She otherwise had normal growth and development of the right leg. No recurrence was found at 12-year follow-up. Although slight contour asymmetry persists, the bone flap has grown much like the native mandible and the patient has no trismus or difficulties with mastication (Figs. 5A–5C). Melanotic neuroectodermal tumor is a rare entity, with sporadic case reports and series in the literature. Less than 400 cases have been reported to date. First described

in 1918, 90% of the cases are seen Selleckchem Vemurafenib in the head and neck region, with the maxilla being the most affected (68.8%). It is accepted to be of neuroectodermal origin, and as a melanin producing tumor, it produces a blue or black, solid, rapidly growing mass, firmly adhered to the bone. Local excision, with total removal of the mass and curettage of the cavity is the adequate treatment of this benign tumor, but a 10–15% recurrency rate and a 3.2% risk of malignancy have been reported in the literature.[2, 3] In the case reported here, the mass was proportionally large, and a complete resection of the affected bone was preferred for adequate treatment. The feasibility of microsurgical reconstruction in children is no longer a discussion, and although technically challenging, the debate has shifted to evaluating the functional outcome of the reconstructed segment.[4, 5] One particular

concern with these complex reconstructions is how the transplanted tissue will respond to the continuous growth Tyrosine Kinase Inhibitor Library cost of the surrounding structures. We were successful in obtaining near normal growth of the neo-mandible in this case. In adults, the harvest of a fibula free flap does not produce significant function morbidity to the donor leg.[6] In a recent report of 18 fibula flaps used for pediatric mandibular reconstruction,[7] the authors state that the flap would not grow concomitantly with the child. These authors

preserved at least 6 cm of the distal fibula at the donor Edoxaban site in an effort to maintain ankle stability. They were successful in preventing ankle deformities in all of their patients, but other procedures were necessary to correct the length of the transplanted bone. In this case, a long segment of the fibula diaphysis had to be harvested due to the extent of the defect. The proximal and distal ends of the diaphysis of long bones are the regions where most of the bone longitudinal growth occurs through endochondral ossification. We believe that incorporating a more distal segment of the bone into the flap is probably the reason for the continuous growth of the flap and the ankle deformity at the donor site in our case. Other authors have reported similar donor site complications, requiring corrective orthopedic procedures.[8, 9] Interestingly, the flap presented with the expected growth of the mandible segment it replaced. We believe that the same stimulus of the surrounding bone structures and soft tissue that would modulate mandibular growth affected the flap.