Hunter Kwok-Lai Yuen
Dr. Hunter Kwok-Lai Yuen is the consultant ophthalmologist and head of the Oculoplastic & Orbital Surgery Service at Hong Kong Eye Hospital. Dr. Yuen was awarded as one of Ten Outstanding Youth Persons in 2007. Subsequently, he received The Fourth Hong Kong Volunteer Award in year 2011. He received Achievement Award and Distinguished Service Award from the Asian Pacific Academy of Ophthalmology in 2012. Moreover, he is the present first Vice President of Asia Pacific Society of Ophthalmic Plastic and Reconstructive Surgery (APSORPS), President of the Hong Kong Society of Ophthalmic Plastic and Reconstructive Surgery, Honorary Secretary of Asia-Pacific Society of Ocular Oncology and Pathology (APSOOP), and editorial member and reviewer of several journals.
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.
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.
Introduction
The orbit is surrounded by a number of significant structures, including the intracranial space and paranasal sinuses. A secondary orbital malignancy refers to a lesion arising from a surrounding structure which extends to the orbit by direct invasion. The primary tumor may arise in the paranasal sinuses, nasal cavity and nasopharynx, eyelid skin and face, eyeball, conjunctiva, lacrimal sac, or intracranial cavity [1]. Commonly encountered entities include epithelial tumors arising from the skin of the forehead, eyelid, and temple region, such as basal cell carcinoma and squamous cell carcinoma; carcinoma arising from glandular tissues of the eyelid such as sebaceous gland carcinoma (Fig. 43.1); carcinoma arising from the external surface of the eye such as conjunctival squamous cell carcinoma or melanoma; nasal cavity and paranasal sinus tumors (Fig. 43.2); and lacrimal sac malignancies (Fig. 43.3) [2].
Fig. 43.1
Left upper lid sebaceous gland carcinoma with orbital extension. There is a visible and palpable mass in the left upper eyelid and anterior orbit, with surrounding skin erythema and blepharoptosis. There is spread of tumor into the lower eyelid resulting in ectropion
Fig. 43.2
Left maxillary sinus carcinoma with orbital extension. The primary sinus mass is displacing the globe upward and causing visible fullness of the left lower eyelid
Fig. 43.3
(a, b) Frontal and sagittal views of squamous cell carcinoma, presumably originating from the left lacrimal sac, with orbital extension. A large mass is seen projecting from the inferomedial orbital area. The tip of the lesion has a scaly, necrotic appearance, consistent with the histopathologic diagnosis
In metastatic orbital disease, the primary malignancy originates at a remote site in the body and travels to the orbit via lymphatic or hematogenous spread. The most common sites of origin include the breast, lung, prostate, kidney, thyroid gland, and alimentary tract. Occasionally, metastasis from an unknown primary site may be encountered [3].
Clinical Presentation
The clinical presentations of various secondary orbital malignancies differ depending on the primary pathology. For example, paranasal sinus and nasal tumors may have rhinologic symptoms, such as epistaxis, nasal obstruction, swelling or pain in the cheek basal cell carcinoma of the skin may be characterized by an ulcerated lesion with rolled edges; sebaceous gland carcinoma can cause a whitish-yellow lesion of the eyelid; lacrimal sac tumors may cause blood stained tears. Orbital extension of these tumors can result in non-axial proptosis, impaired ocular motility, and globe or optic nerve compression. As these secondary tumors originate from surrounding structures, they may develop over a longer period of time and can be quite extensive in size [4].
The clinical presentations of metastatic disease are more variable and include [5–7]:
- 1.
Mass formation causing proptosis and globe or optic nerve compression
- 2.
Infiltrative disease causing ocular motility disturbance, resistance to retropulsion, and enophthalmos (especially in metastatic carcinoma of the breast)
- 3.
Cranial nerve palsies with functional symptoms such as diplopia
- 4.
External eyelid erythema, edema, and pain simulating periorbital inflammatory diseases (Fig. 43.4)
Fig. 43.4
(a, b) Diffuse left upper lid swelling with erythema and secondary blepharoptosis. CT scan showed diffuse infiltrative left upper lid lesion. Subsequent biopsy confirmed eyelid T cell lymphoma
- 5.
Asymptomatic orbital disease detected incidentally by imaging for another reason
Imaging and Investigations
Imaging
Both MRI and CT are highly useful in the diagnosis of secondary and metastatic orbital tumors. CT is best for identifying tumor calcification and the location and extent of bone involvement, while MRI has better soft tissue definition [8]. T1 contrast-enhanced fat-suppressed MRI will show enhancement of the dura if early intracranial extension is present. PET imaging, based on metabolic activity of tissues, can be useful in the assessment of suspected metastatic disease, by identifying involved lymph nodes or sites of potential malignancy elsewhere in the body [9]. Chest x-ray is a simple and quick test to look for pulmonary primary and secondary malignancies. Ultrasonography of the abdomen may be useful to look for primary liver malignancy or liver metastases, as well as major lesions within the abdomen. Hematologic studies and gastrointestinal endoscopy may be helpful when certain diagnoses are suspected. For patients with orbital masses and known systemic metastatic disease, the diagnosis may be simpler, but these studies may be helpful for disease staging. Consultation of colleagues, including oncologists, otolaryngologists, and other specialists, is helpful, depending on the clinical indications.
Role of Biopsy
In cases of secondary orbital tumors, surgical biopsy is usually required to determine the diagnosis [4]. Biopsy may be performed on the orbital portion of the tumor or on the portion of the mass in a surrounding structure, depending on the potential morbidities involved in operating on the various anatomic areas. For example, a sinonasal mass extending into the orbit may be best approached endoscopically via the nose for biopsy.
For orbital metastases with known primary disease or confirmed metastatic disease elsewhere, biopsy may not be needed. Nevertheless, when all investigations are noncontributory or no previous histopathologic diagnosis is available, an incisional biopsy is indicated; histopathologic studies may help identify the primary tumor site. Alternatively, fine-needle aspiration cytology can be considered [10].
Management of Secondary and Metastatic Tumors of the Orbit
The first step for the management of secondary and metastatic orbital tumors should be confirmation of the diagnosis. Even though the patient may arrive with a previously established diagnosis, there are times when additional tissue sampling may be required to confirm the exact nature of the disease. It is important to have a trusted ophthalmic pathologist review the slides, especially in cases where the histopathologic diagnosis is one that is often confused, such as sebaceous gland carcinoma. Proper use of immunohistochemical staining is critical in establishing proper diagnoses.
Various factors should be considered when deciding on curative versus palliative management of the lesion in question, including the age and general status of the patient, co-morbidities, and the histopathologic subtype and stage of disease.
For localized secondary orbital tumors, surgical resection can be considered. Orbital exenteration with resection of the primary lesion may be required. Total exenteration was traditionally the most common type of exenteration, but recently more selective procedures have gained popularity, such as subtotal or skin-sparing exenteration (Fig. 43.5). For extensive cases, surgical resection can be challenging and may involve a craniofacial resection (Fig. 43.6) via a multidisciplinary approach, including oculoplastic surgeons, head and neck surgeons, neurosurgeons, and plastic surgeons. Surgical reconstruction with local or free flaps may be required [11–13]. Recently, preoperative or neoadjuvant chemoreduction has become a possibility [14, 15]. Advanced localized disease is difficult to eradicate and has a high rate of recurrence and a high mortality rate. Postoperative adjuvant chemotherapy and/or radiotherapy may be required [16]. For those tumors highly sensitive to chemotherapy or radiotherapy, such as lymphoma, surgery beyond diagnostic biopsy is generally not required.
Fig. 43.5
Right eyelid-sparing orbital exenteration performed for squamous cell carcinoma of the conjunctiva
Fig. 43.6
Left combined maxillectomy and eyelid-sparing orbital exenteration for the case in Fig. 43.2 with squamous cell carcinoma of the maxillary sinus, 1 week after surgery
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