neurofibromas are tumors of nerve sheath origin and may arise from schwann cells , perineural cells or fibroblasts . they present as individual tumors or in syndromic forms with multiple skin nodules , as in neurofibromatosis and other phakomatosis . the differentiating features include the presence of neurofibromatosis , nerve fibers , and more myxoid tissue ( antoni b tissue ) in the neurofibromas , while schwannomas are well capsulated , have a more compact matrix , a fascicular formation , and the presence of antoni a cells . in the head and scalp , they are more common in the intracranial compartment , or are present as plexiform neurofibromas . malignant degeneration with skull erosion is more common in intracranial lesions and those with background neurofibromatosis . giant forms of neurofibromas of the scalp with erosion of the skull have been documented in adults , and most have been in patients with neurofibromatosis or pre - existing plexiform neurofibromas . such an occurrence is rare in children . we report a case of a five - year - old boy , managed in our center , with a giant frontal neurofibroma with an extensive calvarial defect . a five - year - old boy presented with seven weeks history of a progressive and painless swelling on the right side of the forehead and temporal region [ figure 1 ] . examination revealed a young boy who was not pale , not febrile , had no lymphadenopathy , and there were no other features of neurofibromatosis or phakomatosis . he had a huge irregular right frontal swelling , which was painless , with no skin discoloration over it ; it measured 16  12 cm . it was multinodular with cystic and solid components , not tender , but fixed to the underlying skull [ figure 1 ] . the glasgow coma score was 15 , there were no cranial nerve deficits ; the motor and sensory systems were also normal . preoperative picture of the patient showing a large right frontal swelling laboratory investigations , which included hemogram , electrolytes , and urea , were all within normal limits . a preoperative brain ct scan showed a huge right frontal extradural , which was enhanced with contrast administration . it had a central cystic component and a large right frontotemporal skull defect [ figure 2 ] . preoperative contrast brain ct scan showing a huge extra axial tumor in the right fronto - temporal area , destroying the frontal bone with intra- and extracranial components . the finding at the surgery was a huge soft yellowish extradural tumor , with a cystic ( necrotic ) center . the tumor invaded the frontotemporal bone and extended to the right ethmoidal sinus and the right temporalis muscle . most of the tumor was , however , adherent to parts of the frontal bone . the patient had a one - stage bifrontal craniectomy , with complete tumor excision , and frontal cranioplasty with antibiotic impregnated methyl methacrylate [ figure 3 ] . postoperative treatment included intravenous ceftriaxone 100 mg / kg daily , intravenous phenobarbitone 5 mg / kg eight hourly , and intramuscular pentazocine 1 mg / kg eight hourly , for 48 hours . after removal of the pathological bone , cranioplasty with molded methyl methacrylate was carried out . the implant was kept in place with a nylon 1 suture the histology report was consistent with neurofibroma [ figure 4 ] . brain c t scan at eight weeks post surgery showed no residual tumor [ figure 5 ] . photomicrograph of the excised tumor , showing neurofibroma composed of proliferating spindle - shaped cells , with dark nuclei , disposed in a loose myxoid background . the rapidity of growth and site of occurrence were the other peculiarities of this case . fibrous dysplasia was a differential because of the patient 's age and location of the tumor . it was reported that rapid growth could have been because of acute , severe bleeding into the tumor or malignant transformation , but that was not the case in this patient . most reported cases of giant scalp neurofibroma were in japanese literature.[35 ] the reason for this was not clear because of the relatively small number of cases reported . skull defect with sclerotic margins was more suggestive of an underlying neurogenic tumor ; this patient had a rather osteolytic bony lesion . bone defects were more common in neurofibroma associated with neurofibromatosis.[35 ] the patient did not present with any stigmata of neurofibromatosis . the extent of the tumor and involved parts of the brain could be assessed with a contrast ct scan and magnetic resonance imaging . the aim of the surgery was a one - stage complete tumor excision together with the involved bony margins , and cranioplasty , to protect the brain in areas of bone loss . there may be a need for a second surgery in the future , to put an appropriately sized implant , when the child has reached adulthood . this is because the methyl methacrylate implant has a fixed size and will not adapt with the changing morphology of the child 's skull as the brain grows . other materials that have been used to close the skull defects include split autogenous calvarial bone , rib grafts , and titanium plates . the single stage procedure allowed the patient to undergo only one procedure rather than two or more staged operations and avoided several weeks with a substantial bony defect . one potential problem was the possibility of tumor regrowth in the presence of an implant .
neurofibroma of the scalp are mostly multiple as part of neurofibromatosis or other phakomatosis . 
 de novo solitary types are less common and rarely erode the skull , unlike the intracranial counterpart . 
 skull erosion has been reported in adults with longstanding plexiform neurofibromas . 
 we report a giant neurofibroma on the scalp of a five - year - old boy , managed in our center . 
 although this condition is a rare entity , it should be anticipated and the treatment strategy should include repair of the skull defect .