Tomographic Evaluation of the Odontogenic Maxillary Myxoma. Case Report and Literature Review / Evaluación tomográfica del mixoma odontogénico en maxilar superior. Reporte de caso y revisión de la literatura

Odontogenic myxoma (OM) is a slow-growing, painless, aggressive and non-metastatic central jaw tumor of mesenchymal origin. Radiographically, it can vary from a unilocular radiolucent lesion to a multilocular lesion with well-defined or diffuse margins. The aim of this paper is to recognize the radiographic and tomographic characteristics of OM in a patient who attended the Dental Clinic of the Faculty of Dentistry of the Universidad Nacional Mayor de San Marcos in Lima-Peru. Case presentation: 86 year old male patient, who in the panoramic radiography indicated for his oral rehabilitation, a unilocular radiolucent image was found in the anterosuperior area with partially defined limits, corticalized edges and oval shape. In the volumetric tomography there was evidence of thinning and erosion of both bone tables, thinning of the floor of the nasal cavity. The radiolucent image seems to havean extension close to the alveolar ridge.In adition, there was an effacement of the cortices of the nasopalatine duct. The lesion was enucleated and an anatomopathological examination was performed. Diagnosis was odontogenic myxoma. The patient was evaluated at one year and six months with satisfactory results. The wide variety of radiographic characteristics of odontogenic myxoma leads us to think of a large number of differential diagnoses, being the histological evaluation together with the imaging analysis the ones that provide a definitive diagnosis. Although the anterosuperior area is the least common for its presentation, radiolucent images in this area should be considered as possible odontogenic myxomas, since this condition is more frequent in latín race.

El mixoma odontogénico (MO) es un tumor mandibular central de origen mesenquimal, de crecimiento lento, indoloro, agresivo y no metastásico. Radiográficamente, puede variar desde una lesión unilocular radiolúcida a una lesión multilocular con márgenes bien definidos o difusos. El objetivo de este trabajo es reconocer las características radiográficas y tomográficas del MO en un paciente que acudió a la Clínica Odontológica de la Facultad de Odontología de la Universidad Nacional Mayor de San Marcos en Lima-Perú. Presentación del caso: Paciente masculino de 86 años, en la radiografía panorámica indicada para rehabilitación oral, se encontró una imagen radiolúcida unilocular en la zona anterosuperior con límites parcialmente definidos, bordes corticalizados y forma ovalada. En la tomografía volumétrica se evidenció adelgazamiento y erosión de ambas tablas óseas, adelgazamiento del piso de la cavidad nasal, la imagen radiolúcida parece tener una extensión cercana a la cresta alveolar. Además, había un adelzamiento de las corticales del conducto nasopalatino. La lesión fue enucleada y se realizó un examen anatomopatológico. El diagnóstico fue mixoma odontogénico. La paciente fue evaluada al año y a los seis meses con resultados satisfactorios. La gran variedad de características radiográficas del mixoma odontogénico nos lleva a pensar en un gran número de diagnósticos diferenciales, siendo la evaluación histológica junto con el análisis de imagen los que proporcionan un diagnóstico definitivo. Aunque el área anterosuperior es la menos común para su presentación, las imágenes radiolúcidas en esta área deben ser consideradas como posibles mixomas odontogénicos, ya que esta condición es más frecuente en la raza latina.

Thyroid cancer in patients undergoing thyroidectomy total in the Otorhinolaryngology Department of the Hospital de Clínicas during the period 2018-2022

Introduction: Thyroid cancer, considered one of the most frequent malignant tumors of the endocrine system, accounts for 80%-90% of cases. The most common histological type observed in most studies is papillary cancer. Objective: To determine the frequency of thyroid cancer in patients undergoing total thyroidectomy at the Otorhinolaryngology Department of the Hospital de Clínicas between the period 2018-2022. Methodology: This retrospective study included 260 patients treated during the study period. Data were obtained from the patients' medical records, and sociodemographic and clinical variables related to thyroid cancer were analyzed. Data were analyzed with the SPSS v25 program, using descriptive statistics. Results: The frequency of thyroid cancer was 48.1%. The mean age of the cancer patients was 42.10±13.10 years. A higher frequency was observed in females 34.2%, n=89) and among those aged 25-44 years 20.0%, n=52). The most frequent were papillary (36.9%), follicular (2.7%), medullary (1.5%), and anaplastic (1.2%) types. Discussion: The frequency of thyroid cancer is high; it is more common in women and as age increases.

Introducción: El cáncer de tiroides, considerado uno de los tumores malignos más frecuentes del sistema endocrino, representa entre el 80% y el 90% de los casos. El tipo histológico más común observado en la mayoría de los estudios es el cáncer papilar. Objetivo: Determinar la frecuencia de cáncer de tiroides en pacientes sometidos a tiroidectomía total en la Cátedra y Servicio de Otorrinolaringología del Hospital de Clínicas durante el período 2018-2022. Metodología: se realizó un estudio retrospectivo con un total de 260 pacientes atendidos en el período de estudio. Los datos fueron tomados de las historias clínicas de los pacientes, en el cual se analizan variables sociodemográficas y clínicas relacionadas con el cáncer de tiroides. Los datos se analizaron con el programa SPSS v25, mediante estadística descriptiva. Resultados: La frecuencia del cáncer de tiroides fue del 48.1%. El promedio de edad de los pacientes con cáncer fue de 42.10±13.10 años. se observó una mayor frecuencia en las personas del sexo femenino 34.2% (n=89) y entre quienes tuvieron de 25-44 años 20.0% (n=52). El más frecuente fue el tipo papilar 36.9%, el folicular 2.7%, el medular 1.5% y el anaplásico 1.2%. Discusión: la frecuencia de cáncer de tiroides es alta, es más frecuente en las mujeres y a medida que aumenta la edad.

Phenotype and genotype of patients with multiple endocrine neoplasia type 1 studied in Argentina / Fenotipo y genotipo de pacientes con neoplasia endocrina múltiple tipo 1 estudiados en Argentina

Abstract Introduction: Multiple Endocrine Neoplasia type 1 (MEN1) is an autosomal dominant inherited disease with an estimated prevalence of 2-10:100 000. The main locations of tumors are parathyroid glands (HPT), gas troenteropancreatic tract (GEPT), and anterior pituitary gland (PT). The aim of our investigation was to describe the phe notype and genotype of Argentinian patients with MEN1. Methods: A total of 68 index patients diagnosed with at least two of the three main tumors or one tumor and a relative with MEN1, and 84 first-degree relatives were studied. We sequenced the coding region (exons 2-10); the promoter, exon 1; and the flanking intronic regions of the MEN1 gene, following the Sanger method. We used MLPA in index patients without mutation. Results: Prevalence of tumors: HPT 87.5%, GEPT 49% (p< 0.001). No statistical differences in the prevalence of HPT vs. PT (68%). Prevalence of pathogenic variants: 90% in familial cases and 51% in sporadic cases. Of the different 36 pathogenic variants, 13 (36.2%) were frameshift micro-rearrangement, 8 (22.2%) were mis sense, 9 (25%) were nonsense, 3 (8.3%) were mutations in splicing sites, 2 (5.5%) were large deletions and, 1 in-frame micro-rearrangement. We found 7 novel pathogenic variants. Thirty-nine percent (n = 33) of first-degree relatives of 23 families were found to be mutation carriers. Conclusion: The phenotype and genotype of Argen tinian patients was similar to other MEN1 populations. A high frequency of PT and the identification of seven novel mutations are underscored.

Resumen Introducción: La neoplasia endocrina múltiple tipo 1 (NEM1) es una enfermedad hereditaria autosómica dominante con una prevalencia estimada de 2-10:100 000. Las localizaciones principales de los tumores son glándulas paratiroides (HPT), tracto gastroenteropan creático (TGEP) y glándula pituitaria (TP). El objetivo de nuestra investigación fue describir el fenotipo y genotipo de pacientes argentinos con NEM1. Métodos: Estudiamos 68 casos índices diagnostica dos por presentar al menos dos de los tres tumores principales, o un tumor y un pariente con NEM1, y 84 familiares de primer grado. Secuenciamos la región codificante (exones 2-10); el promotor, exón 1; y las re giones intrónicas flanqueantes del gen MEN1 siguiendo el método de Sanger. Utilizamos MLPA en pacientes índice sin mutación. Resultados: Prevalencia de tumores: HPT 87.5%, TGEP 49% (p < 0.001), sin diferencias estadísticas entre las prevalencias de HPT vs TP (68%). Prevalencia de variantes patogénicas: 90% en casos familiares y 51% en esporádi cos. Hallamos 36 variantes patogénicas, 7 (20%) fueron noveles. Fueron 13 (36.2%) microarreglos con cambio en el marco de lectura, 9 (25%) variantes sin sentido, 8 (22.2%) con cambio de sentido, 3 (8.3%) en sitio de unión de empalme, 2 (5.5%) grandes deleciones y 1 microarre glo sin cambio en el marco de lectura. El 39 % (n = 33) de los parientes de primer grado en 23 familias fueron portadores de mutaciones. Conclusión: El fenotipo y genotipo de los pacientes argentinos con NEM1 fue similar al de otras poblaciones. Destacamos una alta frecuencia de TP y de variaciones patogénicas noveles.

Metástasis hipofisarias: un desafío diagnóstico y terapéutico / Pituitary metastases: a diagnostic and therapeutic chal lenge

Resumen Introducción : La región selar es un sitio infrecuente de metástasis, encontrándose en el 1% de las cirugías hipofisarias. Los tumores primarios más habituales son mama y pulmón. En general son diagnosticadas en pa cientes con enfermedad avanzada, aunque pueden ser el debut de la enfermedad oncológica. Métodos : Análisis retrospectivo de las características clínicas, bioquímicas, radiológicas de pacientes con me tástasis selares o hipofisarias (MS) durante el periodo 2009-2020. Resultados : Se reportaron 18 casos de pacientes, 11 de ellos con confirmación histológica. La mediana de edad fue 53 años (rango: 35-75), 53% hombres. La loca lización del tumor primario fue: 8 pulmón, 6 mama, 1 carcinoma folicular de tiroides, 1 linfoma Hodgkin y 2 carcinomas renales de células claras. La media de tiem po entre el diagnóstico del tumor primario y la aparición de la MS -en los casos de presentación metacrónica- fue 108 meses (rango: 11-180). En 8 pacientes (44.4%), el diagnóstico de la neoplasia primaria se hizo a partir del hallazgo de la masa selar. Diabetes insípida, hipopitui tarismo, trastornos visuales, oftalmoplejía y cefalea se presentaron en el 78, 77, 61, 39 y 39%, respectivamente. Quince pacientes presentaron masas con extensión su pra/paraselar; y 3 lesión limitada a la hipófisis y tallo. Fueron operados 11/18 por vía transesfenoidal, para diagnóstico y/o descompresión. Fallecieron 17, con una mediana de sobrevida de 6 meses (1- 36). Discusión : La sospecha de MS debe estar presente ante una masa selar y supraselar con captación difusa del gadolinio, diabetes insípida, hipopituitarismo y/o disfunción visual, aun en pacientes sin antecedentes oncológicos.

Abstract Introduction : Sellar metastases (SM) are rare mani festations of malignancy. Breast and lung cancer are the most common primary tumors. Most cases are di agnosed in patients with advanced malignant disease; however, symptoms of pituitary involvement can pre cede the diagnosis of the primary tumor. Methods : Retrospective analysis of symptoms at presentation, hormonal, radiological and histological findings, management, and outcome of patients with SM from 2009 to 2020. Results : Eighteen patients´cases were included, 11 with histological confirmation. Median (m) age was 53 years (range 35-75), 53% male. Primary malignant tu mors: 8 lungs, 6 breast, 1 follicular thyroid carcinoma, 1 Hodgkin lymphoma, and 2 clear cell renal carcinomas. The m time between the diagnosis of the primary neo plasm and the occurrence of the SM was 108 months (range: 11-180). In 8 patients the diagnosis of the primary neoplasm was made after the finding of the symptom atic sellar mass. Insipidus diabetes, adenohypophysis deficit, visual disorders, headache, and cranial nerve deficits were evident in 78, 77, 61, 39 and 39% of the cases, respectively. Fifteen patients harbored supra / parasellar masses, in three a lesion was limited to the pituitary gland, and stalk. Eleven out of 18 (61.1%) of the patients were operated on by the trans-sphenoidal approach, for diagnostic and / or decompressive pur poses. Eighteen died, with a median survival time of 6 months (1-36). Discussion : In the presence of a pituitary lesion with diffuse gadolinium uptake, associated with insipidus diabetes and / or visual disorder SM should be suspected even in patients without a history of oncological disease.

Queratoquiste: Expresión clínica mandibular atípica en un caso clínico

El queratoquiste odontogénico constituye del 3 a 11% de los quistes odontogénicos. Se presenta desde la infancia hasta la vejez con mayor incidencia en hombres. La mandíbula está involucrada en el 60% al 80% de los casos, con una frecuencia en cuerpo y rama. Las lesiones de menor tamaño suelen ser asintomáticos, diagnosticados por examen radiográfico, no obstante, las lesiones más grandes pueden estar asociadas con dolor y aumento de volumen. Radiográficamente se observan lesiones uniloculares o multiloculares radiolúcidas de bordes nítidos, corticalizados, asociado a un diente retenido. Se presenta caso clínico de paciente género masculino de 30 años de edad, que exhibe una expresión atípica. Manifestándose como una doble lesión de queratoquistes odontogénicos independientes entre sí, localizados en rama y cuerpo mandibular derecha, tratado en el Servicio de Cirugía Maxilofacial del Hospital San José, Santiago de Chile. Se describe diagnóstico y tratamiento quirúrgico realizado. El interés clínico del caso es la presencia de dos lesiones independientes entre sí, con el mismo diagnóstico. Presentación que nos parece fundamental reportar en la literatura científica debido a su alto alcance e impacto.

The odontogenic keratocyst represents 3 to 11% of all odontogenic cysts. It occurs from childhood to old age with a higher incidence in men. The mandible is involved in 60% to 80% of cases, with a frequency in the body and ramus. Smaller lesions are usually asymptomatic and diagnosed by radiographic examination. However, larger lesions may be associated with pain and increased volume. Radiographically, radiolucent unilocular or multilocular lesions with sharp, corticalized edges are observed, associated with an impacted tooth. A clinical case of a 30-year-old male patient, who exhibits an atypical expression, is presented. A double lesion of odontogenic keratocysts independent of each other appears, located in the right mandibular ramus and body, treated in the Maxillofacial Surgery Service of the San José Hospital, Santiago de Chile. Diagnosis and surgical treatment performed are described. The clinical interest of the case is the presence of two lesions independent of each other, with the same diagnosis. It seems fundamental to us to report it in the scientific literature due to its high scope and impact.

Navigating the uncommon: a comprehensive case study of multiple gastroduodenal neuroendocrine tumor

Neuroendocrine tumors (NETs) have varied pathophysiological characteristics, location, clinical presentation, management & outcome. Unfortunately, most NETs are non-functional and therefore, either remain asymptomatic until incidentally detected or present very late with pressure symptoms, adding up to the associated morbidity and mortality. Here we presented a case of a 43-year gentleman, who presented to gastroenterology OPD with chief complaints of heartburn and pain in the upper abdomen for one year. He had an equivocal clinical examination and laboratory parameters. Upper GI endoscopy and computed tomography revealed multiple nodular growths in the D1 segment and pylorus of the stomach. He was successfully managed by wide local excision of D1 and distal stomach (pylorus) followed by Polya gastrojejunostomy reconstruction. Histopathology confirmed well-differentiated NETs, low Ki67, and positive for chromogranin and synaptophysin. This case draws attention to the early age of presentation of multifocal NETs with vague symptoms and equivocal clinical examination. Coexisting gastric NETs with duodenal NETs in itself is very rare, never the less non-functional status and pre-operative diagnostic dilemma. Here, we have also drawn attention to the pros and cons of various diagnostic tools and how their utility can sometimes limit the approach of clinicians, apart from a high index of suspicion.

A clinicopathological study of histomorphological differentiation and staging of lymph node metastasis in periampullary carcinoma

Background: The periampullary region is an anatomically and histologically complex area that is prone towards malignant transformation. Malignancies in this region have increased in incidence in the last few decades. Classifying these malignancies has become crucial for prognosis and treatment. With the pancreato-biliary type being more aggressive as compared to the intestinal type. Lymph node metastasis and lymph node ratio also aid in prognostication. Methods: This prospective descriptive study was conducted over one year from August 2022 on 30 patients diagnosed with periampullary tumors who underwent pancreatoduodenectomy and biopsy. Histopathological classification and prognostic factors were analyzed based on Kimura et al classification. Results: The study included 18 males and 12 females with a mean age of 53.47 years. The origin of the tumor was the head of the pancreas in 66.66% (n=20) of the patients. Adenocarcinoma was the most common histological type. The pancreato-biliary type of adenocarcinoma was more aggressive than the intestinal type when primary tumor size and staging were compared. Conclusions: Periampullary carcinomas are difficult to classify. Kimura et al classification is an important classification system that helps in the treatment and prognostication of the patient. The pancreato-biliary type of adenocarcinoma is more aggressive as compared to the intestinal type. Lymph node metastasis and lymph node ratio are also important prognostic factors.

Management of challenges with large cervical masses

Cervical adenomyomas are extremely rare benign tumors and are often overlooked as a differential diagnosis of large cervical masses. Herein, we report a case of a 23-year-old nulliparous woman with polypoid adenomyoma arising from the endocervix. The mass was filling up the vagina and stretching the anterior fornix up into the lower abdomen, hence the origin of the mass could not be identified on clinical examination or imaging. Therefore, management of this mass was challenging. Excision of this mass was done using an abdominoperineal approach. Histologically, the polypoid lesion was composed of endocervical epithelium with interlacing fascicles and bundles of tumor cells intermixed with endocervical glands. Clinicians should be aware of such lesions so that they can decide upon the appropriate course of management.

Tumor maligno melanocítico de la vaina neural en mediastino posterior / Malignant melanotic nerve sheath tumor of the posterior mediastinum

Resumen El schwannoma melanótico (SM) es una variante rara e infrecuente caracterizada por el depósito citoplasmáti co de melanosomas (melanina). A diferencia de las otras variantes de schwannomas, tienen capacidad de malig nización. Por poseer características y comportamiento distintos al resto de los schwannomas, fue reclasificado como "tumor maligno melanocítico de la vaina neural" en la 5ta edición de la clasificación de los tumores del sis tema nervioso central de la Organización Mundial de la Salud en 2021. Presentamos dos casos de SM de ubicación mediastinal en los que se realizó una resección quirúrgi ca completa.

Abstract Melanotic schwannoma (MS) is a rare and infrequent subtype of schwannoma characterized by cytoplasmic deposits of melanosomes (melanin). Unlike the other schwannomas, it could have malignant transforma tion. Due to distinctive characteristics and atypical behavior from classic schwannomas subtypes, MS were renamed and reclassified as "melanocytic malignant neural sheath tumor" in the 5th ed. of the World Health Organization's classification of central nervous system tumors in 2021. We present two cases of MS that under went complete surgical resection.

Decoding pituitary tumors: a systematic analysis of diagnostic methods and treatment modalities

Pituitary tumors are growths that form in the gland these tumors are rare representing 10-15% of all brain tumors. They can disrupt the production of hormones, in the body leading to symptoms related to hormone imbalance. This review offers an overview of the methods used for diagnosing and treating tumors. It is worth noting that relying solely on references may restrict the scope and depth of discussions about tumors in this paper. Suggestions for research include exploring diagnostic tools like molecular imaging and liquid biopsy to enhance early detection and accurate assessment of these tumors. Additionally, more research is required to understand the long-term effects and quality of life outcomes for patients undergoing treatment options for tumors. In conclusion, significant progress has been made in diagnosing and treating tumors over time. Advances in imaging technologies such as diffusion-weighted imaging and magnetic resonance spectroscopy have enhanced precision and treatment strategies for these tumors. The discussion also covers the roles of surgery, radiation therapy and medical interventions, in managing tumor growth and hormonal imbalances further advancements, in research and innovation are crucial, for enhancing our knowledge and treatment of tumors ultimately bringing outcomes for both patients and healthcare professionals.

Síndrome de feocromocitoma-paraganglioma de tipo 5 como causa de hipertensión arterial en una paciente colombiana: reporte de caso / Pheochromocytoma/paraganglioma type 5 syndrome as a cause of secondary hypertension in a Colombian patient: case report

El feocromocitoma es un tumor derivado de las células de la cresta neural con la capacidad de producir sustancias simpaticomiméticas y, por ende, un cuadro clínico particular. Causa menos del 1 % de los casos de hipertensión arterial sistémica y su incidencia se estima entre 0,4 y 0,6 casos por 100.000 personas cada año, con una supervivencia media de siete años. De todos los tumores sólidos, el feocromocitoma tiene un mayor componente genético, que puede heredarse hasta en el 40 % de los casos. Una vez diagnosticada la enfermedad, se debe definir el tratamiento y el pronóstico, en parte condicionados por las variantes genéticas asociadas, en especial RET, SDHx, VHL y NF1.Se presenta el caso de una mujer joven con dolor abdominal e hipertensión arterial sistémica, a quien se le diagnosticó feocromocitoma. Al secuenciar el exoma, se identificó una variante patogénica extremadamente rara y de reciente descubrimiento: SDHA: c.1A>C (p.Met1Leu). La paciente respondió adecuadamente al tratamiento quirúrgico y continuó en seguimiento sin recurrencias.El abordaje diagnóstico de los pacientes con feocromocitoma comienza con la sospecha clínica, seguida de la medición de determinados metabolitos en sangre y orina, y, finalmente, los estudios de imagenología. Los desarrollos tecnológicos actuales permiten la aplicación de la medicina de precisión en este campo. En este caso de feocromocitoma, se identificó un componente genético importante que no solo afecta al paciente, sino también, a sus familiares. La tamización adecuada del caso índice permite identificar mutaciones y caracterizar mejor la enfermedad.

Pheochromocytoma is a tumor derived from neural crest cells able to produce sympathomimetic substances and, hence, a particular clinical picture. It is responsible for less than 1% of high blood pressure cases, with an estimated incidence between 0.4 and 0.6 cases per 100,000 people each year, and an average survival of seven years. Pheochromocytoma is a solid tumor with a high genetic component, as heritability can reach 40%. Once diagnosed, its treatment and prognosis are partly conditioned by the associated pathogenic variants that can be documented, especially those related to RET, SDHx, VHL, and NF1 genes.We present the case of a young woman with abdominal pain and high blood pressure, who was found to have a pheochromocytoma. Genetic testing detected a rare and recently discovered pathogenic variant: the SDHA:c.1A>C (p.Met1Leu). The patient responded adequately to the surgical treatment and continued the follow-up without documented recurrences.The diagnostic approach for pheochromocytoma patients must start with a clinical suspicion, followed by metabolite measurement in blood and urine, and finally, imaging. Currently, technology development allows precision medicine applicability. In this case of pheochromocytoma, recent developments in precision medicine resulted in the detection of associated genetic components involving the patient and her family. Adequate screening of the index patient is required for documenting pathogenic variants and better characterizing the disease.

Renal primitive neuroectodermal tumor in an adolescent: a case report and review of literature

Renal primitive neuroectodermal tumor is a highly malignant neoplasm that commonly affects young adults and infrequently presented in children and adolescents. We report a case of a 14-year-old female child who presented with abdominal mass. PET-CT showed an FDG avid cystic lesion in the inter and lower polar regions of the right kidney, extending into the right renal fat and renal vein, with multiple FDG avid lesions in the axial and appendicular skeleton, suggesting renal mass with bony metastases. Biopsy confirmed the diagnosis of primitive neuroectodermal tumor of the kidney. The patient completed six cycles of chemotherapy with partial response and subsequently the patient defaulted and lost to follow-up. To emphasize the critical significance of renal PNET in the differential diagnosis of renal tumors in children.

Tumor odontogénico epitelial calcificante en la rama mandibular. Informe de un caso clínico basado en una actualización bibliográfica / Calcifying epithelial odontogenic tumor in the mandibular ramus. A case report based on a literature update

Objetivo: Presentar un caso clínico de un tumor odon- togénico epitelial calcificante (TOEC), así como una revisión de la literatura disponible sobre esta neoplasia para contribuir al análisis del mejor método de tratamiento de la patología. Caso clínico: Se presenta el caso de una paciente mujer de 35 años con un tumor odontogénico epitelial calcifican- te que recibió tratamiento de enucleación quirúrgica con una evolución favorable y seguimiento de 5 años por medio de evaluación clínica y radiológica. La elección terapéutica se basó en el resultado de un análisis exhaustivo de la literatura para determinar el mejor abordaje de la neoplasia (AU)

Aim: To present a clinical case of a calcifying epithelial odontogenic tumor (CEOT), as well as a review of the availa- ble literature on this neoplasia to contribute to the analysis of the best treatment method for the pathology. Clinical case: The case of a 35-year-old patient with a calcifying epithelial odontogenic tumor who received surgical enucleation treatment with a favorable evolution and 5-year follow-up through clinical and radiological evaluation is pre- sented. The therapeutic choice was based on the result of an exhaustive analysis of the literature to determine the best ap- proach to the neoplasia (AU))

Linfangioma Oral en Paciente con Síndrome Koolen de Vries: Presentación de Caso y Revisión de Literatura

Los linfangiomas son tumores benignos hamartomatosos de los vasos linfáticos, originadas de un secuestro del saco linfático y agrandados por un inadecuado drenaje a la falta de comunicación con los canales linfáticos centrales o a la secreción excesiva de células de revestimiento. La incidencia de estos tumores en el sistema linfático es baja con una frecuencia de 1,2 a 2,8/1.000 en niños, sin predilección por sexo. En el territorio maxilofacial se pueden distinguir tres tipos de linfangioma: linfangioma simple, linfangioma cavernoso e higroma quístico o linfangioma quístico. Clínicamente estas lesiones se presentan como masas de tejido blando indoloras y de crecimiento lento. Su aspecto clínico depende de la extensión de la lesión. Diversos métodos de tratamiento para el linfangioma han sido reportados en la literatura, siendo la escisión quirúrgica la indicada, principalmente cuando estructuras vitales no están involucradas en la lesión. En este artículo se presenta un caso de una paciente femenina de 13 años con un aumento de volumen en el bermellón del labio superior, con antecedente de síndrome Koolen De Vries, a la cual se le realizó la exéresis de la lesión.

Lymphangiomas are benign hamartomatous tumors of the lymphatic vessels, originating from a sequestration of the lymphatic sac and enlarged by inadequate drainage, lack of communication with the central lymphatic channels or excessive secretion of lining cells. The incidence of these tumors in the lymphatic system is low, with a frequency of 1.2 to 2.8/1000 in children, with no predilection for sex. Three types of lymphangioma can be distinguished in the maxillofacial territory: simple lymphangioma, cavernous lymphangioma, and cystic hygroma or cystic lymphangioma. Clinically, these lesions present as painless, slow-growing soft tissue masses. Their clinical appearance depends on the extent of the lesion. Various treatment methods for lymphangioma have been reported in the literature, with surgical excision being indicated mainly when vital structures are not involved in the lesion. This article presents a case of a 13-year-old female patient with an increase in volume in the vermilion of the upper lip, with a history of Koolen De Vries syndrome, in which the excision of the lesion was performed.

Experiencia quirúrgica en el Centro Especializado en Tratamiento de Enfermedades de la Piel (CETEP) del Centro Hospitalario Pereira Rossell, desde 1996 hasta 2019 / Surgical experience at the Specialized Center for the Treatment of Skin Diseases (CETEP) at Pereira Rossell Hospital from 1996 to 2019 / Experiência cirúrgica no Centro Especializado en el Tratamiento de Enfermedades de la Piel (CETEP) do Hospital Pereira Rossell no período 1996 - 2019

Los tumores cutáneos presentan una alta prevalencia en dermatología en el mundo, siendo los benignos más frecuentes que los malignos; sin embargo, estos últimos son más estudiados debido a su morbimortalidad. El objetivo principal de este estudio fue conocer los principales diagnósticos y técnicas quirúrgicas correspondientes desarrolladas en el Centro de Tratamiento de Enfermedades de la Piel (CETEP) entre 1996 y 2019, evaluando aspectos clínicos y demográficos. Se realizó un estudio retrospectivo y observacional que incluyó todo paciente con lesión cutánea y posterior resolución quirúrgica de la misma. En la muestra analizada (N 6.659) hubo un predominio del sexo femenino (68%) y la media de edad fue 53 ± 21 años. Los pacientes residían mayoritariamente en Montevideo (58%). Los tumores benignos fueron los más frecuentes (41%), seguidos de los malignos (28%), dentro de éstos: carcinoma basocelular (CBC) 66%, carcinoma espinocelular (CEC) 21% y melanoma (MM) 5%. Las técnicas quirúrgicas realizadas fueron cirugías convencionales (57%), principalmente losange (93%), seguidas de procedimientos de cirugía dermatológica (42%), predominando biopsias (52%) y afeitado con electrocoagulación (23%). Se destaca que el CETEP resolvió un número mayor de pacientes de centros externos que del propio Centro Hospitalario Pereira Rossell (CHPR): 59% no CHPR vs 41% CHPR. En conclusión, este trabajo proporcionó información nacional sobre la epidemiología de distintos tumores cutáneos, así como las técnicas quirúrgicas más utilizadas en su resolución. Además, estableció la importancia de la cirugía dermatológica y la capacidad del CETEP en dar respuesta a pacientes propios tanto como referenciados desde otros centros del sistema público.

Cutaneous tumors have a high prevalence in dermatology worldwide, with benign tumors being more common than malignant ones. Nevertheless, the latter are more extensively studied due to their associated morbidity and mortality. The main objective of this study was to identify the primary diagnoses and corresponding surgical techniques developed at the Center for the Treatment of Skin Diseases (CETEP) between 1996 and 2019, while assessing clinical and demographic aspects. A retrospective, observational study was conducted, including all patients with cutaneous lesions and subsequent surgical resolution of the same. In the analyzed sample (N 6659), there was a predominance of females (68%), and the mean age was 53 ± 21 years. The majority of patients resided in Montevideo (58%). Benign tumors were the most prevalent (41%), followed by malignant tumors (28%), with the latter comprising basal cell carcinoma (BCC) at 66%, squamous cell carcinoma (SCC) at 21%, and melanoma (MM) at 5%. The performed surgical techniques included conventional surgeries (57%), primarily using the lozenge method (93%), followed by dermatologic surgery procedures (42%), with a predominance of biopsies (52%) and shave excision with electrocoagulation (23%). It is noteworthy that CETEP resolved a greater number of patients from external centers than from its own hospital, Pereira Rossell Hospital Center (CHPR). 59% non-CHPR vs. 41% CHPR. In conclusion, this study provided national information on the epidemiology of various cutaneous tumors, as well as the most commonly employed surgical techniques in their resolution Furthermore, it emphasized the importance of dermatologic surgery and highlighted the capacity of CETEP to respond to both its own patients and those referred from other centers within the public healthcare system.

Os tumores da pele apresentam alta prevalência na dermatologia em todo mundo, sendo os tumores benignos mais frequentes que os malignos, porém estes últimos são mais estudados devido à sua morbidade e mortalidade. O objetivo principal deste estudo foi conhecer os principais diagnósticos e correspondentes técnicas cirúrgicas desenvolvidas no Centro de Tratamento de Doenças da Pele (CETEP) no período 1996-2019, avaliando aspectos clínicos e demográficos. Foi realizado um estudo retrospectivo e observacional, que incluiu todos os pacientes com lesões cutâneas e com posterior tratamento cirúrgico. Foram estudados 659 pacientes com predomínio do sexo feminino (68%) e média de idade de 53 ± 21 anos. A maioria dos pacientes residiam em Montevidéu (58%). Os tumores benignos foram os mais frequentes (41%), seguidos dos tumores malignos (28%), entre estes: carcinoma basocelular (CBC) 66%, carcinoma espinocelular (CEC) 21% e melanoma (MM) 5%. As técnicas cirúrgicas realizadas foram cirurgias convencionais (57%), principalmente em forma de cunha (93%), seguidas de procedimentos cirúrgicos dermatológicos (42%), predominando biópsias (52%) e shaving com eletrocoagulação (23%). Destaca-se que o CETEP atendeu um número maior de pacientes de centros externos do que do próprio Centro Hospitalar Pereira Rossell (CHPR): 59% não-CHPR vs. 41% CHPR. Concluindo, este trabalho forneceu informações sobre a epidemiologia dos diferentes tumores de pele no país, bem como as técnicas cirúrgicas mais utilizadas no seu tratamento. Além disso, estabeleceu a importância da cirurgia dermatológica e a capacidade do CETEP de atender os pacientes do hospital e também os que foram encaminhados de outros centros da rede pública.

Fibroma ameloblástico mandibular: caso clínico / Fibroblastic fibroma mandibular: a clinical case

El fibroma ameloblástico (FA) se describe como una neoplasia benigna de origen odontogénico mixto que suele presentarse entre la primera y segunda década de vida, frecuentemente en los molares permanentes inferiores. Por lo general es asintomático, pero las lesiones de gran tamaño suelen acompañarse con dolor e inflamación. Su tratamiento por lo regular es conservador. Se describe el caso de un fibroma ameloblástico en un paciente de 13 años de edad, que involucraba cuerpo y ángulo mandibular izquierdo, tratado de manera conservadora, se realiza extirpación del tumor, regeneración ósea guiada y rehabilitación con implante dental (AU)

Ameloblastic fibroma (AF) is described as a benign neoplasm of mixed odontogenic origin that usually presents between the first and second decade of life, frequently in lower permanent molars. It is usually asymptomatic, but large lesions are usually accompanied by pain and inflammation. His treatment is generally conservative. The clinical case of an ameloblastic fibroma in a 13-year-old patient is described, involving the left mandibular body and angle, treated conservatively, tumor removal, guided bone regeneration and rehabilitation with dental implants are performed (AU)

Análisis del manejo en tumores presacros en adultos: Experiencia de los últimos 15 años en el Hospital Clínico de la Universidad de Chile

Antecedentes: Los tumores presacros son entidades frecuentes con diferentes etiologías, sin un abordaje quirúrgico establecido, habiendo relativamente poca experiencia en el manejo y en el seguimiento y resultados postoperatorios. Objetivo: Reportar el resultado en forma descriptiva del manejo de este tipo de lesiones en el hospital clínico de la Universidad de Chile. Material y Método: Estudio observacional, descriptivo y de carácter retrospectivo, de serie de casos, analizando la base de datos de cirugías del Hospital clínico de la Universidad de Chile en los últimos 15 años, excluyendo a pacientes con tumores óseos y operados por otros equipos. Se utilizo estadística simple y descriptiva para el análisis. Resultados: Se operaron 23 pacientes en los últimos 15 años, 12 por vía transabdominal, 9 resuelto por vía abierta y 3 por vía laparoscópica. Los restantes 11 fueron resuelto con abordaje posterior, siendo el de kraske usado en todos los pacientes. El seguimiento promedio fue de 16 meses. 4 pacientes registraron recurrencia Conclusiones: Los tumores presacros representan un desafío en términos de diagnóstico y manejo quirúrgico, siendo el estudio preoperatorio con imágenes más examen físico, determinante en el manejo quirúrgico del mismo. Es de considerar abordajes transabdominales en aquellos tumores mayores de 10 cms y en aquellos que se sospeche, fuertemente, malignidad, lo que no contraindica la vía laparoscópica. En caso de abordaje posterior, siempre descartar lesiones incidentales de recto, por lo que el examen anorrectal intraoperatorio debería estar de regla.

Background: Presacral tumors are frequent entities with different etiologies, without an established surgical management or approach, having relatively little experience in management and follow-up and postoperative results. Objective: To report the result in a descriptive way of the management of this type of lesions in the Clinical Hospital of the University of Chile. Material and Method: Observational, descriptive and retrospective study of case series, analyzing the database of surgeries of the Clinical Hospital of the University of Chile in the last 15 years, excluding patients with bone tumors and operated by other units. Simple and descriptive statistics were used for the analysis. Results: 23 patients were operated on in the last 15 years, 12 transabdominally. 9 resolved by open fashion and 3 laparoscopically. The remaining 11 patients was resolved with posterior approach, being the kraske used in all patients. The average follow-up was 16 months with 4 patients showing recurrence. Conclusions: The presacral tumors represent a challenge in terms of diagnosis and surgical management, being the preoperative study with images plus physical examination, determinant in the surgical management of the same. We recommend considering transabdominal approaches in those tumors larger than 10 cm and in those that strongly suspect malignancy, which does not contraindicate the laparoscopic route. In case of subsequent management, never rule out incidental lesions of the rectum, so the intraoperatory anorectal examination should be the rule.

"Penumbra sign" in knee pain: a case of distal femur osteomyelitis

"Penumbra sign" is a characteristic finding in magnetic resonance imaging (MRI) of Brodie's abscess, a rare variant of subacute osteomyelitis. We aimed to discuss the imaging finding penumbra sign that will help in the diagnosis of osteomyelitis and may be useful to clinicians in differential diagnosis. A 26-year-old male patient presented to the emergency department with complaints of pain and limping in the right knee that did not go away. He had a history of arthroscopic debridement and percutaneous fixation surgery due to osteochondral fragment 3 years ago. There were no additional findings in the patient's vital parameters, physical examination, and medical history. X-ray imaging revealed two screws in the distal femur and a well-defined sclerotic rim surrounding a radiolucent lesion anterior to the screws. MRI revealed a lesion in the distal femoral metaphysis with low-density fluid and hyperintense granulation tissue surrounding it. After surgical abscess drainage and local debridement, bone cement was placed in the resulting cavity. Teicoplanin treatment was started. The patient was discharged and complete recovery was achieved in the second month. The diagnosis of osteomyelitis is often missed or confused with bone tumors in non-traumatic cases presenting with persistent bone pain. MRI imaging is frequently used in differential diagnosis, and detection of characteristic imaging signs such as the penumbra sign accelerates the diagnosis. In this context, emergency department clinicians, in particular, should be cautious and not forget that early treatment can be started by recognizing these signs.

Expression of isocitrate dehydrogenase-1 in glioblastoma, Bangladesh perspective

Background: Glioblastoma is the most frequent malignant brain tumor in adults. Various studies have identified IDH (isocitrate dehydrogenase) mutation as a hallmark genetic alteration in glial tumors. The World Health Organization (WHO) has classified glioblastoma based on IDH mutation status, including IDH-mutant glioblastoma, IDH-wildtype glioblastoma along with its variants and glioblastoma, NOS (not otherwise specified) (where IDH mutation status cannot be evaluated). Methods: It was a cross-sectional observational study, conducted on 35 histologically diagnosed cases of glioblastoma, within the period of March, 2018 to December 2019. Results: Among the 35 glioblastoma cases, 6 (17.14%) were found to be IDH-mutant (positive for IDH1 immunostain), while the remaining 29 cases were negative for IDH1 immunostain (therefore designated as IDH-wildtype glioblastoma). In the IDH-mutant group, 3 out of 6 patients were in the younger age group (?40 years). On the other hand, IDH-wildtype glioblastoma was more common in elderly and most frequent was in the age group of 51-60 years (11 out of 29 cases). Conclusions: In this study, IDH1 expression was observed in 17.14% of all glioblastoma cases (designated as IDH-mutant glioblastoma). Whereas, most (~82.86%) of the glioblastoma cases did not express IDH1 (designated as IDH-wildtype).

Hidrocistomas ecrinos múltiples

RESUMEN Los hidrocistomas ecrinos son tumores benignos, únicos o múltiples. Se presentan con mayor frecuencia en la mediana edad y predominan en el sexo femenino. Se presenta un paciente con hidrocistomas ecrinos múltiples, masculino de 74 años.

ABSTRACT Eccrine hydrocystomas are benign tumors, which can be single or multiple. They occur most oftenly in middle aged patients or in the elderly, predominantly female. We present a case of multiple eccrine hydrocystomas, in a 74-year-old male patient.