Dysgenetic Polycystic Disease of the Salivary Glands: A Case Report of This Rare Entity Occurring for the First Time in the Minor Salivary Glands of the Tongue, and a Review of the Literature.

Dysgenetic polycystic disease, also known just as polycystic disease, is a very rare developmental abnormality affecting the salivary gland duct system. This entity has been reported in only 21 patients previously, although a careful review suggests only 16 patients have histological evidence of the disease. In previously reported cases, this lesion most commonly presents as either an incidental finding or as a swelling affecting the parotid glands bilaterally, or rarely the submandibular glands bilaterally. This case report details the first time dysgenetic polycystic disease is found affecting the minor salivary glands of the tongue in a 55-year-old male. Histochemical and immunohistochemical stains are presented and include positivity for AE1/AE3 and p63, and negativity for progesterone receptor, androgen receptor, mammaglobin, S100 and BRAF V600E. PAS-D and Congo Red highlight special microamyloid spheroliths structures intraluminally.

CD1a is not effective in distinguishing calcifying epithelial odontogenic tumors from amyloid-rich central odontogenic fibroma.

OBJECTIVE: Differential diagnosis between the non-calcifying variant of calcifying epithelial odontogenic tumor (NCLC-CEOT) and amyloid-rich central odontogenic fibroma (AR-COdF) has become a debate, particularly regarding the frequency of CD1a positivity in both entities. This has led to the growing consensus that CD1a-positive staining in AR-NC lesions confirms the diagnosis of AR-COdF. Here, we assess the validity of this consensus. STUDY DESIGN: We collected the data of a case series of histopathologically distinct CEOTs, NCLC-CEOTs, and COdFs and stained them for CD1a and amyloid. Of the 9 CEOTs and NCLC-CEOTs, we diagnosed 4 as classic, 3 as associated with a dentigerous cyst, and 2 as combined CEOT/adenomatoid odontogenic tumors. Of the 9 COdFs, we diagnosed 3 as epithelial poor, 3 as epithelial rich (lacking amyloid), 2 as hyalinized with amyloid, and 1 as hyalinized without amyloid and assessed the staining results. RESULTS: Of the 9 CEOTs and NCLC-CEOTs, 7 stained positively for CD1a, 5 diffusely and 2 focally. Notably, 2 classic NCLC-CEOTs stained strongly CD1a positive. All 3 of the epithelial-poor COdFs were predictably CD1a negative. Of the 6 remaining COdFs, 2 were CD1a positive, 1 hyalinized-with-amyloid COdF diffusely and 1 epithelial-rich-without amyloid focally. CONCLUSIONS: CD1a positivity, which occurs in classic CEOT and NCLC-CEOT, does not help distinguish between NCLC-CEOT and AR-COdF and is inconsistent in all AR-COdFs. The diagnosis of CEOT and AR-COdF should be guided by appropriate histopathologic criteria irrespective of CD1a staining or the presence of amyloid or calcifications.

Mucoepidermoid carcinoma showing continuity with the surface mucosa of the oral cavity: a report of 14 cases.

OBJECTIVE: We aimed to characterize the histology and the clinicodemographic features of mucoepidermoid carcinoma (MEC), showing continuity with the oral surface mucosa. STUDY DESIGN: We reviewed 138 cases of intraoral MEC to identify cases that showed continuity with the surface mucosa and compared their clinicodemographic findings with those of MECs not showing continuity. We compared the sex ratio using the 2-sample Z-test and compared the age distribution using the 2-sample Kolmogorov-Smirnov test. RESULTS: Of the 138 cases examined, 14 showed continuity with the surface mucosa. Their histology showed surface mucosa with an apparent transition to an infiltrating tumor with mucous, intermediate, and epidermoid tumor cells growing in solid and cystic patterns. Their clinical appearance ranged from firm submucosal nodules to erythematous to ulcerated lesions. They showed a strong female predilection (6:1) and sharply bimodal age distribution, with sharp peaks in the fourth and seventh decades. CONCLUSIONS: Mucoepidermoid carcinomas that show continuity have a demographic pattern distinct from that of conventional MECs, showing a striking female predilection and bimodal age distribution and suggesting a difference in etiology. Pathologists should remain aware that MEC in the oral cavity can have a histologic appearance of surface origin to reach the correct diagnosis.

Atypical herpetic ulcerations in COVID-19 positive patients: A report of three cases.

Since the global COVID-19 pandemic, numerous reports have been made regarding oral lesions seen in COVID-19 patients. It remains unclear whether or not these are true manifestations of COVID-19. Here we present 3 patients who were hospitalized for COVID-19 and who developed atypical herpetic ulcerations during their treatment with remdesivir (Veklury) and steroids. In healthy patients, recurrent infection by herpes simplex virus (HSV) presents as lesions only on the lips and the attached oral mucosa. Atypical herpetic ulcerations are seen in immunocompromised patients. They present as large, stellate shaped ulcerations with raised borders and may involve movable mucosa. The 3 cases presented in this report resembled the atypical herpetic ulcerations typically seen in patients with immunosuppression. Through our report, we aimed to introduce the possibility of atypical herpetic ulcers in patients being treated for COVID-19, to allow for their timely diagnosis and to raise awareness of the underlying immunocompromised state.

Ectomesenchymal Chondromyxoid Tumor: A Neoplasm Characterized by Recurrent RREB1-MKL2 Fusions.

Ectomesenchymal chondromyxoid tumor is a rare and benign neoplasm with a predilection for the anterior dorsal tongue. Despite morphologic heterogeneity, most cases are characterized by a proliferation of bland spindle cells with a distinctive reticular growth pattern and myxoid stroma. The immunophenotype of these neoplasms is likewise variable; most cases express glial fibrillary acid protein and S100 protein, with inconsistent reports of keratin and myoid marker expression. The molecular pathogenesis is poorly understood; however, a subset of cases has been reported to harbor EWSR1 gene rearrangement. Following identification of an RREB1-MKL2 fusion gene by RNA Sequencing in an index patient, a retrospective review of additional cases of ectomesenchymal chondromyxoid tumors was performed to better characterize the clinical, immunohistochemical, and molecular attributes of this neoplasm. A total of 21 cases were included in this series. A marked predisposition for the dorsal tongue was confirmed. Most cases conformed to prior morphologic descriptions; however, hypercellularity, hyalinized stroma, and necrosis were rare attributes not previously emphasized. The neoplastic cells frequently coexpressed glial fibrillary acid protein, S100 protein, keratin, smooth muscle actin, and/or desmin; a single case was found to contain significant myogenin expression. An RREB1-MKL2 fusion product was identified in 19 tumors (90%), a single tumor (5%) had an EWSR1-CREM fusion product, and the remaining case lacked any known fusion gene by RNA Sequencing. The latter 2 cases subtly differed morphologically from many in the cohort. This series illustrates that recurrent RREB1-MKL2 fusions occur in most, perhaps all, cases of ectomesenchymal chondromyxoid tumor.

Oral Lymphomatoid papulosis type C: A diagnostic pitfall, often confused with T-cell lymphoma.

Eosinophilic ulcer of the oral mucosa (EUOM) is a rare, benign, self-resolving lymphoproliferative disorder, which typically presents with asymptomatic to mildly tender ulcers. Histological findings of EUOM are characterized by a polymorphic infiltrate with many eosinophils often extending into the underlying muscle. Although this entity is well documented within the dental literature, it is not well known to physicians. The pathogenesis of the condition is unclear, although reports dating back to 1997 suggest that at least a subset of EUOM represents CD30 positive lymphoproliferative disorder (CD30+ LPD). More specifically the original report and subsequent authors suggest that the patients fall on the spectrum of CD30+ LPD most reminiscent of Lymphomatoid papulosis (LyP) seen in the skin. This oral variant of LyP would be expected to have the same diverse morphologic spectrum as that seen in cutaneous LyP. We present five EUOM patients whose biopsies showed an atypical lymphocytic infiltrate most compatible with Type C LyP, a histologically unique subset of LyP, reminiscent of the biopsy findings encountered in the reported case by Ficarra and co-workers. (Ficarra, et al., 1997) In four of the five cases, the biopsies were interpreted by expert hematopathologists as an aggressive form of peripheral T cell lymphoma resulting in recommendations to administer systemic chemotherapy to four of the patients, the scheduling of one patient for induction therapy and transplantation before revision of the diagnosis, and administration of chemotherapy to one of the patients. The natural clinical course of spontaneous regression refuted the original diagnoses as a form of aggressive peripheral T cell lymphoma. Recognition of oral LyP is critical to avoid inadvertent exposure to potentially toxic chemotherapeutic regimens intended for the treatment of high grade lymphoma.

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.

Two cases of CD30+, anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma with oral manifestations.

Anaplastic large cell lymphoma (ALCL), a subgroup of T-cell non-Hodgkin's lymphoma, is an uncommon tumor exhibiting CD30 positivity and a characteristic immunophenotypic profile. Histologically, ALCL is characterized by the proliferation of large, anaplastic lymphoid cells with eccentric horseshoe- or kidney-shaped nuclei and one or more prominent nucleoli. Rare cases have been cited in the literature of ALCL presenting primarily in the oral cavity. The purpose of this article was to present 2 instructive cases of CD30+, anaplastic lymphoma kinase-negative ALCL with oral and systemic involvement.

Primary glycogen-rich clear cell squamous cell carcinoma of the mandibular gingiva.

Clear cell squamous cell carcinoma (CCSCC) is a rare variant of squamous cell carcinoma, first reported by Kuo, who described 6 cases of squamous cell carcinoma of the skin of the head and neck. CCSCC is composed of cells with clear cytoplasm, which Kuo attributed to the accumulation of intracellular fluid and not the presence of glycogen, lipid, or mucin. This case describes a 59-year-old woman who presented with an exophytic, hemorrhagic lesion on the posterior mandibular gingiva of 2 months' duration. Histologic examination revealed dysplastic stratified squamous epithelium showing transition to an infiltrating tumor composed of islands of epithelial cells with clear cytoplasm. The cytoplasm stained positive with periodic acid Schiff but was diastase labile. Mucicarmine stains were negative for intracytoplasmic mucin. This is the first reported case describing primary glycogen-rich CCSCC of the mandibular gingiva.

Oral ectomesenchymal chondromyxoid tumor: case report and literature review.

Ectomesenchymal chondromyxoid tumor (ECMT) of the oral cavity is a rare lesion. We describe the 33rd reported case in the current English-language literature. This patient had originally presented 5 years earlier with a tongue neoplasm with biopsy results consistent with nerve sheath myxoma. A general surgical pathologist gave the patient's current lesion a preliminary diagnosis of low-grade sarcoma. After further evaluation by an oral and maxillofacial pathologist, the diagnosis of ectomesenchymal chondromyxoid tumor was established. It is important for clinicians to have a better understanding of ECMT, so as to avoid future misdiagnosis of this entity.

Estrogen and progesterone receptor and p53 gene expression in adenoid cystic cancer.

OBJECTIVES: The current study examined the role of estrogen receptors (ER), progesterone receptors (PR) and p53 expression in adenoid cystic carcinoma (ACC) to determine if simple expression or possible overexpression of these products might influence the development and natural course of this cancer. STUDY DESIGN: ER and PR status and p53 overexpression were retrospectively evaluated utilizing immunohistochemical evaluation of 47 ACC specimens. METHODS: Formalin-fixed paraffin-embedded tissues from 47 ACC specimens and 47 samples of normal salivary gland tissue were evaluated histochemically for the presence of ER, PR and p53. Immunoreactivity was scored using a 0 to +3 scale in which staining was either (0) negative, (+1) spotty, (+2) weakly positive, or (+3) strongly positive. RESULTS: ER was expressed in 8 of 47 tumors while PR was expressed in 4 of 47 tumors. p53 aberrations were demonstrated in 26 of 47 tumors. Tumors showed varying degrees of immunopositivity ranging from 0 to +3. CONCLUSIONS: These studies suggest that p53 aberrations may be involved in ACC tumor progression and that ER and PR may play a role in ACC development.

Nonneural granular cell tumor of the oral cavity: a case report and review of the literature.

Nonneural granular cell tumors (NNGCTs) are rare benign neoplasms originally described in 1991 by Leboit et al. Typical granular cell tumors (GCTs) are commonly encountered in the oral cavity, but NNGCTs, unlike GCTs, are S-100 negative and may display cytologic atypia, allowing for misdiagnosis as a more aggressive lesion. We report a case of a 43-year-old male with a lesion of the mandible that we believe to be the first intraoral example of an NNGCT.

Osteoblastoma of the maxilla and mandible: a report of 24 cases, review of the literature, and discussion of its relationship to osteoid osteoma of the jaws.

Osteoblastoma is a benign neoplasm of bone characterized by a proliferation of osteoblasts forming bone trabeculae set in a vascularized fibrous connective tissue stroma. We report 24 examples of this neoplasm arising in the maxilla and mandible and compare the clinical and radiographic characteristics of this neoplasm to 53 previously reported examples of osteoblastoma and osteoid osteoma in the jaws. Our results reveal more females reported in the new examples of osteoblastoma than in the previously reported examples of osteoblastoma and osteoid osteoma. This raises the overall female percentage from 47.2% to 58.4%. In addition, significantly fewer patients reported pain, tenderness, and discomfort associated with their neoplasms than in previously reported cases. When all cases are combined, a predominant trend is observed whereby osteoblastoma occurs predominantly on the left side of the posterior mandible and is associated with pain, tenderness, and discomfort. We also provide a rationale for use of the term "osteoblastoma" for any benign osteoblastic neoplasm arising in the jaws.

Sporadic sclerotic fibroma of the oral soft tissues.

Sclerotic fibroma (SF) is an uncommon, benign fibrous neoplasm that may present either as a sporadic, small, solitary cutaneous mass, in otherwise healthy individuals, or as solitary or multiple, discrete skin nodules in patients with Cowden syndrome. Oral SF has been reported in patients with Cowden syndrome; however we now report the first documented series of sporadic SF originating within the oral mucosa. We describe 5 cases of SF arising in 3 women and 2 men with an age range of 43 to 66 years. The buccal mucosa was the site of involvement in 4 patients and the lower lip in 1 patient. Microscopically, each of the tumors was characterized by an unencapsulated, well-circumscribed, hypocellular submucosal nodule that was sharply demarcated from the surrounding tissues. The neoplasms were primarily composed of thick collagen bundles that were occasionally arranged in a storiform pattern. Prominent clefts separated many of the collagen bundles. In all cases, spindle and stellate-shaped cells containing fusiform or stellate-shaped nuclei and inconspicuous nucleoli were found scattered throughout the lesion. Occasional stellate-shaped, multinucleated cells were also seen. Many of the cells also exhibited long dendritic cytoplasmic processes. The tumor cells strongly expressed CD34 and vimentin, and occasionally factor XIIIa, but were negative for markers of myofibroblastic, neural or melanocytic differentiation. These findings confirm that oral SF represents a unique entity and should be differentiated from more commonly occurring benign fibrous lesions of the oral soft tissues.

Signet-ring cell (mucin-producing) adenocarcinomas of minor salivary glands.

We report seven cases of minor salivary gland carcinomas characterized by the marked presence of mucin-containing signet-ring cells. These tumors were distinctive in their microscopic appearance and displayed features not seen in any other type of salivary gland malignancy. They typically exhibited invasive growth by narrow parallel strands, randomly scattered small nests, or individually infiltrating cells. Solid, cribriform, or targetoid areas were absent, as well as papillary components. Ductal differentiation was minimal, and seen in only four cases. Degrees of cellularity varied from one area to another. The tumors were cytologically bland. We think that these tumors represent a unique subset of intraoral minor salivary gland carcinomas.

Oral melanoacanthoma: a report of 10 cases, review of the literature, and immunohistochemical analysis for HMB-45 reactivity.

Oral melanoacanthoma (MA) is rare reactive mucosal lesion that, like cutaneous MA, demonstrates hyperplasia of spinous keratinocytes and melanocytes. Unlike MA of the skin, oral MA is unrelated to seborrheic keratosis. This series adds 10 cases to the limited number of previous reports of oral MA. The clinicopathologic features of the cases in this series are generally consistent with those previously reported in the literature; that is, although documented in various intraoral locations in patients of differing ethnicity, oral melanoacanthoma most often presents as an enlarging flat or slightly raised area of hyperpigmentation on the buccal mucosa of adult black women. The current series provides evidence of occurrence over a wider age range (5-77 years) than previously reported. Additionally, the reactivity of oral melanoacanthoma to HMB-45 was investigated. Strong HMB-45 reactivity was present in all cases, thus demonstrating its limited utility in distinguishing oral MA from malignant melanoma.

Intraoral benign mesenchymoma: a report of 10 cases and review of the literature.

A benign mesenchymoma is an unencapsulated soft tissue neoplasm composed of 2 or more mature mesenchymal tissues not normally associated with each other, excluding fibrous connective tissue. No single mesenchymal tissue should predominate with respect to the other mesenchymal elements. Ten well-documented examples of intraoral benign mesenchymoma have been reported in the English language literature. The purpose of this report is to document 10 additional cases and to review the clinicopathologic characteristics of this uncommon tumor.