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Resumen Antecedentes y objetivo El objetivo del estudio ha sido describir los resultados del tratamiento de sinusitis fungica invasiva con cirugia endoscopica nasal en una poblacion oncologica pediatrica con inmunosupresion e informar sobre la seguridad, la eficacia y las complicaciones del procedimiento Metodos Se realizo un estudio retrospectivo de la totalidad de los pacientes con diagnostico de sinusitis fungica invasiva operados en la Unidad Nacional de Oncologia Pediatrica entre los anos 2012 y 2016. Los datos tomados de su historial medico incluyeron: caracteristicas epidemiologicas, diagnostico oncologico, datos hematologicos, sintomas, estudios tomograficos, intervenciones quirurgicas, resultados de enfermedad y cultivos, medicamentos recibidos, complicaciones, evolucion y supervivencia. Los datos fueron analizados utilizando estadistica descriptiva, las variables continuas con medidas de tendencia central y las variables categoricas de forma porcentual. Resultados Se identifico a 18 pacientes, 7 de sexo masculino y 11 de sexo femenino. El promedio de edad fue de 12 anos, 13 tuvieron diagnostico de leucemia linfoide aguda y 5 de leucemia mieloide aguda; 17 pacientes presentaron neutropenia severa en el momento del diagnostico. El agente etiologico mas frecuentemente identificado fue Aspergillus en 13 pacientes. En 16 pacientes (89%) se controlo la enfermedad con cirugia endoscopica nasal. Diez pacientes fallecieron por causas no relacionadas a lo largo del estudio. Discusion y conclusiones La sinusitis fungica invasiva es una enfermedad cuya incidencia va en aumento entre pacientes con inmunosupresion y debe de considerarse una urgencia medica debido a su alta mortalidad. El diagnostico se basa en un alto indice de sospecha en pacientes con factores predisponentes (leucemia, neutropenia, fiebre persistente, sonda nasogastrica) y la evaluacion endoscopica nasal. El tratamiento medico antifungico y cirugia endoscopica nasal agresiva esta indicado independientemente del estado del paciente para disminuir la carga fungica y la alta mortalidad asociada. El tratamiento debe de ser suministrado por un equipo multidisciplinario que incluye pediatria, hemato-oncologia, infectologia y otorrinolaringologia.

Background This study aimed to compare the histological and immunohistochemical characteristics of ameloblastomas (AM) and ameloblastic carcinomas (AC). Material and Methods Fifteen cases of AM and 9 AC were submitted to hematoxilin and eosin (H&E) and immunohistochemical analysis with the following antibodies: cytokeratins 5,7,8,14 and 19, Ki-67, p53, p63 and the cellular adhesion molecules CD138 (Syndecan-1), E-cadherin and β-catenin. The mean score of the expression of Ki-67 and p53 labelling index (LIs) were compared between the groups using the t test. A value of p

Aim: To describe the clinicopathological and immuno-histochemical features of six tumours that do notfulfil the criteria of any of the currently classifiedodontogenic tumours.Methods and results: The patients were three malesand three females, whose ages ranged from 3 yearsto 18 years (mean, 11.5 years). In all cases therewere well-defined radiolucencies associated with une-rupted teeth apparently showing a pericoronal rela-tionship. Microscopically, all tumours were composedof variably cellular loose fibrous tissue with areassimilar to dental papilla, entirely surrounded bycuboidal to columnar epithelium resembling theinternal epithelium of the enamel organ. Mesenchy-mal tissue was positive only for vimentin, and Ki67expression was very low (

Summary Most cystic lesions of the oral cavity are intraosseous and lined by squamous epithelium. Although rare, various developmental cysts occur preferentially in the tongue, lined by dermal, thyroglossal, gastrointestinal or respiratory epithelium. Surgical excision is the treatment of choice for these cysts. We report the clinical, histological and immunohistochemical aspects of one lingual cyst lined by gastric epithelium affecting a 1-year-old boy. Immunohistochemical analysis showed similar profile of normal gastric epithelium, except by negativity for CK20, suggesting well-differentiation states. The pathogenesis lingual cyst lined by gastric epithelium remains uncertain, but it is considered they are derived from entrapment of embryonic epithelial rests.

A 42-year-old woman presented a large, nontender, quickly progressive mass in the left mandible. Radiograph showed extensive destruction of the angle, posterior body, and ramus of the left side of the mandible. The patient was surgically treated by hemimandibulectomy. Microscopically, the tumor was composed of large epithelioid cells, many of them showing polarized nuclei, and evident eosinophilic cytoplasm. The predominant pattern was trabecular, and rosette-like structures were also observed. Typical osteoid-containing cells surrounded by malignant cells were found in a few areas. Immunohistochemistry for a large panel of antibodies showed positivity for osteocalcin, osteonectin, osteopontin, VS38c, and S-100. CD34 saliented the hemangiopericytoma-like distribution of the blood vessels. Collagen I was focally positive for the extracellular matrix and malignant osteoid. All other markers were negative, including vimentin and cytokeratins. To the best of our knowledge, this is the first case of epithelioid osteosarcoma affecting the mandible and the second case affecting the jaws that has been reported in the literature.

Background: Calcifying odontogenic cyst was described first by Gorlin et al. in 1962; since then several hundreds of cases had been reported. In 1981, Praetorius et al. proposed a widely used classification. Afterwards, several authors proposed different classifications and discussed its neoplastic potential. The 2005 WHO Classification of Odontogenic Tumours re-named this entity as calcifying cystic odontogenic tumour (CCOT) and defined the clinico-pathological features of the ghost cell odontogenic tumours, the CCOT, the dentinogenic ghost cell tumour (DGCT) and the ghost cell odontogenic carcinoma (GCOC). Methods: The aim of this paper was to review the clinical-pathological features of 122 CCOT, DGCT and GCOC cases retrieved from the files of the oral pathology laboratories from 14 institutions in Mexico, South Africa, Denmark, the USA, Brazil, Guatemala and Peru. It attempts to clarify and to group the clinico-pathological features of the analysed cases and to propose an objective, comprehensive and useful classification under the 2005 WHO classification guidelines. Results: CCOT cases were divided into four sub-types: (i) simple cystic; (ii) odontoma associated; (iii) ameloblastomatous proliferating; and (iv) CCOT associated with benign odontogenic tumours other than odontomas. DGCT was separated into a central aggressive DGCT and a peripheral non-aggressive counterpart. For GCOC, three variants were identified. The first reported cases of a recurrent peripheral CCOT and a multiple synchronous, CCOT are included. Conclusions: Our results suggest that ghost cell odontogenic tumours comprise a heterogeneous group of neoplasms which need further studies to define more precisely their biological behaviour.

Pathology Department, InstitutoNacional de Cancerologi´a, Me ´xico, D.F. Me´xicoAIM: To classify 163 ameloblastoma cases according tothe new WHO Classification of Odontogenic Tumours(2005) and analyse their clinical and microscopic features.METHODS: We studied the clinico-pathological featuresof 163 ameloblastoma cases from nine regional Latin-American institutions from Mexico and Guatemala.RESULTS: Ameloblastomas comprised 22.7% of allodontogenic tumours. The mean age was 41.4 years forsolid ameloblastoma (SA) and 26.3 years for unicysticameloblastoma (UA) (P < 0.001) and both sexes werealmost equally affected. The mandible was mainly af-fected for both UA and SA. The mean size was 6.2 cm forSA and 6.3 cm for UA cases. The recurrence rate was21.7% for SA and 12.6% for UA. UA was twice as morefrequent than the solid variant.CONCLUSIONS: In this study we found that UA wasfrequently misdiagnosed as SA; however, there are en-ough clinical and microscopic features that allow for anaccurate differentiation between both types of amelob-lastoma that should be recognized for surgical andprognostic purposes. In this study, SA was not found inpatients younger than 20 years, UA had a constantmyxoid stroma while mature connective tissue was morefrequently associated with the solid type.Oral Diseases (2007) 13, 303–307Keywords: odontogenic tumours; ameloblastoma; solid amelob-lastoma; unicystic ameloblastoma; mandibular tumours; maxillarytumours

BACKGROUND: Adenomatoid odontogenic tumour (AOT) is a benign odontogenic jaw lesion. The aim of this study was to update the biological profile of AOT. MATERIAL AND METHODS: Cases published in the literature and cases in files of co-authors were included. RESULTS: 550 new cases were retrieved, and of a total of 1082 cases analysed, 87.2% were found in the second and third decades. The M:F ratio was 1:1.9. 70.8% were of the follicular variant (extrafollicular: 26.9%, peripheral: 2.3%). 64.3% occurred in the maxilla. 60% of follicular AOTs were associated with unerupted canines. Nineteen cases of AOT (2.8%, M:F ratio was 1:1.4) were associated with embedded third molars. Twenty-two peripheral AOTs (2.3%, M:F ratio was 1:5.3) were recorded. The relative frequency (RF) of AOT ranged between 0.6% and 38.5%, revealing a considerably wider AOT/RF range than hitherto reported (2.2–7.1%). CONCLUSIONS: This updated review based on the largest number of AOT cases ever presented, confirms the distinctive, although not pathognomonic clinicopathological profile of the AOT, its worldwide occurrence, and its consistently benign behaviour.

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.

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumour. To date, 60 well-documented cases have been published in the English-language literature. Two additional cases located in the posterior region of the mandible are reported. The relevant clinicopathological features of these cases, as well as those previously reported are discussed. Approximately two-thirds of AFS are malignant tumors de novo, with an average age of the affected patients being 22.9 years. This age is lower than the one observed in AFS developing from a pre-existent benign lesion (mean 33 years). Although regional and distant metastases are very infrequent, AFS is a locally aggressive lesion, with 23 (37%) of the reported cases having at least one recurrence; 12 patients (19.3%) died of the disease. Wide surgical excision with long-term follow-up remains the treatment of choice for this neoplasm.

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.

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.Collaborative retrospective study based on the records of 4 oral pathology diagnostic services in Latin America based on the current World Health Organization classification.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 was1% 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.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.

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.

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.

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.

Lichen sclerosus is a chronic inflammatory mucocutaneous disease, rarely involving the mouth. There are only 20 well-documented cases of oral lichen sclerosus reported in the English-language literature. This report describes the clinicopathological features of 6 cases of oral lichen sclerosus; 5 in women. There were 12 lesions, mainly on the lips (50%) and buccal mucosa (25%). The affected areas appeared as irregular whitish patches, harder than the surrounding tissue. Half of the patients were symptomatic and presented with no associated skin and/or genital lesions. All cases were biopsied, and histopathological features were evaluated using hematoxylin-eosin and Verhoeff's stains, S-100 immunohistochemical reaction and transmission electron microscopy. Management of the oral lesions consisted of surgical excision, intralesional triamcinolone acetonide, oral colchicine, and regular follow-up. There is no effective curative treatment, but there are some options for patient management; and colchicine may be considered an additional choice.

Adenomatoid odontogenic tumour (AOT) is a benign odontogenic jaw lesion. The aim of this study was to update the biological profile of AOT.Cases published in the literature and cases in files of co-authors were included.550 new cases were retrieved, and of a total of 1082 cases analysed, 87.2% were found in the second and third decades. The M:F ratio was 1:1.9. 70.8% were of the follicular variant (extrafollicular: 26.9%, peripheral: 2.3%). 64.3% occurred in the maxilla. 60% of follicular AOTs were associated with unerupted canines. Nineteen cases of AOT (2.8%, M:F ratio was 1:1.4) were associated with embedded third molars. Twenty-two peripheral AOTs (2.3%, M:F ratio was 1:5.3) were recorded. The relative frequency (RF) of AOT ranged between 0.6% and 38.5%, revealing a considerably wider AOT/RF range than hitherto reported (2.2-7.1%).This updated review based on the largest number of AOT cases ever presented, confirms the distinctive, although not pathognomonic clinicopathological profile of the AOT, its worldwide occurrence, and its consistently benign behaviour.

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 (p0.05). Epithelial islands were identified in 5 cases (8%) on HE 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.

We report two cases of an uncommon odontogenic lesion, previously described as adenomatoid dentinoma. They were well-circumscribed unilocular radiolucent lesions exhibiting discrete radiopacities, located in the left mandibular third molar region. Microscopically they were composed of odontogenic hard and soft tissues, similar to a dental germ. Dental papilla and dentin were easily identified. Odontogenic epithelium formed adenomatoid-like structures, and by scanning electron microscopy a layer of enamel was seen in contact with the dentin. Based on these clinical, radiographic, histological and electron microscopical features we proposed the diagnosis of adenomatoid odontogenic hamartoma. Treatment consisted of surgical removal, and no recurrence was observed. In our opinion all similar cases previously reported pertain to the same spectrum of this lesion and thus should be named as suggested above. Moreover, ultrastructural observations using 5 microm sections can be useful to better characterize the presence of hard tissues.

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.

The odontoameloblastoma (OA), is an infrequent neoplasm. To date, there are less than 50 cases reported as OA or ameloblastic odontoma in the English dental literature, but only 14 (including three of our own material), fulfill the histological criteria of the current WHO histological classification of odontogenic tumours. Nine occurred in men and five in women (male to female ratio 1.8:1). Age ranged from 2 to 50 years (mean 20.2 years), and nine cases (64.2%) were diagnosed during the first two decades. Maxilla and mandible were equally involved, and most cases occurred posterior to the canines (71.4%). Follow-up ranged from 6 months to 8 years (mean: 25.5 months). Of the 12 cases with informed follow-up, two recurred once (at 24 and 18 months, respectively), and one case had two documented recurrences, at 6 and 49 months. Although OA tends to occur at an earlier age than conventional ameloblastoma, it has practically the same potential to produce bone expansion, root resorption and recurrence. For these reasons OA should be treated in a similar fashion, with wide surgical excision and close follow-up for at least 5 years.

Background Primordial Odontogenic Tumor (POT) is a recently described odontogenic tumor characterized by a variably cellular loose fibrous tissue with areas similar to the dental papilla, covered by cuboidal to columnar epithelium that resembles the internal epithelium of the enamel organ, surrounded at least partly by a delicate fibrous capsule. The purpose of this study was to investigate the possible histogenesis and biological behavior of this rare tumor by means of a wide immunohistochemical analysis of its epithelial and mesenchymal components. Material and Methods The immunoexpression of twenty-three different antibodies were evaluated in four cases of POT. Results The epithelial cells that cover the periphery of the tumor showed immunopositivity for Cytokeratins 14 and 19, while Amelogenin, Glut-1, MOC-31, Caveolin-1. Galectin-3, PITX2, p53, Bax, Bcl-2, Survivin and PTEN were variably expressed in focal areas. The mesenchymal component of the tumor was positive for Vimentin, Syndecan-1, PITX2, Endoglin (CD105), CD 34, Cyclin D1, Bax, Bcl-2, Survivin and p53. PTEN and CD 90 showed a moderate positivity. BRAF V600E and Calretinin were negative in all samples. Cell proliferation markers (Ki-67, MCM-7) were expressed in