Sebaceoma. Reporte de un caso / Sebaceoma. Case report

RESUMEN El sebaceoma es un tumor de las glándulas sebáceas. Vinculado a una posible alteración en la vía de señalización Wnt/beta-catenina. Se caracteriza clínicamente por ser una lesión exofítica, más comúnmente de aparición solitaria y amarillenta. Cuando en un mismo paciente los tumores son múltiples debemos descartar la presencia del síndrome de Muir-Torre, un trastorno asociado a la presencia de neoplasias malignas internas. En la histopatología, el diagnóstico diferencial se realizaprincipalmente con el adenoma sebáceo, teniendo en cuenta el porcentaje de células germinativas o inmaduras en los lóbulos. El tratamiento de elección es la extirpación quirúrgica, aunque también se puede emplear la electrodesecación, entre otros. Se decide reportar el caso de una paciente femenina con un tumor sebáceo de poca frecuencia, haciendo una revisión de las características clínicas e histopatológicas que nos ayuden a disminuir las dificultades en el diagnóstico.

ABSTRACT Sebaceoma is a rare sebaceous gland tumor, named by Troy and Ackerman in 1984. A possible alteration in the Wnt / beta-catenin signaling pathway has been linked to its etiology and would play an important role in genesis of some tumors, including sebaceous. It is clinically characterized by being an exophytic lesion, most commonly of a solitary, yellowish appearance, which appears in seborrheic areas, although the symptoms can be very heterogeneous. When tumors are multiple in the same patient, we must rule out the presence of Muir-Torre syndrome, an autosomal dominant disorder that is associated with the presence of internal malignancies. In Sebaceoma dermoscopy, an amorphous yellowish erythematous area is generally found, which suggests the sebaceous etiology of the lesion, but currently the definitive diagnosis can only be confirmed with histopathology. The differential diagnosis is made mainly with sebaceous adenoma, taking into account the percentage of germ cells or immature lobes, being less than 50% in sebaceous adenoma and more than 50% in sebaceoma, the latter also tends to be of greater size and depth. The treatment of choice is surgical removal, although electrodesiccation, cryotherapy, or curettage can also be used. It was decided to report the case of a female patient with an infrequent sebaceous tumor, making a review of the clinical and histopathological characteristics that help us decrease the difficulties in diagnosis.

Sebaceoma: Un tumor infrecuente en el conducto auditivo externo / Sebaceoma: an unusual tumor in the external auditory canal

El sebaceoma es un tumor cutáneo poco frecuente que presenta diferenciación sebácea, y se localiza en piel de cabeza y cuello. Puede asociarse con la presencia de tumores malignos, asociación conocida como síndrome de Muir-Torre, por lo que ante su diagnóstico se deben descartar. Su localización en la piel del conducto auditivo externo es muy infrecuente. Presentamos nuestra experiencia en el manejo de esta patología en un varón de 78 años de edad que presentaba una lesión nodular sólida en el conducto auditivo externo izquierdo con pérdida de audición y otorrea como síntomas asociados. Se recomienda la exéresis completa de la lesión, como tratamiento de elección con fines tanto diagnósticos como terapéuticos.

Sebaceoma is a rare benign cutaneous tumor with sebaceous differentiation and it is typically located on the skin of the head and neck. This pathology made appear in association with malignant tumors (known as Muir-Torre syndrome) and must be ruled out. The location in the external auditory canal is very unusual. We present our experience in managing this pathology in a 78-year-old man who complains of hearing loss and otorrhea and presents a solid nodule in the left external auditory canal. Complete surgical removal was performed, as the choice treatment for diagnosis and therapeutic care.

Case for diagnosis. Verrucous plaque on the pubic region

Abstract Muir-Torre syndrome is a rare, autosomal dominant genodermatosis, characterized by sebaceous neoplasms and visceral carcinomas. The authors describe the case of a patient who, 16 years after the diagnosis of colon carcinoma, presented a verrucous plaque on the pubic region, histopathologically compatible with sebaceous adenoma. The need to investigate this syndrome is emphasized, especially in cases of sebaceous neoplasms located outside the head, face, and neck. Screening for neoplasms in these patients and their families is mandatory.

Carlos de la Torre y Huerta. Paradigma del quehacer científico cubano / Carlos de la Torre y Huerta. Paradigma of Cuban scientific activity

RESUMEN La dimensión científica educativa constituye uno de los aportes más sólidos de la obra de Carlos de la Torre y Huerta. Su extensa obra investigativa se nutre de las asignaturas que impartió en la Universidad de la Habana entre las que se desatacan Paleontología, Arqueología, Antropología, Zoología, Geología, Biología y Entomología. Estos estudios favorecieron el descubrimiento de la gran riqueza existente en el país, destacándose una trayectoria ejemplar cuyos cimientos se insertan en la malacología. El descubrimiento de las Polymitas lo llevan a describir una diversidad de subespecies que lo insertan en el escenario internacional aclamado como un referente de obligatoria consulta para los especialistas. La divulgación sistemática de su obra lo conducen a la formación de una escuela cubana de naturalistas en la que los discípulos marcaron impronta en el quehacer científico nacional al apropiarse de las herramientas que le permiten hacer ciencia utilizando medios de enseñanza, imágenes proyectadas, láminas, dibujos y ejemplares autóctonos del país (AU).

ABSTRACT The educational scientific dimension is one of the strongest contributions of the work of Carlos de la Torre and Huerta. His extensive research work draws on the subjects he taught at the University of Havana, including Paleontology, Archeology, Anthropology, Zoology, Geology, Biology and Entomology. These studies favored the discovery of the great wealth existing in the country, highlighting an exemplary trajectory whose foundations are inserted in malacology. The discovery of the Polymites leads him to describe a diversity of subspecies that insert him in the acclaimed international scenario as a mandatory reference for specialists. The systematic dissemination of his work lead him to the formation of a Cuban school of naturalists in which the disciples marked imprint on the national scientific work by appropriating the tools that allow him to do science using teaching means, projected images, prints, drawings and native copies of the country (AU).

Sebaceoma: hallazgos dermatoscópicos a propósito de dos casos / Sebaceoma: dermatoscopic findings at purpose of two cases

El sebaceoma es una neoplasia sebácea benigna inusual, que inicialmente se denominaba epitelioma sebáceo, lo que generaba confusión respecto a su biología tumoral, dado que histopatológicamente no presenta diferenciación suficiente como el adenoma sebáceo y tampoco es tan indeferenciado como el carcinoma sebáceo. Su diagnóstico precoz tiene gran relevancia, dado que, junto con el adenoma sebáceo y el carcinoma sebáceo, tienen una asociación directa con el síndrome de Muirr-Torre y, por lo tanto, con el desarrollo de cáncer de colon, endometrio, entre otros. En este punto, el uso de la dermatoscopia es muy importante. Considerando los pocos reportes en la literatura sobre la dermatoscopia en sebaceomas, presentamos dos casos clínicos en adultos, donde se destaca la presencia de estructuras amarillas homogéneas y vasos en corona o arboriformes.

Sebaceoma is an unusual benign sebaceous neoplasm, initially known as sebaceous epithelioma, which generated confusion regarding its tumor biology, given that it is histopathologically less differentiated than sebaceous adenoma, but with a higher differentiation than sebaceous carcinoma. Early diagnosis becomes relevant, given that together with sebaceous adenoma and sebaceous carcinoma, there is a direct association with Muirr-Torre syndrome and therefore the development of colon and endometrial cancer, among others. Because of this, the use of dermatoscopy becomes very important. Given the few reports in the literature on dermatoscopy in sebaceomas, we present two clinical cases, where the presence of homogeneous yellow structures and crown or arboriform vessels stands out.

Out of the office and into the field: Exploring neuropsychological correlates between search behavior and a traditional desktop task in children and adolescents with ADHD / Del consultorio a la vida cotidiana: Una exploración de los correlatos neuropsicológicos entre una prueba tradicional de escritorio y de la conducta de búsqueda en niños y adolescentes con TDAH

Abstract Introduction Cognitive assessment of patients with attention deficit hyperactivity disorder (ADHD) can help clinicians provide individually tailored treatment and advice, and researchers to identify potential associations between psychopathology and specific cognitive deficits. Assessment instruments, however, have received some criticism regarding their ecological validity, that is, the capacity to extrapolate from the performance on such tasks to aspects of everyday functioning. In order to meet this challenge we developed the Ball Search Field Task (BSFT) that takes place outdoors and uses large, open areas. In the BSFT, the goal is to search for target objects hidden under opaque containers, with experimenters assessing the efficiency of participants' strategies to collect a maximum of these. Objective Here we explore how the measures produced by one of the latest versions of this task (the patchy BSFT) match up with a traditional desktop task often used in clinical environments, the Tower of London (ToLo). Method We applied the BSFT and ToLo to children and adolescents with ADHD and compared the metrics using Spearman correlations. Results We found significant, moderate correlations between instruments, as exemplified by that of balls collected per cones lifted (BSFT) and number of moves (ToLo) (r = -.44). Discussion and conclusion Matching correlates between the BSFT and ToLo suggest these tasks may be tapping into similar cognitive processes. The addition of assessment tools with ecological validity may help provide a more comprehensive evaluation and a better understanding of the day-to-day impact of cognitive afflictions underlying psychiatric disorders such as ADHD.

Resumen Introducción La evaluación cognitiva de pacientes con déficit de atención e hiperactividad (TDAH) puede ayudar al personal clínico a personalizar el tratamiento y a los investigadores a identificar asociaciones entre psicopatología y deficitarios cognitivos específicos. Los instrumentos de evaluación han recibido críticas en cuanto a su validez ecológica, esto es, la capacidad de extrapolar el desempeño en dichos instrumentos a situaciones de la vida diaria. Con este desafío en mente, desarrollamos la Prueba de Búsqueda de Pelotas (BSFT, por sus siglas en inglés) que se lleva a cabo en áreas abiertas y amplias. La BSFT consiste en buscar objetos escondidos bajo contenedores opacos para evaluar la eficiencia de la búsqueda que intenta encontrar el mayor número posible de objetos. Objetivo Exploramos la manera en que una versión de esta tarea (la BSFT en parches) se compara con una tarea de uso común en ambientes clínicos, la Torre de Londres (ToLo, por sus siglas en inglés). Método Aplicamos la BSFT y la ToLo a niños y adolescentes con TDAH y comparamos las métricas resultantes mediante una correlación de Spearman. Resultados Encontramos correlaciones significativas entre estas pruebas, como lo ejemplifica aquella entre el número de conos levantados (BSFT) y el número de secuencias correctas (ToLo) (r = -.48). Discusión y conclusión Correlatos de equivalencia entre la BSFT y la ToLo sugieren que estas tareas demandan procesos cognitivos similares. Investigar tareas con validez ecológica puede ayudarnos a ofrecer una evaluación más completa y a entender mejor el impacto diario de las afectaciones cognitivas subyacentes a trastornos psiquiátricos como el TDAH.

Do we Plan through Gestures? Evidence from Children, Adolescents and Adults in Solving of Tower of Hanoï Task / ¿Planeamos a través de los gestos? Evidencia en niños, adolescentes y adultos en la resolución de la tarea de la Torre de Hanoï

Abstract The aim of this research was to study the planning of the execution of the Tower of Hanoï task (TOH) through gesture and speech. The effects of age and task complexity on gestures-speech mismatches were analyzed in 144 participants (48 children from 8 to 10 years old, 48 adolescents from 12 to 14 years old, and 48 adults from 18 to 20 years old) during their early explanations of the solution to the problem of the TOH. Results suggested effects from task complexity but not from age. Gesture-speech mismatches could be a possible way to analyze early explanations of the tasks, and the level of difficulty could be considered as a developmental indicator. The question of the relationship between gestures and speech during the planning of complex problems is in fact at the center of a passionate debate on the close relationship between thought and language. It is also at the heart of research on multimodal communication and thinking, according to which human cognition is based on verbal and nonverbal aspects of communicative behavior.

Resumen El objetivo de esta investigación fue estudiar la planificación de la ejecución de la tarea de la Torre de Hanoi (TOH) a través de los gestos y la palabra. Se analizaron los efectos de la edad y la complejidad de la tarea en las discordancias gestos-palabras en 144 participantes (48 niños de 8 a 10 años, 48 ​​adolescentes de 12 a 14 años y 48 adultos de 18 a 20 años) durante sus explicaciones anticipadas a la resolución de TOH. Los resultados sugieren efectos de la complejidad de la tarea, pero no de la edad. Las discordancias gestos-palabras podrían constituirse en una manera posible de analizar explicaciones anticipadas a la resolución efectiva de las tareas, y el nivel de dificultad podría ser considerado como un indicador de desarrollo. La pregunta de la relación entre los gestos y las palabras durante la planificación de problemas complejos es, de hecho, el centro de un apasionado debate sobre la estrecha relación entre pensamiento y lenguaje. También está en el centro de la investigación sobre la comunicación y el pensamiento multimodales, según la cual la cognición humana se basa en los aspectos verbales y no verbales del comportamiento comunicativo.

Neoplasias de origen sebáceo asociadas a Síndrome de Muir-Torre. A propósito de un caso / Sebaceous neoplasias associated to Muir-Torre syndrome. A propos of a case

El Síndrome de Muir-Torre (SMT) descrito desde 1967, es una genodermatosis con herencia autosómica dominante y penetrancia variable, caracterizada por la presencia de neoplasias cutáneas de origen sebáceo manifestado en áreas seborreicas, como la cara y cuero cabelludo. Los adenomas son los tumores sebáceos más frecuentes. Se han descrito también hiperplasias, neoformaciones sebáceas quísticas, epiteliomas o carcinoma. Los mismos pueden presentarse de manera aislada o múltiple; en conjunto con al menos una neoplasia visceral maligna. Entre las descritas con frecuencia están las gastrointestinales. Se pueden asociar a otras neoplasias, como las renales, las de endometrio, útero y de laringe. El diagnóstico clínico está dado por la presencia y asociación de un tumor visceral primario y otro de origen sebáceo. El tratamiento de esta entidad se basa en la exéresis de los tumores y el seguimiento especializado según el sistema afectado. Se presentó el caso de un hombre de 62 años de edad, cuya afección cutánea demostró múltiples lesiones de origen sebáceo de siete años de evolución por lo que se le realizaron complementarios para identificar entidades asociadas (AU).

The Muir-Torre syndrome, described since 1967, is a genodermatosis with dominant autosomal inheritance and variable penetrance, characterized by the presence of sebaceous skin neoplasias manifested in seborrheic areas as the face and scalp. Adenomas are the most frequent sebaceous tumors. Hyperplasia, cystic sebaceous neoformations, epitheliomas or carcinomas have been also described. They could appear in an isolated or multiple way, in all with at least one malignant visceral neoplasia. The gastrointestinal ones are among those frequently described. They might be associated to other neoplasias, as the renal ones, or the ones of the endometrium, the uterus and the larynx. The clinical diagnosis is given by the presence and association of one primary visceral tumor and another of sebaceous origin. The treatment of this entity is based on the tumors resection and the specialized follow-up according to the affected system. The case of a man, aged 62 years, is presented; his cutaneous condition showed multiple sebaceous lesions of seven years of evolution. Complementary exams were carried out for identifying associated lesions (AU).

Carcinoma sebáceo asociado al síndrome de Muir-Torre: a propósito de un caso / Sebaceous carcinoma associated with Muir-Torre syndrome: report of a case

El Carcinoma Sebáceo (CS) es una neoplasia maligna, poco frecuente, formada por células que muestran una diferenciación hacia el epitelio sebáceo. Generalmente, se presenta en promedio a los 70 años de edad, es más frecuente en mujeres y principalmente en raza asiática. Presentamos el caso de una paciente de sexo femenino de 69 años, con antecedentes familiares de múltiples cánceres, cáncer de mama y la aparición de numerosos adenomas y carcinomas sebáceos, cuadro compatible con un Síndrome de Muir-Torre (SMT). El 75% de los CS se localizan en la región ocular y el 25% es de ubicación extra-ocular. El de ubicación ocular, se caracteriza por un comportamiento agresivo, con diseminación directa y alto potencial de metástasis regional y a distancia. Los tumores extraoculares generalmente son menos agresivos y se localizan principalmente en cabeza y cuello. La patogénesis es incierta, pero los procesos inflamatorios crónicos, la radiación ultravioleta y la radioterapia favorecerán su desarrollo. El CS es un tumor versátil, que se presenta con diferentes aspectos clínicos y distintos patrones histológicos de crecimiento, que retardan el diagnóstico definitivo. El CS es uno de los marcadores cutáneos diagnósticos del SMT, que es una enfermedad genética asociada a neoplasias viscerales. Por lo tanto, los pacientes con CS deben ser cuidadosamente valorados, realizándose una historia oncológica personal y familiar adecuada. El tratamiento de elección es la cirugía con márgenes amplios.

The Sebaceous Carcinoma (SC) is an uncommon malignant tumor formed by cells that show differentiation toward sebaceous epithelium. It usually occurs in women of average at 70 years of age. We report a case of a 69 years old female with a family history of multiples cancer, breast cancer and the emergence of multiple sebaceous adenomas and carcinomas, features consistent with Syndrome Muir Torre (SMT). The 75% of the SC are located in the ocular region and the 25% rest presents extraocular location. The eye location is characterized by aggressive behavior, with direct extension and high potential for regional and distant metastases. Extraocular are generally less aggressive tumors and are located mainly in the head and neck. The pathogenesis is unclear, but chronic inflammatory processes, ultraviolet radiation and radiation will favor its development. The SC is a versatile tumor that presents with different clinical and histological different growth patterns, which retard the definitive diagnosis. SC is one of the cutaneous diagnostic markers of SMT, which is a genetic disease associated with visceral neoplasms. They are carefully assessed, performing a proper personal and family cancer history. The treatment of choice is the surgery with wide margins.

Syndrome in question

AbstractMuir-Torre syndrome is a rare genodermatosis characterized by the occurrence of at least one sebaceous tumor associated with visceral neoplasia, but with no predisposing factors. The sebaceous neoplasm may appear before, during or after the diagnosis of colorectal cancer. As it is regarded as a subtype of nonpolyposis hereditary colorectal cancer, it is important to evaluate the patient's first-degree relatives. The clinical course of the neoplasm is usually more indolent and the syndrome has a good prognosis. We report the case of a patient who, after a ten-year diagnosis of colorectal cancer, presented with multiple sebaceous neoplasms.

The role of immunohistochemistry in the Muir-Torre Syndrome

Muir-Torre Syndrome is defined by the coexistence of sebaceous skin tumors and internal malignancies. Mutations in the DNA mismatch repair genes are found in the inherited form of the disease, resulting in the absence of crucial enzymes involved with DNA replication process. This case describes a patient with sebaceous adenoma and colorectal carcinoma, meeting the criteria for Muir-Torre Syndrome. The immunohistochemical analysis of the skin lesion was an important tool to confirm the diagnosis, as it revealed nuclear negativity for MSH2 and MSH6.

A Case of Muir-Torre Syndrome Confirmed by Genetic Mutation Analysis / 대한피부과학회지

No abstract available.

Muir-Torre Syndrome: case report and molecular characterization / Sindrome de Muir-Torre: relato de caso e caracterizacao molecular

CONTEXT: Muir-Torre syndrome is a rare autosomal dominant genodermatosis caused by mutations in the mismatch repair genes. It is characterized by the presence of sebaceous skin tumors and internal malignancies, affecting mainly the colon, rectum and urogenital tract. Awareness of this syndrome among physicians can lead to early diagnosis of these malignancies and a better prognosis. CASE REPORT: We report the case of a Chilean patient who, over the course of several years, had multiple skin lesions, endometrial cancer and colon cancer. The syndrome was diagnosed using molecular techniques such as microsatellite instability analysis, immunohistochemistry and DNA sequencing, which allowed us to find the causative mutation. CONCLUSION: Molecular diagnostics is a highly useful tool, since it allows clinicians to confirm the presence of mutations causing Muir-Torre syndrome. It is complementary to the analysis of the clinical data, such as dermatological presentation, presence of visceral malignancies and family history of colorectal tumors, and it provides important knowledge to help physicians and patients choose between treatment options. .

CONTEXTO: A síndrome de Muir-Torre é uma genodermatose autossômica dominante rara causada por mutações nos genes de reparo de incorreções. Caracteriza-se pela presença de tumores sebáceos da pele e doenças malignas internas, afetando principalmente cólon, reto e trato urogenital. A consciência desta síndrome pelos médicos pode levar ao diagnóstico precoce dessas doenças malignas e a um melhor prognóstico. RELATO DE CASO: Relatamos o caso de uma paciente chilena que, ao longo de vários anos, teve lesões cutâneas múltiplas, câncer de endométrio e câncer de cólon. A síndrome foi diagnosticada com técnicas moleculares, como a análise de instabilidade de microssatélites, imunoistoquímica e sequenciamento de DNA, o que nos permitiu encontrar a mutação causadora. CONCLUSÃO: Diagnóstico molecular é uma ferramenta muito útil, uma vez que permite que os clínicos confirmem a presença de mutações causadoras de síndrome de Muir-Torre. É complementar para a análise dos dados clínicos, tais como a apresentação dermatológica, a presença de doenças malignas viscerais e história familiar de tumores colorrectais, e fornece conhecimentos importantes para ajudar os médicos e os pacientes a escolher entre opções de tratamento. .

A case of Muir-Torre syndrome presenting as keratoacanthoma and sebaceous adenoma / 中华皮肤科杂志

A 44-year-old male presented with a neoplasm on the buccal side of the right nasolabial fold for more than two months.Dermatological examination showed a hemispherical bulge sized 1.5 cm × 1.5 cm with central crater-like ulceration on the buccal side of the right nasolabial fold,as well as a crescent-shaped elevation measuring 1.5 cm × 2.5 cm above the hemispherical lesion.Histopathology of the hemispherical lesion revealed irregularly downward proliferation of epidermis,crater-like holes filled with eosinophilic keratinous plug in the center which were surrounded by collar-shaped epithelial cell projections.Small neutrophil abscesses were found in the clumps of epithelial cells,and massive lymphocyte infiltration with a clear bottom boundary was observed around the proliferating epithelial cells.Histopathologic examination of the crescent lesion showed multiple irregularly-shaped lobular-like structures of various sizes with sebaceous glands at different degrees of maturity in the mid dermis,which were surrounded by proliferating connective tissue.Immunohistochemical studies showed that the squamous cells stained positive for cytokeratin (CK),CK5,CK14,CK17,carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA) in the keratoacanthoma,and the sebaceous cells for CK,CK5,CK14 and EMA in the sebaceous adenoma.The pathological diagnosis was keratoacanthoma and sebaceous adenoma.The patient was diagnosed with moderately and poorly differentiated rectal adenocarcinoma in 2008.A diagnosis of Muir-Torre syndrome presenting as keratoacanthoma and sebaceous adenoma was finally made.

Historical review of Lynch syndrome / Revisão histórica da síndrome de Lynch

Lynch syndrome was formerly known as Hereditary Nonpolyposis Colorectal Cancer. Currently, these two nomenclatures each have their unique definitions and are no longer used interchangeably. The history of hereditary nonpolyposis colorectal cancer was first recognized formally in the literature by Henry Lynch in 1967. With advances of molecular genetics, there has been a transformation from clinical phenotype to genotype diagnostics. This has led to the ability to diagnose affected patients before they manifest with cancer, and therefore allow preventative surveillance strategies. Genotype diagnostics has shown a difference in penetrance of different cancer risks dependent on the gene containing the mutation. Surgery is recommended as prevention for some cancers; for others they are reserved for once cancer is noted. Various surveillance strategies are recommended dependent on the relative risk of cancer and the ability to intervene with surgery to impact on survival. Risk reduction through aspirin has shown some recent promise, and continues to be studied. (AU)

A síndrome de Lynch era anteriormente conhecida como "câncer colorretal hereditário não polipose". Atualmente, essas duas nomenclaturas têm, cada uma, sua própria definição original e já não são empregadas de forma intercambiável. O histórico de câncer colorretal hereditário não polipose foi formalmente reconhecido pela primeira vez na literatura por Henry Lynch em 1967. Com os avanços da genética molecular, verificou-se uma mudança do fenótipo clínico para o diagnóstico genotípico. Esse fato levou à capacidade de diagnosticar pacientes afetados antes que o câncer se manifestasse, e, portanto, à utilização de estratégias preventivas de rastreamento. O diagnóstico genotípico mostrou a diferença na penetrância de diferentes riscos de câncer dependendo do gene que contem a mutação. A cirurgia é recomendada para a prevenção de alguns tipos de câncer; para outros, ela é reservada quando há o aparecimento da doença. Várias estratégias de rastreamento são recomendadas, dependendo do risco relativo de câncer, bem como a capacidade para intervir com a cirurgia objetivando um impacto na sobrevivência. A redução do risco através do uso de aspirina recentemente mostrou ser promissor e continua a ser estudada. (AU)

Sebaceous Carcinoma Associated with Breast Cancer, Stomach Cancer, and Colon Cancer: Muir-Torre Syndrome

Muir-Torre syndrome is defined by concurrent or sequential development of internal malignancy and sebaceous neoplasm or multiple keratoacanthomas. Muir-Torre syndrome is very rare, with only 205 cases reported in the literature. We reported a patient with Muir-Torre syndrome with three internal malignancies. A 64-year-old patient with a history of breast cancer, stomach cancer and colon cancer visited our department for treatment of the skin lesion that occurred five years before on the left cheek. The lesion was excised completely with a resection margin of 1 cm, followed by full-thickness skin graft from left postauricular area for reconstruction. Histopathology revealed a 0.2 x 0.2 x 0.1 cm sized sebaceous carcinoma with 4 mm safety margin. The skin graft was well taken within 7 days after surgery and the patient was discharged to outpatient follow-up. There was no complication related with surgery. Muir-Torre syndrome is very rare, as are sebaceous gland tumors. So if a cancer of the sebaceous gland is diagnosed, screening workup for internal malignancy is recommended. Because of its good prognosis, surgical removal of primary or metastatic cancers may be curative and should be attempted where possible.

Adenoma sebáceo en un paciente con síndrome de Muir-Torre / Sebaceous adenoma in a patient with a Muir-Torre syndrome

El síndrome de Muir-Torre comprende la asociación de uno o más tumores de glándulas sebáceas, con una o más malignidades internas y antecedentes familiares de neoplasias. Presentamos el caso de un paciente masculino de 33 años, con antecedentes familiares de cáncer de colon, que consulta a nuestro servicio por lesión en cuero cabelludo de seis meses de evolución, con posterior diagnóstico histopatológico de adenoma sebáceo. Dentro de los exámenes complementarios se observan por colonoscopía, dos lesiones que correspondieron a adenocarcinomas de colon bien diferenciados. Con los antecedentes familiares, el examen clínico y la histopatología se efectúa el diagnóstico de síndrome de Muir-Torre.

Muir-Torre syndrome represents the association of one or more sebaceous tumors, with one or more visceral malignancies and a family history of cancer. We report the case of a 33-year-old man, with a family history of colon cancer, who presented with a scalp tumor. The histopathological diagnosis was sebaceous adenoma. Two lesions were found in the colonoscopy, which corresponded to colon adenocarcionomas. With a compatible personal and family history of cancer and the results of the histopathology, we diagnosed the patient as Muir-Torre syndrome.

Síndrome de Muir-Torre: a propósito de dos casos / Muir-Torre syndrome: speaking of two cases

El síndrome de Muir-Torre (SMT) es una entidad que agrupa a lo menos una neoplasia sebácea y una neoplasia visceral. Es un trastorno genético que involucra un fallo en la transcripción de proteínas de reparación de ADN. Es poco frecuente y habitualmente el diagnóstico requiere una búsqueda exhaustiva de las neoplasias asociadas. El tratamiento de las lesiones cutáneas es la extirpación completa de la lesión, pero requiere un control continuo para detectar a tiempo recurrencias, nuevas neoplasias y posibles metástasis. Se presenta el reporte de dos casos, que presentan todas las características clínicas del SMT, junto con una revisión bibliográfica sobre el tema.

Muir-Torre syndrome (MTS is an entity comprising sebaceous neoplasm and visceral malignancy. It is a genetic disorder that involves a failure in the transcription of DNA repair proteins. It is a uncommon disease and usually the diagnosis requires an exhaustive search of associated neoplasm. The treatment of skin neoplasm is the complete removal of the lesion, but it requires continuous monitoring to detect early recurrence, new neoplasias and possible metastasis. We report two cases, which illustrate all the clinical characteristics of MTS, together with a literature review on the subject.

A Case of Muir-Torre Syndrome

PURPOSE: To present a rare case of Muir-Torre syndrome characterized by the association of sebaceous skin tumors and systemic malignancies. CASE SUMMARY: A 65-year-old female visited our clinic with an irregular nodular mass of the right lower eyelid, which developed 1 year earlier. An excisional biopsy and lower lid reconstruction with Tenzel's semicircular rotational flap was performed under local anesthesia. Histopathologic examination showed well-differentiated sebaceous cells, consistent with sebaceous adenoma. The patient had undergone total abdominal hysterectomy and lower anterior resection due to endometrial cancer and sigmoid colon cancer 5 years before, and nephroureterectomy due to papillary urothelial carcinoma 3 years before. Based on the history of systemic malignancy and sebaceous skin cancer, a diagnosis of Muir-Torre syndrome was made. CONCLUSIONS: When a sebaceous gland tumor of the eyelids is detected, Muir-Torre syndrome should be included in the differential diagnosis, and systemic work-up for the internal malignancy must be performed.

Tabanidae (Diptera) no dossel da floresta amazônica atraídos por luz e descrição de machos de três espécies / Tabanidae (Diptera) in the canopy of the Amazon rainforest attracted by light and description of males of three species

Foram realizadas coletas de tabanídeos a 40 metros de altura, em uma torre metálica, na Estação Experimental de Silvicultura Tropical, Amazonas, Brasil. As coletas foram realizadas de janeiro a dezembro de 2004, durante três noites de transição lunar minguante/nova de cada mês, das 18 às 6 horas. As mutucas foram capturadas em um lençol iluminado com lâmpada de luz mista de vapor de mercúrio de 250 watts e lâmpada de 20 watts BLB. Foram coletados 216 espécimes, dos quais 135 machos e 81 fêmeas, alocados em 29 espécies. Três machos desconhecidos são descritos pela primeira vez: Catachlorops halteratus Kröber, 1931, Leucotabanus janinae Fairchild, 1970 e Leucotabanus pauculus Fairchild, 1951.

Tabanids were collected at a height of 40 meters on a metallic tower, at Estação Experimental de Silvicultura Tropical, Manaus, Amazonas, Brazil. Samples were collected monthly from January to December of 2004, during three nights of lunar transition third quarter moon/ new moon from 06 p.m. to 06 a.m. Horse flies were captured on a vertical sheet illuminated by a 250-watts mixed mercury vapor light and 20-watts black-light (BLB) lamps. 216 specimens were collected, of which 135 were males and 81 were females belonging to 29 species. Males of three species with unknown males are described for the first time, namely: Catachlorops halteratus Kröber, 1931, Leucotabanus janinae Fairchild, 1970 and Leucotabanus pauculus Fairchild, 1951.