise within the orbit, while secondary orbital malignancies are extensions of locally invasive tumors in adjacent structures. Metastatic orbital tumors are the result of hematogenous or lymphatic spread from primary tumors in remote sites.

Malignant neoplasm must be in the differential diagnosis of any patient presenting with proptosis or other globe displacement (Fig. 41.1), restriction of extraocular motility, globe compression, optic neuropathy, or other cranial neuropathies pertaining to the orbit or cavernous sinus. These orbital malignancies require different consideration than when they occur in other parts of the body because the orbit is a small confined space full of structures critical for vision. Prompt diagnosis is important to prevent a rapidly enlarging lesion from causing permanent damage to orbital structures. If not carefully planned, treatments such as surgery or radiation therapy to the orbit may cause loss of vision or visual dysfunction such as diplopia. Certain pediatric neoplasms, such as rhabdomyosarcoma, neuroblastoma, and retinoblastoma, can be rapidly lethal. It is especially critical to recognize a potential neoplasm in this age group and address it immediately. Hence, it is crucial to identify the presence of an orbital lesion when a patient presents with symptoms, to suspect when it may be malignant, and to act carefully but expediently to save vision, eyes, and life.


Fig. 41.1
(a) An 8-year-old female presented with a rapidly progressive mass of the right orbit, causing globe displacement. (b) Coronal T1-weighted MRI with contrast showed a homogenously enhancing mass in the inferior orbit. Emergent biopsy showed embryonal rhabdomyosarcoma


When an orbital neoplasm is suspected, a thorough examination is vital. The clinical history can elucidate helpful information regarding temporal onset of disease, rapidity of progression, and associated symptoms. These may serve as clues regarding tumor location and affected orbital structures. In addition to asking questions about onset, duration, severity, and progression, it is often useful to have the patient bring old photographs or request observations from close friends or family members to determine changes that may have occurred even earlier than the first reported symptoms. Sensory symptoms including pain, numbness, tingling, as well as severity and worsening of these symptoms with eye movements can provide additional information about the type or location of the tumor. For example, adenoid cystic carcinoma of the lacrimal gland is more commonly associated with pain due to its ability to infiltrate adjacent structures and invade orbital nerves. Symptoms suggesting disturbance of the optic nerve include changes in visual acuity or color vision, or awareness of a scotoma. Neoplasms affecting motor abilities of the eye can cause symptoms of diplopia, gaze or head positioning preference, pain with eye movements, and pulling or tugging sensations suggesting a restrictive component. Patients may notice changes in their appearance as a result of tumor growth, such as proptosis or enophthalmos, globe displacement in any direction, or swelling or fullness of eyelids (Fig. 41.2). If the tumor is affecting the orbital vascular system, the patient may notice conjunctival injection or eyelid erythema and edema.


Fig. 41.2
(a) A 69-year-old female presented with swelling of the right brow. (b) Axial CT imaging confirmed lesions of the right and left lids with extension into the right orbit, and biopsy confirmed a diagnosis of MALT lymphoma

A thorough discussion of past medical history and review of systems is also important, as neoplasms within the orbit are often associated with systemic abnormalities [1]. Review of systems may reveal symptoms of systemic illness such as fever or weight loss. Past medical, ocular, surgical, and family history can point to possible etiologies, particularly metastatic cancer or oncologic syndromes.

Physical examination is essential to collecting additional information that can be used to synthesize a plan for further clinical investigations and management. General observations such as facial contour and symmetry of facial, lid, orbital, and ocular structures are useful. The orbital structures should be palpated, as should the preauricular and cervical lymph nodes to check for enlargement, which would be concerning for nodal metastases. If tissues feel firm, nodular, non-mobile, or irregular, they should be investigated further. Resistance to retropulsion of the globe should be assessed and compared to the contralateral orbit. Abnormal lid positioning or structural changes may be secondary to proptosis or other alterations in orbital anatomy. Measurements of interpalpebral fissure height, lid position lid crease position, lagophthalmos, and scleral show should all be documented. Slit lamp biomicroscopy may reveal the presence of tumor on the ocular surface (Fig. 41.3) or conjunctival injection, dilated episcleral vessels, or chemosis, especially when vascular congestion or malformations are present in the orbit.


Fig. 41.3
A 52-year-old male presented with a rapidly growing, melanotic mass of the right eye and was found to have both eyelid and caruncular melanomas. The neoplasm rapidly invaded the nasolacrimal system and metastasized to the lungs

Tests of vision and optic nerve function include best-corrected visual acuity, pupil evaluation for pupil asymmetry or afferent pupillary defect (Fig. 41.4), confrontational visual fields, Amsler grid testing of central vision, and color vision assessment. These measurements are helpful for evaluating compression of or the degree of damage to the optic nerve or other vital ocular structures, helping guide urgency of treatment.
Oct 16, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on General Approach and Algorithm for Managing Emergent Neoplasms of the Orbit and Adnexa

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