Treatment of Primary Lesion
Surgical excision is standard treatment. Clinically, surgical margins around the main lesion should be at least 1-2 cm due to high risk of local recurrence. This can make resection difficult in some anatomical areas (eg. periorbital, nasal, lip) without significant cosmetic deformity. If re-excision is not possible for involved or close margins, then adjuvant radiotherapy should be employed. Adjuvant radiotherapy is given for most cases to the surgical bed, with the exception of 1 cm tumours in immunocompetent individuals which do not exhibit lymphovascular invasion. The margin around the surgical bed is 4-5 cm.
Primary radiotherapy is an option for patients with lesions in difficult sites. Typically a margin of 5 cm is recommended. This can make treatment of facial sites difficult! Primary radiotherapy should be given to 60 Gy.
Treatment of Nodal Sites
Clinically involved nodes can be treated with surgical excision or with radiotherapy (60 Gy). Adjuvant nodal irradiation after surgery should be given in event of extranodal extension or a high number of involved nodes (50 Gy)
Sentinel lymph node biopsy is a popular technique; in patients with negative sentinel node biopsy the risk of malignancy is low and further excision can be avoided. In patients with positive sentinel nodes, lymphadenectomy or radiotherapy is indicated (60 Gy).
In patients without assessment of the sentinel nodes, and clinically negative nodes, radiotherapy should be delivered regardless to 50 Gy.
Chemotherapy
There is minimal evidence for chemotherapy in the adjuvant setting, but this is the subject of clinical trials.
Advanced disease is typically treated with carboplatin/etoposide (similar to small cell lung cancer regimens).
