Oral leiomyomatous hamartoma: presentation of 3 cases and review of the literature.

Oral leiomyomatous hamartoma (OLH) is a rare lesion, with only 40 cases reported in the literature. It typically presents early in life as a nodule on the anterior maxillary alveolar tissues or the tongue. Its growth potential is limited, with few cases reaching dimensions >2.0 cm, and its microscopic composition includes an intact surface mucosa with an underlying fibrovascular stroma possessing an unencapsulated proliferation of smooth muscle fascicles. Excision is considered the definitive treatment. Here we describe the clinical, microscopic, histochemical, and immunohistochemical features and management of 3 cases of OLH and review the literature. The findings we present here can assist in performing differential diagnosis, particularly in discriminating between OLH and similar yet non-hamartomatous processes and in selecting appropriate management.

Tongue Mucoceles: a retrospective clinic-pathological evaluation of 240 cases.

BACKGROUND: Minor salivary glands can be found in the ventral and anterior part of the tongue; these glands can rarely develop mucoceles that, due to their rarity and their unusual clinical appearance, may present an interesting differential diagnosis. Mucoceles appear as an exophytic, sometimes pedunculated, lesion, which is a feature that is due to the absence of a capsule; thus, the glands are right beneath the mucosa and over the muscle tissue. The aim of this article is to retrospectively present and discuss the anatomy, pathology, clinical features and therapy of several cases of Blandin-Nunh mucoceles collected from two different institutions. METHODS: A retrospective case review was carried out in two university institutions, retrieving all cases of tongue mucoceles from 1999 to today. Two oral pathologists reviewed all the slides, confirming the diagnosis. Demographic data of the patient, anatomic location and clinical appearance were retrieved from clinical charts, together with the type of surgical procedure and possible relapses. RESULTS: A total of 240 cases of tongue mucoceles were gathered from the archives: the mean age was 22 years (DS = 14,7; Range 2-83), 126 were females (52,5%, mean age 22,7 years, DS = 16,5; Range 2-83), and 114 were males (47,5%, mean age 20,9 years, DS = 12,4; Range 3-73); in all cases, a history of trauma was reported. The ventral surface was the most frequent location (224 cases - 93,3%), and in the great majority (235 cases - 97,9%), pathology revealed mucous spillage with a wall formed by fibrous connective and granulation tissue with no epithelium lining the cavity. Superficial mucocele and sclerosing sialoadenitis were the more frequent pathological variants (21 cases - 8,8%). All lesions were treated with excision and enucleation of the servicing gland. The healing was uneventful in all cases, but there were four recurrences and two cases of sensory paraesthesia of the border of the tongue, all in males, except one case of paraesthesia in a female. CONCLUSIONS: Tongue mucoceles must be differentiated from many benign and malignant lesions. For this reason, surgical removal of the lesion and of the associated gland with a pathological exam is mandatory. In fact, the anatomical location of the glands and the possible pathological variants must be considered to reach a correct diagnosis and diminish possible relapses. TRIAL REGISTRATION: CE-Muc_Ton_3/2023.

Osteoradionecrosis versus Cancer Recurrence: An Unresolved Clinical Dilemma.

INTRODUCTION: Osteoradionecrosis is a rare and debilitating risk of definitive chemoradiotherapy for head and neck squamous cell carcinoma. It is difficult to distinguish between osteoradionecrosis and recurrent or progressive disease, as clinical and radiologic features may be similar. Our aim was to compare the clinical presentation and radiologic features of osteonecrosis with those of recurrent or progressive cancer. METHODS: We conducted a single-center case series of 19 patients with head and neck squamous cell carcinoma diagnosed between 2011 and 2019 who subsequently developed clinical and/or radiological suspicion of osteoradionecrosis. The population was a referred sample from head and neck cancer physicians at Northwell Health Cancer Institute. Clinician notes and imaging reports were reviewed to assign a final diagnosis of either cancer, osteonecrosis, or indeterminate. RESULTS: No differences were found in the clinical presentation or radiologic features between groups. Median time between treatment and development of symptoms was longer in patients with a final diagnosis of osteoradionecrosis than recurrent or progressive disease (5 vs. 3 months), but this difference was not statistically significant. Radiation dose and type were not associated with diagnosis. Mean standard uptake value maximums on positron emission tomography/computed tomography were significantly higher in the cancer group (median 14.8 vs. 9.1, p < 0.0152). At 1 year after first suspicion of osteoradionecrosis, 100% of osteoradionecrosis patients were alive, versus 28.6% of cancer patients. DISCUSSION/CONCLUSION: There is significant overlap in clinical and radiologic features of osteoradionecrosis and cancer. Standard uptake maximums may be helpful in predicting diagnosis. Occurrence of symptoms within 6 months of completing chemoradiotherapy should raise the concern for malignancy.

Oral Manifestations of Syphilis: a Review of the Clinical and Histopathologic Characteristics of a Reemerging Entity with Report of 19 New Cases.

BACKGROUND: Syphilis is a sexually-transmitted infectious disease caused by Treponema pallidum. Cases of primary and secondary syphilis are on the rise in the United States, with a 14.4% increase in new cases noted from 2017 to 2018 and an escalation of 71% between the years 2014 and 2018. Fulfilling its nickname of "the great imitator," oral manifestations of syphilis may mimic a variety of infectious, neoplastic, or immune-mediated processes, both clinically and histopathologically. This large spectrum of appearances can create a diagnostic challenge to the clinician and/or pathologist, leading to delay in diagnosis or misdiagnosis. METHODS: A database of oral syphilis cases was created from archives at the University of Kentucky, University of Pittsburgh, LIJMC, Columbia University MC, and University of Tennessee. The age, sex, race, location, duration, and clinical description were recorded. Cases without positive reaction upon immunohistochemistry or serologic tests were excluded. RESULTS: We identified 19 new cases of oral syphilis (17 males, one female, and one case unknown sex) and described the clinical and histopathological features of this re-emerging and potentially fatal disease. All cases demonstrated dense lymphoplasmacytic inflammation, often with inflammatory exocytosis or ulceration at the surface, and perivascular inflammation. CONCLUSIONS: Early recognition of the histopathologic and clinical manifestations of oral syphilis is imperative for prompt diagnosis, improved patient outcomes, and disease prevention.

"Ossifying" mucoepidermoid carcinoma: A deceptive clinical presentation.

Mucoepidermoid carcinoma is the most common salivary gland malignancy, accounting for 27% of all salivary gland cancers. Identified in 1921 and first analyzed in 1945, mucoepidermoid carcinoma has demonstrated a widely diverse histology with several morphologic variants having been described. One rare feature is the formation of intratumoral bone, which has been previously reported once in the English language literature. Though the etiology of these calcifications is still not known, it is believed that this finding is independent of overall disease prognosis. This case report illustrates this unusual feature in a 48-year-old Hispanic woman who initially presented with a floor of mouth swelling. Computed tomography examination subsequently revealed a soft tissue mass with intralesional radiopacities. Despite its relative rarity, it is important for practitioners to be aware of this unique presentation in that it may help to avoid misdiagnosis and delays in treatment.

Identifying patterns of failure and secondary primary malignancies in HPV-related oropharyngeal squamous cell carcinomas.

Aim: To compare patterns and rates of recurrence in patients with oropharyngeal squamous cell carcinoma by human papilloma virus (HPV) status. Patients & methods: Retrospective chart review of 155 patients diagnosed with oropharyngeal squamous cell carcinoma between 2012 and 2014 at a single center. Results: Two-year recurrence-free survival was higher in patients with HPV-positive tumors compared with negative (85.2% [standard error = 0.03] versus 59.3% [standard error = 0.09]; p < .001) with the former proportionally less likely to have locoregional recurrence. HPV-positive patients had proportionally higher incidence of second primary malignancies outside of head, neck and lung compared with HPV-negative (74.2 vs 37.5%; p = 0.09). Conclusion: The differences in failure by HPV status indicates a need for modified surveillance guidelines. The differences in second primary malignancies patterns are interesting, warranting further evaluation in larger studies.

USP6 Gene Rearrangement by FISH Analysis in Cranial Fasciitis: A Report of Three Cases.

Cranial fasciitis (CF) is an uncommon benign myofibroblastic proliferation involving the soft and hard tissues of the cranium. It typically occurs in the pediatric population with a male predilection (male-to-female ratio 1.5:1). The clinical presentation is usually a rapidly expanding, painless nodule. Bone erosion may be appreciated radiographically. Histopathologic sections of CF show plump, fibroblast-like cells with pale, oval shaped nuclei and prominent nucleoli in a fibrous or myxoid background. Growth is self-limited and surgical excision is considered curative. Due to these features, CF is thought to be a variant of nodular fasciitis (NF). As with NF, CF may mimic a sarcomatous process and pose a diagnostic challenge to clinicians and pathologists alike. Erickson-Johnson et al. identified rearrangements of the ubiquitin-specific protease 6 (USP6) gene in 44 of 48 cases of NF. MYH9 was the fusion partner in 12 of these cases. To date, the molecular profile of CF has not been studied. Here we present the molecular findings in three cases of CF identified at our institution. Each case was subjected to fluorescence in-situ hybridization with appropriate negative controls. Two of three cases were positive for the USP6 gene rearrangement. The third case failed to hybridize, likely related to nucleic acid damage secondary to decalcification. Negative controls did not demonstrate the genetic rearrangement. These findings warrant further investigation of the USP6 gene rearrangement in CF, as it may prove helpful as a diagnostic adjunct in challenging cases.

Diagnosis of oral lichen planus: a position paper of the American Academy of Oral and Maxillofacial Pathology.

Despite being one of the most common oral mucosal diseases and recognized as early as 1866, oral lichen planus (OLP) is still a disease without a clear etiology or pathogenesis, and with uncertain premalignant potential. More research is urgently needed; however, the research material must be based on an accurate diagnosis. Accurate identification of OLP is often challenging, mandating inclusion of clinico-pathological correlation in the diagnostic process. This article summarizes current knowledge regarding OLP, discusses the challenges of making an accurate diagnosis, and proposes a new set of diagnostic criteria upon which to base future research studies. A checklist is also recommended for clinicians to provide specific information to pathologists when submitting biopsy material. The diagnostic process of OLP requires continued clinical follow-up after initial biopsy, because OLP mimics can manifest, necessitating an additional biopsy for direct immunofluorescence study and/or histopathological evaluation in order to reach a final diagnosis.

Congenital granular cell lesion of the tongue: a report of two cases and review of the literature.

The congenital granular cell lesion most commonly occurs on the maxillary or mandibular alveolus of neonates. Extra-alveolar congenital granular cell lesion is exceptionally rare, with only 10 cases reported. Two additional cases occurring on the tongue are presented with a description of the clinical, histopathologic, and immunohistochemical features. The differential diagnosis is discussed, and the literature reviewed.

The Role of Antiangiogenic Therapy in the Development of Osteonecrosis of the Jaw.

There is an increasing use of established and newer medications that have antiangiogenic properties. Inhibition of angiogenesis likely has either a primary or secondary role in the development of osteonecrosis of the jaw (ONJ). These medications are being used in the treatment of various cancers and in the treatment of several non-oncologic conditions. Antiangiogenic medications when used in combination with antiresorptive medications, such as nitrogen-containing bisphosphonates or denosumab, seem to increase the likelihood of osteonecrosis of the jaw. This review highlights the role of inhibitors of angiogenesis and their role in the development of osteonecrosis of the jaws.

Giant Cell Tumor of the Larynx Treated by Surgery and Adjuvant Denosumab: Case Report and Review of the Literature.

Giant cell tumor of the larynx (GCTL) is a rare entity; only 34 cases have been reported in the literature. We report a case of GCTL in a 46 year-old male presenting clinical, radiographic, histological and therapeutic features. Previously reported cases are also reviewed.

American Association of Oral and Maxillofacial Surgeons position paper on medication-related osteonecrosis of the jaw--2014 update.

Strategies for management of patients with, or at risk for, medication-related osteonecrosis of the jaw (MRONJ) were set forth in the American Association of Oral and Maxillofacial Surgeons (AAOMS) position papers in 2007 and 2009. The position papers were developed by a special committee appointed by the board and composed of clinicians with extensive experience in caring for these patients and basic science researchers. The knowledge base and experience in addressing MRONJ has expanded, necessitating modifications and refinements to the previous position paper. This special committee met in September 2013 to appraise the current literature and revise the guidelines as indicated to reflect current knowledge in this field. This update contains revisions to diagnosis, staging, and management strategies and highlights current research status. The AAOMS considers it vitally important that this information be disseminated to other relevant health care professionals and organizations.

EWSR1 and ATF1 rearrangements in clear cell odontogenic carcinoma: presentation of a case.

Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor of the jaws that is more common in the mandible than maxilla and has a female preponderance with a peak incidence in the sixth decade. It is characterized by locally aggressive behavior and has the potential to metastasize. This tumor was recently reported to have a rearrangement of the Ewing sarcoma breakpoint region 1 gene (EWS RNA-binding protein 1, EWSR1) in 5 of 8 cases tested and of the activating transcription factor 1 gene (ATF1) in 1 case tested. We report a case of CCOC in the premolar area of the mandible in a 59-year-old woman. This case demonstrated the presence of both EWSR1 and ATF1 gene rearrangements by fluorescence in situ hybridization.

Laryngeal myxoma: a case report and review of the literature.

Myxomas are a rare benign neoplasm of uncertain mesenchymal cell origin, typically involving the heart. Laryngeal myxomas are uncommon, and are usually misdiagnosed as laryngeal polyp. To the best of our knowledge, there are only nine reported cases in the English literature. We report a case of a laryngeal myxoma presenting clinically as a left vocal cord polyp in a 77 year old male, and review the literature related to this rare entity.

Calcifying aponeurotic fibroma with bone islands exhibiting hematopoiesis: a case report and review of the literature.

Aponeurotic fibroma (AF) was originally described by Keasbey in 1953 as juvenile aponeurotic fibroma, most commonly occurring in the distal extremities. Initially described in children and adolescents, AF is now recognized to occur over a wide age range and at various anatomic sites. A variant of this lesion, termed calcifying aponeurotic fibroma (CAF) has been described. CAF is a slow-growing, solitary, and painless nodule often adherent to tendon, fascia, or periosteum. We report a patient who presented with a firm lobulated mass, portions of which were fixed to the left ascending mandibular ramus. The lesion represented a CAF arising juxtacortical to the mandible, with the calcified component composed of mature bone exhibiting focal areas of hematopoiesis. Additional reports of CAF occurring in the head and neck region are reviewed.

Labial salivary gland involvement in neonatal hemochromatosis: a report of 2 cases and review of literature.

Neonatal hemochromatosis (NH) is a severe disease of fetal or perinatal onset, in which iron deposition occurs within hepatic and extrahepatic sites without involving the reticuloendothelial system. Labial minor salivary gland biopsy has been suggested as a diagnostic adjunct in patients suspected of having NH, as hemosiderin accumulates in acinar epithelial cells. Prior to this salivary gland pathology, a diagnosis of NH was often delayed, rendered only after the usual causes of neonatal liver failure had been excluded. Recent studies have shown that early diagnosis and treatment can improve survival. Few cases of salivary gland hemosiderosis in NH have been reported in the literature. A positive finding of salivary gland siderosis on biopsy will expedite care. We report 2 cases of NH, of which a labial salivary gland biopsy supported the diagnosis. The clinical and histological features are presented. The NH literature pertaining to labial salivary gland pathology is reviewed.

Review of long-term adverse effects associated with the use of chemically-modified animal and nonanimal source hyaluronic acid dermal fillers.

Although only recently introduced, chemically-modified hyaluronic acid dermal fillers have gained widespread acceptance as "redefining" dermal fillers in the fields of dermatology and cosmetic facial surgery. Although hyaluronic acid-based dermal fillers have a low overall incidence of long-term side effects, occasional adverse outcomes, ranging from chronic lymphoplasmacytic inflammatory reactions to classic foreign body-type granulomatous reactions have been documented. These long-term adverse events are reviewed.