Does childhood chemotherapy affect mandibular bone structures in a lifetime?

BACKGROUND: Chemotherapy, one of the most important treatment modalities for treating childhood cancers, is a major cause of bone loss in patients and survivors. OBJECTIVES: This study aimed to evaluate mandibular bone structures in childhood cancer survivors (CCSs) by means of fractal dimension (FD) analysis and the Klemetti index (KI), and to compare them with regard to the control group. MATERIAL AND METHODS: In this retrospective study, the panoramic radiographs of 49 CCSs were included as the study group and the panoramic radiographs of 49 cancer-free volunteers were included as the control group. Based on the panoramic radiographs, FD and KI were determined. RESULTS: No significant differences were observed between the study and control groups in terms of mean FD values for regions of interest (ROIs) ROI_1, ROI_2 and ROI_3 (p = 0.750, p = 0.490 and p = 0.910, respectively). The mean FD values for ROI_1 for the study and control groups were 1.08 ±0.18 and 1.07 ±0.14, respectively. The mean FD values for ROI_2 for the study and control groups were 1.11 ±0.13 and 1.09 ±0.13, respectively. The mean FD values for ROI_3 for the study and control groups were 1.15 ±0.14 and 1.15 ±0.15, respectively. Statistically significant differences between the study and control groups were noted only in the distribution of the KI categories (p = 0.015). CONCLUSIONS: Childhood chemotherapy may affect mandibular bone structures during a lifetime. The Klemetti index should be considered a useful clinical diagnostic tool for the examination of mandibular bone structures.

Mandibular metastases in neuroblastoma: Outcomes and dental sequelae.

BACKGROUND: Although metastatic involvement of bony sites including cranial bones is common in neuroblastoma (NB), mandibular metastases (MM) are uncommon, and specific outcomes have not been reported upon in the modern therapeutic era. METHODS: In this retrospective study, medical records on patients with MM from NB were reviewed. Statistical analysis was performed using the Kaplan-Meier method. RESULTS: Of 29 patients, nine (31%) had MM at diagnosis, whereas in 20 (69%) MM were first detected at NB relapse at a median time of 26 (6-89) months from diagnosis. Median maximal diameter of lesions was 3 (range 0.8-4.9) cm. MM were unilateral in 83% of patients, with ascending ramus (55%) and mandibular body (38%) being the two most common sites. All patients received systemic chemotherapy, and 26 (93%) patients received radiotherapy to MM. At a median follow-up of 37.3 (24.2-219.5) months, eight of nine patients with MM at diagnosis did not experience mandibular progressive disease. Eighteen of 20 patients with MM at relapse received therapeutic radiotherapy; objective responses were noted in 78%. Seventy-two percent (5/18) had not experienced relapse within the radiation field at a median of 12 (2-276) months postradiotherapy. Dental findings at follow-up after completion of NB therapy included hypodontia, hypocalcification of enamel, and trismus. Median 3-year overall survival in patients with relapsed MM was 51 ± 12% months from relapse. CONCLUSION: MM when detected at diagnosis is associated with a prognosis similar to that for other skeletal metastases of NB. Radiotherapy is effective for control of MM detected both at diagnosis and relapse. Significant dental abnormalities posttherapy warrant regular dental evaluations and appropriate intervention.

Mandibular tumour as the primary manifestation of an occult adenocarcinoma.

Oral cavity is an uncommon site for occurrence of metastasis and usually indicates widespread systemic disease. Primary tumours from the lung most commonly metastasise to oral soft tissues (31.3%) and jaw bones (22%) among men. It is quite challenging to diagnose a solitary metastatic lesion and to determine the site of primary lesion, due to the rarity of these occurrences. This case report describes the jaw bone as a first sign of occult primary lung adenocarcinoma with multiple metastases in 47-year-old man. The patient was treated with the combination of carboplatin and paclitaxel, and has completed two cycles till date with a stable clinical course. Diagnosis of metastatic carcinoma of jaw is very challenging. The pathologist must have knowledge of ancillary techniques along with clinical, radiological and histological correlation to determine the origin of distant primary.

Management of an aggressive giant cell granuloma of the mandible with denosumab: a case report.

The management of giant cell granulomas is challenging, and aggressive lesions have a high tendency to recur after enucleation alone. Based on assumptions regarding cell type and receptors, multiple pharmacological adjuncts have been used to manage them. We describe the use of denosumab, which was successfully used as a single method of treatment, suggesting that it may be a viable alternative or adjunct to operation on giant cell granulomas of the jaws.

Stage IV Neuroblastoma with Metastatic Spread to the Mandible in a Young Child: Case Report and Review of the Literature.

BACKGROUND: Infants and young children with neuroblastoma (NB) may present with metastases. The primary tumor most commonly originates in the abdomen and metastasizes to lymph nodes, liver, and bone marrow. Infants and young children presenting with multiple skull metastases are rare. METHODS: We present a rare case of a 20-month-old child who presented with metastatic neuroblastoma and multiple skull lesions. The child responded well to induction chemotherapy followed by myeloablative busulfan/melphalan consolidation. RESULTS: The child had substantial tumor reduction after chemotherapy was started. There was a significant decrease in tumor sizes and uptake, as seen in the metaiodobenzylguanidine study. The 6-month follow-up examination showed complete remission, and the remission continues. CONCLUSIONS: Infants and young children with neuroblastoma rarely present with metastatic lesions to the skull. Even large lesions involving the skull base may be successfully treated with chemotherapy. The use of myeloablative busulfan/melphalan consolidation after induction chemotherapy can decrease the overall metastatic tumor burden. Craniofacial specialists should be aware of treatment options for these young children.

Desmoid-type fibromatosis of the head and neck in children: A changing situation.

INTRODUCTION: Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies. METHODS: This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. All medical files were reviewed and their outcomes analyzed according to the initial therapies provided. RESULTS: Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XIth cranial nerve sacrifice or sensory impairment. CONCLUSION: HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

Heterozygosity for CMT Type 4 Predicts a Severe Vincristine-induced Polyneuropathy Phenotype: A Case Report and Review of Literature.

Vincristine (VCR) is a common chemotherapeutic agent used in the treatment of multiple types of pediatric tumors. VCR's adverse effects are well documented and commonly involve peripheral neuropathy via axonal degeneration. Neuropathic severity is dose-dependent, with sensory deficits occurring with as little as 4 mg cumulative dose. Severe peripheral neuropathy is generally rare, but its effects become additive when given to patients with undiagnosed hereditary peripheral neuropathy such as Charcot-Marie-Tooth. We report a case of an effect of VCR administration given to a patient who developed grade 4 neuropathy and was found to be a carrier of Charcot-Marie-Tooth disease type 4.

Osteosarcoma de mandíbula. Presentación de un caso / Mandible osteosarcoma. A case report

El osteosarcoma es una neoplasia maligna, poco frecuente. Se observa regularmente en niños y adultos jóvenes, con discreto predominio en los hombres y una incidencia anual de una por cien mil personas. Se puede originar en el hueso de la cavidad oral, representa solamente el 7 por ciento de todos los sarcomas osteogénicos y afecta con igual frecuencia el maxilar y la mandíbula. De etiología idiopática en la mayoría de los casos, se ha asociado a predisposición genética, traumas o radioterapia. El tratamiento generalmente suele ser una combinación de resección quirúrgica, que incluya un ancho margen de hueso normal, seguido de un tratamiento oncoespecífico. Se presenta el caso clínico de un paciente masculino de 43 años de edad con el diagnóstico de osteosarcoma mandibular que se trató con cirugía en una primera fase, luego con quimioterapia y radioterapia adyuvante y el control después de 12 meses sin evidencia de recidiva(AU)

Osteosarcoma is a rare malignancy. It isobserved regularly in children and young adults, with discrete predominance in men and an annual incidence of one per one hundred thousand people. It can originate in the bone of the oral cavity, represents only 7 percent of all osteogenic sarcomas and affects the jaw and maxilla with equal frequency. Of idiopathic etiology in most cases, it has been associated with genetic predisposition, trauma or radiotherapy. The treatment is usually a combination of surgical resection, which includes a wide margin of normal bone, followed by an oncospecific treatment. We present the clinical case of a male patient of 43 years of age with a diagnosis of mandibular osteosarcoma that was treated with surgery in a first phase, then with adjuvant chemotherapy and radiotherapy and control after 12 months without evidence of recurrence(EU)

Immunodeficiency-associated Burkitt's lymphoma in pediatric patients: a clinical case report

Burkitt's lymphoma, a form of non-hodgkin lymphoma, is a neoplastic monoclonal proliferation of lymphoid cells in areas of the immune system. it can occur in HIV-positive patients, as AIDS is related to the development of non- hodgkin lymphoma. burkitt's lymphoma is a rare subtype, highly prevalent in patients with AIDS. incisional biopsy, in situ hybridization and computerized axial tomography are the appropriate tests to determine the characterize of the lesions. the case of a 4-year-old HIV-positive patient, who developed burkitt's lymphoma of the oral cavity, is reported in this paper. the aim of this case report is to describe the course of the pathology, taking into account its clinical imaging characteristics and treatment.

Reversible cicatricial ectropion associated with EGFR inhibitors.

The management of cicatricial ectropion resulting from epidermal growth factor receptor (EGFR) inhibitors is unclear. We describe two cases of bilateral cicatricial ectropion following the use of an EGFR inhibitor who were treated with oral doxycycline, topical ophthalmic steroid and antibiotic ointment to the eyelids, and topical facial steroid cream with lubrication. The first case resolved with discontinuation of panitumumab infusions along with institution of the aforementioned regimen. However, it is unclear whether the resolution was from discontinuation of the infusions or from the instituted regimen. The second case resolved without a dose adjustment of cituximab. This case may provide support for the use of this regimen prior to discontinuation of the offending agent, as there was a successful outcome without alteration of the infusions. Additional cases are necessary to determine if this is a successful means of treating bilateral lower-lid cicatricial ectropion from EGFR inhibitors.

Metastasis in the mandibular condyle: a case report.

BACKGROUND: Most bone metastases are observed in the trunk of the body. Metastasis in the mandibular condyle is rare. In many case reports, temporary common temporomandibular joint disorder-like symptoms can be a sign of relapse and metastasis. CASE PRESENTATION: We report a rare case of breast carcinoma metastatic to the left mandibular condyle in a 55-year-old Japanese woman, who visited our department for a dental check-up prior to chemotherapy. She had almost no symptoms, but radiographs suggested the existence of metastasis. CONCLUSIONS: In many case reports, patients had some symptoms. In this case report, our patient had slight symptoms, but we were able to confirm the metastasis from the symptoms and panoramic dental radiograph. When patients complain about discomfort of the temporomandibular joint, we need to consider the possibility of metastasis and notice changes on the panoramic dental radiograph.

Off-label use of rhBMP-2 as bone regeneration strategies in mandibular ameloblastoma unicystic.

Jawbone reconstruction after tumor resection is one of the most challenging clinical tasks for maxillofacial surgeons. Osteogenic, osteoinductive, osteoconductive and non-antigenic properties of autogenous bone place this bone as the gold standard for solving problems of bone availability. However, the need for a second surgical site to harvest the bone graft increases significantly both the cost and the morbidity associated with the reconstructive procedures. Bone grafting gained an important tool with the discovery of bone morphogenetic proteins in 1960. Benefit of obtaining functional and real bone matrix without need of second surgical site seems to be the great advantage of use bone morphogenetic proteins. This study analyzed the use of rhBMP-2 in unicystic ameloblastoma of the mandible, detailing its structure, mechanisms of cell signaling and biological efficacy, in addition to present possible advantages and disadvantages of clinical use of rhBMP-2 as bone regeneration strategy. RESUMO A reconstrução óssea dos maxilares após ressecções tumorais é uma das tarefas mais difíceis para o cirurgião maxilofacial. As propriedades osteogênicas, osteoindutoras, osteocondutoras e não antigênicas do osso autógeno o colocam como o padrão-ouro para a solução de problemas de disponibilidade óssea. Entretanto a coleta do enxerto ósseo necessita de um segundo sítio cirúrgico, aumentando significativamente o custo e a morbidade associados ao procedimento reconstrutivo. A enxertia óssea ganhou uma excelente ferramenta com a descoberta das proteínas ósseas morfogenéticas na década de 1960. O benefício da obtenção de matriz óssea verdadeira e funcional, sem a necessidade de um segundo sítio cirúrgico, parece ser a grande vantagem do uso das proteínas ósseas morfogenéticas. Neste contexto, o objetivo deste estudo foi analisar a utilização da rhBMP-2 na regeneração óssea de ameloblastoma mandibular unicístico, detalhando sua estrutura, seus mecanismos de sinalização celular e sua eficácia biológica, além de apresentar potenciais vantagens e desvantagens da utilização clínica das rhBMP-2, enquanto estratégia regenerativa.

Off-label use of rhBMP-2 as bone regeneration strategies in mandibular ameloblastoma unicystic / Utilização off-label das rhBMP-2 como estratégia de regeneração do tecido ósseo em ameloblastoma mandibular

ABSTRACT Jawbone reconstruction after tumor resection is one of the most challenging clinical tasks for maxillofacial surgeons. Osteogenic, osteoinductive, osteoconductive and non-antigenic properties of autogenous bone place this bone as the gold standard for solving problems of bone availability. However, the need for a second surgical site to harvest the bone graft increases significantly both the cost and the morbidity associated with the reconstructive procedures. Bone grafting gained an important tool with the discovery of bone morphogenetic proteins in 1960. Benefit of obtaining functional and real bone matrix without need of second surgical site seems to be the great advantage of use bone morphogenetic proteins. This study analyzed the use of rhBMP-2 in unicystic ameloblastoma of the mandible, detailing its structure, mechanisms of cell signaling and biological efficacy, in addition to present possible advantages and disadvantages of clinical use of rhBMP-2 as bone regeneration strategy.

RESUMO A reconstrução óssea dos maxilares após ressecções tumorais é uma das tarefas mais difíceis para o cirurgião maxilofacial. As propriedades osteogênicas, osteoindutoras, osteocondutoras e não antigênicas do osso autógeno o colocam como o padrão-ouro para a solução de problemas de disponibilidade óssea. Entretanto a coleta do enxerto ósseo necessita de um segundo sítio cirúrgico, aumentando significativamente o custo e a morbidade associados ao procedimento reconstrutivo. A enxertia óssea ganhou uma excelente ferramenta com a descoberta das proteínas ósseas morfogenéticas na década de 1960. O benefício da obtenção de matriz óssea verdadeira e funcional, sem a necessidade de um segundo sítio cirúrgico, parece ser a grande vantagem do uso das proteínas ósseas morfogenéticas. Neste contexto, o objetivo deste estudo foi analisar a utilização da rhBMP-2 na regeneração óssea de ameloblastoma mandibular unicístico, detalhando sua estrutura, seus mecanismos de sinalização celular e sua eficácia biológica, além de apresentar potenciais vantagens e desvantagens da utilização clínica das rhBMP-2, enquanto estratégia regenerativa.

Pharmacological and surgical therapy for the central giant cell granuloma: A long-term retrospective cohort study.

PURPOSE: This is a retrospective cohort study of patients with a central giant cell granuloma (CGCG) treated at a single center to assess and compare the different surgical and non-surgical approaches. MATERIAL AND METHODS: A cohort with a single histologically proven non-syndrome-related CGCG was selected and reviewed. Patients were allocated to group I (surgery), group II (pharmacotherapy), and group III (pharmacotherapy and surgery). The primary outcome was long-term radiologic response using computed tomography. Secondary outcomes were intermediate radiologic responses and occurrence and severity of side effects. RESULTS: Thirty-three subjects were included in the study. The surgical group (n = 4) included 1 patient with progression during follow-up and a relatively high post-surgical morbidity. Twenty-nine patients started on various pharmacological treatment regimens (groups II and III). Fourteen patients could be managed without additional surgery. One of these lesions showed progression during follow-up. The other 15 lesions underwent additional surgery, and none showed progression during follow-up. Interferon treatment was associated with the most side effects. CONCLUSION: Pharmacological agents have a role in the treatment of aggressive and non-aggressive CGCGs by limiting the renewed progression during long-term follow up and the extent and morbidity of surgical treatment.

Adjuvant Antiangiogenic Treatment for Aggressive Giant Cell Lesions of the Jaw: A 20-Year Experience at Massachusetts General Hospital.

PURPOSE: To document long-term outcomes using a standardized treatment protocol of enucleation with preservation of vital structures and adjuvant subcutaneous interferon for aggressive giant cell lesions (GCLs) of the jaws. MATERIALS AND METHODS: A retrospective cohort study was designed. We evaluated all patients treated at Massachusetts General Hospital from April 1995 through September 2015 by enucleation with preservation of vital structures and adjuvant daily subcutaneous interferon for aggressive GCLs. The sample included patients with complete medical records consisting of clinical, radiographic, histopathologic, and follow-up data. The exclusion criteria included patients with incomplete records, contraindications to interferon therapy, non-aggressive GCLs, and GCLs associated with syndromes or with hyperparathyroidism. The primary outcome variable was long-term progression-free survival (PFS). The secondary outcome variables were adverse effects and laboratory abnormalities classified by type, frequency, and severity. Predictor variables for recurrence or failure included age, gender, location and features of lesion, type of procedure, duration of interferon treatment, amount of bone fill at end of treatment, and adverse effects. Descriptive statistics, Kaplan-Meier survival analysis, and Cox proportional hazards regression analysis were computed. RESULTS: Of a total of 77 patients, 45 (mean age, 18.8 ± 12.5 years; 29 female patients; 36 in whom the mandible was affected) met the inclusion criteria. The mean duration of interferon therapy was 7.9 ± 2.3 months. After follow-up of 4.8 ± 3.9 years, 6 patients showed progression of the lesion, considered recurrence (13.3% failure rate, 82.6% PFS rate). Most patients had mild (n = 42; 93.3%) and/or moderate (n = 31; 68.8%) side effects, which were readily managed. Adverse effects required stoppage of interferon in 7 patients, whereas no patients had long-term toxicity. No variable was significantly associated with PFS. CONCLUSIONS: The results of this study indicate that enucleation with preservation of vital structures in combination with adjuvant interferon alfa is a reliable treatment for aggressive GCLs of the jaws associated with a low recurrence rate.

Off-Label Use of Bone Morphogenetic Protein 2 in the Reconstructions of Mandibular Continuity Defects.

This paper describes 3 patients of off-label use of bone morphogenetic protein 2 (rhBMP-2) in the reconstruction of mandibular continuity defects. In the first patient, rhBMP-2 was associated with iliac crest bone graft for late mandibular reconstruction after resection of osteosarcoma. In the 2 other patients, rhBMP-2 was used alone. In 1 patient the mandibular continuity defect was due to resection for treatment of osteomyelitis and in the other patient a continuity defect was created by unsuccessful osteogenic distraction for correction of mandibular hypoplasia. Despite the good results in those patients, the off-label use of rhBMP-2 is associated with increased rate of complications, so more studies are needed to assess the predictability of the use of rhBMP-2 in mandibular continuity defects. Therefore, at the moment the off-label use of rhBMP-2 should be restricted to complicated bone defects in which the conventional alternatives of reconstruction were unsuccessful.

Tumor-induced Osteomalacia in a 3-Year-Old With Unresectable Central Giant Cell Lesions.

Tumor-induced osteomalacia (TIO) is a rare cause of hypophosphatemia involving overproduction of fibroblast growth factor 23. TIO has been described largely in adults with small mesenchymal tumors. We report a case of TIO in a child who presented with knee pain and radiographic findings concerning for rickets, and was found to have maxillomandibular giant cell lesions. The patient was treated with oral phosphorus and calcitriol, surgical debulking, and intralesional corticosteroids, which resulted in tumor regression and normalization of serum fibroblast growth factor 23 and phosphorus. This case illustrates the occurrence of this rare paraneoplastic syndrome in children and adds to our knowledge about clinical manifestations and pathologic findings associated with pediatric TIO.

Diagnóstico de mieloma múltiple a raíz de una lesión osteolítica mandibular / Diagnosis of multiple myeloma following an osteolytic mandibular lesion

Objetivo. El mieloma múltiple es una neoplasia de células plasmáticas con repercusión sistémica y lesiones osteolíticas. Su diagnóstico a raíz de una lesión mandibular es bastante infrecuente. Por ello presentamos un caso de mieloma múltiple diagnosticado a partir de sintomatología dolorosa e inflamatoria por una lesión osteolítica en la rama mandibular y revisamos la literatura para establecer el perfil clínico e imagen radiológica típica que nos faciliten sospechar el diagnóstico con más certeza. Material y métodos. Mujer de 46 años acude al servicio de Urgencias por inflamación y dolor preauricular. La ortopantomografía muestra una imagen radiolúcida multilobulada a lo largo de la rama y cóndilo mandibulares. El estudio anatomopatológico y de extensión desenmascaran un mieloma múltiple. Se hace además una revisión de la base de datos Pubmed para casos de diagnóstico de mieloma múltiple a partir de una lesión mandibular en los últimos 5 años. Resultados. Se encontraron 13 casos incluyendo el presente. La edad media era de 61 años con ligero predominio del sexo masculino; la clínica más frecuenta era inflamación indurada de la región mandibular afectada con o sin ulceración mucosa, y la imagen radiológica característica osteólisis uni o multilocular, en un caso con reacción perióstica. Conclusiones. La presentación inicial del mieloma múltiple mediante una lesión mandibular es poco frecuente. Sin embargo, el cirujano maxilofacial debe sospechar una neoplasia de células plasmáticas ante imágenes radiolúcidas mandibulares y no demorar un estudio más exhaustivo (AU)

Purpose. Multiple myeloma is a neoplasic proliferation of plasma cells with systemic repercussion and osteolytic lesions. The initial diagnosis by a mandibular lesion is extremely rare. Therefore we present a clinical case of multiple myeloma diagnosed following pain and swelling from an ostelytic mandibular lesion, and we review the literature in order establish a clinical profile and a typical radiological image that would allow us to suspect the diagnosis with more certainty. Material and methods. A 46 year-old woman comes to the Emergency Department complaining about preauricular pain and swelling. The orthopantomography shows a radiolucid multilolocular image along the mandibulary ramus and condyle. The histology and extension follow up concludes with a multiple myeloma diagnosis. A systematic review of Pubmed database was made searching for mandibular lesion at initial diagnosis of multiple myeloma within the last 5 years. Results. We found 13 cases including ours The mean age was 61 years with a slight male predominance; the most common clinical presentation was a firm to hard mandibular swelling with or without mucosal ulceration; and the most characteristic radiological image was a uni or multilocular osteolysis, with periostic reaction in one case. Conclusions. A mandibular lesion as a first sign of multiple myeloma is infrequent. Nevertheless, a maxilofacial surgeon should suspect a plasma cell neoplasia in cases of mandibular osteolytic images and start further studying without delay (AU)

Plasmablastic lymphoma exclusively involving bones mimicking osteosarcoma in an immunocompetent patient: A case report.

BACKGROUND: It has been known that plasmablastic lymphoma (PBL) is a neoplasm of immunocompromised patients occurring in soft tissue of oral cavity or in the vicinity whereas bone is an unlikely site to harbor PBL. However, its occurrence is increasingly being reported in immunocompetent individuals in either osseous or extra-oral sites. To our best knowledge, F-18 FDG PET/CT findings of PBL involving bones in an immunocompetent patient have not been reported, yet . CASE SUMMARY: We report a case of PBL involving multiple bones in an immunocompetent patient. Features of different imaging modalities including F-18 Fluoro-deoxy glucose (FDG) positron emission tomography/computed tomography (PET/CT) were correlated well as findings of osteosarcoma in mandible with metastatic lesions. However, the histopathology and immunohistochemistry (IHC) of bone tissues from 2 separate biopsy sites revealed features of PBL. CONCLUSION: awareness to F-18 FDG PET/CT findings of PBL involving bones in an immunocompetent patient may prevent misdiagnosis.

BRAF inhibitor treatment of primary BRAF-mutant ameloblastoma with pathologic assessment of response.

OBJECTIVE: Molecular characterization of ameloblastoma has indicated a high frequency of driver mutations in BRAF and SMO. Preclinical data suggest that Food and Drug Administration-approved BRAF-targeted therapies may be immediately relevant for patients with ameloblastoma positive for the BRAF V600E mutation. METHODS: A neoadjuvant treatment regime of dabrafenib was given to a patient with recurrent BRAF-mutant mandibular ameloblastoma. The patient subsequently underwent left mandible composite resection of the tumor and pathologic evaluation of treatment response. RESULTS: The ameloblastoma had a slow but dramatic response with >90% tumor volume reduction. The inner areas of the tumor underwent degeneration and squamous differentiation, and intact ameloblastoma was present in the outer areas associated with bone. CONCLUSIONS: Targeted neoadjuvant therapy for ameloblastoma may be useful in certain clinical settings of primary ameloblastoma. These might include tumors of advanced local stage when a neoadjuvant reduction could alter the extent of surgery and instances of local recurrence when surgical options are limited.