alt While chemotherapy, radiation therapy, curettage and liquid nitrogen have been rarely effective in cases of<unk> , surgical resection or enucleation remains the most definitive treatment for this condition. However, in a detailed study of 345 patients, chemotherapy and radiation therapy was contraindicated for the treatment of<unk> s. Thus, surgery is the most common treatment of this neoplasm. A case of giant<unk>  was recently reported and managed with total mandibulectomy and pectoralis major myocutaneous flap reconstruction. A systematic review found that 79% of desmoplastic<unk>  cases were treated by resection. Conservative treatment requires very careful case selection. Surgical resection The aim of treatment and surgery is to remove the entire tumour with a margin of surrounding tissue (block resection) for a good prognosis. Preferable removal includes 10mm of normal bone around the neoplasm. Larger<unk> s can require partial resection of the jaw bone followed by bone grafting. There is evidence that the treatment of conventional<unk>  is best done by bone resection. A systematic review found that 79% of desmoplastic<unk>  cases were treated by resection. Enucleation Smaller mandibular neoplasms have been enucleated where the cavity of the tumour is curetted, allowing preservation of the bone cortex and the lower border of the mandible. Although, recurrence rate for this type of treatment is higher. Unicystic<unk> s—called intraluminal unicystic or plexiform unicystic<unk> s can be enucleated, as the epithelium is only limited to the inner cyst wall and lumen. Radiation and chemotherapy Radiation is ineffective in many cases of<unk> . There have also been reports of sarcoma being induced as the result of using radiation to treat<unk> . Chemotherapy is also often ineffective. However, there is some controversy regarding this and some indication that some<unk> s might be more responsive to radiation that previously thought. Follow-up and recurrence Persistent follow-up examination including radiographs is essential for managing<unk> . Follow-up should occur at regular intervals for at least 10 years. Follow up is important, because 50% of all recurrences occur within 5 years postoperatively. Recurrence is common, although the recurrence rates for block resection followed by bone graft are lower than those of enucleation and curettage. Follicular variants appear to recur more than plexiform variants. Unicystic lesions recur less frequently than "non-unicystic" lesions. A low recurrence rate of around 10% can be seen in unicystic<unk> s. Recurrence within a bone graft (following resection of the original tumor) does occur, but is less common. Seeding to the bone graft is suspected as a cause of recurrence. The recurrences in these cases seem to stem from the soft tissues, especially the adjacent periosteum. Recurrence has been reported to occur as many as 36 years after treatment. To reduce the likelihood of recurrence within grafted bone, meticulous surgery with attention to the adjacent soft tissues is required. Molecular biology BRAF V600E gene and SMO gene mutations have been found in<unk> s. V600E mutation is also seen in other malignant and benign neoplasms, which activate the MAP kinase pathway required for cell division and differentiation but is the most commonly seen mutation in<unk> . 72% of BRAF mutations are found in the mandible. A recent study discovered a high frequency of BRAF V600E mutations (15 of 24 samples, 63%) in conventional<unk> . These data suggests drugs targeting mutant BRAF as potential novel therapies for<unk> . SMO mutations lead to the activation of the hedgehog pathway giving similar results as V600E but is less frequently seen. 55% of SMO mutations are found in the maxilla. Evidence shows that suppression of matrix metalloproteinase-2 may inhibit the local invasiveness of<unk> , however, this was only demonstrated in vitro. There is also some research suggesting that α5β1 integrin may participate in the local invasiveness of<unk> s.
Ameloblastoma
Medulloblastoma
Hemangioblastoma
Sialoblastoma
Neurothekeoma
Glioblastoma
Pineoblastoma
Neuroma