Orbital Oncology & Ophthalmic Plastic Surgery
Orbital Oncology and Ophthalmic Plastic Surgery is a subspecialty that focuses on the surgical treatment of tumors of the eye, orbit, eyelid, conjunctiva and other ocular adnexal structures, as well as reconstructive surgery in the periocular region in cancer patients. At MD Anderson, the primary goals of this subspecialty are surgical elimination of ocular and orbital cancers while preserving the eye and vision, and restoring function and cosmesis in the periocular region.
In this subspecialty within the Plastic Surgery departmetnt, Bita Esmaeli, M.D., professor of Ophthalmology, sees approximately 350 new patients and consultations each year. Among these are patients with rare cancers of the ocular and orbital area that require multidisciplinary expertise offered by departments across MD Anderson. Specific diseases and cancers treated by physicians in this subspecialty include conjunctival tumors, eyelid cancers, lacrimal gland tumors, orbital tumors and uveal melanoma.
Dr. Esmaeli sees patients in the Ophthalmology Clinic at MD Anderson. For new patient referrals please contact: 713-792-4457.
There are several clinical trials and protocols that are currently underway which are focused on cancers of the eye, orbit, and ocular adnexa are actively recruiting patients. These include:
- Sentinel lymph node biopsy for Conjunctival and Eyelid Melanoma (GSP00-106)
- Blood and Tumor Banking for Uveal Melanoma (Lab 02-693)
- Sentinel lymph node biopsy for sebaceous carcinoma of eyelid and conjunctiva (2008-0266)
- In vivo confocal microscopy for conjunctival melanoma (2010-0330)
- Low-dose radiation therapy for orbital and ocular adnexal lymphoma.
Other research efforts include the use of genomic testing to explore personalized targeted treatments for patients with orbital and ocular cancers; adjuvant radiation therapy for eyelid and orbital cancers after globe preserving surgery; sentinel lymph node biopsy for eyelid and conjunctival tumors to detect early metastasis; and the development of multi-institutional clinical databases for rare cancers of the ocular and orbital region.
Until recently, the treatment of lacrimal gland carcinoma almost
always required removal of the eye. But for some patients with these
tumors, globe-sparing surgery followed by radiation therapy can
preserve vision and decrease ocular morbidity and facial
Carcinomas of the lacrimal gland, although rare, are associated with a high risk of recurrence, perineural invasion, and distant metastasis. About half of patients eventually die of their disease despite treatment.
Treatment for lacrimal gland carcinoma historically entailed orbital exenteration (removal of the eye and all orbital contents), usually followed by high-dose radiation therapy to address perineural invasion or close surgical margins. Surgeons were reluctant to preserve the eye because of concerns that the eye would be severely damaged by the radiation therapy.
In recent years, however, the routine use of orbital exenteration for lacrimal gland carcinoma has been called into question by efforts led by Bita Esmaeli, M.D., a professor of ophthalmology and the director of orbital oncology and ophthalmic plastic surgery at The University of Texas MD Anderson Cancer Center. Retrospective reports from MD Anderson and other centers found poor survival outcomes despite local control of the tumors in patients who underwent orbital exenteration. “Orbital exenteration has not shown any survival benefit,” Dr. Esmaeli said.
The globe-sparing approach
MD Anderson physicians led by Dr. Esmaeli and Steven Frank, M.D., an associate professor in the Department of Radiation Oncology and the medical director of the Proton Therapy Center, use a multidisciplinary globe-sparing approach to manage lacrimal gland carcinoma in selected patients while striving to preserve vision and cosmesis.
This approach involves globe-sparing surgery followed by high-dose adjuvant radiation therapy. Chemotherapy may be given along with radiation to patients with positive surgical margins or recurrent disease.
Dr. Frank said, “With exenteration, a functioning organ is removed because of an adjacent tumor. Our goal is to preserve the eye and its function through surgery and radiation therapy.”
The extent of surgery depends upon the tumor’s location in the lacrimal gland, which comprises the orbital lobe and the palpebral lobe, and the involvement of the orbital soft tissue. For most patients, a significant amount of the orbital lobe must be removed; some patients must also undergo resection of the palpebral part of the gland, which is closest to the eye. Dr. Esmaeli said, “To achieve the goal of resecting the lacrimal gland cancer but preserving the eye and functionally important tissues in the orbit as much as possible, the orbital surgeon must have experience with meticulous oncologic resection.”
The globe-sparing approach requires close collaboration among the orbital surgeon, medical oncologist, and radiation oncologist. This collaboration is especially important in radiation treatment planning, as the treatment field must cover the postoperative surgical bed and often must extend to the base of the skull, toward the brain stem, to prevent perineural invasion and disease recurrence.
In October 2013, I underwent surgery in Arizona to remove a tumor in my left eye. Waking up after surgery, I vividly remember asking my surgeon if he’d been able to remove the entire tumor.
I immediately understood what his “no” meant, and a biopsy confirmed what we all feared: I had adenoid cystic carcinoma of the lacrimal gland, a rare and aggressive form of eye cancer.
My doctor hadn’t treated many adenoid cystic carcinoma...
Tumors of the eyelid and conjunctiva vary in histology and extent of disease, and which treatments, if any, are needed after surgical excision depends on several factors, including the potential for metastasis.
To help guide treatment decisions, physicians at The University of
Texas MD Anderson Cancer Center are using innovative techniques such
as sentinel lymph node biopsy (SLNB) to refine the staging of these
tumors and are conducting molecular studies of rare tumors to improve
the personalized treatment of patients with eyelid and conjunctival
“Treating cancers of the eyelid and conjunctiva requires a multidisciplinary effort that includes surgeons, nuclear radiologists, pathologists, and radiation and medical oncologists,” said Bita Esmaeli, M.D., a professor of ophthalmology and the director of the Orbital Oncology and Ophthalmic Plastic Surgery Fellowship Program at MD Anderson.
Dr. Esmaeli said that the first-line treatment for eyelid or conjunctival tumors is almost always surgical excision, although radiation therapy may be used as the first-line treatment if surgery is contraindicated. In patients whose tumors are excised, decisions about postoperative treatment are determined by factors such as the type of cancer and the risk of lymph node metastases.
Surgery and reconstruction
Many eyelid and conjunctival tumors are caught early; such tumors often can be excised and the wound repaired using oculoplastic surgical techniques with little or no loss of eye function. Neither enucleation nor orbital exenteration has been shown to improve survival outcomes, so these procedures are unnecessary unless there is orbital extension of the tumor. However, large tumors can endanger function and require extensive reconstruction, and surgeons at tertiary referral centers often encounter such difficult-to-treat cases. “We see rare cancers of the eyelid at an advanced stage, where it is questionable whether we can salvage the eye and its function. Such cases are where complex reconstructive procedures and an innovative but cautious multidisciplinary approach can make a big difference,” Dr. Esmaeli said.
At MD Anderson, reconstructive surgery typically is performed immediately after the tumor is excised. A tumor’s size and location determine the techniques used for reconstruction. “Basal cell carcinoma most often occurs in the lower eyelid, and for the larger tumors the wound from excision is repaired with a tarsoconjunctival flap [Hughes flap]—often with excellent functional and cosmetic outcomes,” Dr. Esmaeli said. “But the more rare cancers—sebaceous carcinoma, melanoma, and Merkel cell carcinoma—tend to occur in the upper eyelid, which is more difficult to reconstruct. Patients with these tumors are more likely to come to a referral center, and as a result we end up doing more of these unusual, upper eyelid reconstructions compared with other settings. Eyelid sharing procedures such as Cutler-Beard bridge flaps are often needed to reconstruct large upper eyelid defects.”
Conjunctival carcinomas and melanomas, which can occur in either the bulbar or palpebral region, typically require microscopic ocular surface reconstruction and sometimes the use of amniotic membrane grafts. Special handling of conjunctival surgical specimens by the pathologist and communication between the eye surgeon and the pathologist are very important for correct diagnosis of conjunctival melanomas and carcinomas and for determining the patient’s prognosis.
For tumors that have a significant risk of regional lymph node metastasis—such as conjunctival or eyelid melanomas that are thicker than 1 mm or demonstrate other high-risk histologic features such as ulceration or high mitotic figures, sebaceous carcinomas of the eyelid, or Merkel cell carcinomas of the eyelid—SLNB is typically done at the time of primary tumor excision.
Sentinel lymph node biopsy
SLNB entails the removal and examination of one or two sentinel lymph nodes (i.e., the draining lymph nodes nearest the tumor) to determine whether they contain metastatic disease. The use of SLNB for eyelid and conjunctival cancers was developed and modified over the past 15 years by Dr. Esmaeli and her colleagues at MD Anderson—including Merrick Ross, M.D., a professor in the Department of Surgical Oncology, and Jeffrey Myers, M.D., Ph.D., a professor in the Department of Head and Neck Surgery. The technique has now been adopted at other centers, mostly outside the United States.
“The chief benefits of SLNB are accurate staging and early diagnosis of metastatic disease. In melanomas and other cutaneous tumors of the eyelid and conjunctiva with a significant risk of nodal metastasis, it is important to identify micrometastases early rather than wait for them to get big enough to be palpable on a physical exam or show up on imaging,” Dr. Esmaeli said. “If we can find metastasis early, we can offer treatments earlier.”
Several decades ago, studies were done of elective neck dissection in patients with head and neck cutaneous melanoma. The procedure was abandoned for patients with no signs of metastasis because the morbidity of such major surgery was not justified by the small proportion of patients who were found to have positive lymph nodes.
Another drawback of elective neck dissection is the number of lymph nodes that must be examined. “The techniques we use to examine lymph nodes are exhaustive and expensive,” said Victor Prieto, M.D., Ph.D., a professor in and chair of the Department of Pathology. “It would be unfeasible to apply them to up to 30 lymph nodes that can be retrieved in a procedure to remove all the lymph nodes of an anatomic region. With SLNB, by examining only the nodes that are most likely to be positive, we can increase our sensitivity.”
Sentinel lymph node(s) typically are removed at the time of primary eyelid/conjunctival tumor excision, although SLNB can also be done later. Before surgery, a radioactive tracer such as technetium-99m is injected into the tissue around the tumor. Preoperative lymphoscintigraphy may be performed to determine which of the region’s multiple nodal basins contains the sentinel node(s). Lymphoscintigraphy can be especially helpful in patients with scarring from prior surgery or radiation therapy.
To remove the sentinel lymph node(s), the surgeon first uses a gamma probe to locate the tracer taken up by the sentinel node(s). A small incision is made, and the sentinel nodes(s) are removed and sent to pathologists for examination.
Pathologists cut the lymph node into multiple slices, like a bread loaf, rather than in half longitudinally. “In 2002 we proved that the two techniques are equivalent,” Dr. Prieto said. “The advantage of the bread loaf technique is that you can put more tissue in one cassette, so it’s less expensive and requires less time to process.”