Primary intraosseous squamous cell carcinoma arising from an odontogenic keratocyst: case series and literature review

BACKGROUND: The aim of this study was to investigate the clinicopathologic features of primary intraosseous squamous cell carcinoma arising from an odontogenic keratocyst (PIOSCC ex OKC) and comprehensively improve the understanding of this disease. MATERIAL AND METHODS: We retrospectively investigated five cases of PIOSCC ex OKC at Peking University School and Hospital of Stomatology. We also conducted a systematic review of studies on PIOSCC ex OKC by using online databases from their inception until February 2020. RESULTS: In our series of five cases, all lesions were located in the mandible. Three cases (60%) showed recurrent OKCs and two cases (40%) showed primary OKCs. During the follow-up period, one patient died of local relapse. No patients developed metastasis. On the basis of our literature survey, we selected 22 articles reporting 29 patients with PIOSCC ex OKC. Seven of these patients (24.1%) showed local recurrence, three patients (10.3%) developed cervical metastasis, three patients (10.3%) developed distant metastasis (in the pleura in one case and in the lung in two cases), and seven patients died from the disease during the follow-up period. The disease-specific 5-year survival rate in the study group was 53.2%. Through univariate and multivariate analysis, local recurrence was identified as the only significant independent prognostic factor for survival (P < 0.05). CONCLUSIONS: The results suggest that PIOSCC ex OKC is a rare intermediate-grade malignancy. Although elective neck dissection is typically unnecessary, adequate therapy should be applied to achieve the lowest local recurrence rate possible to ensure a favorable survival rate

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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.

Ameloblastic carcinoma: Clinicopathological analysis of 18 cases and a systematic review.

OBJECTIVES: Exploring the clinicopathological features of ameloblastic carcinoma (AC) and reviewing the literature to improve the diagnosis and treatment of the disease. MATERIALS AND METHODS: Clinical data and pathological features of 18 cases of AC were retrospectively analyzed. A systematic review was carried out by searching PubMed and Medline databases using the MeSH terms "ameloblastic" and "carcinoma." RESULTS: In the systematic analysis, 125 cases of AC from 81 eligible original studies and 18 cases of AC from this research were included. The male-to-female ratio was 2.58:1, and the mandible-to-maxilla ratio was 1.80:1. Mean age of patients was 45.3 years. Thirty-seven cases of recurrence and 27 cases of metastasis were recorded. CONCLUSION: AC is a rare neoplasm of the odontogenic epithelium. A systematic review indicates that diagnoses at the early phase and a close periodic assessment for recurrence and metastasis are necessary.

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.

Endoscope-Assisted Enucleation of Mandibular Odontogenic Keratocyst Tumors.

The keratocyst odontogenic tumor (KCOT) represents a rare and benign but locally aggressive developmental cystic lesion usually affecting the posterior aspect of the mandible bone, the treatment of which has always been raising debate, since Philipsen first described it as a distinct pathological entity in 1956.Recent studies have proposed the use of endoscope-assisted surgical technique, due to the possibility given by the endoscope of improving the effectiveness of the treatment of these lesions thanks to a better visualization of operative field and though a better understanding of the pathology. In this article, we would like to present our experience with the endoscope-assisted treatment of KCOT of the posterior region of the mandible.From April 2000 to April 2012, 32 patients treated for KCOT were enrolled in our retrospective study: patients were divided in 2 groups according to the type of treatment, that is, 18 were treated with traditional enucleation surgery (TES), and 14 patients underwent endoscopic assisted enucleation surgery (EES).Fischer exact test and Kaplan-Meier curves were used to compare the outcomes between the 2 focusing on the recurrence and complication rates. In the TES group, patients we found a higher recurrence rate (39%) and higher postoperative complication rate at 5-year follow-up.Our data suggested, though, that EES seems to be a feasible alternative for the treatment of posterior mandibular KCOT. Further studies and larger series are needed to confirm these results.

Clinicopathological Study of Primary Intraosseous Squamous Cell Carcinoma of the Jaw and a Review of the Literature.

PURPOSE: Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare malignant odontogenic tumor that originates from odontogenic epithelial remnants. It is often difficult to diagnose PIOSCC definitively; hence, extraction or surgical treatment is performed before the initial diagnosis in most cases. The present study examined new insights into and prognostic factors of patients with PIOSCC admitted to the authors' department. MATERIALS AND METHODS: An extensive record review was conducted of patients who underwent radical surgery for PIOSCC from January 2001 through December 2014. RESULTS: Of all cases of OSCC, the frequency of PIOSCC was 1.45%. The 2-year relapse-free survival (RFS) and overall survival (OS) rates were 50.0 and 41.6% in all cases, respectively. Three patients underwent surgery or tooth extraction before the initial diagnosis; in fact, intervention before initial diagnosis was found to be an important poor prognostic factor for RFS and OS. In contrast, patients who were not treated before the initial diagnosis was made did not exhibit any locoregional recurrence. CONCLUSIONS: The treatment of PIOSCC should be similar to that for oral cancer with at least clinical stage T3N0 in the National Comprehensive Cancer Network clinical practice guidelines. In addition, cases of PIOSCC that are not treated before the initial diagnosis are more likely to obtain a good prognosis.

Survival and Prognosis for Malignant Tumors of Odontogenic Origin.

OBJECTIVE: Determine survival and factors affecting survival for patients with malignant tumors of odontogenic origin. STUDY DESIGN: Retrospective analysis of the National Cancer Institute's SEER database (Surveillance, Epidemiology, and End Results). SETTING: Tertiary medical center. SUBJECTS AND METHODS: All cases of malignant tumors of odontogenic origin were extracted from the SEER database for the period of 1973 to 2011. Demographic, tumor-specific, and survival data were tabulated and Kaplan-Meier survival analysis conducted according to histopathologic results. Cox regression analysis stratified for histopathology was conducted to determine factors that influenced survival. RESULTS: A total of 308 cases of malignant tumors with odontogenic origin were analyzed. Malignant ameloblastoma accounted for 59.7% of cases, followed by malignant odontogenic tumor (35.4%; including odontogenic carcinoma, odontogenic sarcoma, primary intraosseous carcinoma, and ameloblastic carcinoma) and ameloblastic fibrosarcoma (2.9%). The overall mean and median were 229 and 227 months, respectively, while the 5-year survival rate was 81% for the entire cohort. Malignant ameloblastoma exhibited the best mean survival (237 months), whereas malignant odontogenic tumor (139 months) and ameloblastic fibrosarcoma (42 months) had lower mean survival rates. Younger age, surgery with adjuvant radiation, and smaller tumor size were found to improve survival. CONCLUSIONS: Significantly different survival can be expected depending on individual tumor histopathology, tumor size, age at diagnosis, and treatment modality.

The use of vismodegib to shrink keratocystic odontogenic tumors in patients with basal cell nevus syndrome.

IMPORTANCE: Keratocystic odontogenic tumors (KCOTs) of the jaw affect more than 65% of patients with basal cell nevus syndrome (BCNS). Surgery frequently causes facial disfigurement and is not always curative. Most BCNS-related and some sporadic KCOTs have malignant activation of the Hedgehog signaling pathway. OBSERVATIONS: We examined the effect of vismodegib (an oral Hedgehog pathway inhibitor) on KCOT size in patients with BCNS enrolled in a clinical trial testing vismodegib for basal cell carcinoma prevention (NCT00957229), using pretreatment and posttreatment magnetic resonance imaging. Four men and 2 women had pretreatment KCOTs (mean longest diameter, 2.0 cm; range, 0.7-3.3 cm), occurring primarily in the mandible. Patients were treated with vismodegib, 150 mg/d, for a mean (SD) of 18.0 (4.8) months (range, 11-24 months). Four patients experienced a size reduction and 2 had no change. Vismodegib reduced the mean longest diameter of KCOTs in all patients by 1.0 cm (95% CI, 0.03-1.94; P = .02) or 50% from baseline. We observed no enlargement of existing KCOTs or new KCOT development. CONCLUSIONS AND RELEVANCE: Vismodegib shrinks some KCOTs in patients with BCNS and may offer an alternative to surgical therapy. These effects were maintained for at least 9 months after drug cessation in 1 patient. Further studies assessing long-term efficacy and optimal maintenance regimens should be performed.

Recurrence rate of keratocystic odontogenic tumor after conservative surgical treatment without adjunctive therapies - A 35-year single institution experience.

The recurrence rate in conservative surgical treatment, without adjunctive treatment, of the keratocystic odontogenic tumor (KCOT) has been studied. A total number of 68 patients, previously untreated and fulfilling the histopathological criteria provided by the 2005 WHO classification, has been retrospectively reviewed. The study period lasted from 1975 to 2009. Treatment consisted of enucleation (n=58) or marsupialization (n=10). The mean follow-up period was 65 months. No involved or adjacent teeth were removed, except for wisdom teeth, if applicable, and badly decayed teeth. After enucleation, the recurrence rate was 20.7% in a mean follow-up period of 46 months, while 40% of the marsupialized KCOTs recurred in a mean follow-up period of 58 months. In none of the patients permanent loss of nerve function has been observed. Due to the recurrence rate observed in the present study, and in view of the potential benefit of adjunctive treatment in KCOT, particularly with regard to the use of Carnoy's solution, there is a need for prospective studies to test its usefulness with regard to the risk of recurrences.

Tumor cell proliferation and microsatellite alterations in human ameloblastoma.

Ameloblastoma is the most common odontogenic tumor. It can exhibit a variety of histological patterns, a great infiltrative potential and a high recurrence rate. Mutations in microsatellite sequences are a hallmark of neoplastic transformation but little is known about their role in ameloblastoma development. In this study DNA was extracted from laser-microdissected samples of 24 ameloblastomas and was analyzed for the status of 22 microsatellite loci. The occurrence and the pattern of microsatellite alterations, in form of loss or length variation, was evaluated and correlated with the Ki67 labeling index and with other clinicopathologic parameters. The prognostic significance of these alterations was also evaluated. High Ki67 expression was significantly associated with a shorter disease-free survival (p=0.003 by log-rank test). Alterations of at least one of the selected loci was observed in all (100%) the ameloblastomas analyzed with a mean of 4 altered microsatellites for each tumor. The microsatellites most frequently altered were D9S747 and D11S488 (42%). All the other loci analyzed were altered in less than 40% of cases and some of them (D3S1312, D3S1300, IFNA, D9S164, D13S176 and TP53) did not show alterations in any of the ameloblastomas analyzed. No relationship was observed between the occurrence of microsatellite alterations and other parameters, such as patients age and gender, tumor size, localization and histotype. The occurrence of microsatellite alterations was more frequent in tumors displaying a high Ki67 labeling index (p=0.03) and in a univariate analysis was predictor of an increased risk of disease recurrence (p=0.039 by log-rank test). These findings demonstrate that microsatellite alterations are frequent event in ameloblastomas. They also suggest that evaluation of tumor cells proliferative activity and microsatellite alterations may be helpful to stratify ameloblastomas prognostically and to predict the clinical behavior of these tumors.

[Odontogenic tumors and neoplastic-like changes of the jaw bone. Clinical study and evaluation of treatment results]. / Nowotwory zebopochodne i zmiany nowotworopodobne kosci szczekowych. Studium kliniczne i ocena wyników leczenia.

The purpose of the study was clinical analysis of 164 odontogenic tumours and tumour-like lesions of the jaws treated at the Department of Maxillofacial and Oral Surgery, Jagiellonian University College of Medicine between 1956 and 1996, and to evaluate early late and therapeutic results. Odontogenic tumours have a specific histological structure reflecting various stages of odontogenesis and are located mainly in the jaws, exceptionally in other parts of the skeletal system. Due to their specific structure and location they have been identified and classified by pathologists into a separate group of neoplasms differing from other tumours developing in the oral cavity and facial bones first of all in histogenesis, but also in biology, clinical manifestations and radiological sings. The currently recommended histological classification of odontogenic tumours established by the WHO in 1992 comprises a variety of odontogenic tumours and tumour-like lesions of bones of ectodermal, ecto-mesodermal and mesodermal origin as well as lesions histogenetically not associated with the dental organ, but constituting this group. They include histologically benign tumours, locally malignant and malignant tumours, the latter making up the least numerous group. Due to marked polymorphism histological diagnosis of odontogenic tumours is difficult, therefore co-operation between a clinicist, radiologist and pathologist is especially important. Treatment of choice in patients with odontogenic tumours is surgical operation: in case of benign tumours extirpation and curretage, whereas in case of malignant and locally malignant tumours--segmental resection of the jaws. Removal of extensive tumours is associated with the need of surgical tissue repair and prosthetic rehabilitation, and in young patients surgical treatment must be followed by orthodontic treatment. Clinical analysis included case histories of 164 patients admitted to the Department of Maxillofacial and Oral Surgery (Head: Professor MD S. B. Bartkowski) between 1956 and 1996. Additional control examinations were performed to analyze late results of therapy. The 40-year clinical material comprised 164 odontogenic tumours including 91 (55.5%) in women, and 73 (44.5%) in men. The mean age of the patients was 32.5 years, but about 30% of the patients were below 18. The tumour was most frequently located in the mandible i.e. in 118 (72.0%) patients, and in the maxilla in 44 (26.8%), and in both jaws in 2 (1.2%) patients. Due to tumour recurrence 19 (11.6%) patients were referred to our clinic from other centres, the remaining ones received their first treatment. The most frequent reason for patient referral was deformity of the jaws, maleruption or lack of teeth or their dislocation, and in case of extensive lesions face deformation and bulging of the tumour into the oral cavity. Radiological examination showed noncharacteristic transparencies, round or oval in shape, single- or multiventricular or foci of noncircumscribed bone densities usually in the vicinity of the teeth, frequently dislocated or unerupted. Rarely osteolysis of the roots of teeth or their amputation was found. Histological examination of the 164 tumours revealed (in order of frequency): ameloblastoma--60 (36.6%), central reparative giant cell granuloma--52 (31.7%), cementoma--15 (9.2%), odontoma--13 (7.9%), odontogenic myxoma--12 (7.3%), ameloblastic fibroma--4 (2.4%), calcifying epithelial odontogenic tumour--3 (1.8%), odontogenic fibroma--2 (1.2%), adenomatoid odontogenic tumour--2 (1.2%) and odontoameloblastoma--1 (0.6%). Of the 60 ameloblastomas three tumours were histologically malignant. Of the 164 patients 152 were selected for surgical treatment. Surgical treatment of histologically and clinically benign tumours included tumour extirpation, curettage, excision and/or electrocoagulation, whereas malignant and locally malignant tumours such as ameloblastomas, myxomas, Pindborg's tumours, invasive reparative giant c

Cancer de cabeza y cuello: Pautas de diognostico y tratamiento / Head Cancer and neck: Rules of I diagnose and treatment

contiene: Consideraciones generales de cancer de cabeza y cuello,pautas para tumoraciones de cuello,evaluacion del paciente con cancer de cabeza y cuello,carcinomas de piel de cara y cuello,malanoma de cabeza y cuello,cancer de cavidad bucal y orofaringe,cancer de labio,tumores malignos de glandulas salivales,consideraciones practicas en la parotidectomia,protocolo y diagnostico y tratamiento de la laringe,cancerde hipofaringe,tumores de glandulas tiroides,cancer de nasofaringe,cancer de seno maxilar,linfomas de hodgking y no hodgkin presente en la cabeza y cuello,reparaciones y defectos en cirugia oncologica cervicofacial mediante colgafos miocutaneos del musculo del pectoral mayor,diseccion funcional del cuello

Cancer of the mouth in an isolated community--the results of treatment.

A survey is presented of all known cases of cancer of the mouth in a population of nearly half a million in a relatively isolated part of Yorkshire, with the results of treatment of squamous carcinoma, adeno-carcinoma, lympho-sarcoma, malignant tumours of odontogenic origin and sarcomas.