SLN biopsy is feasible for eyelid and conjunctival tumors, and continued use of the procedure is recommended. Future multi-institutional trials are needed to expand on currently available data, fine-tune patient selection criteria, and elucidate the relationships between SLN status and patient survival and tumor recurrence.
SLNB in patients with ocular adnexal melanoma is safe and identifies nodal micrometastasis in approximately 20% of cases. Histologic features associated with a positive SLN included greater tumour thickness, greater number of mitotic figures and ulceration.
Purpose
To review the literature on targeted therapy for orbital and periocular basal cell carcinoma (BCC) and cutaneous squamous cell carcinoma (SCC) and provide examples of patients recently treated with such therapy.
Methods
We reviewed the literature on clinical results of targeted therapy and the molecular basis for targeted therapy in orbital and periocular BCC and cutaneous SCC. We also present representative cases from our practice.
Results
Mutation in the patched gene (PTCH1) has been implicated in BCC, and overexpression of epidermal growth factor receptor (EGFR) has been shown in SCC. Vismodegib, an inhibitor of smoothened, which is activated upon binding of hedgehog to Ptc, has been shown to significantly decrease BCC tumor size or even produce complete resolution, especially in cases of basal cell nevus syndrome. Similarly, EGFR inhibitors have been shown to significantly decrease SCC tumor size in cases of locally advanced and metastatic disease. We describe successful outcomes after vismodegib treatment in a patient with basal cell nevus syndrome with numerous bulky lesions of the eyelid and periocular region and erlotinib (EGFR inhibitor) treatment in a patient with SCC who was deemed not to be a good surgical candidate because of advanced SCC of the orbit with metastasis to the regional lymph nodes, advanced age, and multiple medical comorbidities.
Conclusion
Targeted therapy using hedgehog pathway and EGFR inhibitors shows significant promise in treatment of orbital and periocular BCC and cutaneous SCC, respectively. Such targeted therapy may be appropriate for patients who are not good candidates for surgery.
Accurate and reliable staging methods are crucial for optimal care of patients with ocular and orbital malignancies. Positron emission tomography/computed tomography (PET/CT) has recently emerged as a staging tool in the field of ophthalmic oncology. For detecting primary ocular or orbital lesions, PET/CT does not seem to provide an advantage over clinical ophthalmologic examination or conventional imaging studies such as CT or magnetic resonance imaging of the orbit. However, PET/CT may detect distant metastatic lesions that conventional imaging studies miss. For orbital and ocular adnexal lymphoma, use of PET/CT has been proven to be feasible and is now accepted both as a standard part of the initial staging work-up and for the assessment of response to therapy. For other ophthalmic tumors, PET/CT seems most appropriate for advanced metastatic tumors of the orbit, eyelid, and eye, for which the detection of distant metastasis with 1 comprehensive study may be preferable to performing multiple CT scans with contrast.
A variety of chemotherapeutic agents have been reported to cause epiphora, and some of these drugs have also been documented to cause obstructions of the lacrimal drainage system. Early recognition and management of epiphora is important and leads to better outcomes.
The objective of this study was to assess the value of magnetic resonance imaging (MRI) of the orbit for conjunctival mucosa-associated lymphoid tissue (MALT) lymphoma. The yield of other staging tests at baseline were also evaluated. Twenty-one consecutive patients treated for conjunctival MALT lymphoma were retrospectively studied. Lymphoma was staged according to both the Ann Arbor system and the seventh edition of the AJCC [American Joint Committee on Cancer] cancer staging manual. Findings on MRI of the orbit, whole-body positron emission tomography/computed tomography (PET/CT), CT of the chest/abdomen/pelvis, bone marrow (BM) biopsy and gastrointestinal (GI) endoscopy were recorded. Seventeen patients had orbital MRI. Fourteen of 17 patients (82%) with obvious conjunctival MALT lymphoma on clinical examination had a negative MRI scan. Only three patients had subtle conjunctival enhancement on orbital MRI. Ann Arbor stage at presentation was as follows: stage IE (15 patients), stage IIE (two patients) and stage IV (four patients). Eighteen of 21 patients had total-body PET/CT; four patients (22%) had hypermetabolic activity evident on PET scan. All 21 patients had bilateral BM biopsies. Fifteen of 21 patients (71%) had GI endoscopy. None of the patients had a positive BM biopsy or findings on GI endoscopy. Our data suggest that orbital MRI has a very low yield for identification of conjunctival MALT lymphoma. Clinical examination is critical in diagnosing and assessing treatment response in conjunctival MALT lymphoma. The yield for GI endoscopy and BM biopsy may also be low in staging of conjunctival MALT lymphoma.
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