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Mandibular malignancies are rare in the pediatric population and subsequently not well characterized. SEER 18 registry data was collected, applying age 0-18 years and ICD-O-3 code C41.1 ('mandible'). Univariate Cox regression analysis was conducted, and hazard ratios (HR) were calculated for overall survival (OS) and disease-specific survival (DSS) according to patient demographics, tumor characteristics, and treatment. Kaplan-Meier survival curves were generated for OS and DSS. Sixty-four patients met the inclusion criteria. The median age at diagnosis was 13.0 years, and median survival was 8.7 years. Osteosarcoma was the most common histological diagnosis (n = 22). Sex, race, age (<13 vs ≥13 years), histological type, odontogenic origin, and treatment modality were found not to be associated with OS or DSS. The SEER stage 'distant' was significantly associated with an elevated HR of 6.28 for DSS (P = 0.027) and 5.29 for OS (P = 0.025). Kaplan-Meier survival curves demonstrated significantly lower 5-year DSS (P<0.001) and OS (P<0.001) for SEER 'distant' stage. This study includes the analysis of a large number of pediatric mandibular malignancies when compared to previous studies. 'Distant' stage was associated with decreased survival. Early clinical suspicion and diagnosis are paramount for improved survival.
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BACKGROUND: Currently, the differentiation of jaw tumors is mainly based on the lesion's morphology rather than the enhancement characteristics, which are important in the differentiation of neoplasms across the body. There is a paucity of literature on the enhancement characteristics of jaw tumors. This is mainly because, even though computed tomography (CT) is used to evaluate these lesions, they are often imaged without intravenous contrast. This study hypothesised that the enhancement characteristics of the solid component of jaw tumors can aid in the differentiation of these lesions in addition to their morphology by dual-energy CT, therefore improving the ability to differentiate between various pathologies. AIM: To evaluate the role of contrast enhancement and dual-energy quantitative parameters in CT in the differentiation of jaw tumors. METHODS: Fifty-seven patients with jaw tumors underwent contrast-enhanced dual-energy CT. Morphological analysis of the tumor, including the enhancing solid component, was done, followed by quantitative analysis of iodine concentration (IC), water concentration (WC), HU, and normalized IC. The study population was divided into four subgroups based on histopathological analysis-central giant cell granuloma (CGCG), ameloblastoma, odontogenic keratocyst (OKC), and other jaw tumors. A one-way ANOVA test for parametric variables and the Kruskal-Wallis test for non-parametric variables were used. If significant differences were found, a series of independent t-tests or Mann-Whitney U tests were used. RESULTS: Ameloblastoma was the most common pathology (n = 20), followed by CGCG (n = 11) and OKC. CGCG showed a higher mean concentration of all quantitative parameters than ameloblastomas (P < 0.05). An IC threshold of 31.35 × 100 µg/cm3 had the maximum sensitivity (81.8%) and specificity (65%). Between ameloblastomas and OKC, the former showed a higher mean concentration of all quantitative parameters (P < 0.001), however when comparing unilocular ameloblastomas with OKCs, the latter showed significantly higher WC. Also, ameloblastoma had a higher IC and lower WC compared to "other jaw tumors" group. CONCLUSION: Enhancement characteristics of solid components combined with dual-energy parameters offer a more precise way to differentiate between jaw tumors.
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Background: Reconstruction of large mandibular defects requires reestablishment of mandibular continuity with bone and soft tissue. The microvascularized fibula flap (MFF) has the advantage of providing both, with adequate length, low resorption rate, low infection risk and possibility of dental implant insertion. It can be adapted to mandibular defects in many different ways. Purpose: This retrospective study will present and evaluate the results of the male-female joint technique for flap positioning and fixation. Methods: The technique consists of designing osteotomies on the binding edge of the MFF and recipient jaw, increasing bone contact from one to five faces. Patients submitted to mandibular reconstruction through this technique were included and evaluated regarding systemic compromise, complication occurrence as well as primary and long-term stability. Results: Ten patients underwent mandibular reconstruction with the male-female joint technique. Sixteen joints were applied, and excluding an early loss due to vascular failure, all remaining 14 joints healed uneventfully. None showed signs of early or late mobility. Conclusion: Fitting the MFF through a male-female joint that provides greater bone contact may improve stability and reduce screw loosening and mini-plate removal rates.
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The purpose of this multicentre study was to evaluate the efficacy of the 'dredging-marsupialization-curettage' (D-M-C) strategy in the treatment of conventional intraosseous ameloblastoma of the mandible. A total of 31 patients from three institutions, who had a pathological diagnosis of conventional ameloblastoma of the mandible, were treated with the D-M-C strategy. The surgical protocol comprised a dredging and marsupialization (D-M) step, with additional D-M steps as required. The patients then underwent curettage (C) once an obvious effect of the D-M step had been achieved during follow-up. Eight patients were followed up for ≥36 months but <60 months, while 23 were followed up for ≥60 months. Nineteen of the 23 patients followed up for ≥60 months were disease-free at the last follow-up, with no evidence of recurrence. The D-M step is effective for reducing the tumour size and preserving vital structures. The D-M-C surgical strategy may be a feasible treatment option for conventional ameloblastoma of the mandible.
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Ameloblastoma , Curetagem , Neoplasias Mandibulares , Humanos , Ameloblastoma/cirurgia , Ameloblastoma/diagnóstico por imagem , Ameloblastoma/patologia , Neoplasias Mandibulares/cirurgia , Neoplasias Mandibulares/diagnóstico por imagem , Neoplasias Mandibulares/patologia , Estudos Retrospectivos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Resultado do Tratamento , Adolescente , IdosoRESUMO
AIM: To summarize the features of condylar kinematics in patients with condylar reconstruction using a mandibular motion simulation method based on intraoral scanning registration. MATERIALS AND METHODS: Patients undergoing unilateral segmental mandibulectomy and autogenous bone reconstruction as well as healthy volunteers were enrolled in the study. Patients were grouped based on whether the condyles were reconstructed. Mandibular movements were recorded using a jaw tracking system, and kinematic models were simulated after registration. The path inclination of the condyle point, margin of border movement, deviation, and chewing cycle were analyzed. A t test and one-way analysis of variance (ANOVA) were carried out. RESULTS: A total of 20 patients, including 6 with condylar reconstruction and 14 with condylar preservation as well as 10 healthy volunteers were included. The patients with condylar reconstruction showed flatter movement paths of the condyle points. The mean inclination angle of the condylar movement paths of the patients with condylar reconstruction (0.57 ± 12.54 degrees) was significantly smaller than that of those with condylar preservation (24.70 ± 3.90 degrees, P = 0.014) during both maximum opening and protrusion (7.04 ± 12.21 degrees and 31.12 ± 6.79 degrees, respectively, P = 0.022). The inclination angle of the condylar movement paths of the healthy volunteers was 16.81 ± 3.97 degrees during maximum opening and 21.54 ± 2.80 degrees during protrusion; no significant difference compared with the patients. The condyles of the affected side tended to deviate laterally in all patients during mouth opening and protrusion. Patients with condylar reconstruction showed more severe symptoms of mouth opening limitation and mandibular movement deviation as well as shorter chewing cycles than patients with condylar preservation. CONCLUSION: Patients with condylar reconstruction showed flatter movement paths of the condyle points, greater lateral motion range, and shorter chewing cycles than those with condylar preservation. The method of mandibular motion simulation based on intraoral scanning registration was feasible to simulate condylar movement.
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Côndilo Mandibular , Transtornos da Articulação Temporomandibular , Humanos , Côndilo Mandibular/cirurgia , Fenômenos Biomecânicos , Registro da Relação Maxilomandibular , Movimento , Rotação , Articulação Temporomandibular , Amplitude de Movimento ArticularRESUMO
Background: Mucoepidermoid carcinoma starts in the salivary glands and accounts for 5%-10% of all salivary gland tumours. Its intraosseous occurrence is rare and only accounts for 2%-3% of all mucoepidermoid carcinomas. This neoplasm often follows a long and indolent course. Also, given its ambiguous presentation and similarities to other dental pathologies, it often has a late diagnosis. In this instance, we present the case of a patient with an asymptomatic mandibular lesion, who underwent mandibular resection and reconstruction with fibula free flap. Case report: A 32-year-old male patient reported discomfort when chewing, which was attributable to a self-detected mass localised in proximity to teeth 47 and 48. The lesion presented as a slight swelling without clear expression in the oral cavity mucosa. The rest of the physical examination revealed no abnormalities. Both the panoramic radiograph and computed tomography of the maxillary bones revealed lytic lesions in proximity to teeth 47 and 48, close to the mandibular angle. An incisional biopsy was performed, for which the pathological anatomy showed low-grade mucoepidermoid carcinoma. A resection was then performed, which involved a right hemimandibulectomy with ipsilateral cervical lymphadenectomy. The reconstruction was performed with a right fibula-free flap. Upon histological evaluation of the surgical specimen, an intermediate-grade mucoepidermoid carcinoma was found. The patient presented good post-operative evolution. Following a multidisciplinary assessment, the use of adjuvant radiation therapy was deemed necessary. The patient currently presents good evolution and has regular check-ups. Conclusion: Intraosseous mucoepidermoid carcinoma is a rare salivary gland tumour. Given its low frequency, there are no studies that accurately describe its biological behaviour and prognosis.
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Osteoblastoma is a rare benign neoplasm formed by osteoid tissue and well-vascularized bone that occurs mainly in children and adolescents. It appears primarily in the long bones, vertebral column, and small bones of the hands and feet, and not typically in the skull and maxillary bones. The purpose of this study is to present the case of an 8-year-old girl with a diagnosis of right mandibular osteoblastoma and a review of the relevant literature. The goals of treatment were to preserve dental occlusion, masticatory function and facial symmetry while minimizing the effects on patient body image and quality of life. Osteoblastoma, although it is benign, can be aggressive, and its treatment will depend on the timing of diagnosis, size and location. Early diagnosis is essential to avoid not only radical surgery as in the case presented, but also to help minimize the risk of possible relapse and potential malignancy of a benign osteoblastoma.
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Surgical treatment of tumours in the anterior mandible and surrounding tissues may result in defects which can be restored by a fibula free flap. The upper airway may change during this process. The purpose of this retrospective study was to evaluate upper airway changes after fibula free flap reconstruction. A total of 37 patients who underwent anterior mandibulectomy and fibula free flap reconstruction between 2012 and 2020 were recruited. Patients with benign and malignant tumours involving the anterior mandible were included. Spiral computed tomography was performed 1 week preoperatively, 1 week postoperatively, and at> 1 year (range 12-23 months) after surgery. Cross-sectional areas and volumes of the upper airway were measured. Data were analysed by two-way analysis of variance. The upper airway in the malignant tumour group showed an increasing trend, especially at the soft palate and tongue base levels (P < 0.01). In the benign tumour group, the upper airway showed no significant changes. The location of the minimum cross-sectional area moved downwards in both groups, and the area increased in the malignant tumour group during long-term follow-up. Upper airway obstruction is less likely to occur in the long term after surgical resection of anterior mandible malignancies and fibula free flap reconstruction.
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Retalhos de Tecido Biológico , Neoplasias Mandibulares , Neoplasias , Humanos , Estudos Retrospectivos , Neoplasias Mandibulares/diagnóstico por imagem , Neoplasias Mandibulares/cirurgia , Neoplasias Mandibulares/patologia , Resultado do Tratamento , Mandíbula/diagnóstico por imagem , Mandíbula/cirurgia , Mandíbula/patologiaRESUMO
Nuclear protein of the testis (NUT) carcinoma is a rare, undifferentiated carcinoma that is characterized by NUTM1 gene rearrangements. Patients with NUT carcinoma have an overall survival of approximately 5% at 5 years despite a multimodal treatment approach. This report illustrates the management of mandibular NUT carcinoma in a pediatric patient, complemented by a systematic review of head and neck NUT carcinoma. A 5-year-old female presented with an enlarging jaw mass that was diagnosed as BRD4-NUTM1 carcinoma and was treated with hemimandibulectomy and chemoradiation. She remains disease-free 21 months after completion of therapy. A total of 63 patient cases reported in 34 articles were identified in the review. Only 26.9% (14/52) of tumors were correctly diagnosed initially as NUT carcinoma, whereas 73.1% (38/52) were incorrectly diagnosed as another malignancy; the initial diagnosis was not reported for 11 patients. The mandibular tumor subtype was among the rarest reported (n = 1; 1.6%). Combination therapy, including surgery and chemoradiation, was the most common treatment (55.2%). The patient case presented here is a novel case of pediatric mandibular NUT carcinoma. Due to the poor overall survival of patients with NUT carcinoma, aggressive upfront resection with 2-cm margins followed by adjuvant chemoradiation is advocated.
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Carcinoma , Proteínas de Neoplasias , Masculino , Feminino , Humanos , Criança , Pré-Escolar , Proteínas de Neoplasias/genética , Proteínas Nucleares/genética , Fatores de Transcrição/genética , Carcinoma/patologia , Mandíbula/patologia , Proteínas de Ciclo Celular/genéticaRESUMO
Ameloblastoma is one of the most common benign odontogenic tumors of the jaw that constitutes about 10% of all tumors that arise in the mandible and maxilla. It is a slow-growing but locally invasive tumor that presents with painless swelling of the mandible or maxilla. The World Health Organization (WHO) classification of 2017 describes ameloblastomas of the following four types: ameloblastoma; unicystic ameloblastoma; extraosseous/peripheral ameloblastoma; and metastasizing ameloblastoma. The diagnosis of ameloblastoma requires computerized tomography (CT) imaging as well as a biopsy. A biopsy is helpful in differentiating ameloblastoma from ossifying fibroma, osteomyelitis, giant cell tumor, cystic fibrous dysplasia, myeloma, and sarcoma. The best treatment of ameloblastoma is aggressive en bloc resection with simultaneous reconstruction. The high recurrence rate and large tissue defects have been long-standing issues in the treatment of ameloblastoma. Recent molecular developments strongly suggest the possibility of targeted therapy with better outcomes in ameloblastomas. We present a detailed updated narrative review of our current understanding and management of this enigmatic tumor.
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INTRODUCTION: importance: Gingival squamous cell carcinoma (GSCC) is a rare neoplasm. GSCC has nonspecific features which commonly misdiagnosed with a simple toothache. CASE PRESENTATION: These are two cases; the first one describes a 62-year-old female who presented with pain in her right posterior mandibular teeth, and she was misdiagnosed with periodontal disease, but later histopathological tests confirmed gingival squamous cell carcinoma (GSCC). Consequently, the patient underwent surgery, and the gingival tumor was totally resected. Afterward, the patient was receiving chemotherapy, and the radiotherapy was postponed until the chemotherapy completion.The second case is of a 58-year-old female who presented with pain in her mandibular incisors, and she developed a dermal fistula on her chin and therefore underwent several gingival curettages. The following histopathologic tests confirmed GSCC so she underwent surgery, after the surgery it was planned to give her chemotherapy. CLINICAL DISCUSSION: GSCC is a rare neoplasm with a 6% incidence of all oral malignancies. GSCC is a very challenging cancer for a physician or a pathologist to diagnose because GSCC usually mimics the characteristics of a large variety of diseases and abnormalities. Unlike oral neoplasms, GSCC has the least association with smoking. This may lead to make mistakes in the treatment or misdiagnose it until the late-stage of GSCC. CONCLUSION: Despite the rare incidence of Gingival squamous cell carcinoma (GSCC), clinicians should consider GSCC while investigating any localized lesion with nonspecific oral symptoms.
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This report describes the management of a case of calcifying epithelial odontogenic tumour (CEOT) that underwent malignant transformation and metastasized to the lung. The solitary pulmonary metastasis was discovered incidentally on computed tomography (CT) imaging of the neck. It appears that only one previous case with proven pulmonary metastasis has been reported in the literature, which involved multiple pulmonary deposits managed with platinum chemotherapy. The long-term prognosis of metastatic CEOT is therefore unknown. In the case presented here, the patient was managed successfully with surgery alone. There is often diagnostic uncertainty because histological features of benign, recurrent, and malignant CEOT are not dissimilar. Ki-67 immunohistochemistry is helpful, as higher levels are more indicative of malignancy. We consider that in cases of suspected recurrent and malignant CEOT, CT imaging of the thorax and abdomen as part of follow-up may identify metastases early, resulting in earlier treatment, an improved prognosis, and reduced morbidity and mortality.
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Tumores Odontogênicos , Neoplasias Cutâneas , Transformação Celular Neoplásica , Humanos , Recidiva Local de NeoplasiaRESUMO
ABSTRACT Central giant cell granuloma is a rare osseous tumor affecting young patients with anatomical and functional compromise of the maxilla and mandible. Steroid injection therapy constitutes a less invasive treatment modality for disease control in selected cases. Retinal ischemia is a reported complication of multiple medical procedures, including dental interventions, and may lead to loss of vision with poor prognosis. We report a case of retinal arteriolar ischemic disease following central giant cell granuloma management with local injected corticosteroids.
RESUMO O granuloma central de células gigantes é um tumor ósseo raro que afeta pacientes jovens com comprometimento anatômico e funcional da maxila e mandíbula. A terapia com injeção de esteroides constitui uma modalidade de tratamento menos invasiva para o controle da doença em casos selecionados. A isquemia retiniana é uma complicação relatada em vários procedimentos médicos, incluindo intervenções odontológicas, e pode levar à perda da visão com mau prognóstico. Relatamos um caso de doença isquêmica arteriolar da retina após o tratamento com granuloma central de células gigantes com corticosteroides injetados locais.
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Humanos , Feminino , Adolescente , Neoplasias Ósseas , Corticosteroides , Isquemia/induzido quimicamente , Neoplasias Ósseas/tratamento farmacológico , Granuloma de Células Gigantes , Granuloma de Células Gigantes/tratamento farmacológico , MandíbulaRESUMO
PURPOSE: The purpose of this study was to investigate the utility of the width-to-length ratio for the differentiation of ameloblastomas and odontogenic keratocysts in the body of the mandible. MATERIALS AND METHODS: This study retrospectively reviewed 9 patients with ameloblastomas and 9 patients with odontogenic keratocysts using cone-beam computed tomography. The width-to-length ratio was determined by measuring the ratio between the greatest buccolingual dimension and the greatest perpendicular anteroposterior dimension of the lesion on the axial view. One-way analysis of variance was used to examine the difference in the width-to-length ratio between the 2 types of lesions. Statistical significance was tested at P<0.05. RESULTS: Ameloblastomas showed a mean width-to-length ratio of 0.64, whereas odontogenic keratocysts showed a mean width-to-length ratio of 0.41. The cut-off value with which the 2 types of lesions were differentiated was 0.5. The width-to-length ratios of ameloblastomas were significantly higher than those of odontogenic keratocysts (P<0.05). CONCLUSION: The width-to-length ratio might be used to differentiate between ameloblastomas and odontogenic keratocysts.
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Ameloblastic carcinoma is a rare odontogenic malignant tumor with the histologic features of both ameloblastoma and carcinoma. It occurs more frequently in the mandible than in the maxilla and it may appear de novo or develop from a preexisting ameloblastoma or odontogenic cyst. Rapidly progressing, painful swelling is the most common symptom, and radiographically, it shows significant bone resorption and cortical perforation. This report described a case of ameloblastic carcinoma in a 45-year-old man who presented with swelling in the left mandible. The lesion showed combined features of benign findings, such as an expansile cortex with a distinct border, and malignant findings, such as a large exophytic mass with frank bone resorption. Excisional biopsy was performed and a final diagnosis of ameloblastic carcinoma was made.
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Ameloblastoma is an odontogenic tumor that affects the bones of the jaw, often affecting posterior region of the mandible. It is a benign neoplasm and is frequently associated to an unerupted tooth. The purpose of this article is to report a case of failure in making an immediate full denture in a patient that had an ameloblastoma. A female patient aged 67 years complained of a hard swelling in the right mandible at the premolar region, that she noted after teeth extraction and confection of an immediate denture. Intraoral examination showed buccal and lingual cortical plate expansion and radiograph examination showed multilocular radiolucency with a well-defined margin. Aspiration was nonproductive and the provisional diagnosis was ameloblastoma. An incisional biopsy was performed and the histopathological report was conclusive of an acanthomatous ameloblastoma. The patient was sent for surgical excision of the lesion and after three years, the patient returned reporting that she was operated elsewhere in the past year and had an unsuccessful bone graft. She was sent to a prosthodontist to make a complete denture. In the present case the ameloblastoma was diagnosed only after the teeth extraction and immediate denture (AU)
O ameloblastoma é um tumor odontogênico que afeta os ossos maxilares, geralmente a região posterior de mandíbula. É uma neoplasia benigna frequentemente associada a um dente não irrompido. O objetivo deste artigo é relatar um caso de prótese total imediata confeccionada para uma paciente com ameloblastoma na região anterior da mandíbula. Paciente do sexo feminino, 67 anos, queixou-se de aumento de volume na região de pré-molares inferiores do lado direito após extração dentária e confecção de prótese total imediata. Ao exame clínico intrabucal foi observada expansão da cortical vestibular e lingual e o exame radiográfico revelou radiolucência multilocular com margem bem definida. A punção aspirativa não foi produtiva e o diagnóstico provisório foi de ameloblastoma. Foi realizada biópsia incisional e o laudo histopatológico foi conclusivo para ameloblastoma acantomatoso. A paciente foi encaminhada para ressecção cirúrgica da lesão e não retornou. Depois de três anos procurou atendimento e relatou que tinha sido submetida a cirurgia com colocação de enxerto ósseo para implante sem sucesso. O enxerto ósseo foi perdido e a paciente necessitava de nova prótese total. Ela foi encaminhada a um protesista. No presente caso, o diagnóstico de ameloblastoma foi feito somente após a instalação da prótese total imediata, quando a paciente notou aumento de volume (AU).
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Humanos , Feminino , Idoso , Ameloblastoma , Neoplasias Mandibulares , Tumores Odontogênicos , DiagnósticoRESUMO
ABSTRACT Introduction: Ameloblastoma is a neoplasm usually found in the maxilla and mandible with progressive intraosseous growth and a tendency to local recurrence. Malignant or metastatic ameloblastoma is one of its rare variants; a histopathological study is required for diagnosis confirmation. The common sites of dissemination are the lungs and cervical lymph nodes. The treatment is surgical and radiotherapy or chemotherapy are palliative options. Case report: A case of malignant ameloblastoma was reported in a 39-year-old man with metastasis to the scalp and multiple local recurrences. The patient required mandibular osteotomy, multiple local resections, partial maxillary resection, free flap reconstruction, local scalp resection and free flap coverage. Due to a new relapse that could not be treated with a surgical approach, radiotherapy was ordered. Discussion: The site most frequently affected by malignant ameloblastoma due to hematog-enous dissemination is the lung. This paper reports the case of a patient with compromised scalp. To date, according to a search conducted, only one case report about this type of met-astatic neoplasm has been published. Conclusion: Histopathological diagnosis of ameloblastoma associated with surgical resection of the primary tumor and the metastatic tumor is the only approach that can offer disease-free survival.
RESUMEN Introducción. El ameloblastoma es una neoplasia maxilomandibular con crecimiento progresivo intraóseo y tendencia a la recidiva local. El ameloblastoma maligno, o metastásico, es una de sus variantes raras y para su diagnóstico se requiere confirmación con estudio histo-patológico. Los sitios frecuentes de diseminación son el pulmón y los ganglios cervicales. El tratamiento es quirúrgico y la radioterapia o quimioterapia son paliativas. Presentación del caso. Paciente masculino de 39 años con ameloblastoma maligno que hace metástasis a cuero cabelludo y quien requirió osteotomía mandibular, resecciones locales, resección parcial de maxilar, reconstrucción con colgajo libre, resección local en cuero cabelludo y cubrimiento con colgajo libre. Por nueva recaída no susceptible de abordaje quirúrgico, se ordenó radioterapia. Discusión. En ameloblastoma maligno por diseminación hematógena, el sitio más frecuentemente afectado es el pulmón. En el caso reportado se presenta un paciente con compromiso del cuero cabelludo, lo que lo hace inusual, pues, según la búsqueda realizada, hasta la fecha solo se encontró un reporte de caso publicado con este tipo de metástasis. Conclusión. El diagnostico histopatológico del ameloblastoma asociado a una resección quirúrgica del tumor primario como del tumor metastásico es lo único que puede ofrecer una supervivencia libre de enfermedad.
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BACKGROUND: Adenoid cystic carcinoma (ACC) is a relatively rare malignant neoplasm that occurs in salivary glands and various other organs. Recent studies have revealed that a significant proportion of ACCs harbor gene alterations involving MYB or MYBL1 (mostly fusions with NFIB) in a mutually-exclusive manner. However, its clinical significance remains to be well-established. METHODS: We investigated clinicopathological and molecular features of 36 ACCs with special emphasis on the significance of MYBL1 alterations. Reverse-transcription polymerase-chain reaction (RT-PCR) and fluorescence in-situ hybridization (FISH) were performed to detect MYB/MYBL1-NFIB fusions and MYBL1 alterations, respectively. Immunohistochemistry was performed to evaluate MYB expression in the tumors. The results were correlated with clinicopathological profiles of the patients. RESULTS: RT-PCR revealed MYB-NFIB and MYBL1-NFIB fusions in 10 (27.8%) and 7 (19.4%) ACCs, respectively, in a mutually-exclusive manner. FISH for MYBL1 rearrangements was successfully performed in 11 cases, and the results were concordant with those of RT-PCR. Immunohistochemically, strong MYB expression was observed in 23 (63.9%) tumors, none of which showed MYBL1 alterations. Clinicopathologically, a trend of a better disease-specific survival was noted in patients with MYBL1 alterations than in those with MYB-NFIB fusions and/or strong MYB expression; however, the difference was not significant. Interestingly, we found tumors with MYBL1 alterations significantly frequently occurred in the mandibular regions (P = 0.012). Moreover, literature review revealed a similar tendency in a previous study. CONCLUSION: Our results suggest that there are some biological or etiological differences between ACCs with MYB and MYBL1 alterations. Moreover, the frequent occurrence of MYBL1-associated ACC in the mandibular regions suggests that MYB immunohistochemistry is less useful in diagnosing ACCs arising in these regions. Further studies are warranted to verify our findings.
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Mucoepidermoid carcinoma (MEC) is the most common malignant tumor of the salivary glands and is seen most commonly in the parotid gland. Intraosseous MECs can be rarely seen and is mostly detected in the posterior part of the mandible. This condition can be acknowledged as an entity different from salivary gland MECs. In this case series, we delineated three patients of intraosseous MEC, two mandible tumors and one maxillary tumor and discussed the diagnostic characteristics and treatment methods of this rare entity.
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O ameloblastoma é o tumor odontogênico de origem epitelial, de maior significado clínico dos ossos gnáticos. A variante unicística está de 10 a 46% dos casos dos ameloblastomas intraósseos. Devido às suas características, a abordagem terapêutica do ameloblastoma, ainda, é controversa, sendo classificada em radical ou conservadora, cada qual com suas vantagens e desvantagens. Na escolha da abordagem cirúrgica, vários fatores devem ser levados em consideração, como localização, extensão, características histopatológicas, idade, entre outros. Este trabalho tem como objetivo demonstrar um caso de abordagem conservadora de um ameloblastoma unicístico com proliferação mural em paciente jovem. Ele queixava-se de aumento de volume em face, em região posterior esquerda de mandíbula. Os exames clínicos, radiográficos e histopatológicos confirmaram o diagnóstico de ameloblastoma unicístico do subtipo mural. Por se tratar de um paciente jovem e com uma lesão benigna, mas com caráter infiltrativo, foi submetido à marsupialização com posterior enucleação associada à ostectomia periférica e tratamento do leito cirúrgico com solução de Carnoy. O paciente encontra se atualmente em rígido controle clínico-radiográfico com doze meses de pós-operatório, observando-se, diante do tratamento eleito, relevante neoformação óssea e regressão total da lesão... (AU)
Ameloblastoma is the odontogenic epithelial origin tumor with the most common clinical significance of the gnatic bones. The unicystic variant is between 10 and 46% of cases of intraosseous ameloblastomas. Due to its characteristics, the therapeutic approach of ameloblastoma is still controversial being classified as radical or conservative, each with its advantages and disadvantages. In choosing the surgical approach, several factors must be taken into account such as location, extent, histopathological characteristics, age among others. This work aims to demonstrate a case of a conservative approach of a unicystic ameloblastoma with mural proliferation in a young patient. The same complained of volumetric increase in the face and posterior region of mandible. Clinical, radiographic and histopathological exams confirmed the diagnosis of unicystic ameloblastoma with the mural subtype. Because it is a young patient with a benign but infiltrative lesion, he was submitted to marsupialization with subsequent enucleation associated with the peripheral osteotomy and treatment of the surgical bed with Carnoy's solution. The patient is currently in rigid clinical-radiographic control with twelve months after surgery we observe, with the chosen treatment, relevant bone neoformation and total regression of the lesion... (AU)