Appeal type: Experimental/Investigational
Decision: Upheld

Physician 1:  This patient is a 42-year-old male with a previous diagnosis of myoepithelial sarcoma of the paranasal sinus. He has apparently been treated with surgery, radiation, and multiple chemotherapeutic agents including Cisplatin, Ifosfamide, Taxol, Carboplatinum, Adriamycin, and Temodar. He has also been treated with Cyberknife radiotherapy.  The patients tumor was found positive for the epithelial growth factor (EGF) receptor and it is proposed that he be treated with Erbitux.  The providers request for authorization of Erbitux was denied by the Health Plan citing the experimental nature of the therapy.Erbitux is one of a new class of drugs that inhibits the EGF receptors on a cancer cell and presumably interferes with angiogenesis. Erbitux has been approved by the FDA for use, along with chemotherapy, in the treatment of colon cancer. Additionally, there is one randomized study demonstrating that, when combined with radiation, in the primary treatment of squamous cell cancer of the head and neck, Erbitux does appear to increase survival.  There is however, no data available that is applicable to this patients case.  He does not have the same histology as the patients in the randomized study and the proposed treatment is not a primary therapy. Though his tumor does stain for the EGF receptor, this finding does not correlate with a demonstrated response to the drug. Accordingly, the use of Erbitux is not likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. The Health Plans denial should be upheld. Physician 2:  The patient is a 42-year-old with clinically aggressive and recurrent myoepithelial carcinoma of the sinus and orbits. The patient has recently taken Temodar (temozolomide) and undergone Cyberknife radiotherapy to multiple areas of the scalp and left orbit. His provider has prescribed Erbitux, based on the 2+ expression of EGFR on the majority of cells in a biopsy sample and submitted a request for authorization.  The Health Plan denied authorization indicating there is insufficient evidence in the peer-reviewed literature of the safety and utility of Erbitux therapy for metastatic myoepithelial cancer.This patient has a rare disease characterized by soft tissue myoepithelial carcinoma involving the critical structures of the face.  The bulk of the scientific and peer-reviewed data concerning myoepithelial carcinoma is in the breast cancer literature, where myoepithelial carcinoma features form a subset of clinically aggressive breast carcinomas. Similarly affected soft tissue structures of the head and neck (tongue, salivary glands) are rare, but have been sited in case reports. Of note, EGF receptor over expression is also a feature of this histologic subset of carcinoma from breast origin, and denotes clinically aggressive disease relative to other subtypes of cancers.The standard treatments that were available to this young patient have been exhausted, and therefore the choices that remain are supportive care or experimental/investigational approaches undertaken with strong clinical rationale and a reasonable safety index.  In this regard, Erbitux therapy seems appropriate. It is unclear whether chemotherapy is planned in conjunction with Erbitux; Erbitux monotherapy in most clinical scenarios has only modest activity compared to combination therapy.  However, given the aggressive and recurrent nature of the patients disease and the generally acceptable toxicity profile, the possibility of even modest activity is more than standard therapies can offer.Based upon the information set forth above, I have determined that the requested therapy is likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. The Health Plans denial should be overturned.Physician 3:  The patient is a 42-year-old male with myoepithelial carcinoma of the paranasal sinuses. This tumor arose in the left maxillary sinus and orbit with multiple subsequent recurrences. The patient has undergone resections, radiation and different courses of chemotherapy.  Most recently he received his first cycle of Temodar and underwent Cyberknife treatment to his left orbit and subcutaneous nodules on his scalp. The patients provider indicates the tumor is EGF receptor positive and has recommended the patient receive Erbitux therapy.  The Health Plan has determined that the use of Erbitux remains unproven as a treatment for metastatic myoepithelial carcinoma and therefore has denied coverage for the therapy.  In the treatment of cancers of the head and neck region there is current optimism with regard to the benefits of Erbitux monoclonal antibody therapy for patients with squamous cell cancers.  These clinical trials have expressly excluded other tissue types of head and neck cancer, and therefore, the results of these trials should not be extrapolated to other types of cancer. At this time there is insufficient peer-reviewed scientific literature to support the use of Erbitux for the treatment of metastatic myoepithelial cancer of the paranasal sinuses. Because of the multiple side effects of Erbitux, such clinical experience should be gathered only in the setting of carefully designed clinical trials.  Therefore, I have determined that the requested therapy is not likely to be more beneficial for treatment of the patients medical condition than any available standard therapy. The Health Plans denial should be upheld.