An oral lesion masquerading as lichen planus.

Proliferative verrucous leukoplakia (PVL) is a rare, aggressive form of oral leukoplakia with a substantial risk of malignant transformation. The slowly progressive course and the lack of a single defining histopathologic characteristic for PVL make this entity a diagnostic challenge. We report on a patient who presented with a 7-year history of worsening oral lesions.

A subset of ectomesenchymal chondromyxoid tumours of the tongue show EWSR1 rearrangements and are genetically linked to soft tissue myoepithelial neoplasms: a study of 11 cases.

AIMS: Ectomesenchymal chondromyxoid tumour (ECT) is a rare, benign intraoral neoplasm showing a predilection for the anterior dorsum of the tongue. The World Health Organization includes ECT in the pathological spectrum of soft tissue myoepithelioma. EWS RNA-binding protein 1 gene (EWSR1) rearrangement is found in 45% of cutaneous, soft tissue and bone myoepithelial neoplasms, and pleomorphic adenoma gene 1 (PLAG1) aberrations are found in 37% of EWSR1-negative soft tissue myoepitheliomas. The aim of this study was to evaluate the presence of EWSR1 and PLAG1 rearrangements in ECTs. METHODS AND RESULTS: Eleven formalin-fixed, paraffin-embedded ECTs were evaluated with fluorescence in-situ hybridization probes for EWSR1 (22q12) and PLAG1 (8q12). Among the 11 ECTs tested, three (27.3%) showed EWSR1 rearrangement in >15% of tumour cells, whereas eight (72.7%) cases did not show EWSR1 rearrangement. Eight of nine (89%) ECTs showed gain of EWSR1, probably representing gain of all or part of chromosome 22, in a varying proportion of neoplastic cells ranging between 1.4% and 27.9%. PLAG1 rearrangement was not detected in the successfully hybridized tissue sections (7/11). No correlation was observed between the molecular and histopathological findings, such as morphology of the neoplastic cells, the presence of atypia, and matrical type. CONCLUSIONS: We identified EWSR1 rearrangement in >25% of ECTs. These results suggest that some ECTs are at least genetically related to myoepithelioma of the soft parts. Finally, PLAG1 aberrations do not appear to be critical in the pathogenesis of ECT of the tongue.

Oral Potentially Malignant Disorders.

Oral potentially malignant disorders (OPMDs) are precursor lesions that may undergo malignant transformation to oral cancer. These lesions most commonly present clinically as white patches (leukoplakia). However, they may also be red (erythroplakia), or red and white (erythroleukoplakia). There are many risk factors associated with the development of an OPMD, and with the risk of malignant transformation of the lesion. A biopsy with subsequent microscopic examination from the lesional tissue is necessary in identification of OPMD. This article reviews the clinical appearance of OPMDs, associated risk factors, diagnosis and histologic appearance, and treatment.

Primordial Odontogenic Cyst with Induction Phenomenon (Zonal Fibroblastic Hypercellularity) and Dentinoid Material Versus Archegonous Cystic Odontoma: You Choose!

The most recent A.F.I.P. fascicle defines primordial odontogenic cyst (POC) as a distinct, nonkeratinized, odontogenic cyst of "undetermined origin" forming in the place of a developing normal or supernumerary tooth. However, the majority of examples reported in the literature under this term represent odontogenic keratocysts (keratocystic odontogenic tumors). In addition, there are rare reported cases of cystic odontomas. An 18-year-old Caucasian male presented with a unilocular mandibular radiolucent lesion in the place of a congenitally missing molar. Histologically, it featured nonkeratinizing, thin stratified squamous epithelial lining with areas of spongiosis and foci of vacuolization of individual basal cells without significant nuclear palisading. Focally, budding of the basal cell layer was identified. A zone of increased cellularity featuring induction-type fibroblasts was present subepithelially as well as dentinoid deposits with odontogenic epithelial nests. Immunohistochemically, the epithelial lining was negative for calretinin and the induction-like zone negative for S100 protein, smooth muscle actin, and CD34. The case was externally reviewed by five oral pathologists who provided various diagnostic interpretations including primordial cyst, odontogenic cyst not otherwise specified (NOS), cyst with ameloblastic changes, and unicystic ameloblastoma. At that time, a final diagnosis of odontogenic cyst NOS was rendered with a comment that it may represent a true example of POC or a cystic odontoma. The lesion has not recurred within a 13 year follow-up period after initial excision. An unusual cystic lesion is presented that may represent a true example of POC with dentinoid formation or an archegonous cystic odontoma.

Clinical utility of myb rearrangement detection and p63/p40 immunophenotyping in the diagnosis of adenoid cystic carcinoma of minor salivary glands: a pilot study.

OBJECTIVES: MYB rearrangement is observed in approximately 28% to 86% of adenoid cystic carcinomas (ACCs). Also, ACC features a p63+/p40+ immunophenotype in greater than 90% of cases, compared with p63+/p40- polymorphous low-grade adenocarcinoma (PLGA). Our aim was to investigate the incidence of (1) MYB rearrangement and (2) p63/p40 immunoreactivity in ACC and PLGA of minor salivary glands (MSGs). STUDY DESIGN: Seven cases of ACC as well as five of PLGA were evaluated by using a MYB (6 q23.3) break-apart fluorescence in situ hybridization (FISH) probe. In addition, all cases were immunohistochemically stained with p63 and p40 antibodies. RESULTS: All five successfully hybridized ACCs featured MYB rearrangement, whereas PLGAs did not show MYB rearrangement. Interestingly, one case of PLGA demonstrated a single intact copy of MYB in greater than 88% of the neoplastic cells. All ACCs exhibited consistent p63+/p40+ staining, whereas PLGAs demonstrated a p63+/p40- immunophenotype. CONCLUSIONS: (1) MYB rearrangement is encountered in ACCs but not PLGAs of MSGs; (2) MYB aberrations, for example, monosomy or deletion, can be seen in PLGAs; (3) combined p63/p40 immunostaining can be used to differentiate ACC from PLGA in incisionally biopsied specimens; and (4) performance of either FISH or p63/p40 immunohistochemistry is expected to be able to confirm the diagnosis of ACC or PLGA in small intraoral biopsies, since both techniques appeared to be diagnostically accurate in this pilot study.

Malignant rhabdoid tumor of the floor of mouth: first reported case in the oral cavity of an adult.

Malignant rhabdoid tumors (MRTs) are exceedingly rare lesions. To our knowledge, only 2 cases have been reported in the oral cavity, with both examples occurring in infants. The current case is the third reported case of MRT of the oral cavity and the first reported case to occur in an adult at this location. The following report describes the clinical, histologic and immunohistochemical features of this tumor.