Pindborg tumor associated with a supernumerary tooth: a case report

The calcifying epithelial odontogenic tumor is a rare benign neoplasm that accounts for approximately 1% of all odontogenic tumors. Most of the cases occur in the posterior mandible, and a few involve the maxilla. Despite their relatively indolent biological behavior, tumors in the maxilla tend to grow fast. We report the case of a 33-year-old female patient exhibiting swelling in the right maxilla. An isodense area associated with an impacted supernumerary tooth was found on imaging examination. The histopathologic diagnosis was a calcifying epithelial odontogenic tumor. The treatment of choice was surgical removal of the lesion and associated dental elements. The patient has been followed up for 11 months and shows no signs of recurrence. Besides describing this case, we reviewed the literature on the association of calcifying epithelial odontogenic tumors with supernumerary teeth and found two case reports addressing this subject.

Squamous cell carcinoma at sites of old maxillary fractures.

Malignancies have been reported to occasionally arise in scar tissue following injury. One hypothesis involves prolonged overactivation of tissue repair systems due to chronic inflammation and irritation, although the pathogenesis of cancers occurring in scars is not fully understood. We describe here two cases with a history of maxillary fracture at the site where squamous cell carcinoma (SCC) subsequently developed. The first patient developed SCC 7 years after right maxillary fractures resulting from a traffic accident. He underwent chemoradiotherapy (70 Gy in 35 fractions) and maintained complete response (CR) for 10 months. The second patient developed SCC 3 years after sustaining right maxillary fractures in an ice hockey game. Radiotherapy and total maxillectomy were performed, but local recurrence arose and he has since been receiving chemotherapy.

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.

Maxillofacial brown tumours: Series of 5 cases.

OBJECTIVES: Brown tumours are benign bone tumours secondary to hyperparathyroidism. The authors describe the various clinical features, diagnostic methods and treatment modalities for maxillofacial brown tumours. MATERIAL AND METHODS: This multicentre retrospective study comprised 5 patients (four women and one man, between the ages of 29 and 70 years) with one or several maxillofacial brown tumours observed over a 16-year period from January 2000 to December 2016. RESULTS: Four patients presented secondary hyperparathyroidism in a context of chronic renal failure, one patient presented primary hyperparathyroidism due to parathyroid adenoma. Three patients presented a mandibular brown tumour, and two patients presented a maxillary brown tumour. The diagnosis was based on histological examination and laboratory tests. Brown tumours were treated either surgically or conservatively. A favourable outcome was observed in all cases. CONCLUSION: Brown tumours are rare lesions. This diagnosis must be considered in a context of giant cell tumour associated with hyperparathyroidism. Brown tumours should be treated conservatively.

Squamous cell carcinoma of the maxilla: Analysis of clinicopathological predictors for disease recurrence and metastatic behavior.

INTRODUCTION: Squamous cell carcinoma of the maxilla only constitutes a small fraction of Head and Neck Cancers. There is thereby a lack of information about frequent tumor staging and localization and their effect on patients' outcome. The main factors that influence longterm survival in HNSCC are the extent of the primary disease and recurrence rate, including local neck metastases. PATIENTS AND METHODS: In this study, clinical outcome and rates of disease recurrence in 68 surgically treated patients with maxillary SCC were evaluated in terms of primary tumor staging and localization. RESULTS: It could be demonstrated that maxillary cancer is mostly located in the posterior region of the upper jaw (70%). The rate of neck node metastasis was 35.3%, which is equivalent to the rest of the oral cavity and supports the role of elective neck dissection for patients with clinically negative neck node status. Staging, tumor differentiation, and infiltration of lymphatic structures correlated significantly with the development of local neck node metastases (r = 0.321, p = 0.01; r = 0.348, p < 0.01; r = 0.64; p < 0.01). CONCLUSION: Maxillary carcinomas exhibit similar rates of locoregional disease recurrence as the rest of the oral cavity. The existence of cervical metastases even in patients with T1 tumors supports the concept of elective neck dissection in early tumors with clinically negative neck status.

Melanotic neuroectodermal tumor of infancy (MNTI) of the head and neck: A French multicenter study.

OBJECTIVES: Melanotic neuroectodermal tumor of infancy (MNTI) of the head and neck is a rare entity with uncertain clinical behavior. Radical surgical resection is the current recommended treatment, however this can cause severe aesthetic and functional sequelae. The aim of this study was to clinically characterize MNTIs and to stratify risk factors that may influence locoregional recurrence. METHODS: A retrospective multicenter study, including 11 patients from eight centers with a confirmed diagnosis of MNTI, was conducted. Epidemiological, clinical, radiological, pathological, and immunohistochemical examinations were reviewed. A statistical analysis using a t-test was conducted to calculate parameters correlating with tumor recurrence. RESULTS: MNTIs mainly occurred in the maxilla, with a mean age at diagnosis of 3.18 months (range: 0-6 months). Primary surgery was performed on 10 patients, with a clear margin resection on two patients. Overall recurrence rate was 27% with a survival of 100% at time of follow-up. No statistical correlation between recurrence rate, age at diagnosis, localization, resection margins, and pathological and immunohistochemical characteristics could be established. CONCLUSION: In our study, locoregional tumor recurrence did not seem to correlate with resection margins, so a conservative surgical approach may need to be considered to avoid functional and aesthetic sequelae.

The localization and risk factors of squamous cell carcinoma in the oral cavity: A retrospective study of 1501 cases.

Head and neck cancer is the tenth leading cause of cancer mortality. Ninety percent of tumours in the oral cavity are squamous cell carcinomas. Information about the exact localisation of OSCC is missing in the literature. In the present study, we retrospectively analysed a total of 1501 OSCC patients, who were treated between 1975 and 2009. The purpose of this study was to examine the localisation of OSCC tumours and to analyse the influence of various parameters on tumour localisation. 71.5% of these patients were male and 28.5% were female. The mean age was 60 years. The most common sites of OSCC occurrence were the floor of the mouth and the anterior base of the mouth. The hard palate was the most affected anatomical area of the maxilla. Descriptive statistical analysis, chi-square testing and a multivariate analysis using a multinomial logistical model showed a significant correlation of younger age and female gender with tumour occurrence in the maxilla and the tongue. We provide a very detailed anatomical mapping of OSCC.

Cáncer Bucomaxilofacial en Niños III: Tumores Malignos del Tejido Blando / Oral and Maxillofacial Cancer in Children III: Malignant Soft Tissue Tumors

RESUMEN: El grupo de neoplasias malignas de tejido blando de la región de cabeza y cuello en pacientes pediátricos está representado por carcinomas, sarcomas, melanomas y tumores de diferenciación incierta. La neoplasia más prevalente en la población pediátrica es el Rabdomiosarcoma, seguido por el carcinoma de células escamosas. Los rangos de presentación son muy amplios, siendo los grupos entre 2-6 años y 15-19 años los que presentan mayor incidencia. Se ha planteado que la etiología de estas neoplasias es incierta. El tratamiento de estas neoplasias es comúnmente de enfoque multimodal, combinando un procedimiento quirúrgico con quimioterapia y radioterapia. El pronóstico y sobrevida del paciente dependerán principalmente del momento en que se realice el diagnóstico de la lesión. Un diagnóstico y tratamiento temprano favorecen las posibilidades de sobrevida y el pronóstico del paciente. Este estudio corresponde a la 3ra parte de "Cáncer bucomaxilofacial en niños". Se hará referencia a los distintos tumores malignos del tejido blando en la población pediátrica en el territorio de cabeza y cuello, abarcando sus generalidades, etiología, epidemiología, tratamiento y pronóstico.

ABSTRACT: Head and neck malignant tumors in pediatric patients comprise carcinoma, sarcoma, melanoma and tumours of uncertain differentiation. Within the pediatric population, the most prevalent neoplasm is rhabdomyosarcoma, followed by squamous cell carcinoma. There is a wide range in the presentation, and it varies significantly with age groups of 2-6 and 15-19 year-olds who present the higher incidence rates. For this reason, it has been suggested that the etiology of head and neck neoplasms remains unclear. Treating these pathologies usually involves a multimodal approach that combines surgery, radiation and chemotherapy. Prognosis and survival rates depend mainly of the stage at the time of diagnosis. Early diagnosis and treatment can improve prognosis and survival rates. In this 3rd part of "Maxillofacial Cancer in Pediatric Patients", we studied a variety of malignant tumors in head and neck soft tissue from a paediatric sample. Specifically, we aim to analyze their etiology, epidemiology, treatment and prognosis.

Radiation-induced osteosarcoma of the maxilla and mandible after radiotherapy for nasopharyngeal carcinoma.

BACKGROUND: The increasing incidence of radiation-induced osteosarcoma of the maxilla and mandible (RIOSM) has become a significant problem that can limit long-term survival. The purpose of this study was to analyze the association of clinicopathologic characteristics with treatment outcomes and prognostic factors of patients who developed RIOSM after undergoing radiotherapy for nasopharyngeal carcinoma (NPC). METHODS: We reviewed the medical records of 53,760 NPC patients admitted to Sun Yat-sen University Cancer Center during the period August 1964 to August 2012. Of these patients, 47 who developed RISOM and met inclusion criteria were included in this study. Two of these 47 patients refused treatment and were then excluded. RESULTS: For all patients treated for NPC at Sun Yat-sen University Cancer Center during the study period, the total incidence of RIOSM after radiotherapy was 0.084% (47/53,760). Two patients (4.4%) had metastases at the diagnosis of RIOSM. Thirty-nine of the 45 (86.7%) patients underwent surgery for RIOSM; most patients (24/39; 61.5%) who underwent resection had gross clear margins, with 15 patients (38.5%) having either a gross or microscopic positive margin. All patients died. The 1-, 2-, and 3-year overall survival (OS) rates for the entire cohort of 45 patients were 53.3%, 35.6% and 13.5%, respectively. The independent prognostic factors associated with high OS rate were tumor size and treatment type. CONCLUSIONS: RISOM after radiotherapy for NPC is aggressive and often eludes early detection and timely intervention. Surgery combined with postoperative chemotherapy might be an effective treatment to improve patient survival.

Maxillary mesenchymal chondrosarcoma presenting with epistaxis in a child.

Mesenchymal chondrosarcomas are a rare variant of primary chondrosarcomas and can pose a diagnostic dilemma, especially when the features on conventional imaging are equivocal for an aggressive lesion. There is very little PET-CT experience in mesenchymal chondrosarcomas as per the literature and to the best of our knowledge, we are the first to describe a maxillary mesenchymal chondrosarcoma on PET-CT imaging. We report a case where PET-CT not only complemented conventional imaging in suspecting a malignant osseous lesion, but also was indicative of the grade of the tumor.

Elodontoma in Two Guinea Pigs.

Elodontoma was diagnosed in two pet guinea pigs, one involving a maxillary premolar tooth and the other affecting a mandibular incisor tooth. Diagnostic imaging, including radiographs, computed tomography, and oral endoscopy was performed in order to quantify dental disease. Diagnostic imaging was also used to guide treatment of acquired dental disease, which included intraoral restoration of normal occlusal plane and tooth extraction using an extraoral approach. These are the first histologically confirmed cases of elodontoma in guinea pigs.

An idiosyncratic post-traumatic tetrad: compound odontome, dentigerous cyst, impaction, and double-dilaceration.

OBJECTIVE: This report describes the case of a 13-year-old patient who experienced traumatic intrusion of the primary maxillary right central incisor and subsequently suffered an atypical tetrad, comprising of an unerupted compound odontoma associated with a dentigerous cyst, and an impacted, doubly dilacerated permanent maxillary right central incisor; however, the high interconnectivity of the occurrence of four pathologies together is unusual has not previously been reported. SUMMARY: The pathologies were detected 7 years after trauma; surgical removal of odontome along with the dentigerous cyst was performed, followed by orthodontic extrusion of the impacted double-dilacerated permanent central incisor. The 18-month follow-up shows no pathology, no gingival recession, and normal probing depth.

Maxillary brown tumor as initial presentation of parathyroid adenoma: a case report.

Brown tumor is a rare late-stage skeletal change caused by long-term stimulation of excess parathyroid hormone. It is not neoplastic, but a reparative cellular process. Common sites of brown tumor are the ribs, clavicle, long bones and pelvic girdle. Solitary maxillary brown tumor as initial presentation of primary hyperparathyroidism is rare; it is often accompanied by brown tumors of the other facial bones. Here, we present the first case of solitary maxillary brown tumor in a 29-year-old ethnic Chinese woman with initial presentation of a large tumor filling the left maxillary sinus. Underlying long-standing primary hyperparathyroidism caused by a large parathyroid adenoma was finally diagnosed. Brown tumor tends to be misdiagnosed as malignancy, and delayed diagnosis of the underlying hyperparathyroidism is common. Our case validates the suggestion that young women have a higher probability of brown tumor. Biopsy of the suspicious bone tumor and blood tests for calcium and parathyroid hormone level are crucial and essential to reach the correct diagnosis. Most brown tumors show spontaneous regression after parathyroidectomy. However, direct excision of the brown tumor may be indicated to avoid the risk of facial deformity and orbital compression at a special anatomical site, as in our case.

Intraoral desmoplastic fibroma: a manifestation of tuberous sclerosis.

Desmoplastic fibroma (DF) is a benign but locally aggressive tumor usually recognized as an intraosseous counterpart of soft tissue fibromatosis in both gnathic and extra-gnathic sites. The most common site is mandible followed by pelvis and long bones. Maxilla has been reported to be involved very rarely. Desmoplastic fibroma is recently being considered to be one of the oral manifestations of tuberous sclerosis rather than a coincidental finding. We report an unusual case of a girl with a previous diagnosis of tuberosclerosis who presented at 8 years with an oral lesion diagnosed as desmoplastic fibroma.

Squamous cell carcinoma in association with dental implants: an assessment of previously hypothesized carcinogenic mechanisms and a case report.

Although dental implants have seen tremendous clinical success over the past few decades, there are some worrying reports in literature describing squamous cell carcinoma (SCC) in close association with dental implants. This article also provides a critical assessment of the published literature relating to the presence of carcinoma in association with dental implants, analyzing the previously published and hypothesized carcinogenic responses to an implant, to try and come to a conclusion regarding the plausibility and clinical risk for cancer formation in association with dental implants. An unusual case of an SCC noted in close proximity to a dental implant is also presented. A systematic search was conducted using Medline (PubMed), Cochrane Database, and Google Scholar with the search terms "cancer," "squamous cell carcinoma," "dental implant," "SCC," "peri-implantitis," "oral cancer," and "implantology" and using multiple combinations using Boolean operators "or" and "and." The search was not limited to dental literature; orthopedic and biomedical literature was also included. The results were then hand screened to pick out the relevant articles. In total, 14 previous published reports were found, where 24 dental implants were reported to be associated with SCC. Not all the reported patients had a history of cancer, but contributory factors such as smoking were found. An analysis of the biological plausibility of previously proposed carcinogenic mechanisms, such as corrosion, metallic ion release, and particulate debris, did not support the etiologic role for dental implants in cancer development, and the standardized incidence ratio was found to be extremely low (0.00017). Peri-implantitis should be assessed cautiously in patients receiving implants who have a previous history of cancer. Dental implants are a safe treatment modality based on the published data, and any change in surgical protocol is not mandated.

Radiation-induced malignant fibrous histiocytoma of the maxilla.

Malignant fibrous histiocytoma (MFH) originates from primitive mesenchymal cells and has the capacity for dual histiocytic and fibroblastic differentiation. We report on an MFH of the left maxilla that developed in a 79-year old woman 20 years after surgery and radiation for squamous cell carcinoma (SCC). Postoperative radiotherapy with 70 Gy was administered for a primary neoplasm of SCC of the left maxilla to a localized field through two lateral ports. This secondary neoplasm arose at the site of tumor resection (partial maxillectomy) within the irradiated field, and was resected. The development of sarcomas is a recognized complication of radiation therapy. The final diagnosis after the operation was MFH. The patient died of tumor recurrence at the skull base and within the cranium, 19 months after the operation. Radiation-induced sarcoma is well known, but radiation-induced MFH is relatively rare in the head and neck region. The details of this case are presented with a review of literature.

Developmental dental alterations in permanent teeth after intrusion of the predecessors: clinical and microscopic evaluation.

This case report describes the management of developmental dental alterations in permanent dentition as a consequence of severe intrusive luxation in its predecessors in a child of 2 years. At 10 years of age, this patient was referred for consultation due to lack of permanent maxillary right central and lateral incisors. Radiographic examination revealed impaction of hypoplasic permanent maxillary central incisor, absence of the lateral incisor and compound odontoma in region of the permanent maxillary lateral incisor. The odontoma was surgically removed and unerupted central incisor was placed in orthodontic traction over a period of 8 months. The central incisor presented with abnormal shape and was restored with composite resin. Odontoma histologic analysis was carried out through Hematoxylin and Eosin coloration and Scanning Electron Microscopy. Cement and osteocement formations were found in soft tissue, as well as some irregularly distributed dentine islands of tooth-like structures, indicative of compound odontoma. We followed up this patient for 5 years and orthodontic management was successfully performed for correct alignment of the maxillary right central incisor impacted by compound odontoma.

Multiple keratocystic odontogenic tumors associated with nevoid basal cell carcinoma syndrome having distinct PTCH1 mutations: a case report.

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant disorder characterized by developmental abnormalities and a predisposition to cancers. Although multiple jaw tumors, such as keratocystic odontogenic tumors (KCOTs), are one of the most frequent complications in NBCCS, the molecular mechanism for how KCOTs develop in NBCCS is poorly understood. A 15-year-old girl with 2 jaw tumors was diagnosed as NBCCS according to the clinical criteria. The pathologic findings indicated that the 2 tumors were consistent with KCOTs. A PTCH1 mutation, c.1472delT, was detected in her peripheral blood as well as in the 2 tumors. Interestingly, an additional PTCH1 mutation, c.264_265insAATA, that was not present in the peripheral blood, was found in the maxillary tumor but not the mandibular tumor. The Ki-67 labeling index was significantly higher in the maxillary KCOT (17.7%) than in the mandibular KCOT (14.3%). These findings indicate distinct molecular mechanisms of tumorigenesis in these KCOTs.