The management of perineural spread of squamous cell carcinoma to the ocular adnexae

Purpose: To analyze a series of patients with periorbital perineural spread of squamous cell carcinoma and propose treatment guidelines. Methods: Retrospective, noncomparative, interventional case series of 17 patients with clinical, radiologic, or histologic evidence of distant perineural spread. Treatment, recurrence, progression, and mortality rates were recorded. Results: Numbness and pain were the most common symptoms, whereas ophthalmoplegia, ptosis, and facial palsy were the most frequent signs. All cases received wide-field radiotherapy to at least 50 Gy. Chemotherapy and surgery (biopsy, debulking, exenteration) were used in selected cases. Disease progression occurred in 6 patients, 4 of whom died. Median disease-free survival in the remainder was 37.5 months. Conclusions: The diagnosis of perineural spread is largely clinical and may be confirmed with imaging findings in the majority of cases. The role of biopsy is usually complementary but may be essential in some cases. In patients with evidence of distant perineural spread, radiotherapy volumes inclusive of potential antegrade and retrograde spread are recommended. Three-dimensional conformal planning or intensity-modulated radiation therapy assists in minimizing damage to adjacent structures. Synchronous chemotherapy should be considered to potentiate the effectiveness of radiation. The place of surgery in the treatment of perineural spread is palliative