Chondromyxoid fibroma affecting the maxilla in a 1-year-old child: Immunohistochemical analysis and literature review.

Chondromyxoid fibroma (CMF) is a benign chondroid/myxoid matrix-producing tumor that often develops in the long bones of young adults. CMF is rarely reported in the craniofacial skeleton, with most cases presenting with bone erosion or destruction, which may lead to a misdiagnosis. To date, approximately 129 cases of CMF in the craniofacial region have been reported, with only three cases in patients aged less than 1 year. Of these 129 cases, only 34 affected the jaws. A 1-year-old boy presented with a mass in the left anterior maxilla, extending and compressing the ipsilateral nasal cavity. After surgical excision of the lesion, microscopy revealed spindle-to-stellate tumor cells surrounded by a predominant myxoid stroma containing focal slit-like vascular channels and hemorrhagic areas. Immunohistochemistry showed positivity for vimentin, CD10, and α-SMA (focal). The Ki-67 labeling index was 6%. CFM should be included in the differential diagnosis when assessing maxillary tumors in pediatric patients.

Jaw osteosarcoma and pregnancy: a rare coexistence

Osteosarcoma of the jaw represents less than 1% of all head and neck malignancies. This malignancy in pregnant women occurs in one per 1000 deliveries. We report a case of a 29-year-old woman, in the 33rd week of gestation, who presented with an expansive tumor destroying the maxillary alveolar bone, histologically composed of pleomorphic, round, spindle, or epithelioid cells and osteoid/chondroid matrix. Upon final diagnosis of osteosarcoma, the lesion was excised. To the best of our knowledge, only 10 cases of jaw osteosarcoma in pregnant women have been reported to date in the English language literature. The use of ancillary examinations, malignancy diagnosis, and cancer treatment can be challenging during pregnancy. Knowledge about jaw osteosarcoma in pregnancy can increase healthcare providers' awareness, avoid delays and misdiagnosis and potentially improve maternal and neonatal outcomes.

A Rare Case of an Aggressive Clear Cell Variant of Calcifying Epithelial Odontogenic Tumor in the Posterior Maxilla.

A clear cell variant of calcifying epithelial odontogenic tumor is a rare benign odontogenic neoplasm, accounting for 33 cases described in the literature. In this article, we report a challenging example of clear cell variant of calcifying epithelial odontogenic tumor of the posterior maxilla in a 45-year-old female patient showing locally aggressive growth and recurrence. Microscopically, islands of polyhedral cells containing abundant cytoplasm, well-developed intercellular bridges blended with clear cells were observed. The nuclei were frequently pleomorphic and permeated by hyaline calcified material. Immunohistochemistry revealed positivity for pan-cytokeratin (AE1/AE3), cytokeratins (CK-14 and CK-19), Bcl-2, p53, and p63. The Ki-67 proliferative index was ~10%. As odontogenic tumors are rare, when a significant clear cell component is observed, the differential diagnosis with other lesions of the jaws with similar morphology, including other odontogenic tumors with prominent clear cell component, clear cell odontogenic carcinomas, and metastatic tumors, is difficult.

Calcifying Odontogenic Cyst Associated With Dentigerous Cyst in a 15-Year-Old Girl.

Calcifying odontogenic cyst (COC) is a rare odontogenic cyst with ameloblastic epithelial lining containing clusters of ghost cells. COCs have been described in association with several odontogenic tumors, more commonly odontomas and rarely with dentigerous cyst (DC). In this article, we describe a case of COC associated with DC in a 15-year-old girl, who presented with a swelling on the right middle third of the face, producing facial asymmetry. Panoramic radiography showed a well-circumscribed, corticated, and unilocular radiolucency at the level of the right maxillary sinus, involving 2 unerupted premolars. The lesion was enucleated and histologically revealed a COC associated with DC, which presented mucous metaplasia. Immunohistochemical reactions were performed to better illustrate this rare synchronous occurrence of COC and DC, showing positivity for CK5, CK14, CK19, and p63 in both lesions. CK18 was negative in COC, and Bcl-2 was negative in DC. Periodic acid Schiff highlighted the mucous cells in the DC lining.

Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla.

Melanotic neuroectodermal tumor of infancy is a rare and fast-growing neoplasm. In this study, we describe the case of a 6-month-old female patient, who presented swelling in the anterior maxilla. Tomographic reconstruction showed an unilocular hypodense and expansive area associated with the upper right central primary incisor. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. Microscopically, the lesion revealed a biphasic cell population, consisting of small, ovoid, neuroblastic-like cells and epithelioid cells containing melanin. Immunohistochemically, the melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. Then, enucleation of the lesion was performed by careful curettage. After 2 year follow-up, no clinical or radiographical evidence of recurrence was verified. The present case highlights the importance of early diagnosis and therapeutic intervention at the appropriate time to achieve a favorable outcome for the patient.

Management of compound odontoma with guided bone regeneration: a case report

An odontoma is a mixed benign odontogenic tumor, being a hamartomatous neoplastic lesion of odontogenic epithelial and mesenchymal cells that form enamel, dentine and cement, which occurs in the second to third decade of life, and is commonly a radiographic finding. The aim of this paper is to describe the management of a compound odontoma with guided bone regeneration. A 20-year-old asymptomatic male patient with no relevant surgical medical history underwent surgery for the enucleation of a compound odontoma followed by guided bone regeneration of the defect, and histopathological study of the specimen. The evolution was favorable, without any sign of recurrence. It is important to carry out a previous surgical plan, taking into account the size of the defect resulting from its enucleation, assess the need for guided bone regeneration and use complementary diagnostic resources for a satisfactory recovery.

Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla / Estudo clínico e imuno-histoquímico de tumor neuroectodérmico melanocítico da infância em maxila

ABSTRACT Melanotic neuroectodermal tumor of infancy is a rare and fast-growing neoplasm. In this study, we describe the case of a 6-month-old female patient, who presented swelling in the anterior maxilla. Tomographic reconstruction showed an unilocular hypodense and expansive area associated with the upper right central primary incisor. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. Microscopically, the lesion revealed a biphasic cell population, consisting of small, ovoid, neuroblastic-like cells and epithelioid cells containing melanin. Immunohistochemically, the melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. Then, enucleation of the lesion was performed by careful curettage. After 2 year follow-up, no clinical or radiographical evidence of recurrence was verified. The present case highlights the importance of early diagnosis and therapeutic intervention at the appropriate time to achieve a favorable outcome for the patient.

RESUMO O tumor neuroectodérmico melanocítico da infância é uma neoplasia rara e de crescimento rápido. Neste estudo, relata-se o caso de uma paciente do sexo feminino de 6 meses de idade, que apresentou tumefação na região anterior de maxila. A reconstrução tomográfica revelou área unilocular hipodensa e expansiva associada ao incisivo central superior direito decíduo. Realizou-se biópsia incisional, considerando as hipóteses diagnósticas de cisto dentígero, tumor odontogênico adenomatoide, tumor neuroectodérmico melanocítico da infância e rabdomiossarcoma. Microscopicamente, a lesão revelou população celular bifásica, consistindo de células pequenas, ovoides, de aparência neuroblástica, e de células epitelioides, contendo melanina. A análise imuno-histoquímica demonstrou que o componente celular contendo melanina era positivo de forma intensa e difusa para HMB-45 e Melan-A, mas levemente positivo para S100. Com base nestes achados, foi estabelecido o diagnóstico definitivo de tumor neuroectodérmico melanocítico da infância. Em seguida, foi realizada a enucleação da lesão com curetagem cuidadosa. Após 2 anos de acompanhamento, não foram verificadas evidências clínicas ou radiográficas de recorrência. O presente caso destaca a importância do diagnóstico precoce e da intervenção terapêutica no momento apropriado, a fim de alcançar um desfecho favorável para o paciente.

Aggressive Osteoblastoma in the Maxilla: Unusual Lesion in the Craniofacial Skeleton.

Osteoblastomas are benign bone tumors, which are unusual in the craniofacial skeleton, being most often observed in the axial skeleton and long bones. The most common site in the maxillofacial region is the mandible and the involvement of the maxilla and paranasal sinuses is extremely rare. Although it is a benign lesion, the aggressive variant raises concerns due to its huge local destructive potential and tendency to relapse. In this clinical case, an aggressive osteoblastoma is described in a 7-year-old patient. The lesion was large and fully involved the left maxilla, including the maxillary sinus and the nasal cavity. Recurrent volume increase was observed 2 months following enucleation of the lesion and en bloc resection of the maxillary segment was performed. Histological and immunohistochemical evaluation associated with clinical and imaging findings allowed to define the tumor as an aggressive variant of osteoblastoma and not osteosarcoma, despite the aggressive behavior. The patient recovered well and no relapses were observed after 12 months following maxillary resection.

Odontogenic Mixoma in a Ten-Year-Old Patient.

Odontogenic myxomas are benign, rarely seen intraosseous tumors arising from the embryonic connective tissue associated to tooth formation. The authors present the case of a 10-year-old patient affected by this entity.

An update on peripheral ossifying fibroma: case report and literature review.

The purpose of the present article was to present a clinical case of an 11-year-old girl with peripheral ossifying fibroma (POF). Additionally, after performing a literature review, we identified clinical information that occurs more frequently in association with POF, such evidence would help professionals in yielding a specific diagnosis and tailor a more specific therapeutic approach with the objective to decrease morbidities' associated with POF. This lesion represents the third most common lesion of all localized reactive hyperplastic lesions. Clinical aspects related to this pathology include the fact that it occurs most frequently in women between the first and second decades of life. It affects anterior maxillary region and interferes with normal functioning of this anatomical structure. After conducting the literature search, we found that it can also be presented in a considerable number of males with pain and hyperemia being the most common clinical manifestations. We found that often clinical cases are presented with incomplete information. It is important that in order to get to a consensus with respect to updates about information related to this lesion, new case series that include complete clinical information, radiographic analysis, and histopathology tests could be presented.

Epidemiologia das lesões fibro-ósseas benignas dos maxilares / Epidemiological study of the benign fibro-osseous lesions (BFOLs) of the jaws

As lesões fibro-ósseas benignas (LFOBs) são condições raras caracterizadas pelo desenvolvimento de doenças neoplásicas, reativas ou displásicas, nas quais o osso normal é substituído por tecido conjuntivo composto por colágeno, fibroblastos e diferentes materiais mineralizados semelhantes ao osso ou cemento. Os principais tipos são: displasia fibrosa (DF): monostótica (DFM) ou poliostótica (DFP); displasia óssea (DO): periapical (DOP), focal (DOFoc) ou florida (DOFlor); e o fibroma ossificante (FO): convencional (FOC) ou juvenil (FOJ). O objetivo deste estudo foi descrever o perfil epidemiológico das LFOBs dos maxilares diagnosticados no serviço de referência em Patologia, Estomatologia e Radiologia da Faculdade de Odontologia da Universidade Federal de Minas Gerais e comparar com os resultados encontrados em outros estudos. Os dados foram obtidos dos arquivos dos serviços clínico e laboratorial, no período de 26 anos (1990-2015). Informações de gênero, idade, raça dos pacientes e principais características clínicas e radiográficas da lesão foram coletadas. Foi realizada a análise estatística descritiva das variáveis e teste de associação entre os grupos de lesões e o gênero, faixa etária, aumento de volume e osteomielite. Entre todos os diagnósticos do período avaliado (27998 prontuários), as LFOBs representaram 1,36% (n=383). As DOs (n=187, 48,8%) foram as mais frequentes, seguidas pela DF (n=103, 26,9%) e FO (n=93, 24,3%). Quanto aos subtipos de LFOBs, as mais frequentes foram a DOFlor e a DFM, ambas com 101 casos (26,4%). As mulheres foram a maioria no estudo, com frequência de 82,0% (n=314), assim como os pacientes não negros (59,0%, n=226). Entre todas as LFOBs a média de idade foi de 38,52 ± 17,54, acometendo mais a 4ª e 5ª décadas. As características radiográficas mais comuns foram imagens mistas: radiopaca e radiolúcida (51,7%). A mandíbula foi a região mais acometida (n=247, 64,5%), exceto para DF que envolveu mais a maxila (n=68, 66,0%). A DF foi observada mais entre os homens que em mulheres, assim como entre os pacientes mais jovens. DOs foram mais comuns em mulheres e em pacientes mais velhos. Enquanto que no FO não foi observada diferença significativa entre o gênero. A associação entre a presença de osteomielite e DO foi estatisticamente significante (p=0,0001). O aumento de volume foi significante na DF e FO (p=0,0001). A frequência de LFOB é semelhante à previamente reportada na literatura no mesmo país, mas difere dos dados observados em outras populações. É importante considerar a correlação de dados clínicos, radiográficos e histopatológico para o diagnóstico definitivo de LFOBs. Estudos epidemiólogicos podem orientar o clínico sobre a perfil do paciente acometido por uma doença. Nossos resultados são importantes para auxiliar no diagnóstico e manejo clínico das lesões fibro-ósseas benignas

Benign fibro-osseous lesions (BFOLs) are rare conditions characterized by the development of neoplastic, reactive or dysplastic disease, in which is observed replacement of the normal bone by collagen, fibroblasts and different mineralized materials like bone and cementum. The main types are: fibrous dysplasia (FD): monostotic (MFD) or polyostotic (PFD); osseous dysplasia (OD): periapical (POD), focal (FocOD) or florid (FlorOD); and ossifying fibroma (OF): conventional (COF) or juvenile (JOF). The aim of this study was to describe the epidemiological profile of BFOLs of the jaws diagnosed in the reference service in oral pathology at the Universidade Federal of Minas Gerais and compare it with the results found in other studies. Data were obtained from the archives of clinical and laboratory services in the period of 26 years (1990-2015). Gender, age, race and main clinical and radiographic characteristics of the lesion were collected. Descriptive statistical analysis of each variable and associations test between groups of lesions and gender, age, swelling and osteomyelitis were made. Among all diagnoses in the period (n=27998 records), the BFOLs represented 1.36% (n=383). The ODs (n=187, 48.8%) were the most frequently diagnosed, followed by FD (n=103, 26.9%) and OF (n= 93, 24.3%). Regarding the subtypes, the most common were FlorOD and MFD, both with 101 cases. Women were the majority in the study, with frequency of 82.0% (n=314), as well as non-black patients with (n=226, 59.0%). Among all LFOBs the average age was 38.52 ± 17.54, affecting more the 4th and 5th decades. The most common radiographic features were mixed images: radiopaque and radiolucent (51.7%). The mandible was the most affected region (n=247, 64.5%), except for DF involving more maxilla (n=68, 66.0%). The DF was observed more in men than in women, as well as among younger patients. DOs were more common in women and older patients. The FO was no significant difference between genders. The association between the presence of osteomyelitis and OD was statistically significant (p=0.0001). The swelling was significant in FD and FO (p=0.0001). The frequency of LFOB is similar to that previously reported in the literature in the same country, but differs from data observed in other populations. It is important to consider the correlation of clinical, radiographic and histopathologic for definitive diagnosis of LFOBs. Epidemiological studies can guide the clinician on the patient's profile affected by a disease. Our results are important to assist in the diagnosis and clinical management of benign fibro-osseous lesions

Tumor odontogénico adenomatoide: presentación característica de un tumor infrecuente; revisión bibliográfica y presentación de un caso clínico / Adenomatoid odontogenic tumor: characteristic presentation of an unusual tumor; review of the literature and report of a case

El tumor odontogénico adenomatoide (TOA) es una neoplasia benigna epitelial de origen odontogénico que se manifiesta con mayor predisposición en el sextante anterior del maxilar superior, aunque puede presentarse en otras localizaciones. Su nombre refleja el rasgo histológico característico de estructuras parecidas a conductos entremezclados con todo el componente epitelial, dando a la lesión un aspecto glandular o adenomatoso. Sigue patrones clínicos y radiográficos característicos. Dado que la lesión no es agresiva biológicamente y requiere de tratamiento conservador, es de extrema importancia su identificación y diferenciación de otras lesiones, especialmente del ameloblastoma...

Mixed periapical lesion: an atypical radicular cyst with extensive calcifications.

The radicular cyst is an inflammatory odontogenic cyst of endodontic origin. Radiographically, the lesion appears as a periapical radiolucent image. This report describes a very rare case of a mixed periapical radiographic image diagnosed as a radicular cyst. A 37-year-old female patient presented a mixed, well-circumscribed image located in the periapical region of the left maxillary central incisor, which presented unsatisfactory endodontic treatment. Microscopic examination revealed a cavity lined by non-keratinized squamous epithelium and extensive calcifications in the cystic lumen and lining epithelium. Diagnosis of radicular cyst with extensive calcifications was established. Endodontic retreatment was performed and no radiographic signs of recurrence were observed 18 months after treatment. Although very rare, a radicular cyst should be considered in the differential diagnosis of a mixed periapical image associated to teeth with pulp necrosis.

Melanotic neuroectodermal tumor of infancy in an African-indigenous patient from the Amazon: a case report.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare condition that occurs normally in the anterior maxilla of infants aged <1 year. The use of clinical and imaging tools for MNTI is extremely important to prevent problems with function as well as the aesthetic features in a breastfeeding child. Brazil is a multiethnic country with a poor epidemiological policy and little data to track the incidence of certain diseases, including MNTI. It is important to study this pathology with ethnicity as a factor to improve the current epidemiological programs and establish better post-treatment management. This paper describes a case of a 2-month-old male of African-indigenous descent and Brazilian Amazon residency, who presented to our unit in 2009 with a history of an expanding mass involving the anterior maxilla. Clinical and computerized tomography scans were used to diagnose the mass as MNTI, which was removed by total excision. A biopsy later confirmed the MNTI diagnosis.

Synchronous ossifying fibromas of maxilla and mandible.

Ossifying fibroma is a benign neoplasm composed of fibrocellular tissue and mineralized material of varying appearance, which is most commonly seen in the posterior region of the mandible as a solitary lesion. We report an unusual case of synchronous ossifying fibroma of maxilla and mandible. To the best of our knowledge, only 13 similar cases have been previously reported.

Extensive central ossifying fibroma of the maxilla: a case report.

Central ossifying fibroma (COF) is a benign osteogenic neoplasm, with fibrous tissue and calcifications similar to bone, which has the clinical presentation of an asymptomatic bulging. It is more common in the mandibular premolar and molar region, in the third and fourth decades of life, and in women more frequently than men. Surgical removal is the treatment of choice and additional reconstruction is essential due to the functional and esthetic problems faced by the patient. This article describes the surgical treatment and subsequent reconstruction in a 22-year-old man with COF and an expansile lesion of the anterior maxilla. Enucleation of the lesion was performed and porous high-density polyethylene biomaterial was used for reconstruction.

Maxillary desmoplastic fibroma with initial symptoms suggestive of sinusitis.

Desmoplastic fibroma (DF) is a benign intra-osseous neoplasm characterized by the formation of abundant collagen fibers. It arises most commonly in the jaws and exhibits local aggressiveness and high recurrence rates after local resection. An uncommon case of expansive DF involving the right maxilla, maxillary sinus, and inferior orbital wall of a 49-year-old man whose initial symptoms were similar to acute sinusitis is presented, and the criteria for diagnosis and clinical management are discussed.

[Maxillary osteosarcoma. A woman with 12 years of survival]. / Osteosarcoma maxilar. Mujer con supervivencia de 12 años.

BACKGROUND: The osteosarcomas of the jaws are infrequently tumors. The accelerated bone growthing and the swelling with nervous sensibility alterations are suggestive of malignancy. The diagnosis is established only by the histological study and the standard treatment used is surgery with a poor survival prognosis of only 25% at five years for those cases with margins free of tumor. CLINICAL CASE: A female with an osteosarcoma of the jaw limited to the anterior left face of the maxillary and malar area without involve of the alveolar bone. A modified maxillectomy wasdone. Chemotherapy and radiotherapy were given after surgery. At the present, the patient is free of malignancy activity after twelve years of survival. CONCLUSIONS: No matter the systemic chemotherapy in maxillar and jaw osteosarcomas is little effective, the early diagnosis and treatment was the key in the clinical evolution of the patient presented and in who the first elected treatment was surgery.