Head and Neck Neurothekeoma of Lower Lip with Aggressive Reconstruction using Bengt-Johanson’s Step Technique: Case Presentation and Systematic Review of Literature
Aim: Neurothekeoma is a benign lesion of superficial tissue mainly localized in the head-neck area. The aim of the article is to provide a systematic literature review on the tumor’s main characteristics and its correct surgical treatment. We support our theory with the report of an aggressive case of the lower lip region which required a large excision and reconstruction with local flaps. Materials and Methods: The literature review is based on the scientific materials produced from December 1980 to March 2020 on the head and neck neurothekeomas. In total, 76 papers were included in the study. We presented a classic neurothekeoma S-100 protein positive case that required wide local excision with healthy margins and reconstruction with Bengt-Johanson’s step technique. Results: Most of the papers were case reports (47,4 %) and reviews (19,7 %) with 721 evaluated neurothekeomas. The male to female ratio was 1:1,8 with a mean age of 26,4 years. The most frequent site of lesions was the head (36,1%), and the three subtypes were divided in cellular (65,7%) mixed (17,5%) and classic (16,8%). The classic neurothekeoma resulted more immunoreactive for S-100 Protein while cellular neurothekeomas for NKI/C3. Our case was successful without recurrence at 1 year follow-up. Conclusion: Our review highlights that neurothekeoma mainly occurs in young women in the superficial planes of the head and neck. The most frequent type is the cellular one, but the most aggressive is the classic type due to a high local recurrence of protein S-100. A local excision is sufficient for the cellular neurothekeoma, while for the classic type a wide local excision with healthy tissue margins is necessary. The treatment of our case demonstrates that by following this guide, relapse can be avoided.