Surgical Skin Tumors in Neurofibromatosis 1 (NF1) at the Dakar Cancer Institute
Sidy KA Department Of Oncology, Dakar Cancer Institute, Senegal
Published on: 2018-11-02
Objectives: To evaluate the epidemiological, diagnostic and evolutionary aspects of surgical skin tumors associated with neurofibromatosis type 1, the cancer institute of Dakar. Patients and Methods: We performed a retrospective study of six (06) years (July 2009 - December 2015) on surgical skin manifestations in Neurofibromatosis 1 (NF1). Results: There were 8 cases of Nf1 according to the bethesda criteria associated with skin tumors requiring surgical management. We found 6 women and 2 men. The average age was 35.4 years. We noted 05 cases (71.4%) of royal tumors and 03 cases (28.5%) of malignant tumors. In functional terms, 1 case of neurofibroma obscuring it was found. We conducted two shoulder disarticulation and 1 mastectomy. neo adjuvant radiotherapy was performed in a case of axillary tumor. After a 48-month decline, 1 case of malignant nerve sheath died. Conclusion: Surgical skin tumors in nf1 are rare. Surgery plays an important functional role in the royal tumors and is very mutilating in malignant tumors.
Neurofibromatosis Type 1; Surgery; Skin tumors
Neurofibromatosis type 1 (NF1) or Von Recklinghausen disease is a genetic condition with autosomal dominant transmission. Signs of Von Recklinghausen's disease are many and skin tumors represent an important part. The majority of these tumors are benign, but what make the severity of NF1 is the large tumors or royal tumors and the high frequency of malignant nerve sheath tumors.