The changing clinical pattern of endemic Burkitt lymphoma in Western Africa: Experience from a tertiary center in Ghana.

BACKGROUND: Burkitt lymphoma (BL) is the most common childhood cancer in Ghana, where the endemic variant is the predominant subtype and historically presents as a highly chemo-sensitive jaw tumor. This study aimed to update the current epidemiological characteristics of childhood BL in our institution. PROCEDURE: Patient data for all children diagnosed with BL and seen at Korle Bu Teaching Hospital between January 2007 and December 2012 were retrospectively analyzed. RESULTS: BL was diagnosed in 173 children (<13 years) during the study period, with the abdomen as the most common tumor site (46%) followed by the jaw (31%). Abdominal tumors were associated with advanced/disseminated disease (P = 0.002), and were more likely to occur in females irrespective of tumor stage (relative risk = 1.56 [95% CI; 1.1-12.3]). Twenty-five percent (43/173) of the study cohort died and mortality was influenced by increasing age (P = 0.02) and advanced disease (P = 0.03). Treatment delay was experienced by nine in ten patients primarily due to familial financial constraint (75%). Treatment abandonment was observed as a first event in 94% of patients and two thirds of children in the study were eventually lost to follow-up. CONCLUSION: The predominance of primary abdominal tumors in our study cohort may indicate a changing epidemiological pattern of BL in Ghana. High rates of treatment delay and abandonment were evident and are likely to be contributing factors to the poor childhood cancer survival outcomes seen in resource-limited countries in Africa.

A squamous odontogenic tumor following an orthodontic micro-screw: A rare case report and review of the literature.

We reported a very rare case of squamous odontogenic tumor(SOT) in a 23-year-old female. The tumor arose after an implanting operation of an orthodontic micro-screw, and was definitely diagnosed by the histopathological examination. Based on the case report and a review of the literature, we discussed about the general features, differential diagnosis and pathogenesis of SOT.

Ameloblastoma: current etiopathological concepts and management.

Ameloblastoma is a benign odontogenic tumor of epithelial origin. It is locally aggressive with unlimited growth capacity and has a high potential for malignant transformation as well as metastasis. Ameloblastoma has no established preventive measures although majority of patients are between ages 30 and 60 years. Molecular and genetic factors that promote oncogenic transformation of odontogenic epithelium to ameloblastoma are strongly linked to dysregulation of multiple genes associated with mitogen-activated protein kinase, sonic hedgehog, and WNT/ß-catenin signaling pathways. Treatment of ameloblastoma is focused on surgical resection with a wide margin of normal tissue because of its high propensity for locoregional invasion; but this is often associated with significant patient morbidity. The relatively high recurrence rate of ameloblastoma is influenced by the type of molecular etiological factors, the management approach, and how early the patient presents for treatment. It is expected that further elucidation of molecular factors that orchestrate pathogenesis and recurrence of ameloblastoma will lead to new diagnostic markers and targeted drug therapies for ameloblastoma.

Unusual jaw metastasis from squamous cell lung cancer in heavy smoker: Two case reports and review of the literature.

RATIONALE: Jaw metastasis is a very rare condition associated with lung cancer evolution. In this paper we present two cases of patients who underwent different approach.Lung cancer is the leading cause of cancer related death worldwide. Survival depends on the staging and biology of tumor. Smoking may affect the prognosis. PATIENT CONCERNS: There are herein reported the cases of two patients affected by squamous cell lung cancer with spread to mandibular bone. DIAGNOSES: In the first case a computed tomography (CT scan) showed a large mass located in upper right lung , then bronchoscopy was performed with biopsies and the histology revealed a squamous cell carcinoma P63+ and TTF-. In the second case upper right lung mass with metastases to mediastinal bilateral lymph nodes was found at CT scan. A bronchial biopsy revealed a squamous cell carcinoma p63 positive and TTF-1 negative. INTERVENTIONS: The first, a 65-years old man, current heavy smoker who quit and relapsed, at initial stage of IIa (T2aN1M0), after initial neo-adjuvant chemotherapy underwent lung lobectomy and lymph nodes resection. Three months later a metastasis located on mandibular bone was found out. The second case, a 68-years old female heavy smoker, at initial stage IIIb not available for surgery because of involvement of bilateral lymph nodes, underwent first line course chemotherapy with cisplatin and vinorelbine and second line with erlotinib. OUTCOMES: The first patient underwent hyperthermia and radiotherapy but a sepsis developed and patients died. In the second case, after two months from the beginning of II line the patient developed a jaw metastasis and underwent surgery with resection of hemi-mandible, but soon after she died because of pulmonary embolism. LESSONS: These cases highlight the poor prognosis of patients current smokers affected by squamous cell lung cancer. The jaw bone localization is very rare and different approach could be applied.

Clinicopathologic evaluation of malignancy adjacent to dental implants.

OBJECTIVE: The aim of this study was to describe a new case series of peri-implant malignancy, review the literature, and discuss the implications of malignancies resembling peri-implantitis. STUDY DESIGN: This study was a retrospective analysis of cases from 2000 to 2016. RESULTS: Seven patients (two males and five females), aged 44 to 89 years, were included, representing 1.5% of oral malignancy cases. Five cases were squamous carcinoma, one of basal cell carcinoma, and one of carcinoma of metastatic origin. Six cases presented with nonulcerated overgrowth, with bone loss in three and massive osteolysis in one. Misinterpretation as peri-implantitis delayed diagnosis in six cases. Risk factors included previous oral malignancy (2), potentially malignant conditions (2), and smoking (1). Of the 47 cases in the English language literature, 85% were squamous cell carcinoma and 8.5% had distant metastasis. Most cases had one or more risk factors. CONCLUSIONS: Peri-implant malignancy may represent up to 1.5% of oral malignancy cases. Clinical features imitating peri-implantitis may delay diagnosis. Lesions failing to respond to treatment, especially in patients with pre-existing risk factors, should significantly increase suspicion. Histopathology is crucial for diagnosis.

Orthokeratinized Odontogenic Cyst with an Associated Keratocystic Odontogenic Tumor Component and Ghost Cell Keratinization and Calcifications in a Patient with Gardner Syndrome.

Gardner syndrome (GS) is caused by mutations in the APC and besides adenomatous colorectal polyps includes such manifestations as osteomas, epidermoid cysts (ECs) and occasionally multiple pilomatricomas. More than 50 % of ECs in patients with GS exhibit pilomatricoma-like ghost cell keratinization. The latter may be explained by the fact that the development of both GS and pilomatricoma is driven by activation of the Wnt/ß-catenin signaling pathway. A 62-year-old, Caucasian male with history of GS presented with a unilocular, mixed radiopaque/radiolucent mandibular lesion causing divergence and external root resorption of involved teeth. Histopathologically, the lesion was composed of two cystic components, an orthokeratinized odontogenic cyst (OOC) and a smaller one with characteristics of keratocystic odontogenic tumor (KCOT) featuring, focally, ghost cells and an epithelial morule-like structure. Dystrophic calcifications essentially similar to those seen in pilomatricomas were observed in the fibrous connective tissue wall. The KCOT and OOC epithelia revealed strong and diffuse cytokeratin (AE1/AE3) and ß-catenin immunoreactivity. CD10 positive immunostaining was seen in the keratin and superficial spinous cell layers in both OOC and KCOT. The intraepithelial and mural ghost cells showed a cytokeratin (+), ß-catenin and CD10 (-) immunophenotype. The diagnosis of OOC with ghost cell calcifications in association with KCOT was rendered. The patient was lost to follow-up. Although a coincidental co-existence cannot be excluded, ghost cell calcifications mimicking pilomatricoma-like changes in an unusual odontogenic cyst combining OOC and KCOT features as seen in this patient with GS may be explained by the common molecular mechanisms underlying the pathogenesis of cutaneous pilomatricomas and GS.

Insight into the pathogenesis and nature of Central Giant Cell Lesions of the Jaws.

Central giant cell lesions of the jaws are not uncommon. While the majority of these represent single, sporadic lesions, histologically identical lesions are seen in association with a number of other bone lesions, as well as in certain syndromes. This manuscript offers a brief update on recent developments in this area that provide new insight into the pathogenesis and nature of Central Giant Cell Lesions of the Jaws.

Pseudoepitheliomatous hyperplasia associated with bisphosphonate-related osteonecrosis of the jaw.

Bisphosphonate-related osteonecrosis of the jaw (BRONJ) is a well-characterized oral complication of systemic therapy with bisphosphonates. Pseudoepitheliomatous hyperplasia was observed in some of the lesions. Because podoplanin expression has been linked to malignant lesions of the oral mucosa, we aimed to investigate podoplanin expression in the pseudoepitheliomatous hyperplasia. We analyzed archival paraffin- and plastic-embedded specimens from BRONJ using both conventional and immunohistochemical (AE1/AE3, D2-40) staining methods. Eleven out of seventeen BRONJ cases showed pseudoepitheliomatous hyperplasia. All these cases were positive for AE1/AE3 and pseudoepitheliomatous hyperplasia displayed a strong basal and parabasal reaction against podoplanin. The podoplanin expression in pseudoepitheliomatous hyperplasia in BRONJ specimens should not be considered a sign of malignancy. We discuss the current and possible future roles of surgical pathologists in diagnosing morphological changes associated with the development and therapy of BRONJ lesions.

[The influence of risk factors for malignant tumors of maxillo-facial area on the expression of matrix metalloproteinases and tissue inhibitors of matrix metalloproteinases in patients of elderly and senile age period].

Morbidity and mortality from malignant neoplasms increases each year, and the average age of patients with first diagnosed decreases. The peak of incidence of malignant tumors of maxillofacial region falls on the elderly life. Not only age-related changes of the organism contribute in the development of this pathology, but also harmful habits. A comprehensive approach to solving the problem requires monitoring of this group of patients to identify risk factors for the development of pathologies in the oral and maxillofacial region.

Primary intraosseous squamous cell carcinoma arising in odontogenic cysts: an insight in pathogenesis.

PURPOSE: To present a case series of primary intraosseous squamous cell carcinoma arising in odontogenic cysts and review the published data to understand the pathogenesis of the lesion. MATERIALS AND METHODS: The files of the Department of Oral and Maxillofacial Surgery, Government Dental College and Hospital (Jaipur, India) were reviewed, and 3 cases of primary intraosseous squamous cell carcinoma ex odontogenic cyst diagnosed from 2006 to 2011 were identified. The diagnostic inclusion criteria were the presence of a completely intraosseous lesion; the absence of ulceration of the oral mucosa, except when caused by other factors such as tooth extraction or trauma; and the absence of a distant primary tumor. The malignant transformation of the benign cystic lining of an odontogenic cyst to squamous cell carcinoma was identified, and the diagnosis of primary intraosseous squamous cell carcinoma ex odontogenic cyst was confirmed in all cases. The relevant English data were reviewed to determine the possible etiopathologic factors associated with the lesion. RESULTS: It was observed that long-standing chronic inflammation in the benign odontogenic cyst was the most probable etiopathologic factor responsible for malignant transformation of a benign cystic lining. CONCLUSIONS: The signs of chronic inflammation in odontogenic cysts should be taken into consideration, and such lesions should be treated promptly. By doing so, we might reduce the rate of malignant transformation of benign odontogenic cysts.

The keratocystic odontogenic tumor.

In 2005, the World Health Organization renamed the lesion previously known as an odontogenic keratocyst as the keratocystic odontogenic tumor. The clinical features associated with the keratocystic odontogenic tumor show it to be a unilocular or multilocular radiolucency, occurring most frequently in the posterior mandible. These tumors are normally diagnosed histologically from a sample of the lining. With simple enucleation, it seems that the recurrence rate may be from 25% to 60%.

Bifosfonatos en relación con la oncología: ¿nuevo efecto adverso? / Biphosphonates in oncology: a new adverse effect?

Objetivo: presentar el caso clínico de un paciente con diagnóstico de osteonecrosis maxilar aosicada a un tratamiento crónico con bifosfonatos. Se presentó a la consulta una paciente de 76 años de edad con una lesión en el sector 3, en el reborde mandibular supraneural, de tipo crónica, asintomática. Como patología de base la paciente estaba diagnosticada como portadora de cáncer de mama desde el año 2011. la biopsia confirmó el diagnóstico de osteonecrosis maxilar por tratamiento crónico con bifosfonatos, por lo cual se siguieron las reglamentaciones del Consenso de Canadá indicadas para el tratamiento de dicha patología. Conclusión: es indispensable realizar un diagnóstico clínico, radiográfico anatomo-patológico y bioquímico exhaustioçiv, con el fin de de identificar esta nueva patología que obliga a tomar ciertos recaudos clínicos en el momemnto de la atención a los pacientes

Bifosfonatos en relación con la oncología: ¿nuevo efecto adverso? / Biphosphonates in oncology: a new adverse effect?

Objetivo: presentar el caso clínico de un paciente con diagnóstico de osteonecrosis maxilar aosicada a un tratamiento crónico con bifosfonatos. Se presentó a la consulta una paciente de 76 años de edad con una lesión en el sector 3, en el reborde mandibular supraneural, de tipo crónica, asintomática. Como patología de base la paciente estaba diagnosticada como portadora de cáncer de mama desde el año 2011. la biopsia confirmó el diagnóstico de osteonecrosis maxilar por tratamiento crónico con bifosfonatos, por lo cual se siguieron las reglamentaciones del Consenso de Canadá indicadas para el tratamiento de dicha patología. Conclusión: es indispensable realizar un diagnóstico clínico, radiográfico anatomo-patológico y bioquímico exhaustioþiv, con el fin de de identificar esta nueva patología que obliga a tomar ciertos recaudos clínicos en el momemnto de la atención a los pacientes (AU)

Odontogenic myxomas are not associated with GNAS1 mutations.

UNLABELLED: Myxomas are rare tumors of unknown aetiology arising in the jaws. Myxomas are also diagnosed in soft tissues. Recent reports on Gs alpha subunit gene (GNAS1) mutations occasionally being identified in soft tissue myxomas in non-syndromatic patients and the effects of myxoma on bone in variants of fibrous dysplasia led us to re-examine the putative role of GNAS1 mutations in odontogenic myxoma. MATERIAL AND METHODS: Seven biopsies from patients with confirmed diagnosis of odontogenic myxoma and two cases of fibrous dyplasia of the jaw were investigated for GNAS1 mutations by polymerase chain reaction. RESULTS: Although GNAS1 was mutated in cases of fibrous dysplasia, no GNAS1 mutations were detected in odontogenic myxomas. CONCLUSION: The development of odontogenic myxoma is independent of mutations of GNAS1.

Radiographic findings in the jaws of patients with neurofibromatosis 1.

PURPOSE: Neurofibromatosis 1 (NF1) is a neurocutaneous-skeletal disorder with variable phenotypic expression and an incidence of 1:3,000 worldwide. The objective was to characterize the NF1-related radiologic alterations found in the jaws of these patients. PATIENTS AND METHODS: In total, 102 patients with NF1 were included in the present study. Six patients had a plexiform neurofibroma in the craniofacial region. RESULTS: Radiologic abnormalities in the jaws were found in 29 of 102 patients with NF1, including 6 patients with plexiform neurofibroma in the head and neck region. The most common radiologic finding was enlargement of the mandibular canal. The most prominent skeletal deformities and alterations of varying severity were detected in the jaws of 6 patients with plexiform neurofibroma. In these patients, the skeletal deformities were seen on the side affected by the tumor and possibly caused by the tumor. In 1 patient, however, the skeletal changes were on the opposite side. CONCLUSIONS: Radiologic abnormalities were found in 29 of 102 patients. The most significant findings were profound deformities of the mandible and maxilla in all 6 patients with plexiform neurofibroma, but not in the other patients. The facial bone deformities found in young patients did not progress markedly at older ages with cessation of the patients' growth.

Genetic screening for nevoid basal cell carcinoma syndrome in patients with a solitary keratocystic odontogenic tumour is not useful.

AIM: The aim of this study was to investigate the need for routine genetic counselling for identification of features of nevoid basal cell carcinoma syndrome (NBCCS) in patients presenting with a solitairy keratocystic odontogenic tumour (KCOT) of the jaws. METHODS: Sixty-nine patients treated for a solitary KCOT have been followed for the possible development of second KCOTs or other signs indicative of NBCCS. In addition, 11 randomly selected patients of this group were referred for genetic counselling, including identification of germ-line mutations in the Patched gene (PTCH gene). RESULTS: In none of the 69 patients clinical and radiographic manifestations of second KCOTs and/or other features associated with NBCCS were found during a follow-up period of 49.8 months. In the 11 patients referred for genetic counselling, there were no features indicative of the presence of NBCCS. No mutations in the PTCH gene could be identified. CONCLUSION: This study does not support the need for routine genetic counselling in patients presenting with a solitairy keratocystic odontogenic tumour of the jaws.

Multiple brown tumours of tertiary hyperparathyroidism in a renaltransplant recipient: A case report

Tertiary Hyperparathyroidism (HPT) is an uncommon condition that affects patients with secondary HPT aftera successful kidney transplant. Bone alterations are the main consequences of this endocrine condition, includingthe development of an osteolytic lesion called brown tumour. This paper reports an unusual case of multiplebrown tumours located in the maxilla and mandible in a 58-year-old renal transplant recipient, with tertiary HPT.Incisional biopsies were performed and, in both samples, the histopathological diagnoses were giant cell lesions.Due to these diagnoses, the medical history of chronic renal failure, and high levels of serum calcium and PTH,lesions were diagnosed as multiple brown tumours of tertiary HPT. The lesions showed regression within the twomonths following a total parathyroidectomy. Minimal local interventions were established to make an early diagnosisand to treat these lesions. The diagnosis of primary oral lesions was very important in order to demonstratethe relevance of interdisciplinary activity and the inclusion of dentists as part of organ transplant units (AU)

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Multiple brown tumours of tertiary hyperparathyroidism in a renal transplant recipient: a case report.

Tertiary Hyperparathyroidism (HPT) is an uncommon condition that affects patients with secondary HPT after a successful kidney transplant. Bone alterations are the main consequences of this endocrine condition, including the development of an osteolytic lesion called brown tumour. This paper reports an unusual case of multiple brown tumours located in the maxilla and mandible in a 58-year-old renal transplant recipient, with tertiary HPT. Incisional biopsies were performed and, in both samples, the histopathological diagnoses were giant cell lesions. Due to these diagnoses, the medical history of chronic renal failure, and high levels of serum calcium and PTH, lesions were diagnosed as multiple brown tumours of tertiary HPT. The lesions showed regression within the two months following a total parathyroidectomy. Minimal local interventions were established to make an early diagnosis and to treat these lesions. The diagnosis of primary oral lesions was very important in order to demonstrate the relevance of interdisciplinary activity and the inclusion of dentists as part of organ transplant units.