Conservative management of multiple odontogenic keratocysts in a child with nevoid basal cell carcinoma syndrome: A case report.

BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant multisystemic disorder characterized by the presence of multiple odontogenic keratocysts (OKC), which are a hallmark feature of the syndrome. The treatment of these OKC poses challenges due to their high recurrence rates and the myriad of management options available. CASE REPORT: We describe here a case of NBCCS diagnosed in an 11-year-old girl who presented with multiple OKC in the jaws. Chest and cranial radiographs showed no abnormalities in the ribs and the cerebral falx, respectively. Cephalometric analysis indicated mandibular retrusion, a skeletal class II relationship, and a convex profile. The treatment approach involved a personalized strategy tailored for each cyst, comprising marsupialization followed by enucleation. This approach aimed to minimize surgical trauma and to reduce the risk of recurrence. The patient underwent regular follow-up appointments, demonstrating successful outcomes with no signs of recurrence or de novo OKC observed over a 32-month period. CONCLUSION: Clinicians should consider lesion characteristics and patient cooperation when determining treatment strategies for the optimization of outcomes for children and adolescents with NBCCS and multiple OKC.

Gingival metastasis of angiosarcoma of the breast as a first manifestation of spreading disease: Case report and review of the literature.

BACKGROUND: Angiosarcoma is an aggressive malignant neoplasm of vascular origin. Oral metastases of angiosarcoma are rare and have a non-specific clinical presentation, thus the diagnosis may be challenging. CASE REPORT: Herein we report a case of a 34-year-old female patient after treatment of a high-grade angiosarcoma of the breast, who presented an asymptomatic bleeding purplish nodule in the maxillary interdental papilla between the first and second premolar. A biopsy was performed, and the histological examination revealed infiltration by malignant neoplasm of epithelioid and fusocellular pattern. Immunohistochemical analysis demonstrated that neoplastic cells were positive for ERG and CD31, and negative for cytokeratins AE1/AE3, confirming the diagnosis of metastatic angiosarcoma. After investigation, multiple metastases were discovered. The patient is under management with chemotherapy and palliative radiotherapy for the bone lesions. CONCLUSION: Metastases should be considered in the differential diagnosis of oral lesions in patients with a previous history of cancer. Due to the morphology of angiosarcomas, the metastatic lesions may resemble benign vascular lesions, therefore, biopsy is mandatory to exclude malignancy.

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)

Drug-induced sarcoidosis-like reaction to adalimumab in the oral mucosa of a patient with Crohn's Disease.

BACKGROUND: The drug-induced sarcoidosis-like reaction (DISR) is a condition clinically and pathologically similar to sarcoidosis but is induced by certain drugs. A few cases of DISR associated with the use of TNF-α antagonists have been reported in the literature. CASE REPORT: A 49-year-old female patient with a diagnosis of Crohn's Disease under treatment with adalimumab presented with a 2-month-long ulcerated swelling in the left lower fornix. Histological analysis of the biopsy specimen revealed multiple non-caseating granulomas multinucleated cells and epithelioid macrophages surrounded by lymphocytes. The lesion is under symptomatic control with a topical corticosteroid, and the patient is being monitored for manifestation in other organs and systems. CONCLUSION: Lesions of DISR may occur isolated in the oral mucosa. Therefore, this complication must be considered in the differential diagnosis of oral granulomatous lesions in patients under treatment with anti-TNF-α drugs.

Influence of different methods for classification of lymph node metastases on the survival of patients with oral squamous cell carcinoma.

BACKGROUND: Despite the advances in the classification of oral squamous cell carcinoma (OSCC) based on its extension by the TNM system, there is still a need for methods to better classify the patients to predict prognosis and indicate adjuvant therapy. OBJECTIVES: To analyze the influence of the number of positive lymph nodes (PN), lymph node ratio (LNR), and log odds of positive lymph nodes (LODDS) in survival of patients with OSCC. METHODS: Clinicopathologic data from patients with OSCC who were treated with curative purposes by surgery and neck dissection (ND) with or without subsequent adjuvant therapies from 1991 to 2015 was retrospectively assessed. The impact of the PN, LNR, LODDS, and other variables on overall survival (OS) and disease-free survival (DFS) was analyzed in univariate and multivariate analyses. RESULTS: One hundred nineteen patients were included in this study. In the univariate analysis the PN had a significant impact on OS (p = 0.001) and DFS (p = 0.020), and the LNR had a significant impact on the OS (p = 0.042). In the multivariate analysis with other relevant clinicopathologic variables, the PN was the only significantly independent factor influencing in the OS (p = 0.017) but not in DFS (p = 0.096). CONCLUSIONS: The PN is an independent prognostic indicator for OS and DFS in patients with OSCC and has the potential to aggregate the current AJCC classification. The LNR has potential to be an important prognostic indicator, but the methods for this classification require lapidation. The LODDS did not demonstrate prognostic potential.

Mucosal Leishmaniasis of the lip: Report of an Exuberant case in a Young man.

BACKGROUND: Leishmaniasis is a tropical disease caused by protozoan parasites of the genus Leishmania. Mucosal leishmaniasis has been described as secondary to the cutaneous form; however, isolated mucosal involvement can also occur. Specifically, mucosal leishmaniasis of the lip is poorly described and its diagnosis challenges clinicians. METHODS: We herein report a case of mucosal leishmaniasis affecting the lower lip without cutaneous involvement in a 20-year-old Venezuelan man. The patient had no relevant past medical history. Clinically, a mass-like lesion with ulcerations and crusts was observed. RESULTS: Microscopically, the lesion was composed of granulomatous inflammation along with macrophages containing intracytoplasmic inclusions similar to round-shaped Leishmania. The species Leishmania (Viannia) braziliensis was confirmed. Treatment with meglumine antimonate was effective. The lesion healed satisfactorily, and no side effects or recurrences were observed. CONCLUSION: Clinicians should be aware of isolated forms of mucosal leishmaniasis of the lip, even in cases where the cutaneous lesion is undetected or clinically manifests as self-limiting. Knowing the endemic areas in the scenario of the dynamics of the ecoepidemiology of leishmaniasis is also essential for surveillance and counselling of the population.

Potential of lymph-node ratio as a prognostic factor for patients with oral squamous cell carcinoma.

BACKGROUND: To analyze the influence of the lymph node ratio (LNR) in survival of patients with OSCC METHODS: Clinicopathologic data from patients with OSCC who were treated with curative surgery and neck dissection (ND) with or without adjuvant therapies from 1991 to 2015 was retrospectively assessed. The impact of LNR and other variables on overall survival (OS) and disease-free survival (DFS) was analyzed in univariate and multivariate analyses. RESULTS: One hundred nineteen patients were included. In the univariate analysis the LNR had a significant impact on OS (p = 0.01) and DFS (p = 0.01). In the multivariate analysis, the LNR was the only significantly independent factor influencing in the OS (p = 0.03). The adjuvant therapies did not influence on the OS (p = 0.42) and DFS (p = 0.10). CONCLUSIONS: The LNR is an independent prognostic factor in patients with OSCC. The LNR alone is not recommended to indicate the performance of adjuvant therapies.

Garlic burn trauma of the oral mucosa in a patient with trigeminal neuralgia: A case report.

Garlic is one of the most common medicinal plants used since ancient times to treat diverse diseases within several cultures worldwide. Nevertheless, this plant has the potential to cause chemical burns of the skin and mucosa. This report presents a case of a garlic burn in the palate of a 57-year-old woman who applied garlic to the palate to relieve the pain of trigeminal neuralgia. This case demonstrates the potential of garlic to cause chemical burns to the oral mucosa and raises an alert to oral health professionals regarding inappropriate self-treatment methods.

An extensive intraoral blue nevus in an elder patient.

OBJECTIVE: To report a rare case of intraoral extensive blue nevus in an elder patient. BACKGROUND: Oral blue nevi is a well-recognised skin melanocytic neoplasm that rarely occurs in the oral cavity and may mimic melanoma in the early stages. METHODS: An incisional biopsy was performed, and the diagnosis was blue nevus. CONCLUSION: Both the clinician and pathologist must be aware of such a presentation to diagnose and treat appropriately.

Primary intraosseous malignant peripheral nerve sheath tumor of the mandible: An unusual presentation mimicking a benign lesion.

The malignant peripheral nerve sheath tumor (MPNST) is a rare, aggressive malignant tumor that usually develops in the context of neurofibromatosis type 1. In the oral cavity, these tumors are excelling rare, especially in intraosseous sites. Herein, we report an unusual presentation of intraosseous MPNST affecting the mandible posterior region in a 28-year-old male without neurofibromatosis type 1 discovered as an incidental find on imaging exam. CT scan evaluation showed a solitary, well-defined, round hypodense lesion in the posterior mandibular region extending from tooth 45 to 46. Microscopic evaluation showed a tumor composed of atypical spindle-shaped cells arranged in fascicles and a storiform pattern. Tumor cells were positive for S-100 protein. Epithelial membrane antigen (EMA), pan-cytokeratin AE1/AE3, desmin, alpha-smooth muscle actin (α-SMA), HMB-45, MART-1, MUC4, and CD56 were negative. The diagnosis was low-grade MPNST. The patient underwent wide surgical resection of the tumor. After three years of follow-up, the patient remained with no evidence of recurrence or metastatic disease. When an intraosseous neurogenic tumor is suspected based on radiological characteristics, despite the apparent benign nature, an incisional biopsy is mandatory to rule out malignancy before treatment planning to avoid inadequate conservative treatment.

Fat-free pleomorphic lipoma of the buccal mucosa: An immunohistochemical study and literature review.

Pleomorphic lipomas are extremely rare in the oral cavity. Due to the significant overlap of morphological findings with several benign and malignant soft tissue tumors, especially in the absence of adipocytes, the diagnosis is challenging. We reported the clinicopathological and immunohistochemical features of an uncommon case of a fat-free variant of pleomorphic lipoma in a 48-year-old female presenting clinically as a painless nodule on the buccal mucosa. Microscopically, the lesion showed atypical spindle cells, numerous floret-like giant multinucleated cells, and abundant ropey collagen fibers bundles. Immunohistochemistry showed strong positivity for vimentin and CD34. Mast cell tryptase highlighted numerous mast cells distributed throughout all tumor stroma. S-100 protein, pan-cytokeratin, desmin, α-SMA, EMA, CD68, STAT6, Bcl-2, MDM2, and CDK4 were negative. Conservative surgical excision was carried out, and no recurrence was observed after 13 months of follow-up. Careful histopathological and immunohistochemistry analysis of these lesions is recommended to ensure the correct diagnosis and provide adequate management through a conservative surgical approach. To the best of our knowledge, this is the second case of fat-free pleomorphic lipoma in the oral cavity.

Survival analysis of different methods for classification of lymph node metastases in patients with oral squamous cell carcinoma / Análise de sobrevida com diferentes métodos de classificação de metástases em linfonodos em pacientes com carcinoma espinocelular oral

Introdução: Apesar dos avanços no estadiamento clínico do carcinoma espinocelular (CEC) oral a partir de sua extensão pelo sistema TNM, ainda há necessidade de métodos que classifiquem melhor a doença para predizer o prognóstico e indicar terapia adjuvante. Objetivos: Analisar a influência do número de linfonodos positivos (PN), razão de linfonodos (LNR) e probabilidade log de linfonodos positivos (LODDS) na sobrevida de pacientes com CEC de boca. Pacientes e Métodos: Dados clínico-patológicos de pacientes com CEC de boca tratados com fins curativos por cirurgia e esvaziamento cervical (ND) associado ou não a terapias adjuvantes de 1991 a 2015 foram avaliados retrospectivamente. O impacto do PN, LNR, LODDS e outras variáveis na sobrevida global (OS) e sobrevida livre de doença (DFS) foi avaliado por meio de análises univariada e multivariada. Resultados: Cento e dezenove pacientes foram incluídos neste estudo. Na análise univariada, o PN teve um impacto significativo na OS (p = 0,001) e DFS (p = 0,020), e o LNR teve um impacto estatisticamente significante na OS (p = 0,042). Na análise multivariada com outras variáveis clínicopatológicas relevantes, o PN foi o único fator significativamente independente com influência na OS (p = 0,017), mas não na DFS (p = 0,096). Conclusões: O estudo sugere que a NP é um indicador prognóstico independente para OS e DFS em pacientes com CEC de boca e tem o potencial de complementar a classificação AJCC (2017). O LNR tem potencial para ser um importante indicador prognóstico, mas os métodos para essa classificação requerem mais estudos. O LODDS não demonstrou potencial prognóstico(AU)

Background: Despite the advances in the classification of oral squamous cell carcinoma (OSCC) based on its extension by the TNM system, there is still a need for methods to better segregate the patients to predict prognosis and indicate adjuvant therapy. Objectives: To analyze the influence of the number of positive lymph nodes (PN), lymph node ratio (LNR), and log odds of positive lymph nodes (LODDS) in survival of patients with OSCC Methods: Clinicopathologic data from patients with OSCC who were treated with curative purposes by surgery and neck dissection (ND) with or without subsequent adjuvant therapies from 1991 to 2015 was retrospectively assessed. The impact of the PN, LNR, LODDS, and other variables on overall survival (OS) and disease-free survival (DFS) was analyzed in univariate and multivariate analyses. Results: One hundred nineteen patients were included in this study. In the univariate analysis the PN had a significant impact on OS (p=0.001) and DFS (p=0.020), and the LNR had a significant impact on the OS (p=0.042). In the multivariate analysis with other relevant clinicopathologic variables, the PN was the only significantly independent factor influencing in the OS (p=0.017) but not in DFS (p=0.096). Conclusions: The PN is an independent prognostic indicator for OS and DFS in patients with OSCC and has the potential to aggregate the current AJCC classification. The LNR has potential to be an important prognostic indicator, but the methods for this classification require lapidation. The LODDS did not demonstrate prognostic potential(AU)

Fluorescence-guided surgical management of medication-related osteonecrosis of the jaws.

Medication-related osteonecrosis of the jaw (MRONJ) results in progressive destruction of the jawbones, and advanced cases demand surgical intervention. The total removal of necrotic bone is required to prevent recurrence and infection. However, determining the limits between necrotic and healthy bone is a challenge. The use of fluorescence to detect tissue alterations and determine necrosis extension is a promising method to avoid inadequate bone debridement. In the literature, there are several studies and reported cases that successfully use fluorescence-guided surgery (FGS) of MRONJ. The objective of this study was to present a critical review of the literature regarding the intraoperative use of optic fluorescence to differentiate healthy and necrotic bone in MRONJ. Studies that evaluated the intraoperative use of optic fluorescence to determine the surgical margins of MRONJ were searched in Pubmed/Medline and Scopus databases using the following terms: "medication-related osteonecrosis of the jaws" and "fluorescence". Eighteen papers describing the intraoperative use of FGS in ONJ management were selected, totalizing 218 patients. Of those 18 papers, only 5 were prospective studies, and the other 13 were isolated case reports, case series, and technical notes. The use of FGS to delimitate the resection margin of MRONJ is a promising method. There is no need for the application of exogenous fluorophore to perform FGS and the most often used light device was the VELScope® system. Further prospective studies with larger samples are still required to ascertain the fluorescence validity as a supporting method in MRONJ surgical treatment and to establish clinical protocols.