Effectiveness of Molecular?Targeted Chemotherapy in Ameloblastomas: A Systematic Review

Ameloblastoma is a benign, locally aggressive neoplasm that needs extensive surgical resection. The goal of this article is to obtain an in?depth review of benign ameloblastomas to determine the available level of evidence and the possible benefit of targeted therapeutics for the treatment of ameloblastoma and BRAF V600E mutation in ameloblastoma. An electronic literature search was conducted according to PRISMA guidelines in PubMed/MEDLINE, EBSCO, and Web of Science for eligible studies published between 1975 and 2021. The systematic review is registered with INPLASY (INPLASY202260018). The review included 2 case series and 17 case reports. The histopathological type, anatomic location, expression of BRAF mutation, additional mutations, and molecular?targeted therapies of the 19 reviewed articles were summarized and tabulated. Interestingly, the majority of the primary site of ameloblastoma was located in the mandible (80.9%) compared to the maxilla (17%). The tumour size was reported in nine of the included studies. Most of the included studies in the review exhibited ameloblastoma with BRAF V600E mutations and responded to molecular?targeted therapies. Molecular therapies employing BRAF and/or MEK inhibitors in ameloblastoma with BRAF V600E mutations proved to be an appropriate treatment based on the limited available evidence. It is essential further to deepen our understanding at th

Logic and clinical significance of the new WHO classification of ameloblastoma in 2017 / 口腔疾病防治

@#A substantial revision of the classification of ameloblastoma was made by the World Health Organization (WHO) in the fourth edition of the Classification of Head and Neck Tumors in 2017, which was based on the review and summary of much clinical research data and prospective evaluation of the latest results of genetic research. The new classification is simpler and more practical. It retains two subtypes, the unicystic type and extraosseous/peripheral type, classifies the remaining types as ameloblastoma (classic), defines metastatic ameloblastoma as a benign tumor and simplifies the classification of ameloblastic carcinoma, which has important guiding significance for clinical diagnosis and treatment. Moreover, the new classification included the latest advances in the genetic research on ameloblastoma, demonstrating that the BRAF gene mutation was found in approximately 60% of ameloblastoma cases. The classification provides a new concept and direction for studying the pathogenesis of ameloblastoma, and BRAF-targeted therapy may be an emerging therapy for some ameloblastoma patients with multiple recurrence or surgical contraindications. This article analyzes the intrinsic logic of these changes via a review of the relevant literature and combination of clinical experiences to better understand the new classification. In 2017, the WHO′s new classification of ameloblastoma summarized the experience and achievements in histopathology and clinical treatment of ameloblastoma in the prior 10 years, indicating that BRAF-targeted treatment may bring new treatment options and hope for patients with recurrent or inoperable ameloblastoma.

Mandibular ameloblastoma with lung metastasis 10 years after resection

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To present a case of mandibular ameloblastoma with pulmonary metastasis after ten years and discuss the possible pathophysiology, diagnostic and therapeutic options.<br /><strong>METHODS:</strong><br /><strong> Design:</strong> Case Report<br /> <strong>Setting:</strong> Tertiary Private Hospital<br /><strong> Patient:</strong> One<br /><strong>RESULTS:</strong> A  27-year-old  woman  diagnosed  with  follicular  variant  ameloblastoma  underwent left  segmental  mandibulectomy  with  iliac  bone reconstruction  in  2004.  The  titanium  plates were  removed  in  2008  because  of  a  recurrent  orocutaneous  fistula.  She  was  apparently  well until 2014, when she complained of intermittent, non-radiating, sharp and piercing, right upper back pains. Work-ups revealed multiple bilateral lung nodules.  A CT scan-guided percutaneous needle  biopsy  of  the  right  upper  lung  nodule  revealed  metastatic  ameloblastoma.  Opting  for observation  instead  of  chemoradiation,  she  remains  asymptomatic  on  regular  follow-ups  with medical oncology, pulmonary medicine and otorhinolaryngology.<br /><strong>CONCLUSION:</strong> Though  benign,  ameloblastoma  has  a  high  propensity  for  local  invasion  and  may metastasize.  It  is  difficult  to  predict metastasis, even  with  adequate  treatment  of  the  primary lesion. There is no standard protocol to prevent or detect metastatic ameloblastoma, but regular and close follow up may ensure early diagnosis.</p>