RESUMO
The aim of this study was to describe the prevalence, clinicopathological, and prognostic features of acinic cell carcinoma (AciCC) of the oral and maxillofacial region. AciCC cases were retrospectively retrieved from 11 pathology centers of three different countries. Medical records were examined to extract demographic, clinical, pathologic, and follow-up information. A total of 75 cases were included. Females (65.33%) with a mean age of 45.51 years were mostly affected. The lesions usually presented as an asymptomatic (64.28%) nodule (95.66%) in the parotid gland (70.68%). The association of two histopathological patterns was the most common finding (48.93%) and the tumors presented mainly conventional histopathological grades (86.11%). Surgical treatment was performed in the majority of the cases (59.19%). Local recurrence was observed in 20% of the informed cases, regional metastasis in 30.43%, and distant metastasis in 12.50%. The statistical analysis showed that the cases with a solid histopathological pattern (p=0.01), high-grade transformation (p=0.008), recurrence (p=0.007), and regional metastasis (p=0.03) were associated with poor survival. In conclusion, high histopathological transformation, presence of nodal metastasis, and recurrence were prognostic factors for AciCC of the oral and maxillofacial region.
Assuntos
Carcinoma de Células Acinares , Neoplasias das Glândulas Salivares , Feminino , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Carcinoma de Células Acinares/epidemiologia , Carcinoma de Células Acinares/cirurgia , Carcinoma de Células Acinares/patologia , Prognóstico , Neoplasias das Glândulas Salivares/patologiaRESUMO
Abstract The aim of this study was to describe the prevalence, clinicopathological, and prognostic features of acinic cell carcinoma (AciCC) of the oral and maxillofacial region. AciCC cases were retrospectively retrieved from 11 pathology centers of three different countries. Medical records were examined to extract demographic, clinical, pathologic, and follow-up information. A total of 75 cases were included. Females (65.33%) with a mean age of 45.51 years were mostly affected. The lesions usually presented as an asymptomatic (64.28%) nodule (95.66%) in the parotid gland (70.68%). The association of two histopathological patterns was the most common finding (48.93%) and the tumors presented mainly conventional histopathological grades (86.11%). Surgical treatment was performed in the majority of the cases (59.19%). Local recurrence was observed in 20% of the informed cases, regional metastasis in 30.43%, and distant metastasis in 12.50%. The statistical analysis showed that the cases with a solid histopathological pattern (p=0.01), high-grade transformation (p=0.008), recurrence (p=0.007), and regional metastasis (p=0.03) were associated with poor survival. In conclusion, high histopathological transformation, presence of nodal metastasis, and recurrence were prognostic factors for AciCC of the oral and maxillofacial region.
RESUMO
BACKGROUND AND OBJECTIVE: to describe the results of the treatment of invasive fungal sinusitis with nasal endoscopic surgery in an immunocompromised paediatric oncological population. METHODS: retrospective study of all patients diagnosed with invasive fungal sinusitis operated in the National Paediatric Oncology Unit between 2012 and 2016. Data taken from their medical history included: epidemiological characteristics, oncological diagnosis, haematological data, symptoms, tomographic studies, surgical interventions, results of pathology and cultures, medications received, complications, evolution and survival. RESULTS: 18 patients were identified, 7 male and 11 female. The average age was 12 years, 13 had a diagnosis of acute lymphocytic leukemia and 5 of acute myeloid leukemia. Seventeen patients presented severe neutropenia at the time of diagnosis. The most frequently identified aetiological agent was Aspergillus in 13 patients. In 16 patients (89%) the disease was controlled with nasal endoscopic surgery. Ten patients died due to unrelated causes throughout the study. DISCUSSION AND CONCLUSIONS: Invasive fungal sinusitis should be considered a medical emergency due to its high mortality. The diagnosis is based on a high index of suspicion in patients with predisposing factors (leukaemia, neutropenia, persistent fever, nasogastric tube) and endoscopic nasal evaluation. Antifungal medical treatment and aggressive nasal endoscopic surgery is indicated regardless of the patient's condition to reduce the fungal burden and associated high mortality. The treatment must be provided by a multidisciplinary team that includes paediatrics, haemato-oncology, infectology and otorhinolaryngology.
Assuntos
Aspergilose/cirurgia , Endoscopia/métodos , Infecções Fúngicas Invasivas/cirurgia , Procedimentos Cirúrgicos Nasais/métodos , Sinusite/cirurgia , Adolescente , Antifúngicos/uso terapêutico , Aspergilose/diagnóstico , Aspergilose/tratamento farmacológico , Criança , Pré-Escolar , Terapia Combinada , Epistaxe/etiologia , Feminino , Febre/etiologia , Humanos , Hospedeiro Imunocomprometido , Infecções Fúngicas Invasivas/diagnóstico , Infecções Fúngicas Invasivas/microbiologia , Leucemia Mieloide Aguda/complicações , Masculino , Equipe de Assistência ao Paciente , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Estudos Retrospectivos , Sinusite/diagnóstico , Sinusite/tratamento farmacológico , Avaliação de SintomasRESUMO
Kohlschütter-Tönz Syndrome is a rare disorder clinically characterized by amelogenesis imperfecta, epilepsy and progressive mental deterioration. We present an additional case of this syndrome of a nine year-old boy who was referred by pigmented teeth. The mental deterioration was associated with speech delay, impulsive behavior, attention-deficit/hyperactivity disorder, and learning problems. The physical examination revealed a reduction of lower third, slightly palpebral fissures, low ear and hair implantation, coarse hair and hypertrichosis. The intraoral examination showed alteration in teeth pigmentation diagnosed as amelogenesis imperfecta. Although rare, the present case report illustrates a syndrome that has dental anomalies and systemic alterations. It is important to recognize this syndrome as early as possible and paediatric dentist may contribute to the diagnosis and consequently to better manage the patients. Key words:Kohlschütter-Tönz syndrome, amelogenesis imperfecta, seizures, mental deterioration.
RESUMO
Non-Hodgkin lymphomas (NHLs) of the oral cavity and oropharynx constitute 13% of all primary extranodal NHLs. Marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) in the palatine tonsil is rare, corresponding to 6% of the NHLs of the Waldeyer ring. Some cases of MALT lymphoma can present prominent plasma cell differentiation, and less commonly, monoclonal gammopathy. The differential diagnosis of these cases from other NHLs with plasmacytic differentiation or plasma cell neoplasms is very difficult. In this article, we describe a rare case of MALT lymphoma in a 34-year-old man presenting as a swelling of the palatine tonsil. The tumor mass was diagnosed as MALT lymphoma with prominent plasma cell differentiation. Systemic evaluation was noncontributory. This is the first report of MALT lymphoma showing extensive plasmacytic differentiation of the palatine tonsil, and reinforces a possible relationship between extramedullary plasmacytoma and MALT lymphoma.
Assuntos
Linfoma de Zona Marginal Tipo Células B/patologia , Tonsila Palatina/patologia , Plasmócitos/patologia , Plasmocitoma/patologia , Neoplasias Tonsilares/patologia , Adulto , Diferenciação Celular , Diagnóstico Diferencial , Humanos , Imunidade nas Mucosas , Linfoma de Zona Marginal Tipo Células B/cirurgia , Masculino , Tonsila Palatina/cirurgia , Plasmocitoma/cirurgia , Neoplasias Tonsilares/cirurgia , TonsilectomiaRESUMO
OBJECTIVE: The aim of this study was to describe the clinicopathologic and immunohistochemical characteristics of 14 cases of central odontogenic fibroma (COF), and the ultrastructural features of 2 of them. STUDY DESIGN: Collaborative retrospective study based on the records of 4 oral pathology diagnostic services in Latin America based on the current World Health Organization classification. RESULTS: There were 7 male and 7 female patients (mean age 31.8 years). Eight tumors occurred in the maxilla and 6 in the mandible. Thirteen cases were epithelium-rich and 1 epithelium-poor COF. Three were classified as hybrid COF with giant cell lesion. Mean size of the hybrid lesions were larger than pure COF (3.8 vs. 2.4 cm). Odontogenic epithelial islands were immunoreactive for cytokeratin (CK) AE1/AE3, CK5, CK14, CK19, and 34BE12 and negative for CK1 and CK18. Langerhans cells positive for S-100 and CD1a were found within the epithelial islands in 6/6 tested cases. CD68 was expressed in the giant cells of the hybrid lesions and in a few mononuclear cells of 2 cases of COF. Ki-67 index was <1% in all cases. In 6 tumors (42.8%), there were small globular eosinophilic droplets within the epithelial islands, which were positive for collagen type IV, and 9/13 cases (69.2%) were focally positive for smooth muscle actin. In addition to fibroblasts, myofibroblastic differentiation was found in the 2 cases studied ultrastructurally. CONCLUSIONS: Immunohistochemistry was useful to confirm the presence of epithelium and to exclude other central fibrous tumors. COF also contains a variable number of mast cells, Langerhans cells, and myofibroblasts, and further studies are needed to better understand the participation of these cells in COF histogenesis.
Assuntos
Fibroma/patologia , Queratinas/metabolismo , Neoplasias Mandibulares/patologia , Neoplasias Maxilares/patologia , Tumores Odontogênicos/patologia , Adolescente , Adulto , Epitélio/patologia , Feminino , Fibroma/diagnóstico por imagem , Fibroma/metabolismo , Humanos , Imuno-Histoquímica , Queratinas/classificação , Masculino , Neoplasias Mandibulares/diagnóstico por imagem , Neoplasias Mandibulares/metabolismo , Neoplasias Maxilares/diagnóstico por imagem , Neoplasias Maxilares/metabolismo , Pessoa de Meia-Idade , Tumores Odontogênicos/diagnóstico por imagem , Tumores Odontogênicos/metabolismo , Estudos Retrospectivos , Ultrassonografia , Adulto JovemRESUMO
Pleomorphic adenoma (PA), the most common salivary gland tumor, accounts for 54 to 65% of all salivary gland neoplasias and 80% of the benign salivary gland tumors. It most frequently affects the parotid gland, followed by the submandibular and the minor salivary glands. Microscopically, mucous, sebaceous, oncocytic and squamous metaplasia, sometimes with the formation of keratin pearls, may be present, but the latter rarely results in the formation of extensive keratin-filled cysts lined by squamous epithelium. Extensive squamous metaplasia can be mistaken for malignancy, including mucoepidermoid carcinoma and squamous cell carcinoma. Here, we present an unusual case of PA with extensive squamous metaplasia and keratin cyst formations in a minor salivary gland, and discuss its microscopic features, including the immunohistochemical characteristics, and differential diagnosis of this uncommon presentation.
Assuntos
Adenoma Pleomorfo/patologia , Neoplasias das Glândulas Salivares/patologia , Glândulas Salivares Menores/patologia , Adulto , Humanos , Imuno-Histoquímica , Queratinas/biossíntese , Masculino , MetaplasiaRESUMO
Pleomorphic adenoma (PA), the most common salivary gland tumor, accounts for 54 to 65 percent of all salivary gland neoplasias and 80 percent of the benign salivary gland tumors. It most frequently affects the parotid gland, followed by the submandibular and the minor salivary glands. Microscopically, mucous, sebaceous, oncocytic and squamous metaplasia, sometimes with the formation of keratin pearls, may be present, but the latter rarely results in the formation of extensive keratin-flled cysts lined by squamous epithelium. Extensive squamous metaplasia can be mistaken for malignancy, including mucoepidermoid carcinoma and squamous cell carcinoma. Here, we present an unusual case of PA with extensive squamous metaplasia and keratin cyst formations in a minor salivary gland, and discuss its microscopic features, including the immunohistochemical characteristics, and differential diagnosis of this uncommon presentation.
Assuntos
Adulto , Humanos , Masculino , Adenoma Pleomorfo/patologia , Neoplasias das Glândulas Salivares/patologia , Glândulas Salivares Menores/patologia , Imuno-Histoquímica , Queratinas/biossíntese , MetaplasiaRESUMO
Extra-osseous odontogenic tumors are rarely observed. However, it is widely accepted that the remains of odontogenic epithelium entrapped in the oral soft tissues may be a possible source for peripheral odontogenic tumors differentiation. Peripheral developing odontoma is considered exceptionally rare, since few similar cases are described in the English-related literature under diverse nomenclature, such as irregular eruption, ectopic tooth, ectopic soft-tissue mesiodens, ectopic odontoma and extra-osseous tooth germ. Previously reported cases invariably affected children and surgical exploration revealed tooth germs exclusively embedded in the soft tissue without bone involvement. Microscopically, all these cases exhibited developing tooth germs composed of ameloblasts, enamel matrix, odontoblastic layer, dentin and dental papilla and the morphological findings seem to depend on the developmental stage of each tooth germ at discovery. Thus, we believe that it is relevant to report two additional cases that were recently diagnosed in Brazil and Guatemala, focusing on their nomenclature, correct diagnosis and further treatment.
Assuntos
Odontoma , Neoplasias Palatinas , Humanos , Lactente , Masculino , Odontoma/patologia , Odontoma/cirurgia , Neoplasias Palatinas/patologia , Neoplasias Palatinas/cirurgiaRESUMO
Primary intraosseous salivary gland tumors of the mandible are rare, with mucopidermoid carcinoma being the most frequent, followed by adenoid cystic carcinoma (ACC). We present a case of a central ACC involving the mandible of a 46-year-old man. He presented an indurated swelling on the vestibular aspect of the left mandibular body and ipsilateral paraesthesia of the lower lip. A panoramic radiography revealed a large radiolucent area, with irregular margins, involving the body and ramus of the left mandible, and CT scan confirmed that the lesion was confined within the mandibular bone. The histopathological features were of an ACC. CT scan also revealed multiple nodular lesions in both lungs suggestive of metastases. The patient was surgically treated by hemi-mandibulectomy. The patient is well with no evidences of recurrences in the mandible. The present case shows that the clinical and immunohistochemical profile of primary intraosseous ACC is similar to what is found in ACC involving the salivary glands.
Assuntos
Carcinoma Adenoide Cístico/metabolismo , Carcinoma Adenoide Cístico/patologia , Neoplasias Mandibulares/metabolismo , Neoplasias Mandibulares/patologia , Caderinas/metabolismo , Carcinoma Adenoide Cístico/cirurgia , Humanos , Queratinas/metabolismo , Masculino , Mandíbula/diagnóstico por imagem , Mandíbula/metabolismo , Mandíbula/patologia , Neoplasias Mandibulares/cirurgia , Pessoa de Meia-Idade , Radiografia , Resultado do Tratamento , Vimentina/metabolismo , beta Catenina/metabolismoRESUMO
The aim of this study was to analyze the clinico-pathological and immunohistochemical features of 62 cases of odontogenic myxoma (OM) diagnosed in three Oral Pathology Diagnostic Services in Latin America, as well as to describe the ultrastructural features of three of these cases. OM showed a wide age range (9-71 years), with a mean of 27.97 yr (SD: 11.01) and a male to female ratio of 1:2.2. Mandible was affected in 37 cases (59.6%) and maxilla in 25 (40.4%), with 61.3% located in the posterior region. Thirty-nine cases (62.9%) were multilocular and 23 (37.1%) unilocular. Size ranged from 1 to 13 cm, (mean: 5.2 cm). Thirty-seven multilocular (54.8%) and 6 unilocular lesions (26%) were larger than 4 cm (p<0.05). Epithelial islands were identified in 5 cases (8%) on H&E stained sections, but AE1/AE3 and CK14 disclosed these structures in 15 cases each (24.2%); CK5 was positive in 8 (12.9%); CK7 in 2 (3.2%) and CK19 in only 3 cases (4.8%). All cases were negative for CKs 8 and 18, S-100 protein, NSE and CD68, and showed a low index of expression of Bcl2 and ki-67 proteins (<1%). Mast cell antibodies showed these cells in 45 cases (72.6%). Myofibroblastic differentiation evidenced by myofilaments and fibronexi was found in one case out of the three studied by TEM and 29 cases (46.7%) were positive by immunohistochemistry for alpha actin. In conclusion, only a minority of OM had epithelial islands, and only 3 cases expressed CK 19, indicating an odontogenic epithelium origin. Immunohistochemical and ultrastructural findings suggest that OM is a mesenchymal neoplasm in which several factors may contribute to its pathogenesis, including myofibroblastic differentiation and the participation of mast cell products. However, further investigations are needed to better understand the participation of these elements in this particular neoplasm.
Assuntos
Neoplasias Mandibulares , Neoplasias Maxilares , Tumores Odontogênicos , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/metabolismo , Criança , Feminino , Humanos , Imuno-Histoquímica , Masculino , Neoplasias Mandibulares/metabolismo , Neoplasias Mandibulares/patologia , Neoplasias Mandibulares/ultraestrutura , Neoplasias Maxilares/metabolismo , Neoplasias Maxilares/patologia , Neoplasias Maxilares/ultraestrutura , Pessoa de Meia-Idade , Tumores Odontogênicos/metabolismo , Tumores Odontogênicos/patologia , Tumores Odontogênicos/ultraestrutura , Adulto JovemRESUMO
Oral melanoacanthoma (MA) is a rare, benign pigmented lesion, similar to cutaneous MA, characterized by hyperplasia of spinous keratinocytes and dendritic melanocytes. The pathogenesis of oral MA remains uncertain, although its clinical behavior is suggestive of a reactive origin. The most common intraoral sites are the buccal mucosa, lip, palate and gingiva. The average age of presentation is 28 years, mainly in blacks, with a strong female predilection. The oral melanotic macule (MM) is a small, well-circumscribed brown-to-black macule that occurs on the lips and mucous membranes. The etiology is not clear and it may represent a physiologic or reactive process. The average age of presentation is 43 years, with a female predilection. A biopsy is recommended to distinguish these lesions from each other and from other oral melanocytic lesions. We depict four cases each of oral MA and MM, affecting Caucasian and Latin American mestizo patients. The clinicopathological features of these cases reflect its ample spectrum, and to the best of our knowledge, it is the first example of oral MA affecting a Caucasian boy reported in the English literature. Therefore oral MA and MM should be considered in the differential diagnosis of pigmented lesions in the oral mucosa in these populations.
Assuntos
Acantoma/patologia , Melanose/patologia , Doenças da Boca/patologia , Neoplasias Bucais/patologia , Acantoma/complicações , Adulto , Criança , Feminino , Humanos , Imuno-Histoquímica , Masculino , Melanose/complicações , Doenças da Boca/complicações , Neoplasias Bucais/complicaçõesRESUMO
Cysticercosis is a potentially fatal parasitic disease that rarely involves the oral region in humans. This study includes 21 lesions seen in 16 Latin American patients. There were 8 men and 8 women, with an age range from 6 to 67 years (mean 33.6 years). Most cases appeared as asymptomatic submucosal nodules that resembled mucous cysts or benign mesenchymal neoplasms. Most were found in the tongue (11 cases), followed by buccal mucosa and the lower lip (4 cases each) and upper lip (2 cases). These were well circumscribed, and ranged in size from 1 to 2.5 cm in diameter. Typical histologic features of viable cysticerci were observed in 15 cases, and the presence of colloid degeneration and granular mineralization were detected in only 1 case. In all cases, simple surgical excision was sufficient to ensure complete removal of the lesions without postoperative complications. It is important to carry out a detailed study in every case, in order to exclude the presence of the parasite in other sites.
Assuntos
Cisticercose/patologia , Doenças da Boca/parasitologia , Taenia solium , Adolescente , Adulto , Idoso , Animais , Criança , Cisticercose/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Doenças Labiais/parasitologia , Doenças Labiais/cirurgia , Masculino , Pessoa de Meia-Idade , Doenças da Boca/cirurgia , Mucosa Bucal/parasitologia , Mucosa Bucal/cirurgia , Doenças da Língua/parasitologia , Doenças da Língua/cirurgiaRESUMO
Adenomatoid Odontogenic Tumour (AOT) is a relatively uncommon odontogenic lesion and few studies describing its cytokeratin profile have been reported in the English-language literature. Thirty-nine cases of AOT from three Oral Diagnosis services (Brazil, Mexico and Guatemala) were studied, considering their clinical, radiographic, and histological features and immunohistochemical expression of cytokeratins (AE1/AE3, 34betaE12, CK1, CK5, CK6, CK7, CK8, CK10, CK13, CK14, CK16, CK18, and CK19), vimentin and Ki-67. Sixty five percent of cases affected women, anterior maxilla was the preferred site and radiographically most cases showed unilocular radiolucency with well defined sclerotic borders. Calcifying epithelial odontogenic tumour (CEOT)-like areas were found in 36 out of 39 cases, and 10 cases showed positivity for Congo red in polarized light. All cases were positive for AE1/AE3, 34betaE12, CK5, CK14 and CK19. CEOT-like areas were negative for CK 19. Vimentin was also expressed in 27 cases and this profile may indicate the existence of a variable phenotype in certain areas of the tumour. There were no recurrences after surgical treatment, and this can be related to the low proliferative activity observed in all cases with Ki-67 marker.