title: Role of chemotherapy and molecularly targeted agents in the treatment of adenoid cystic carcinoma of the lacrimal gland.
abstract: Adenoid cystic carcinoma (ACC) is the most common malignant epithelial cancer of the lacrimal gland. Despite a slow rate of growth, ACCs are ultimately associated with poor clinical outcome. Given the rarity of this disease, most recommendations regarding therapy are guided by expert opinion and retrospective data rather than level 1 evidence. Surgery and postoperative radiation therapy are commonly used as initial local treatment. In patients at high risk of recurrence, concomitant platinum-based chemotherapy may be added to postoperative radiotherapy in an attempt to enhance radio-sensitivity. While encouraging responses have been reported with intra-arterial neoadjuvant chemotherapy, this strategy is associated with substantial toxicity and should be considered investigational. For patients with metastatic disease not amenable to surgery or radiotherapy, chemotherapy may have a role based on its modest efficacy in non-lacrimal ACC. Similarly, molecular targeted agents may have a role, although the agents tested to date in non-lacrimal ACC have been disappointing. A better understanding of the biology of ACC will be crucial to the future success of developing targeted agents for this disease.
authors.affiliation: Département d'Oncologie Médicale - Unité d'Investigation Clinique, Institut Curie, 26, rue d'Ulm - 75248 Paris Cedex 05, France. christophe.letourneau@curie.net
authors.forename: Christophe
authors.initials: C
authors.lastname: Le Tourneau
authors.forename: Albiruni R A
authors.initials: AR
authors.lastname: Razak
authors.forename: Christine
authors.initials: C
authors.lastname: Levy
authors.forename: Valentin
authors.initials: V
authors.lastname: Calugaru
authors.forename: Olivier
authors.initials: O
authors.lastname: Galatoire
authors.forename: Rémi
authors.initials: R
authors.lastname: Dendale
authors.forename: Laurence
authors.initials: L
authors.lastname: Desjardins
authors.forename: Hui K
authors.initials: HK
authors.lastname: Gan
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D003528:Carcinoma, Adenoid Cystic / Q000188:drug therapy* / Q000378:metabolism / Q000532:radiotherapy / Q000556:secondary; D017024:Chemotherapy, Adjuvant; D005134:Eye Neoplasms / Q000188:drug therapy* / Q000378:metabolism / Q000532:radiotherapy; D006801:Humans; D007766:Lacrimal Apparatus Diseases / Q000188:drug therapy* / Q000378:metabolism / Q000532:radiotherapy; D058990:Molecular Targeted Therapy / Q000379:methods*; D009363:Neoplasm Proteins / Q000037:antagonists & inhibitors / Q000378:metabolism
chemical_list: D000970:Antineoplastic Agents; D009363:Neoplasm Proteins