Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified, in the Oral Cavity.

Lymphomas of the oral cavity are rare and the most frequent type is diffuse large B-cell lymphoma (DLBCL). Epstein-Barr virus (EBV) is known to be associated with the development of different lymphomas. In 2008, the World Health Organization provisionally included the EBV-positive DLBCL of the elderly in the classification of hematopoietic and lymphoid tumors as a lymphoma occurring in older individuals without any known immunodeficiency. However, it has since been recognized that this entity may occur in younger individuals and present similar clinical parameters in both age groups. As a result, the 2017 revision has declined the term elderly and modified it to EBV-positive DLBCL, not otherwise specified (NOS). In this report, we describe a rare case of EBV-positive DLBCL, NOS, presenting as a painless swelling in the oral cavity. This entity shows a more aggressive clinical course than EBV-negative DLBCL, and other lymphoproliferative disorders should be considered in the differential diagnosis.

Rabdomyosarcoma of the Mandible: An Uncommon Clinical Presentation.

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents. Most patients present with a mass in the head and neck region, urogenital region, or with distal extremity involvement. The authors describe a challenging case of a 6-year-old male patient presenting with mandibular RMS. The clinical/radiographic/tomographic evaluations classified the tumor as an advanced stage (stage IV), with a mass of 6.0 cm involving the left side of the mandible and parotid region. The biopsy revealed round, spindled, and pleomorphic cells with hyperchromatic nuclei and rare larger rhabdomyoblasts with eosinophilic cytoplasm. The diagnosis was of embryonal RMS. The patient was referred for treatment with cycles of chemotherapy; however, pulmonary and bone marrow metastasis were identified. Radiotherapy and local surgery with microvascular reconstruction were performed later; however, the patient died after a few months. Early diagnosis is critical for a good prognosis and cure of patients with RMS. Correct diagnosis considering also the histological subtype is important for adequate treatment, which according to the literature is not uniform probably because of the rarity of this neoplasm.

Transition from glass to digital slide microscopy in the teaching of oral pathology in a Brazilian dental school.

OBJECTIVES: Several medical and dental schools have described their experience in the transition from conventional to digital microscopy in the teaching of general pathology and histology disciplines; however, this transitional process has scarcely been reported in the teaching of oral pathology. Therefore, the objective of the current study is to report the transition from conventional glass slide to virtual microscopy in oral pathology teaching, a unique experience in Latin America. STUDY DESIGN: An Aperio ScanScope® scanner was used to digitalize histological slides used in practical lectures of oral pathology. The challenges and benefits observed by the group of Professors from the Piracicaba Dental School (Brazil) are described and a questionnaire to evaluate the students' compliance to this new methodology was applied. RESULTS: An improvement in the classes was described by the Professors who mainly dealt with questions related to pathological changes instead of technical problems; also, a higher interaction with the students was described. The simplicity of the software used and the high quality of the virtual slides, requiring a smaller time to identify microscopic structures, were considered important for a better teaching process. CONCLUSIONS: Virtual microscopy used to teach oral pathology represents a useful educational methodology, with an excellent compliance of the dental students.

Burkitt's lymphoma of the jaws in the Amazon region of Brazil.

OBJECTIVE: To describe the clinicopathologic and immunohistochemical features of Burkitt's lymphoma of the jaws in 7 patients of Northern Brazil. STUDY DESIGN: Clinical data concerning gender, age, affected site, clinical presentation, symptomatology and follow-up were collected from the clinical files. Histopathology was complemented with a broad immunohistochemical panel and in situ hybridization for Epstein-Barr virus (EBV). RESULTS: Most of the patients were infants and 5 out of 7 were males. The mandible was affected in 5 cases and all patients also presented abdominal involvement. All cases were positive for CD45, CD20, CD79a, CD10, Bcl-6 and EBV. Ki-67 proliferative index was approximately 100%. Six patients were treated with R-CHOP (Rituximab + Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone) chemotherapy, and 2 of these died of the disease. One young adult patient refused treatment and died 3 months after initial diagnosis. CONCLUSION: Burkitt's lymphoma of the jaws diagnosed in the Amazon region of Brazil present similar clinicopathologic features to those described in endemic areas of Africa, including EBV positivity.

Reactive post-radiotherapy bone formation in the maxilla.

OBJECTIVE: High-dose radiation therapy affects bone metabolism, and therefore post-radiotherapy bone formation is an uncommon finding. This case describes an unusual ossification in the maxillary region identified after head and neck radiotherapy. RESULTS: A 45-year-old female patient was submitted to maxillary surgical resection and orbital exenteration due to squamous cell carcinoma. Radiotherapy post-surgery was performed because of compromised surgical margins. After 4 months of the conclusion of radiotherapy, the patient presented a bone exposition at surgical site. Surprisingly, the oral examination and computed tomography revealed a new formation of the right palatine vault and bone formation filling the nasal and orbital cavity. The incisional biopsy discarded residual disease or osteoradionecrosis and showed normal tissue. CONCLUSIONS: To the best of the authors' knowledge, this is the first case of wide bone formation as an early effect of head and neck radiotherapy.

Hybrid Neurofibroma/Schwannoma of the Oral Cavity: A Rare Case Report and Literature Review.

Hybrid peripheral nerve sheath tumors show combined features of more than one type of conventional benign peripheral nerve sheath tumors. There are few cases reported of hybrid peripheral nerve sheath tumors in the head and neck region. A 68-year-old female patient was referred for evaluation of an oral swelling lasting five years. Intraoral examination revealed a small mobile nodule located in the lower vestibule. The patient underwent excisional biopsy and microscopic evaluation showed typical features of neurofibroma enclosing areas with palisading nuclei compatible with Antoni A pattern, which are seen in schwannomas. These regions showed strong and diffuse immunoreactivity for S100 protein and moderate positivity in the neurofibroma area. CD34 was positive in the neurofibroma area and entrapped axons were positive for neurofilament. The final diagnosis was oral hybrid neurofibroma-schwannoma tumor. Hybrid peripheral nerve sheath tumors, although extremely rare, may arise within the oral cavity. To the best of our knowledge, this is the first neurofibroma-schwannoma tumor reported in the oral cavity. Recognizing hybrid peripheral nerve sheath tumors as a distinct clinicopathological entity is important because they may also be associated with syndromic disorders.

Contribution of public oral pathology services to the diagnosis of oral and oropharyngeal cancer in Brazil.

This study aimed to evaluate the contribution of oral and maxillofacial pathology laboratories (OMPLs) in Brazilian public universities to the diagnosis of lip, oral cavity, and oropharyngeal squamous cell carcinoma (SCC). A cross-sectional study was performed using biopsy records from a consortium of sixteen public OMPLs from all regions of Brazil (North, Northeast, Central-West, Southeast, and South). Clinical and demographic data of patients diagnosed with lip, oral cavity, and oropharyngeal SCC between 2010 and 2019 were collected from the patients' histopathological records. Of the 120,010 oral and maxillofacial biopsies (2010-2019), 6.9% (8,321 cases) were diagnosed as lip (0.8%, 951 cases), oral cavity (4.9%, 5,971 cases), and oropharyngeal (1.2%, 1,399 cases) SCCs. Most cases were from Brazil's Southeast (64.5%), where six of the OMPLs analyzed are located. The predominant profile of patients with lip and oral cavity SCC was Caucasian men, with a mean age over 60 years, low schooling level, and a previous history of heavy tobacco consumption. In the oropharyngeal group, the majority were non-Caucasian men, with a mean age under 60 years, had a low education level, and were former/current tobacco and alcohol users. According to data from the Brazilian National Cancer Institute, approximately 9.9% of the total lip, oral cavity, and oropharyngeal SCCs reported over the last decade in Brazil may have been diagnosed at the OMPLs included in the current study. Therefore, this data confirms the contribution of public OMPLs with respect to the important diagnostic support they provide to the oral healthcare services extended by the Brazilian Public Health System.

Oncocytic Lipoadenoma: Report of 3 Rare Cases Involving the Parotid Gland, Including a Synchronous Presentation With Paraganglioma of the Right Carotid Bifurcation and Literature Review.

Oncocytic lipoadenoma (OL) is a rare salivary gland tumor characterized by the presence of oncocytic cells and mature adipose tissue. To date, only 30 cases of OL have been reported in the English-language literature. We present 3 additional OL cases involving the parotid, including a synchronous presentation with paraganglioma of the right carotid bifurcation. Microscopically, both the OLs were composed of a mixed population of oncocytes and adipocytes in varying proportions surrounded by a thin, connective tissue fibrous capsule. Oncocytes were positive for pan-cytokeratins (CKs) AE1/AE3, epithelial membrane antigen, CK5, CK7, CK14, CK18, and CK19. Calponin, p63, alpha-smooth muscle actin, and carcinoembryonic antigen were negative. Vimentin and S-100 protein were positive only in adipose cells. Despite distinctive morphologic features, OL is often misdiagnosed, given its rarity. We hope to contribute to surgeons' and pathologists' awareness and knowledge regarding the existence of this tumor and provide adequate management through conservative surgical excision.

The need for communication between clinicians and pathologists in the context of oral and maxillofacial diseases.

Good communication between clinicians and pathologists is a vital element in the diagnostic process, and poor communication can adversely affect patient care. There is a lack of research about communication in diagnostic oral and maxillofacial pathology. This narrative review explores different aspects of the quality of communication between clinicians and oral pathologists, with a focus on the diagnosis of oral and maxillofacial diseases. An electronic search was carried out in MEDLINE through the PubMed, Scopus, and Embase databases up to April 2021. No studies reporting communication, its adequacy or the required skills between clinicians and pathologists in oral diagnosis were found. According to studies published in medicine, strategies for improving communication skills include clinician-pathologist collaboration; a well-formatted, clear and thorough report; training in communication skills; and patient-centered care. Further studies evaluating the current practices and quality in oral and maxillofacial pathology are required to identify barriers and encourage optimal communication to facilitate diagnosis, as well as patient safety.

Oral Amelanotic Melanomas: Clinicopathologic Features of 8 Cases and Review of the Literature.

Mucosal melanomas are aggressive tumors, rarely observed in the oral cavity. The diagnosis is based on the clinical and microscopical features. Often these tumors had variable amounts of melanin pigmentation. However, when melanin is absent, the tumors are denominated amelanotic, presenting a tendency to misdiagnosis and delayed treatment. The aim of this study was to describe the clinicopathologic features of a series of oral amelanotic melanomas (OAM). Records of all cases of OAM were retrospectively retrieved from oral pathology services from January 2002 to January 2019. Data regarding the clinical features, morphological aspects, immunohistochemical reactions, treatment, and follow-up status were collected. Eight cases of OAM were included, 6 in men and 2 in women (ratio of 3:1) ranging in age from 33 to 77 years (mean 53.6 years). Clinically, the tumors presented as masses or ulcerated swellings. The most common intraoral locations of the tumors were gingiva and palate. Cervical lymph node metastasis was detected in 3 patients at the first examination. All but one patient died from complications of the tumors after a mean follow-up period of 8.5 months. In conclusion, OAM is a very aggressive malignant tumor, and when melanin is absent, an immunohistochemical panel comprising S100, melan A, HMB45, and SOX10 should be performed.

Clinicopathologic study of 6 cases of epithelioid osteoblastoma of the jaws with immunoexpression analysis of FOS and FOSB.

OBJECTIVE: The aim of this study was to describe the clinicopathologic features of a series of gnathic epithelioid osteoblastomas. As high levels of Proto-oncogene c-Fos proteins resulting from FOS-FOSB translocation were recently demonstrated in osteoblastomas, we also evaluated the immunoexpression of these proteins. STUDY DESIGN: Records of all cases of epithelioid osteoblastoma of the jaws were retrieved from oral pathology services, and their clinicopathologic and immunohistochemical data were collected. Immunohistochemistry was also performed by using anti-FOS and anti-FOSB antibodies. RESULTS: Six cases of epithelioid osteoblastomas were obtained, 4 in men and 2 in women, and they were mainly located in the posterior body of the mandible (n = 4). Radiographically, the tumors showed mixed radiolucent and radiopaque images, most with poorly defined margins. Microscopically, large epithelioid cells with eccentrically located nuclei predominated among osteoid and immature bone trabeculae. Sharp delineation from adjacent normal bone was observed in all cases. FOS immunostaining was diffuse and strong in the cytoplasm and nucleus of neoplastic cells in all cases, whereas FOSB was only focally positive, with few epithelioid osteoblasts showing nuclear staining. CONCLUSIONS: Although epithelioid osteoblastomas of the jaws are locally aggressive, widespread metastasis does not occur, and, as with conventional osteoblastomas, there is wide expression of the FOS protein.

Glomus Tumor of the Oral Cavity: Report of a Rare Case and Literature Review.

Glomus tumor is a benign neoplasm composed of a perivascular proliferation of glomic cells that resembles the normal glomus body. Usually, it appears as a solitary, symptomatic small blue-red nodule, located in the deep dermis or subcutis of upper or lower extremities of young to middle-aged adults. Cases affecting the oral cavity are very rare, with only 23 well-documented cases reported in the English-language literature. Herein, we present a rare case of glomus tumor of the upper lip, and review the literature of cases involving the mouth.

Leiomyomatous Hamartomas of the Oral Cavity: Clinicopathological and Immunohistochemical Features of 4 Cases and Literature Review.

Objective. To present 4 new cases of oral leiomyomatous hamartomas (OLH), describing its clinical, microscopical, and immunohistochemical features, and a literature review. Methods. The OLH cases were retrieved from the files of 2 Brazilian and 1 Peruvian oral pathology services. Clinical data were obtained from the pathology reports. Microscopical features were reviewed, and immunohistochemical reactions were performed. A review of the English-language literature about OLH was done. Results. The sample was composed of 3 females (4-, 10-, and 15-year-old) and 1 male (3-year-old). Three cases affected the dorsum of the tongue and one the incisive papilla, and all presented sessile or pedunculated smooth nodules. Microscopically, the lesions were composed of varying-size fascicles of smooth muscle fibers in a fibrous connective tissue background. Immunohistochemistry revealed positivity of the smooth muscle bundles for myogenic markers. The English-language literature review revealed 38 OLH cases reported, most were congenital or diagnosed up to 5 years old. The main locations were tongue and anterior maxilla. Conclusions. Although OLH is a rare lesion, it must be considered in the differential diagnosis of midline tongue or maxillary masses in newborns, infants, and youngsters.

Diffuse cutaneous leishmaniasis with oral involvement in a patient of Northern Mexico.

Diffuse cutaneous leishmaniasis is a rare chronic infectious disease, associated with Leishmania mexicana and L. amazonensis, presenting as multiple non-ulcerative painless nodules, with a tendency to relapse soon after treatment. We report a case of a 56-year-old Mexican woman exhibiting nodular lesions, plaques, crusts and scars involving the whole body. A solitary nodule was present at the junction between hard and soft palates. Diffuse cutaneous leishmaniasis is a disfiguring disease resulting in severe scarring if untreated.

Lymphomas affecting the submandibular glands / Linfomas que afectan las glándulas submandibulares

Background: Lymphomas affecting the submandibular glands are very uncommon and few reports are currentlyavailable in the literature. Therefore, the aim of the current study is to describe the clinical and microscopic fea-tures of an original series of lymphomas affecting the submandibular glands.Material and Methods: The pathology files of two institutions were searched for lymphoma cases affecting thesubmandibular glands. The original hematoxylin and eosin, and immunohistochemical slides were revised by apathologist for diagnosis confirmation following the revised 4th edition of the World Health Organization classification of tumours of haematopoietic and lymphoid tissues. Clinical data regarding age, sex, clinical manifestation,treatment, follow-up and status at last appointment were retrieved from the patients’ medical charts.Results: During the period investigated, 16 cases were included in the study. Females predominated (10:6) with amean age of 57.8 years-old. Tumors usually presented as asymptomatic swellings. MALT lymphoma representedthe most common subtype, followed by diffuse large B cell lymphoma and follicular lymphoma. Three patientsdied, one of them affected by plasmablastic lymphoma, one by DLBCL and one by MALT lymphoma.Conclusions: Low-grade B cell lymphomas predominate in the submandibular glands, but DLBCL and other sub-types may also be rarely diagnosed in this salivary gland.(AU)

Superficial mucoceles in cancer patients: a retrospective series from a Stomatology unit

Background: The aim of this study is to relate all the superficial mucoceles found in a cancer center, described the association with oncological conditions, and discuss its etiology and pathology that we found in the past few years. Material and Methods: Sixteen cases of superficial mucocele were retrieved from the patients’ records of the Stomatology Department of the A. C. Camargo Cancer Center, São Paulo, Brazil, and demographic and clinical data were collected from electronic medical records. Results: There were 16 patients, 8 patients were men and 8 women, with ages varying from 26 to 70 years old. Superficial mucoceles were observed in patients submitted to head and neck radiotherapy (n=6), graft versus host disease (n=4), one associated with oral mucositis related to allogenic bone marrow stem cells transplantation (n=1), systemic lupus (n=1), Sjögren’s syndrome (n=1), oral lichenoid lesion associated with pembrolizumab (n=1) and no local or systemic inflammatory associated found (n=2).Conclusions: This study reports a series of superficial mucoceles from a single stomatology unit. Most patients had superficial mucoceles secondary to head and neck radiotherapy and graft versus host diseases. However, two patients (12.5%) had mucoceles related to systemic inflammatory conditions (Sjögren’s Syndrome and Systemic Lupus). (AU)

Contribution of public oral pathology services to the diagnosis of oral and oropharyngeal cancer in Brazil

Abstract This study aimed to evaluate the contribution of oral and maxillofacial pathology laboratories (OMPLs) in Brazilian public universities to the diagnosis of lip, oral cavity, and oropharyngeal squamous cell carcinoma (SCC). A cross-sectional study was performed using biopsy records from a consortium of sixteen public OMPLs from all regions of Brazil (North, Northeast, Central-West, Southeast, and South). Clinical and demographic data of patients diagnosed with lip, oral cavity, and oropharyngeal SCC between 2010 and 2019 were collected from the patients' histopathological records. Of the 120,010 oral and maxillofacial biopsies (2010-2019), 6.9% (8,321 cases) were diagnosed as lip (0.8%, 951 cases), oral cavity (4.9%, 5,971 cases), and oropharyngeal (1.2%, 1,399 cases) SCCs. Most cases were from Brazil's Southeast (64.5%), where six of the OMPLs analyzed are located. The predominant profile of patients with lip and oral cavity SCC was Caucasian men, with a mean age over 60 years, low schooling level, and a previous history of heavy tobacco consumption. In the oropharyngeal group, the majority were non-Caucasian men, with a mean age under 60 years, had a low education level, and were former/current tobacco and alcohol users. According to data from the Brazilian National Cancer Institute, approximately 9.9% of the total lip, oral cavity, and oropharyngeal SCCs reported over the last decade in Brazil may have been diagnosed at the OMPLs included in the current study. Therefore, this data confirms the contribution of public OMPLs with respect to the important diagnostic support they provide to the oral healthcare services extended by the Brazilian Public Health System.

Chondroid Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Rare Case Report.

Tenosynovial giant cell tumor of diffuse type (TGCT-d) or pigmented villonodular synovitis (PVNS) is a locally aggressive lesion that mostly affects the joints of long bones. Chondroid tenosynovial giant cell tumor (CTGCT) or PVNS with chondroid metaplasia is a rare distinct subset of synovial tumors that has a predilection for the TMJ. We report a rare case of CTGCT in the TMJ, initially misdiagnosed as temporomandibular disorder (TMD). A 51-year-old woman was referred to the surgeon with the chief complaint of TMJ pain for 5 years and a past history of an unsuccessful TMD treatment. Extraoral examination revealed discrete preauricular swelling and restricted mandibular range of motion. Panoramic radiograph and computerized tomography showed destruction of the mandibular fossa and condyle. Histologically, the tumor was composed by large mononuclear cells with prominent eosinophilic cytoplasm and grooved nuclei, small histiocytoid cells, osteoclast-like multinucleated cells, brown pigmentation and areas of chondroid metaplasia. Morphological and immunohistochemical characteristics lead to the final diagnosis of CTGCT. The rarity of CTGCT could be attributed to the lack of recognition of this lesion, with cases diagnosed as chondroblastomas, synovial chodromatosis and chondrosarcoma. The patient received immediate reconstruction and recurrence was found 22 months after initial intervention. TGCT-d and CTGCT of the TMJ can present similar symptoms to TMD, but clinicians must distinguish both lesions by complete examination, imaging and, when necessary, histopathologic evaluation.

Primary oral melanoma: A clinicopathologic review and case presentation.

OBJECTIVE: The purpose of this manuscript is to present a clinicopathologic review of the literature concerning all the detailed cases of primary oral melanoma (OM) that were confirmed by immunohistochemistry. In addition, a pertinent case presentation is addressed. DATA SOURCES: An extensive electronic search of the literature was performed using PubMed/Medline from 1953 to 2017. Relevant articles were selected based on specific inclusion criteria. Statistical analyses were conducted by the Shapiro-Wilk, Fisher's exact, chi-square, and Z tests (α = .05). RESULTS: Forty-nine cases of primary OM reported in the literature plus the current case were analyzed; patient ages ranged from 17 to 89 years with a mean of 60.8 years; male to female ratio was 1.5:1; Caucasian patients were the most affected and the most frequent locations were maxillary alveolar mucosa and palate; the majority of the lesions were pigmented (62%); differences among the proportions of positivity to S-100, HMB-45, Melan-A, NKFC3, vimentin, tyrosinase, CK, microphthalmia transcription factor (MITF), and Ki-67 were found (P < .0001), especially when comparing with vimentin (P < .05) and CK (P < .01); recurrence was reported in 11.6% and mortality in 54.8%. The case presented is of a 71-year-old mixed-race woman who presented multiple pigmented lesions over the maxillary alveolar mucosa and palate. Positivity for S-100, HMB-45, Melan-A, and Ki-67 confirmed the diagnosis of primary OM. CONCLUSION: Primary OM is rare and very aggressive, with only 49 detailed cases confirmed by immunohistochemistry existing within the English literature, in addition to the present case. S-100 and HMB-45 are excellent markers to confirm the diagnosis of primary OM, although the use of adjuvant specific markers such as Melan-A, tyrosinase, and MITF should be also encouraged.

Prognostic significance of hMSH2, hMSH3, and hMSH6 expression in ameloblastoma.

OBJECTIVE: The aim of this study was to investigate hMutS proteins in developing human tooth, ameloblastomas, and ameloblastic carcinoma and to determine whether the expression of these proteins has any prognostic potential. STUDY DESIGN: Ten cases of developing human tooth, 39 ameloblastomas, and 2 ameloblastic carcinomas were used to determine the distribution of the proteins during the process of carcinogenesis. Simultaneously, another sample of 73 ameloblastomas was arranged in tissue microarray, and their clinical, microscopic, and radiographic features; treatment outcome; presence of BRAF-V600E mutation; and follow-up data were assessed to determine the prognostic relevance of the expression of hMutS (hMSH2, hMSH3, hMSH6) and Ki-67. hMSH2 and hMSH6 were significantly downexpressed in ameloblastomas (P = .0059) compared with developing human tooth (P < .0001). RESULTS: hMSH2, hMSH3 expression were significantly associated with BRAF-V600E mutation (P < .05). Simultaneous overexpression of hMutS was associated with recurrence (P = .035); however, these did not predict the disease-free survival of patients (P > .05). CONCLUSIONS: hMutS proteins are downregulated in ameloblastoma; moreover, simultaneous overexpression of these proteins in ameloblastoma was associated with recurrence but did not predict disease-free survival.