RESUMO
Juvenile aggressive ossifying fibromas (JAOF) are rare, typically benign pediatric tumors that are locally aggressive and have high recurrence rates. A 7-year old male presented with a palatal mass and a 3D printed model was created and used as a visual aide to highlight the importance of management in terms of functional, cosmetic, and disease-free outcomes with the family. The patient ultimately underwent successful enucleation with final pathology consistent with JAOF. To our knowledge, this is the first description of the use of 3D printing to help in the shared decision-making process for the treatment of this aggressive tumor.
Assuntos
Neoplasias Ósseas/patologia , Tomada de Decisão Compartilhada , Fibroma Ossificante/patologia , Palato Duro/patologia , Impressão Tridimensional , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/cirurgia , Criança , Fibroma Ossificante/diagnóstico , Fibroma Ossificante/cirurgia , Humanos , Masculino , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Palato Duro/diagnóstico por imagem , Doenças Raras , Medição de Risco , Resultado do TratamentoRESUMO
Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor of the jaws that is more common in the mandible than maxilla and has a female preponderance with a peak incidence in the sixth decade. It is characterized by locally aggressive behavior and has the potential to metastasize. This tumor was recently reported to have a rearrangement of the Ewing sarcoma breakpoint region 1 gene (EWS RNA-binding protein 1, EWSR1) in 5 of 8 cases tested and of the activating transcription factor 1 gene (ATF1) in 1 case tested. We report a case of CCOC in the premolar area of the mandible in a 59-year-old woman. This case demonstrated the presence of both EWSR1 and ATF1 gene rearrangements by fluorescence in situ hybridization.
Assuntos
Adenocarcinoma de Células Claras/genética , Adenocarcinoma de Células Claras/cirurgia , Proteínas de Ligação a Calmodulina/genética , Neoplasias Mandibulares/genética , Neoplasias Mandibulares/cirurgia , Tumores Odontogênicos/genética , Tumores Odontogênicos/cirurgia , Proteínas de Ligação a RNA/genética , Adenocarcinoma de Células Claras/diagnóstico , Adenocarcinoma de Células Claras/patologia , Biópsia , Tomografia Computadorizada de Feixe Cônico , Diagnóstico Diferencial , Diagnóstico por Imagem , Feminino , Rearranjo Gênico , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Neoplasias Mandibulares/diagnóstico , Neoplasias Mandibulares/patologia , Pessoa de Meia-Idade , Tumores Odontogênicos/diagnóstico , Tumores Odontogênicos/patologia , Proteína EWS de Ligação a RNARESUMO
Human mesenchymal stem cells form the supportive structure in which the functional cells of a differentiated tissue reside. We describe the creation of ectopic niches within polyurethane scaffolds coated with human mesenchymal stem cells. When implanted subcutaneously in NOD/SCID mice, these niches supported engraftment of primary human acute myeloid leukemia cells. The scaffolds showed vascularization and presence of osteoclasts and adipocytes, suggestive of an organizing human bone marrow microenvironment in the murine host. The chemokine stromal-derived factor-1 (SDF-1 or CXCL12) and its receptor CXCR4 are critical for homing and migration of acute myeloid leukemia. We found that a CXCR4 antagonist could disrupt homing to the ectopic niches, possibly by modulation of the mesenchymal stroma. We believe that these scaffold niches provide a new and powerful tool to study the leukemia stem cell microenvironment and may be useful for identification of novel drug targets.