Diagnostic delay in autoimmune oral diseases.

Autoimmune diseases (ADs) affect about 5% of the general population, causing various systemic and/or topical clinical manifestations. The oral mucosa is often affected, sometimes as the only involved site. The misdiagnosis of oral ADs is an underreported issue. This narrative review focuses on diagnostic delay (DD) in oral ADs (oral lichen planus [OLP], oral Pemphigus Vulgaris, mucous membrane pemphigoid, oral lupus erythematosus, orofacial granulomatosis, oral erythema multiforme [EM], and Sjogren syndrome). Extensive literature research was conducted via MEDLINE, Embase and Google Scholar databases for articles reporting the time spent to achieve the correct diagnosis of oral ADs. Only 16 studies reported DD in oral ADs. Oral autoimmune vesiculobullous diseases are usually diagnosed after 8 months from the initial signs/symptoms, the Sjogren Syndrome diagnosis usually requires about 73 months. No data exist about the DD in OLP, oral lupus erythematosus, orofacial granulomatosis, and oral EM. The diagnosis of oral ADs can be difficult due to the non-specificity of their manifestations and the unawareness of dentists, physicians, and dental and medical specialists about these diseases. This can lead to a professional DD and a consequential treatment delay. The delay can be attributed to the physicians or/and the healthcare system (Professional Delay) or the patient (Patient's Delay).

The role of the dentist in the diagnosis and management of patients with oral mucosal diseases / El papel del dentista en el diagnóstico y tratamiento de pacientes con enfermedades de la mucosa oral

Based on a few case reports of oral mucosal diseases a number of questions is raised about the role of dentists-general practitioners in the diagnostic procedure and management of patients with such diseases. For instance, aredentists prepared to prescribe topical corticosteroids and should dentists be taught how and when to take a biopsy?And how about palpation of the neck? A strong recommendation is made to take clinical pictures for proper docu-mentation and, if needed, for telediagnostic procedures.Another issue relates to the communication between dentists and dental specialists when dealing with patientswith oral diseases. In case of a patient suffering from burning mouth syndrome or any other type of chronicorofacial pain, the question is raised whether dentists-general practitioners are prepared to manage such patients.Furthermore, there is a call for structuring the collaboration between dentists-general practitioners and dentalspecialists, including oral and maxillofacial surgeons.(AU)

Oral leukoplakia; a proposal for simplification and consistency of the clinical classification and terminology

There is a distinct lack of uniformity in the definitions and clinical terminologies related to oral leukoplakia and leukoplakialike lesions and disorders. Proposals have been put forward to subclassify leukoplakia into a homo-geneous and a non-homogeneous type based on color only, being either predominantly white or mixed white-and-red, respectively, irrespective of the texture of the lesion. In this proposal there is no need anymore to regard the poorly defined proliferative verrucous leukoplakia as a separate entity. Since keratosis is primarily a histo-pathological term, its clinical use is discouraged. Alternative terminology for these so-called keratotic lesions and disorders has been put forward. Finally, a suggestion has been made to rename the term hairy leukoplakia, being a well defined, not potentially malignant disorder particularly related to HIV-infection, into 'EBV-positive white lesion of the tongue' (EBVposWLT)

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Historical perspective and nomenclature of potentially malignant or potentially premalignant oral epithelial lesions with emphasis on leukoplakia-some suggestions for modifications.

Of the potentially (pre)maligant oral epithelial lesions, leukoplakia is the most common. A brief overview of the various definitions of leukoplakia that have been used in the past is presented here. A proposal has been made to modify the current definition. Clinically, for decades, leukoplakias have been divided into homogeneous and nonhomogeneous leukoplakias and further into different subtypes. A proposal has been made to slightly rearrange these subtypes. Furthermore, attention has been paid to a number of keratotic lesions that have been reported in the literature. It is expected that the increasing knowledge on carcinogenesis, including various genetic aspects, will be reflected in the definition of oral potentially (pre)malignant lesions in the near future.

Follow-up of oral cancer patients: three uneventful years may be enough.

OBJECTIVE: To explore the possibility of shortening the length of follow-up from 5 to 3 years in patients who have undergone curative treatment of an oral squamous cell carcinoma. STUDY DESIGN: The medical records of 225 patients who had undergone initial curative treatment at the VU University Medical Center in Amsterdam, the Netherlands, between 2004 and 2009 were analyzed. RESULTS: In 96 patients (42.7%) a secondary event occurred. Eighty-six percent of the secondary events were detected within 3 years. In the fourth and fifth years, 14% of the secondary events were found, consisting mainly of second primary tumors. CONCLUSIONS: Most secondary events occurred within the first 3 years of follow-up. Therefore, the case can be made for an altered regimen of follow-up beyond 3 years based on certain risk factors unique to the patient as well as the patient's ability to adequately report symptoms that may be associated with recurrent disease.

Oral leukoplakia, the ongoing discussion on definition and terminology.

In the past decades several definitions of oral leukoplakia have been proposed, the last one, being authorized by the World Health Organization (WHO), dating from 2005. In the present treatise an adjustment of that definition and the 1978 WHO definition is suggested, being : "A predominantly white patch or plaque that cannot be characterized clinically or pathologically as any other disorder; oral leukoplakia carries an increased risk of cancer development either in or close to the area of the leukoplakia or elsewhere in the oral cavity or the head-and-neck region". Furthermore, the use of strict diagnostic criteria is recommended for predominantly white lesions for which a causative factor has been identified, e.g. smokers' lesion, frictional lesion and dental restoration associated lesion. A final diagnosis of such leukoplakic lesions can only be made in retrospect after successful elimination of the causative factor within a somewhat arbitrarily chosen period of 4-8 weeks. It seems questionable to exclude "frictional keratosis" and "alveolar ridge keratosis" from the category of leukoplakia as has been suggested in the literature. Finally, brief attention has been paid to some histopathological issues that may cause confusion in establishing a final diagnosis of leukoplakia.

Disease scoring systems for oral lichen planus; a critical appraisal.

The aim of the present study has been to critically review 22 disease scoring systems (DSSs) on oral lichen planus (OLP) that have been reported in the literature during the past decades. Although the presently available DSSs may all have some merit, particularly for research purposes, the diversity of both the objective and subjective parameters used in these systems and the lack of acceptance of one of these systems for uniform use, there is a need for an international, authorized consensus meeting on this subject. Because of the natural course of OLP characterized by remissions and exacerbations and also due to the varying distribution pattern and the varying clinical types, e.g. reticular and erosive, the relevance of a DSS based on morphologic parameters is somewhat questionable. Instead, one may consider to only look for a quality of life scoring system adapted for use in OLP patients.

Granular cell tumor of the oral cavity; a case series including a case of metachronous occurrence in the tongue and the lung.

The granular cell tumor (GCT) is a rare, benign tumor that most commonly occurs in the oral cavity, particularly in the anterior part of the tongue. In this study the experience with 16 patients with a GCT observed in a single Institution will be discussed. Although no radicality has been obtained in most cases, recurrences are rare. In one patient, a recurrence was noted four years after excision of the primary. In the same patient a pulmonary lesion occurred five years after excision of the recurrence in the oral cavity, most likely representing an example of metachronous occurrence and not a distant metastasis. Since recurrences and metachronous lesions are rare, as are distant metastases, routine follow-up does not seem warranted in patients treated for a granular cell tumor of the oral cavity.

Histopathological grading of adenoid cystic carcinoma of the head and neck: analysis of currently used grading systems and proposal for a simplified grading scheme.

BACKGROUND: Histopathological grading of adenoid cystic carcinoma (ACC) is a controversial issue. It is generally agreed that solid type ACC has a relatively poor prognosis. However, the amount of solid regions within this often mixed type tumor that predicts a poor prognosis is not firmly established. Some authors stipulate that the presence of a solid component regardless of the amount is a poor prognosticator where others argue that the amount should be taken into consideration. Two grading systems most commonly used are those described by Perzin et al./Szanto et al. and Spiro et al., respectively. They report that prognosis of ACC is poor if >30% and >50% of the tumor volume has a solid growth pattern, respectively. MATERIAL AND METHODS: The described grading systems are applied to a series of 81 surgically treated cases of ACC at the VU University Medical Center, Amsterdam, The Netherlands. Moreover, we introduced an alternative grading system, in which the presence of a solid component, irrespective of its amount, is considered. All three systems of grading were tested for inter-observer concordance and prediction of prognosis. RESULTS: Inter-observer concordance for grading ACC according to Perzin et al./Szanto et al. and Spiro et al., proved to be moderate with Kappa Scores of 0.393 and 0.433, respectively. Our alternative grading system yielded inter-observer concordance with a Cohen's kappa result of 0.990. All systems were comparable in discriminating patients with poor clinical outcome. Histopathological grade proved to be an independent prognosticator. CONCLUSION: The presence of any solid component in ACC is a negative prognosticator, and can histopathologically be diagnosed with a high reliability. These results suggest to merely register the presence or absence of a solid tumor component since its inter-observer variability is very low, its reproducibility is high and its predictive value is comparable to the traditional grading systems used.

Epidemiology of ameloblastomas of the jaws; A report from the Netherlands

OBJECTIVES: To provide epidemiological data of ameloblastomas of the jaws in the Netherlands over a 25-year time period (1985-2010) and to compare these data with data from other parts of the world. MATERIAL AND METHODS: The data of all patients diagnosed with a primary ameloblastoma of the jaws in the Netherlands in the period 1985-2010 have been retrieved from the nationwide histopathology and cytopathology network and registry in the Netherlands (PALGA). The pathology reports were screened and only those cases were included in which a distinct diagnosis of primary, histopathologically benign, intraosseous ameloblastoma was rendered. The average population in The Netherlands during this period amounted approximately 15 million people. RESULTS: An annual incidence rate was approximately 1,5 per million population, the male-female ratio being 1.4: 1. The age at the time of diagnosis was 44.1 years. The average age in males was 46.3 years compared to anaverage age in females of 41.3 years, the difference being significant (p≤ 0.05). The results were compared with those available in only a small number of publications worldwide. CONCLUSIONS: There is no strong evidence for significant differences of the true incidence of ameloblastomas worldwide, neither for a gender predilection. The diagnosis is generally made at a somewhat lower age in women; this phenomenon is even much stronger in the Black population, irrespective of gender. No proper explanation for this finding can be provided

Epidemiology of ameloblastomas of the jaws; a report from the Netherlands.

OBJECTIVES: To provide epidemiological data of ameloblastomas of the jaws in the Netherlands over a 25-year time period (1985-2010) and to compare these data with data from other parts of the world. MATERIAL AND METHODS: The data of all patients diagnosed with a primary ameloblastoma of the jaws in the Netherlands in the period 1985-2010 have been retrieved from the nationwide histopathology and cytopathology network and registry in the Netherlands (PALGA). The pathology reports were screened and only those cases were included in which a distinct diagnosis of primary, histopathologically benign, intraosseous ameloblastoma was rendered. The average population in The Netherlands during this period amounted approximately 15 million people. RESULTS: An annual incidence rate was approximately 1,5 per million population, the male-female ratio being 1.4: 1. The age at the time of diagnosis was 44.1 years. The average age in males was 46.3 years compared to an average age in females of 41.3 years, the difference being significant (p≤ 0.05). The results were compared with those available in only a small number of publications worldwide. CONCLUSIONS: There is no strong evidence for significant differences of the true incidence of ameloblastomas worldwide, neither for a gender predilection. The diagnosis is generally made at a somewhat lower age in women; this phenomenon is even much stronger in the Black population, irrespective of gender. No proper explanation for this finding can be provided.

The role of a labial salivary gland biopsy in the diagnostic procedure for Sjögren's syndrome; a study of 94 cases.

OBJECTIVES: The purpose of the present study is to examine the role of the outcome of the labial salivary gland biopsy (LSGB) in the diagnostic procedure of patients suspected of suffering from Sjögren's syndrome (SS). MATERIAL AND METHODS: In a retrospective study the result of histopathological assessment of 94 consecutively taken labial salivary gland biopsies has been examined. For the diagnosis of SS the American-European Consensus Group classification (AECG, 2002) have been used. The outcome of the assessment has been discussed in relation to a recently reported classification provided by the American College of Rheumatology (ACR, 2012). RESULTS: In the 94 LSGBs support for a diagnosis of SS has been encountered in 24 out of 26 patients with SS. In the 68 patients with a negative diagnosis of SS only six positive LSGBs were observed. The sensitivity of the labial biopsy amounted 0.92; the specificity was 0.91, while the positive predictive value and the negative predictive value amounted 0.80 and 0.97 respectively. LSGBs taken by or on the request of the departments of Rheumatology or Internal Medicine had a significant higher yield compared to LSGBs taken in other clinical departments. CONCLUSIONS: The LSGB may play a role in the diagnostic procedure of Sjögren's syndrome when using either the AECG classification or the ACR classification. A LSGB should preferably taken after counseling for the possible presence of SS by a department of Rheumatology or Internal Medicine since the yield of such biopsies is much higher than in patients who have not been counseled by these departments prior to the taking of a LSGB. When using the ACR classification, a positive serologic result and a positive ocular test make the taking of a LSGB redundant. Only in case of a negative serologic outcome or a negative result of the ocular test a LSGB is indicated. Since both the serologic test and the ocular test carry hardly any morbidity, these tests should, indeed, be performed first before considering to take a LSGB.

Oral potentially malignant disorders: is malignant transformation predictable and preventable?

Leukoplakia is the most common potentially malignant disorder of the oral mucosa. The prevalence is approximately 1% while the annual malignant transformation ranges from 2% to 3%. At present, there are no reliable clinicopathological or molecular predicting factors of malignant transformation that can be used in an individual patient and such event can not truly be prevented. Furthermore, follow-up programs are of questionable value in this respect. Cessation of smoking habits may result in regression or even disappearance of the leukoplakia and will diminish the risk of cancer development either at the site of the leukoplakia or elsewhere in the mouth or the upper aerodigestive tract. The debate on the allegedly potentially malignant character of oral lichen planus is going on already for several decades. At present, there is a tendency to accept its potentially malignant behaviour, the annual malignant transformation rate amounting less than 0.5%. As in leukoplakia, there are no reliable predicting factors of malignant transformation that can be used in an individual patient and such event can not truly be prevented either. Follow-up visits, e.g twice a year, may be of some value. It is probably beyond the scope of most dentists to manage patients with these lesions in their own office. Timely referral to a specialist seems most appropriate, indeed.

Proposal for a new staging system for osteoradionecrosis of the mandible.

A new staging system for osteoradionecrosis of the mandible has been retrospectively applied to a group of 31 patients. In this system clinicoradiographic signs and symptoms are incorporated in a simplified manner. For imaging purposes the use of plain radiographs such as periapical films and panoramic radiographs is recommended, mainly because of their readily availability. The presented staging system seems well reproducible, facilitating the comparison of study groups dealing with the various issues of osteoradionecrosis of the mandible. It is yet to be evaluated whether the presently proposed staging system is useful for management purposes.

Oral squamous cell carcinoma in patients twenty years of age or younger--review and analysis of 186 reported cases.

To review the literature on reported cases of squamous cell carcinoma (SCC) of the oral cavity in patients twenty-years-of-age or younger. All well-documented cases of oral SCC in patients twenty-years-of-age or less, published between 1936 and 2012, were collected and the clinicopathologic features were evaluated. Primary cases of oral SCC were selected. Age, gender, intra-oral subsite were recorded. A total of 88 articles describing 186 cases were included. The group of otherwise healthy patients had a mean age of 14.08years (range newborn-20years), the m:f ratio was 1.36; the oral subsites were the tongue, gingiva, and lower lip. A second group of patients who have disorders that predispose to cancer development, such as xeroderma pigmentosum, Fanconi's anemia, and a history of bone marrow transplant, had a mean age of 13.17years (range 5-20years); the m:f ratio was 1.23; the oral subsite was mainly the tongue. There was a slight difference between otherwise healthy patients and patients with predisposing systemic factors, but this difference was not statistically significant. It contrast to adults, there is only a weak predilection for males (m:f ratio of 1.23-1.36). In the young population SCC occurs most frequently in the tongue, followed by gingiva and lips. Unlike in adults, SCC is very uncommon in the floor of mouth. Oral SCC may, indeed, occur in patients younger than 20years and clinicians should take cognizance of this. Periodical examination of the oral cavity of young patients is recommended in cases of systemic diseases that predispose to cancer development such as xeroderma pigmentosum, Fanconi's anemia, and a history of bone marrow transplant.

Molecular screening of oral precancer.

OBJECTIVES: Early detection and treatment of high risk premalignant mucosal changes of the oral cavity, will expectedly improve survival and reduce treatment-related morbidity. Aims of this study were to evaluate a non-invasive screening approach and to assess the value of molecular markers to identify patients at risk for oral cancer. MATERIALS AND METHODS: Exfoliated cells and biopsies were obtained from oral leukoplakia lesions of 43 patients, of whom six developed oral cancer. All samples were investigated for loss of heterozygosity (LOH) at chromosomes 3p, 9p, 11q and 17p using microsatellite markers. On the biopsy specimen additional immunohistochemical staining for p53, TP53 mutation analysis and histopathological grading were performed. RESULTS: The analytical sensitivity of the non-invasive assay using exfoliated cells to detect genetic changes present in the lesions was 45% (9 of 20), the specificity was 100% (19 of 19), and the positive predictive value was also 100% (9 of 9). LOH was present in 20 of 39 (51%) of the biopsies with uniformly LOH at 9p. Mutated TP53 and LOH at 9p in the biopsy, as single markers and in combination, were significant risk factors for malignant progression of leukoplakia to oral cancer (Kaplan-Meier analysis, p<0.05). CONCLUSION: A non-invasive genetic screening approach using LOH in exfoliated cells has limited value for monitoring patients with leukoplakia. However, LOH at 9p, but also mutated TP53 in biopsies of oral leukoplakia have a significant association with malignant transformation and are promising candidate biomarkers to predict the risk for malignant progression.

Management recommendations for invasive dental treatment in patients using oral antithrombotic medication, including novel oral anticoagulants.

OBJECTIVE: The aims were (1) to search the scientific literature from 2007 to 2012 for guidelines and new studies on the dental management of patients using oral antithrombotic medication; (2) to summarize the articles' evidence and recommendations; and (3) to propose an updated clinical practice guideline for general dentists. STUDY DESIGN: A systematic literature search in MEDLINE, Embase, and the Guideline websites, from October 2007 to October 2012, produced articles that were critically evaluated. RESULTS: The systematic literature search for guidelines yielded 74 citations (MEDLINE, 45; Embase, 22; and the Guideline websites, 7). Of these, only 2 guideline publications and 2 systematic reviews met the inclusion criteria. They yielded 32 recommendations. CONCLUSIONS: The evidence and subsequent recommendations from published guidelines all point in the same direction: do not interrupt oral antithrombotic medication, not even dual antiplatelet therapy, in simple dental procedures.

Adenoid cystic carcinoma of the head and neck: a single-center analysis of 105 consecutive cases over a 30-year period.

BACKGROUND: Adenoid cystic carcinoma is a rare salivary gland malignancy with a poor disease free survival due to frequent distant metastases and late local recurrences. Previous single-center reports on outcome mostly encompass small series. In this report a relative large series of 105 cases is analyzed, all treated at the VU University Medical Center, Amsterdam, The Netherlands over a 30-year period in which treatment strategies remained unchanged. METHODS: All cases of ACC of the head and neck between 1979 and 2009 at our institution were analyzed through a medical chart review. Recurrence patterns and possible prognostic factors (T-stage, N-status, age, gender, type of salivary gland involved, histological grade, surgical margins, perineural invasion (PNI) and postoperative radiotherapy (RT)) were analyzed. RESULTS: One-hundred and five cases of ACC of the head and neck were identified. Five-, ten- and twenty-year survival rates for overall survival were 68%, 52% and 28%, respectively. T-stage, N-status, surgical margins, histological subtype and age were highly significant predictors for survival. PNI was not a negative prognosticator. CONCLUSIONS: T-stage, N-status, surgical margins, histological grade and age are the main predictors of survival-outcome in ACC of the head and neck. Distant metastasis frequently develop, mainly in the first 5 years post treatment. Local recurrences often develop even later on, warranting long term follow up of patients treated for ACC. Grade III ACC should be considered a specific entity within the group of ACC due to its typical aggressive biological behavior and relatively poor outcome, implicating the need for an improved adjuvant treatment.