Heterotopic gastrointestinal cyst within the submandibular space in an adult: A case report and review of the literature.

Heterotopic gastrointestinal cysts (HGIC) are rare congenital cysts that arise anywhere along the gastrointestinal tract. HGICs are infrequently reported in the oral cavity; 0.3% of HGICs are reported in the tongue and even more rarely in the submandibular space. Oral HCIGs are more common in children with only 13 reported cases in adults. In the present report, we discuss the differential diagnoses of the submandibular space lesions and describe a rare case of a very large submandibular space HGIC in an adult patient.

Ameloblastoma with adenoid features: Case report with cyto-histopathologic correlation and molecular findings.

Ameloblastomas are benign but locally aggressive odontogenic tumors that commonly present as expansile lesions in the tooth-bearing areas. Fine-needle aspiration (FNA) biopsies of ameloblastomas are rare in clinical practice, and only a handful of case reports and series have described their cytologic features. We present the case of a 70-year-old woman with a large and disfiguring maxillary sinus soft tissue mass sampled via transcutaneous FNA. Aspirate smears were composed of small clusters of cohesive and monotonous basaloid cells. The accompanying cellblock showed similar clusters of basaloid cells in gland-like, or "adenoid," configurations, eliciting a differential diagnosis that included sinonasal and salivary gland neoplasms. Excisional surgery material was consistent with ameloblastoma with adenoid morphology. Next-generation sequencing (NGS) analysis demonstrated FGFR2 and SMO pathogenic variants. This case exemplifies several uncommonly described features of ameloblastomas in cytology, including cyto-histologic correlation, adenoid morphology, and NGS findings. Awareness of the cytologic features of this neoplasm are important for cytopathologists confronted with maxillary sinus lesions.

Spontaneous resolution of an eosinophilic granuloma of the mandible following open biopsy.

Langerhans cell histiocytosis X is a clonal proliferation of dendritic cells of the immune system, which can affect multiple organ systems and range in behavior from a benign inflammatory process to a much more aggressive process. Only few isolated cases have been reported in the jaws. This case represents an example of Langerhans cell histiocytosis X in the mandible, which resolved following incisional biopsy without any further surgical intervention.

Primary Xanthoma of the Mandible: Report of a Rare Case.

Xanthoma is a lesion most commonly seen in soft tissues such as the skin, subcutis, or tendon sheaths. Xanthoma formation is often associated with primary or secondary hyperlipidemia. Primary bone xanthomas are extremely rare benign bone lesions not associated with hyperlipidemia, histopathologically characterized by histiocytes, abundant lipid containing macrophages (foam cells), and multinucleated giant cells. Cholesterol clefts can be found in the medullary bone. Less than ten cases of xanthoma in the mandible have been reported. We present a rare primary intrabony xanthoma in a normolipidemic patient.

Multiple Radiolucencies of the Jaws in a 6 Year Old.

Radiolucencies of the jaws are a common finding. Mostly, they are isolated findings associated with inflammatory processes and consistent with inflammatory periapical disease, such as periapical cysts and periapical granulomas. If associated with the crowns of unerupted teeth, they may represent dentigerous cysts. However, occasionally, larger lytic/radiolucent lesions are identified; even more infrequently, these radiographic findings may be multiple and concurrent. The purpose of this report is to present the case of a 6-year-old female patient with no known concomitant syndrome who presented with multiple intraoral swellings and to discuss the treatment of multiple, bilateral mandibular and maxillary radiolucencies.

Surgical management of the buccal bifurcation cyst: bone grafting as a treatment adjunct to enucleation and curettage.

The buccal bifurcation cyst (BBC) is a rare inflammatory odontogenic cyst of unknown etiology. It typically develops on the buccal aspect of the permanent mandibular first molar and occasionally on the permanent mandibular second molar in children 4 to 14 years old. Distinct clinical findings of the BBC include involvement of a vital partially or fully erupted mandibular first or second molar, swelling in the affected mandibular molar region, delayed or altered eruption pattern of the involved tooth, and an increase in periodontal pocket depth when the affected tooth is partially erupted. Specific radiographic features include a radiolucent lesion on the buccal aspect of the tooth involving the roots to a variable extent, tilting of the involved molar so that the root apices are toward the lingual cortical plate, an intact periodontal ligament space and lamina dura, a periosteal reaction on the buccal surface, and an intact inferior border of the mandible. The histopathology of the lesion has been described as similar to a radicular or inflammatory odontogenic cyst. Most of the current literature supports simple enucleation and curettage of the cyst without extraction of the involved tooth as the treatment of choice. This report presents 3 cases of BBCs that were treated with enucleation and curettage without extraction of the involved tooth, in addition to a bone graft placed primarily or secondarily as an adjunctive treatment approach to the current therapies.

Gingival squamous cell carcinoma in adolescence.

Squamous cell carcinoma (SCC) is a rare finding in the adolescent population, with most cases occurring in patients with underlying heritable diseases or immunologic conditions. Moreover, the incidence of oral SCC in this age group is extremely low. While isolated cases of adolescent oral SCC have been documented, most have been primary tongue or lip lesions. We report 4 cases of gingival SCC occurring in otherwise healthy adolescent patients. The preliminary clinical impressions ranged from factitial injury to inflammatory tissue. Microscopic similarities, including overlap with pseudoepitheliomatous hyperplasia and keratoacanthoma, were seen. Review of the literature indicates that adolescent gingival SCC is extremely rare and a challenging diagnosis for the clinician and pathologist alike. Diagnostic pitfalls, possible etiologic factors, and the prognostic outlook of this condition are discussed.

Assessment of CD43 expression in adenoid cystic carcinomas, polymorphous low-grade adenocarcinomas, and monomorphic adenomas.

OBJECTIVE: Distinguishing between adenoid cystic carcinoma (ACC), polymorphous low-grade adenocarcinoma (PLGA), and monomorphic adenoma (MA) can occasionally pose a diagnostic challenge. It is of interest to identify a marker that can differentiate between these tumors. CD43 is a sialoglycoprotein that is typically expressed by hematopoietic cells and their derivative neoplasms, although positivity in epithelial tumors has been recently recognized. Our aim was to investigate CD43 immunoreactivity in ACCs, PLGAs, and MAs. STUDY DESIGN: Formalin-fixed paraffin-embedded sections from 40 salivary gland tumors (12 ACCs, 14 PLGAs, and 14 MAs) accessioned from 1989 to 2002 were retrieved from the files at the Department of Pathology, Long Island Jewish Medical Center. Immunohistochemical staining with anti-CD43 monoclonal antibody was performed. RESULTS: Cytoplasmic and membranous immunoreactivity was detected in 12/12 ACCs (100%), 1/14 PLGAs (7.1%), and 3/14 MAs (21.4%). CONCLUSIONS: CD43 appears to be preferentially expressed in salivary gland ACCs compared to PLGAs and MAs. Although the mechanism of this overexpression remains obscure at this time, our results suggest that the use of CD43 immunostaining as an adjunct to histological examination may be helpful in differentiating ACC from its mimics.

Bilateral central giant cell granulomas of the mandible in an 8-year-old girl with Noonan syndrome (Noonan-like/multiple giant cell lesion syndrome).

We report a case of an 8-year-old girl who presented with bilateral central giant cell granulomas of the posterior mandible. Characteristic facial features and a history of pulmonary stenosis led us to suspect a diagnosis of Noonan syndrome. A medical geneticist confirmed this. This case report will discuss the salient features of this diagnosis.

Assessment of p63 expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous low-grade adenocarcinoma, and basal cell and canalicular adenomas.

PURPOSE: The purpose of this study was to determine the extent of p63 immunoreactivity in the malignant salivary gland neoplasms adenoid cystic carcinoma (ACC) and polymorphous low-grade adenocarcinoma (PLGA) and to compare this to the expression of this marker in the benign salivary gland tumors canalicular adenoma and basal cell adenoma. Few studies on the expression of p63 in head and neck salivary gland tumors have been published to date. P63, a selective immunohistochemical marker of basal/stem cells of stratified epithelium and of myoepithelial cells, is a p53 homologue that plays an essential role in both morphogenesis of epidermis and limb development. P63 immunoreactivity has been demonstrated in squamous cell and urothelial carcinomas. It is generally absent in most nonsquamous cell carcinomas. Study design Formalin-fixed paraffin-embedded sections from 49 salivary gland neoplasms, representing 6 canalicular adenomas, 11 basal cell adenomas, 17 PLGA and 15 ACC accessioned from 1989 to 2002 by the Department of Pathology, Long Island Jewish Medical Center, New Hyde Park, NY, were stained with an anti-p63 monoclonal antibody. RESULTS: Nuclear p63 reactivity was uniformly positive in PLGA (17/17, 100%). Positive reactivity was also identified in the majority of cases of ACC (13/15, 87%), primarily in the nonluminal myoepithelial-like cells surrounding luminal cells. Canalicular adenoma did not exhibit any p63 immunoreactivity. All basal cell adenomas of parotid origin stained strongly for p63, with staining localized to the peripheral tumor cells situated adjacent to the connective tissue stroma. None of the basal cell adenomas originating in the upper lip stained with p63. In native adjacent salivary gland tissue, p63 reactivity was identified focally in the nuclei of myoepithelial and basal duct cells. CONCLUSIONS: P63 is strongly expressed in basal cell adenoma of parotid origin, and in ACC and PLGA. Canalicular adenoma did not demonstrate p63 staining, consistent with this tumor's putative luminal ductal cell differentiation. Our results suggest that the neoplastic cells in PLGA may represent either a population of p63-positive epithelial stem/reserve cells similar to the basal cells of stratified epithelium, or modified myoepithelial cells. Given the staining pattern of the tumors examined, p63 does not appear to be an ideal marker for distinguishing between ACC, PLGA, and basal cell adenoma.

C-kit expression in the salivary gland neoplasms adenoid cystic carcinoma, polymorphous low-grade adenocarcinoma, and monomorphic adenoma.

OBJECTIVE: Differentiating between adenoid cystic carcinomas (ACCs), polymorphous low-grade adenocarcinomas (PLGAs), and the monomorphic adenomas (including canalicular adenomas, trabecular adenomas, and basal cell adenomas) can present a diagnostic challenge, especially when examining tissue obtained from small incisional or fragmented biopsies. Recent studies have revealed that overexpression of the tyrosine kinase receptor protein c-kit occurs in a narrow subset of malignant neoplasms, including gastrointestinal stromal tumors, myeloid leukemias, seminomas, and ACCs. C-kit reportedly is not expressed in PLGAs. We compared the expression of the c-kit antigen in the malignant salivary gland neoplasms ACC and PLGA with its expression in salivary gland monomorphic adenoma (including canalicular adenoma and basal cell adenoma). STUDY DESIGN: Formalin-fixed paraffin-embedded sections of 49 salivary gland neoplasms (17 monomorphic adenomas, 17 PLGAs, and 15 ACCs) accessioned between 1989 and 2002 were retrieved from the files of the Department of Pathology, Long Island Jewish Medical Center, and were stained with an anti-c-kit polyclonal antibody. RESULTS: C-kit reactivity was uniformly positive in the cytoplasm of luminal neoplastic cells in ACCs (15/15, 100%). Positive reactivity was also identified in the majority of PLGAs (16/17, 94%), with at least 25% of the tumor cells being positive. Similar reactivity was seen in monomorphic adenomas (16/17, 94%). CONCLUSIONS: In contrast to previous reports, we find that c-kit expression was not restricted to ACC but was expressed in all 3 tumor types evaluated (ACC, PLGA, and monomorphic adenoma). Therefore, c-kit does not appear to be a useful marker for distinguishing between either ACC and PLGA in equivocal cases, or in benign and malignant salivary gland neoplasms.

Oral lichen planus: clinical presentation and management.

Oral lichen planus (OLP) is a chronic mucosal condition commonly encountered in clinical dental practice. Lichen planus is believed to represent an abnormal immune response in which epithelial cells are recognized as foreign, secondary to changes in the antigenicity of the cell surface. It has various oral manifestations, the reticular form being the most common. The erosive and atrophic forms of OLP are less common, yet are most likely to cause symptoms. Topical corticosteroids constitute the mainstay of treatment for symptomatic lesions of OLP. Recalcitrant lesions can be treated with systemic steroids or other systemic medications. However, there is only weak evidence that these treatments are superior to placebo. Given reports of a slightly greater risk of squamous cell carcinoma developing in areas of erosive OLP, it is important for clinicians to maintain a high index of suspicion for all intraoral lichenoid lesions. Periodic follow-up of all patients with OLP is recommended.