Ghost cell odontogenic carcinoma: A rare case report and review of literature.

RATIONALE: Ghost cell odontogenic carcinoma is a rare malignant odontogenic carcinoma characterized by the presence of ghost cells. It has a nonspecific clinical and radiographic presentation and can be locally destructive and invasive, sometimes with distant metastases. However, no effective systemic therapy is currently recommended for such patients. PATIENT CONCERNS: The patient has been unable to undergo surgery or radiotherapy again. Therefore, he was referred to our department for a more aggressive, multimodal systematic treatment program. DIAGNOSES: The histopathological examination was morphologically suggestive of ghost cell odontogenic carcinomas. INTERVENTIONS: We report a case of locally invasive primary inoperable odontogenic shadow cell carcinoma in a 31-year-old Chinese man who achieved treatment with Toripalimab and chemotherapy, followed by Toripalimab maintenance therapy after 6 cycles. OUTCOMES: He achieved partial remission after treatment. The quality of life significantly improved after treatment. There were no grade 3/4 treatment-related adverse events during treatment. LESSONS: This case presented that Toripalimab and chemotherapy may be a safe and effective systemic therapy for ghost cell odontogenic carcinoma.

Patterns of Care and Survival Outcomes for Odontogenic Cancers.

OBJECTIVES: Our understanding of odontogenic cancers is limited primarily to case studies given the rarity of these head and neck neoplasms. Using the National Cancer Database, we report the treatment patterns and survival outcomes for one of the largest cohorts of patients with odontogenic cancers. METHODS: Patients with odontogenic tumors who did not have metastatic disease and received at least part of their care at the reporting facility were included. Patient and treatment variables were assessed using logistic regression. Survival was assessed using Cox proportional hazard models. RESULTS: We identified 437 patients with odontogenic cancers, the majority of which had malignant ameloblastoma (n = 203) or odontogenic carcinoma (n = 217). Median follow-up was 44.8 months. On multivariate analysis, improved survival was associated with age <57 years (Hazard ratios [HR] 0.44, P = .012), lower comorbidity scores (HR 0.40, P = .008), surgical resection (HR 0.08, P < .001) and absence of lymph node metastasis (HR 0.23, P < .001). The 5-year overall survival was 87.1% for debulking surgery, 88.6% for radical resection and 26.6% for no surgical resection (P < .001). Lymph node metastases were associated with tumor size ≥5 cm (P = .006), malignant odontogenic histology (P = .025), and moderate/poor differentiation (P < .001). CONCLUSION: In this large series of odontogenic cancers, any type of surgical resection was associated with improved survival. Lymph node metastases, although infrequent, were associated with significantly worse survival. LEVEL OF EVIDENCE: Level 3 Laryngoscope, 131:E1496-E1502, 2021.

Malignant fibrous histiocytoma - a case report.

Fibrous histiocytoma is a mesenchymal neoplasm with benign and malignant varieties. This tumor mainly affects the skin of extremities in adults and may on rare occasions affect the oral cavity. The tumor has radiographic features in very rare cases. The present case report aims to conduct a clinicopathological-radiographic and immunohistochemical assessment and treatment of a patient with this lesion.

Reconstruction of Mandibular Defects With Bone Marrow-Derived Stem Cells in Odontogenic Myxoma.

Odontogenic myxoma is a benign tumor, mostly located in the mandible. It shows locally aggressive behavior and requires surgical removal. Common treatment options for reconstructing the bone defects are immediate or delayed autologous bone graft or free flap. In this article, the authors present the successful reconstruction with autogenous bone graft and autologous human bone marrow mesenchymal stem, followed by distraction osteogenesis, dental implant placement and prosthodontic restoration in the mandibular defect.

[Clinical and imaging features of 36 odontogenic keratocysts associated with an impacted tooth].

PURPOSE: To analyse the imaging features of odontogenic keratocysts (OKCs) associated with an impacted tooth. METHODS: Clinical and radiological data of 235 cases with OKCs were respectively investigated, with emphasis on imaging features of 36 OKCs, which were associated with an unerupted tooth. RESULTS: In 36 cases of OKCs associated with an impacted tooth, the ratio of male to female was 1.77:1, and molar-ramus was involved in 26 cases (72.22％). OKCs in association with an unerupted tooth occurred mostly in patients ranging from 20 to 30 years (19 cases, 52.8％). There were 27 cases (75％) of unilocular and 9 cases (25％) multilocualr radiolucency. Thirteen cases (36.11%) were related to the crown of the impacted teeth, and the unerupted teeth also were impacted as a result of malposition in which the entire teeth appeared to be enveloped by cysts (15 cases, 41.67%), or adjacent to cyst wall (8 cases, 22.22%). CONCLUSIONS: Radiographically, one of the most imaging features of OKCs in association with an unerupted tooth is that its entire tooth appears to be enveloped by cyst or adjacent to cyst, while pericoronal radiolucencies surrounding an impacted tooth are rarely seen.

Odontogenic myxomas: what is the ideal treatment?

There is no consensus regarding the ideal treatment for odontogenic myxomas, an odontogenic mesenchymal neoplasm. Various authors have suggested en bloc resection due to a concern regarding inadequate clearance while others have suggested more conservative treatment. We present a case managed by buccal cortical resection and an iliac crest bone graft. The patient had no recurrence for over 7 years.

The dentigerous cyst: two different treatment options illustrated by two cases / Die follikuläre Zyste: zwei unterschiedliche Behandlungsmöglichkeiten, präsentiert anhand zweier Fallbeispiele

The dentigerous cyst is the second most frequent odontogenic cyst after the radicular one. Typically, dentigerous cysts are asymptomatic and are commonly diagnosed incidentally. Due to the slow, expansive growth dentigerous cysts have the potential for dislocation of adjacent structures (neighbouring teeth, canalis retromolaris), root resorptions and in an extreme case could cause jaw fractures. A histological examination represents a sine qua non, because other more alarming pathologies (odontogenic keratocyst, unicystic ameloblastoma, myxoma, primordial odontogenic tumour etc.) can exhibit clinically and radiologically a similar appearance. This brief overview aims to illustrate, by two clinical cases, the examination, the diagnosis and decision-making for either a cystostomy or a cystectomy. The surgical procedure of both techniques is presented in a step-by-step manner.

Effect of the sonic hedgehog inhibitor GDC-0449 on an in vitro isogenic cellular model simulating odontogenic keratocysts.

Odontogenic keratocysts (OKCs) are common cystic lesions of odontogenic epithelial origin that can occur sporadically or in association with naevoid basal cell carcinoma syndrome (NBCCS). OKCs are locally aggressive, cause marked destruction of the jaw bones and have a propensity to recur. PTCH1 mutations (at ∼80%) are frequently detected in the epithelia of both NBCCS-related and sporadic OKCs, suggesting that PTCH1 inactivation might constitutively activate sonic hedgehog (SHH) signalling and play a major role in disease pathogenesis. Thus, small molecule inhibitors of SHH signalling might represent a new treatment strategy for OKCs. However, studies on the molecular mechanisms associated with OKCs have been hampered by limited epithelial cell yields during OKC explant culture. Here, we constructed an isogenic PTCH1R135X/+ cellular model of PTCH1 inactivation by introducing a heterozygous mutation, namely, c.403C>T (p.R135X), which has been identified in OKC patients, into a human embryonic stem cell line using the clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated 9 (Cas9) system. This was followed by the induction of epithelial differentiation. Using this in vitro isogenic cellular model, we verified that the PTCH1R135X/+ heterozygous mutation causes ligand-independent activation of SHH signalling due to PTCH1 haploinsufficiency. This activation was found to be downregulated in a dose-dependent manner by the SHH pathway inhibitor GDC-0449. In addition, through inhibition of activated SHH signalling, the enhanced proliferation observed in these induced cells was suppressed, suggesting that GDC-0449 might represent an effective inhibitor of the SHH pathway for use during OKC treatment.

Sclerosing Odontogenic Carcinoma with Local Recurrence: Case Report and Review of Literature.

Sclerosing odontogenic carcinoma is a rare locally destructive neoplasm with many histologic mimics. Here the diagnostic challenges are presented of a case of sclerosing odontogenic carcinoma with variable histologic features, including unusual and unexpected negative immunostaining for CK19.

Odontogenic tumors and lesions treated in a single specialist oral and maxillofacial pathology unit in the United Kingdom in 1992-2016.

OBJECTIVES: Odontogenic tumors (ODTs) are a heterogeneous group of lesions derived from elements of tooth-forming tissues. No detailed data on the incidence of odontogenic tumors in the United Kingdom have been published. The aim of this study was to retrospectively describe the range and incidence of odontogenic tumors from 1992 to 2016 in a single specialist unit and to compare this population with others. STUDY DESIGN: By using the Oral and Maxillofacial Pathology database, Sheffield (UK), we included both local and referred consultation cases. A proportion of diagnoses were reclassified in accordance with the 2017 World Health Organization classification. RESULTS: In total, 559 odontogenic tumors were diagnosed. Overall, the most common lesions were ameloblastoma (196 [33.8%]), odontoma (148 [25.5%]), and odontogenic myxoma (37 [6.3%]), but this varied between local and referral case populations, with odontomas being most common in the local population (43%). The sites affected and the gender and age of patients were similar to other Western populations. Malignant ODTs comprised 33 cases (5.7%), of which 9 (27.3%) were ameloblastic carcinoma. The majority of the malignant ODTs comprised referral cases. CONCLUSIONS: Here, we present the first detailed data on ODTs within a UK population, and the pattern of incidence from the local population is similar to other Western populations. The exceptional rarity of malignant ODTs emphasizes the need for specialist centers for their treatment to gain diagnostic experience.

Risk factors and prognosis for the primary intraosseous carcinoma of the jaw.

Primary intraosseous carcinoma (PIOC) is a rare but aggressive type of odontogenic tumour arising within the jawbone. Diagnosis criteria and treatment strategy remain difficult and controversial. The present study aimed to clarify the clinicopathological features and determine prognostic factors in management of PIOC. A retrospective study of 30 patients with PIOC, treated at the Hospital of Stomatology of Sun Yat-sen University between 2009 and 2017, was conducted. Clinical, histopathological and treatment modality data were collected. Follow-up data were recorded to determine prognostic factors. There were 19 males and 11 females with a mean age of 52.3 years. The most common location of the tumour was the mandible (90%). Having a history of tooth extraction or tooth mobility was the major characteristic symptom (63.3%), jaw swelling coming in second (53.3%). Half of the patients underwent surgery alone. The estimated 2-year overall survival rate (OS) and recurrence-free survival rate (RFS) were 61.3% and 40.1%, respectively. Higher histological grade was an independent risk factor for poor OS (hazard ratio (HR) 0.233 [0.059-0.915], P=0.037), while at pN+ stage for RFS, HR=5.627 [1.199-26.409], P=0.029. Because of its rarity and intrabony site, the classification, staging and treatment guidelines for PIOC should be further studied and established.

Maxillofacial Radiographic study of Gardner's syndrome presenting with odontogenic myxoma: A rare case report.

Gardner syndrome is an autosomal dominant disease. It is characterized by a combination of familial adenomatous polyposis (FAP) of the intestine with extraintestinal changes as multiple osteomas and fibromas. Odontogenic Myxoma is a benign, aggressive intraosseous neoplasm. We report a rare case of a 14-year-old male patient with Gardner's syndrome and odontogenic myxoma, which involved the entire left half of the mandible, resulting in a gross facial deformity, within a span of one year.

Secondary Ameloblastic Carcinoma of Mandible with Pulmonary Metastasis.

Ameloblastic carcinoma (AC) is a very rare odontogenic malignancy. Primary AC is more common in maxilla, while secondary AC is more common in mandible. Histologically, it is an ameloblastoma with features of cancer. Presence of metastasis is not necessary to establish the diagnosis. This tumor usually metastasises in bone or lungs. We report a case of aggressive secondary AC of mandible with lung metastasis. There are few cases in medical literature discussing management of AC with lung metastasis. In this case, a 42-year woman, who was diagnosed with ameloblastoma of right mandible, was treated with right hemi-mandibulectomy. However, 10 years later, the patient developed AC with pulmonary metastasis. Patient received six cycles of carboplatin-paclitaxel-based regimen. There was noted a partial response to the therapy. However, the patient went into respiratory failure, although she was provided adequate analgesic control as part of end-of-life care. Further, oncological or surgical management was not possible as patient had poor performance status.

Jaw bones regeneration using mesenchymal stem cells. A single-center experience.

PURPOSE: Mesenchymal stem cells (MSC), which are multipotent stromal cells, are considered to be a promising resource in tissue engineering and tissue regeneration. MSCs have been used to generate new maxillary bone with clinically successful results. The aim of this study was to determine the role of MSC in bone regeneration procedures in patients with benign maxillary lesions. METHODS: A study was conducted on five patients treated for maxillary bone defects resulting from biopsy of benign lesions at the University Hospital of Magna Græcia, Catanzaro, Italy from January 2015 to October 2016. MSC from autologous bone marrow were used for bone regeneration. The bone mineral density was compared, using the Hounsfield scale, before and after treatment. Follow-up was monthly for six months, and the patients underwent a computed tomography scan of the maxilla at 6 months. RESULTS: Five patients, who underwent biopsy of osteolytic odontogenic benign tumors, were included in the study. There were no intraoperative or postoperative complications. The mean volume of the newly formed bone was 2.44cm3 (range 2,0-3,1) and the mean bone density was 1137 Hounsfield Units (range 898-1355). CONCLUSIONS: Bone regeneration with MSC from autologous bone marrow appears to be a valid treatment option for maxillary bone defects. KEY WORDS: Bone regeneration, Mesenchymal stem cells, BM-MSC, Upper jaw, Mandible.

Ghost cell odontogenic carcinoma with suspected cholesterol granuloma of the maxillary sinus in a patient treated with combined modality therapy: A case report and the review of literature.

RATIONALE: Ghost cell odontogenic carcinoma (GCOC) is a rare malignant odontogenic tumor with aggressive growth characteristics. PATIENT CONCERNS: A 41-year-old Chinese male visited our hospital in 2013, with a 4-month history of bloody purulent rhinorrhea with a peculiar smell in the right nasal cavity. DIAGNOSES: The patient suffered from recurrent GCOC with suspected cholesterol granuloma of the maxillary sinus. The patient was incorrectly diagnosed with a calcifying epithelial odontogenic tumor at his first recurrence. Physical examination, radiological examination, and histopathology were used to identify GCOC. INTERVENTIONS: Considering the recurrence of GCOC and poor effects of single surgery, postoperative adjuvant chemotherapy and concurrent chemoradiotherapy were performed after radical surgical excision. OUTCOMES: So far, no significant evidence has suggested recurrence or metastasis after a long-term follow-up. LESSONS: We advocate wide surgery with clean margins at the first accurate diagnosis. Combined modality therapy could be taken for the recurrent GCOC. We expect to provide a new treatment strategy to prevent the growth of this neoplasm.

Primary intraosseous squamous cell carcinoma of the mandible: locoregional control and survival is significantly reduced if the tumour is more than 4cm in size.

To establish the prognostic factors for primary intraosseous squamous cell carcinoma we designed a retrospective study of patients treated in the head and neck department of a tertiary referral centre in China from 2010-2015. We collected clinical, radiological, and histopathological data from 36 patients treated during the given time period, among which 34 were followed up. There were 22 male and 12 female patients, 13 of whom gave a history of smoking tobacco and four who drank alcohol. All 34 patients were treated by segmental mandibulectomy and neck dissection. Nine had cervical lymph node metastases on histopathological examination, and none had invaded surgical margins. Twenty-eight were treated with radiotherapy postoperatively. During follow up nine died of locoregional recurrence or metastases. Specific factors such as cervical lymph node metastases were related to a greater likelihood of locoregional recurrence. Patients who drank alcohol were also more likely to develop metastases postoperatively. Tumours more than 4cm in size were significantly associated with reductions in locoregional control and survival.

Granular ameloblastoma: report of two cases with atypical cytological aspects.

Ameloblastoma is an aggressive odontogenic tumor, which arises from odontogenic epithelium. Ameloblastomas can present in several clinical and histomorphological patterns. The granular cell variant accounts for only 3.5% to 5% of ameloblastomas. We have presented two cases of granular cell ameloblastoma (GCA) occurring in a 44-year-old and 50-year-old man, respectively. Case No. 1 on incisional biopsy was diagnosed as unicystic ameloblastoma (UA), which later after excisional biopsy was finally diagnosed as GCA owing to the features observed in excisional biopsied tissue. Case No. 2 on incisional biopsy showed darker and lighter stained cells arranged in small follicular pattern, and anastomosing cords. Meticulous immunohistochemistry, histochemical examination and careful literature search helped us to diagnose it as GCA. We have made an attempt to elucidate the diagnosis of GCA especially in cases of GCA with unusual granular component.

Keratocystic odontogenic tumor / Tumor odontogênico ceratocístico

ABSTRACT Objective: to evaluate the frequency of keratocystic odontogenic tumor (KOT) in the Oral Surgery Service (OSS) of the University Hospital Clementino Fraga Filho of the Federal University of Rio de Janeiro (HUCFF / UFRJ), with respect to recurrence rate, gender, age of recurrence and location of the injury Methods: clinical records were reviewed and histopathological reports of KOT patients of the HUCFF/UFRJ between 2002 and 2012. Patients diagnosed with KOT were divided into two groups for the occurrence of relapse: positive (n=6) and negative (n=19) Results: regarding the location, there was a predilection for the mandible. In the average age of patients in the positive group was 40.5 and the negative group, 35.53. In the distribution by gender, positive group showed equal distribution, different from that observed in the negative group, which showed a predilection for males Conclusion: KOT was the second most frequent injury in our patients, recurrence was lower among males and had the jaw as most affected location

RESUMO Objetivo: avaliar a frequência do tumor odontogênico ceratocístico (TOC) no Serviço de Cirurgia Oral (SCO) do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro (HUCFF/UFRJ), no que diz respeito à taxa de recidiva, ao sexo, à idade de recorrência e à localização da lesão. Métodos: foram examinados os prontuários clínicos e laudos histopatológicos de pacientes do SCO do HUCFF/UFRJ no período de 2002 a 2012. Os pacientes diagnosticados com TOC foram divididos em dois grupos quanto à ocorrência de recidiva: positivo (n=6) e negativo (n=19) . Resultados: com relação à localização, houve predileção pela mandíbula. Em relação à média de idade dos pacientes, no grupo positivo foi 40,5, e no grupo negativo, de 35,53. Na distribuição por sexo, o grupo positivo apresentou distribuição igualitária, diferentemente do observado no grupo negativo, em que predominou o sexo masculino. Conclusões: o TOC representou a segunda lesão mais frequente em nossos pacientes, tem menor recidiva no sexo masculino e tem a mandíbula como localização mais acometida.