Current treatment of lacrimal gland carcinoma.
OBJECTIVE
The traditional treatment for lacrimal gland carcinoma is orbital exenteration followed by radiation therapy. However, orbital exenteration does not prevent distant relapse and death, and some patients experience local-regional recurrence after exenteration. More recently, eye-sparing surgery and adjuvant radiation therapy and chemotherapy have gained popularity in the treatment of lacrimal gland carcinoma. Preliminary studies show that these approaches are associated with reasonable local control rates and ocular toxicity profiles. In this review, we discuss recent studies of treatment of lacrimal gland carcinoma, including studies of potential molecular treatment targets.


RESULTS
Recent studies suggest promising results for neoadjuvant intra-arterial chemotherapy followed by orbital exenteration and adjuvant intravenous chemotherapy, but only in patients with an 'intact lacrimal artery'. Recent studies of globe-sparing surgery followed by adjuvant proton radiation therapy or concurrent chemoradiation suggest good local control, reasonable rates of eye preservation, and low risk of serious ocular toxic effects. Larger tumor size and predominant basaloid histology seem to be associated with higher risk of local-regional recurrence and distant metastasis. One study showed oncogenic mutations in more than half of cases of lacrimal gland adenoid cystic carcinoma, with KRAS mutations in 10 of 24 patients, suggesting potential benefit of treatments targeting the EGFR-RAS-RAF cascade.


CONCLUSIONS
In selected patients with lacrimal gland carcinoma, eye-sparing surgery with eye-sparing radiation therapy offers preservation of visual function with good local control and minimal radiation-induced ocular toxic effects. In patients with recurrent or metastatic disease, a search for actionable cancer-associated mutations may be prudent.
aDepartment of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine bDepartment of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea cOrbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Kyung In
KI
Woo
Yoon-Duck
YD
Kim
Ho-Seok
HS
Sa
Bita
B
Esmaeli
D003528:Carcinoma, Adenoid Cystic / Q000473:pathology / Q000628:therapy*; D059186:Chemoradiotherapy, Adjuvant; D003131:Combined Modality Therapy; D005134:Eye Neoplasms / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007765:Lacrimal Apparatus; D007766:Lacrimal Apparatus Diseases / Q000473:pathology / Q000628:therapy*; D013508:Ophthalmologic Surgical Procedures