Voluminous Plexiform Neurofibroma of the Foot in a Newborn - A Case Report Orthopedic-Traumatology Surgery Department - Batna Hospital Laboratory of Acquired and Constitutional Genetic Diseases (MAGECA) Faculty of Medicine -Batna 2 University

Abstract

Neurofibromas are the cardinal dermatological signs of neurofibromatosis type I or Von Recklinghausen disease; also, they are benign tumors of the nerve sheaths. We can distinguish three types: cutaneous neurofibromas, subcutaneous neurofibromas and plexiform neurofibromas.1 Concerning the last type “plexiform neurofibroma (PNF)”, it arises as a diffuse mass from the nerve trunk and its branches habitually extending to the soft parts, the fat, muscles, and adjacent cutaneous and subcutaneous tissue. It affects 20-40% of individuals with neurofibromatosis type I.2 The diagnosis is based on history, physical examination, imaging and especially the anatomicopathological study. Histologically, it corresponds to a fibromyxoid proliferation of the nerve sheath and it is composed of Schwann cells, perineural cells, mast cells, and fibroblasts.2,3 Plexiform neurofibroma has two major complications, the first one is a higher risk of degeneration that is produced in approximately 10%4 and the second one is the overgrowth with its locoregional side effects. Thus, to avoid these two complications, the treatment can only be carcinologic, which means a complete resection of the tumor. In this paper, we report a rare case of a voluminous congenital plexiform neurofibroma in a 7-month-old infant at the level of his left foot, to whom a surgical resection was performed.

Keywords

• Neurofibromatosis type 1
• taches café au lait
• Plexiform Neurofibroma
• foot
• baby