Primary Anorectal Melanoma: Case Report and Review of a Rare Neoplasm

Primary anorectal melanoma is a rare malignant melanocytic neoplasm; its principal manifestation is rectal bleeding. It has an ominous prognosis with a five-year survival rate of 10%. The case of a 56-year-old woman with rectal bleeding and the sensation of a rectal mass is presented. A polypoid lesion, resected transanally, was documented in the distal rectum during the colonoscopy. The histological study confirmed a primary anorectal melanoma.

El melanoma anorrectal primario es una neoplasia melanocítica maligna poco frecuente, su principal manifestación es el sangrado rectal. Tiene un pronóstico ominoso con una tasa de sobrevida del 10% a 5 años. Se presenta el caso de una mujer de 56 años con rectorragia y sensación de masa rectal. Durante la colonoscopia se documentó una lesión polipoide en el recto distal, que se resecó por vía transanal. El estudio histológico confirmó la presencia de un melanoma anorrectal primario.

Melanoma maligno cutáneo con metástasis sistémica en una hembra de tigre de bengala blanca (Panthera tigris tigris) / Cutaneous Malignant Melanoma with Systemic Metastasis in a Female White Bengal Tiger (Panthera tigris tigris)

Resumen La presencia del melanoma maligno cutáneo en felinos exóticos está pobremente reportada. Una hembra de tigre de Bengala blanca (Panthera tigris tigris), de 6 años, no castrada, solitaria y viviendo en un ambiente adecuado, fue evaluada por la presencia de una masa cerca del ojo izquierdo. La histopatología de la biopsia remitida mostró la presencia de melanocitos con características anaplásicas (pleomorfismo, citomegalia, cariomegalia, binucleación), área hemorrágica y foco inflamatorio fibrinoso. El diagnóstico histopatológico fue melanoma maligno. Dos meses después del diagnóstico, la tigresa manifestó un deterioro considerable con pérdida de peso y presencia de una masa redonda en el cuello. En aquel momento, por su tamaño, el melanoma obstruía totalmente la visión. Con base en esto, y por razones humanitarias, el animal fue eutanasiado. Al momento de la necropsia, se observó metástasis en pulmones, pleura y riñones. Este caso evidencia la importancia de dar a conocer la presentación de melanomas en especies poco reportadas.

Abstract The occurrence of cutaneous malignant melanoma in exotic felids is poorly reported. A 6-year-old, unneutered, solitary, female white Bengal tiger (Panthera tigris tigris), living in a suitable environment, was evaluated for the presence of a mass near the left eye. Histopathology of the referred biopsy showed the presence of melanocytes with anaplastic features (pleomorphism, cytomegaly, karyomegaly, binucleation), hemorrhagic area and fibrinous inflammatory focus. The histopathological diagnosis was malignant melanoma. Two months after diagnosis, the tigress manifested considerable deterioration with weight loss and the presence of a round mass on the neck. At that time, because of its size, the melanoma totally obstructed vision. Based on this, and for humane reasons, the animal was euthanized. At the time of necropsy, metastasis was observed in the lungs, pleura and kidneys. This case demonstrates the importance of raising awareness of melanomas in underreported species.

Melanoma maligno primario de pulmón / Primary malignant lung melanoma

Introducción: El melanoma maligno es un tumor de etiología multifactorial, cuando afecta al sistema respiratorio su origen es casi siempre metastásico, y como tumor primario es muy infrecuente, constituye el 0,07 por ciento de los tumores pulmonares malignos. Objetivo: Presentar un caso clínico con diagnóstico de melanoma maligno primario de pulmón. Presentación del caso: Se presenta el caso de una mujer de 64 años de edad, exfumadora, sin antecedentes de melanoma, que presenta tos seca asociada a disnea, los esfuerzos físicos y dolor torácico. Además de astenia y pérdida de peso. Se le realizó biopsia pulmonar mediante broncoscopia y se le diagnosticó melanoma. Conclusiones: El melanoma primario pulmonar es uno de los más raros del tipo de melanoma visceral, es una entidad neumológica infrecuente y el diagnóstico preciso requiere una investigación detallada y el cumplimiento de criterios clínicos y anatomopatológicos específicos(AU)

Introduction: Malignant melanoma is a tumor of multifactorial etiology, when it affects the respiratory system its origin is almost always metastatic, and as a primary tumor it is very infrequent, it constitutes 0.07 por ciento of malignant lung tumors. Objective: To report a clinical case with a diagnosis of primary malignant melanoma of the lung. Case report: We report the case of a 64-year-old woman, former smoker, with no history of melanoma, who had dry cough associated with dyspnea with physical exertion and chest pain. In addition to asthenia and weight loss, she had a lung biopsy by bronchoscopy and was diagnosed with melanoma. Conclusions: Primary pulmonary melanoma is one of the rarest of the visceral melanoma type, this is an infrequent pneumological entity and the precise diagnosis requires detailed investigation and the fulfillment of specific clinical and pathological criteria(AU)

Melanoma amelanótico diseminado / Disseminated amelanotic melanoma

El melanoma amelanótico es una de las neoplasias con mayor índice de mortalidad por su alta agresividad y baja probabilidad diagnóstica. Afecta a la población de todo el orbe, más frecuente en caucásicos, con predisposición genética y factores de riesgo como la exposición al sol. Presenta tasas de supervivencia menor a 10 por ciento a 5 años y de recurrencia elevadas; con evidencia de procesos metastásicos a distancia en órganos como cerebro, tejido celular subcutáneo, pulmón, peritoneo, hueso, lo que ensombrece el pronóstico. Se presenta el caso de una paciente de 21 años de edad que acude al hospital por presentar lesiones equimóticas, nódulos subcutáneos y cefalea hemicránea izquierda de dos meses de evolución. Se le realizó tomografías de tórax abdomen y resonancia magnética de cráneo y evidenciaron diseminación metastásica. Se realizó estudio histopatológico con inmunohistoquímica que informó melanoma amelanótico(AU)

Amelanotic melanoma is one of the neoplasms with the highest mortality rate because it is highly aggressive and the diagnostic probability is low. It affects the population of the entire globe, more frequent in Caucasians, with genetic predisposition and risk factors such as sun exposure. It presents survival rates of less than 10 percent at 5 years and high recurrence rates; with evidence of distant metastatic processes in organs such as brain, subcutaneous cellular tissue, lung, peritoneum, bone, which casts a shadow on the prognosis. We report the case of a 21-year-old patient who came to the hospital due to ecchymotic lesions, subcutaneous nodules and a two-month evolution of left hemicrania headache. She underwent chest and abdomen tomography and MRI of the skull. They showed metastatic spread. Histopathological study was performed with immunohistochemistry that reported amelanotic melanoma(AU)

Melanoma primario de cuello uterino: Reporte de caso / Primary malignant melanoma of the cervix: Case report

Resumen El melanoma primario de mucosas representa el 1% de todos los cánceres. Su localización en cuello uterino es rara y existen menos de 100 casos reportados en la literatura hasta la fecha. Los datos son limitados en cuanto su estadificación y tratamiento y su pronóstico es malo con tasas de supervivencia del 10% a 5 años. Se presenta el caso clínico de una paciente de 82 años con sangrado vaginal, con evidencia de una lesión melanótica en cuello uterino, la biopsia de la lesión reportó compromiso por tumor maligno pobremente diferenciado, con inmuno perfil que confirma melanoma maligno. Los estudios de extensión no mostraron enfermedad metastásica a distancia, se presentó el caso en junta multidisciplinaria de ginecología oncológica por lo que se indicó tratamiento con radioterapia pélvica externa exclusiva con intención paliativa para control de síntomas, teniendo en cuenta: la edad, las comorbilidades y el estado funcional ECOG (Eastern Cooperative Oncology Group) 3; luego de 10 meses de seguimiento la paciente falleció.

Abstract Primary mucosal melanoma represents 1% of all cancers, the location in the cervix is rare, there are less than 100 cases reported in the literature to date, the data is limited in terms of staging and treatment, its prognosis is poor with survival rates of 10% at 5 years. We present a clinical case of a primary melanoma of the cervix in an 82-year-old patient with vaginal bleeding with evidence of a melanotic lesion in the cervix. The biopsy of the lesion reported poorly differentiated malignant tumor involvement, with an immuno-profile that favors melanoma. Extension studies were performed that did not show distant metastatic disease, the case was presented in a multidisciplinary oncological gynecology meeting, indicating treatment with exclusive external pelvic radiotherapy with palliative intention for symptom control taking into account patient comorbidities and ECOG functional status. (Eastern Cooperative Oncology Group) 3, after 10 months of follow-up the patient died.

Melanoma maligno metatastásico: reporte de caso / Metastasic malignament melanoma a case report

El Melanoma Maligno es una de las neoplasias más agresivas, siendo responsable del 90% de las muertes por cáncer de piel; Su incidencia fue incrementándose durante las últimas décadas a nivel mundial. La progresión de la enfermedad puede presentarse en forma de recurrencia local, por diseminación linfática hacia los ganglios regionales, o por metástasis hematógena a sitios distantes: piel, órganos y hueso entre otros. Las metástasis regionales y a distancia, se presentan aproximadamente en 10% de los pacientes con melanoma, al momento del diagnóstico, conllevando peor pronóstico. La supervivencia a 5 y 10 años en pacientes con metástasis a distancia es de 12%-15% y 8%, respectivamente. Se presenta el caso de un paciente que rechaza tratamiento inicial de ablación de lesión primaria, presentándose múltiples metástasis y desenlace fatal.

The malignant melanoma is one of the most aggressive neoplasias, being responsible for 90% of the deaths due to skin cancer; its incidence was increasing during the last decades worldwide. The progression of the disease can occur in the form of local recurrence, by lymphatic dissemination to the regional ganglia, or by hematogenous metastasis to distant sites: skin, organs and bone among others. Regional and distant metastasis occurs in approximately 10% of patients with melanoma at the time of diagnosis, leading to a worse prognosis. The 5-year and 10-year survival in patients with distant metastasis is 12% - 15% and 8%, respectively. The case of a patient who refuses initial treatment of primary lesion is presented, presenting multiple metastasis and fatal outcome.

Formas cutáneas y mucosas infrecuentes de melanoma: parte I / Skin and mucosal forms infrequent melanoma: part I

Se han descrito varios subtipos infrecuentes de melanoma maligno en la literatura médica. A pesar de su baja frecuencia ­ que no suele superar el dos por ciento en varias series de melanoma ­ es muy importante reconocerlos precozmente pues un diagnóstico tardío está asociado a un muy mal pronóstico. En esta primera parte incluiremos; melanoma animal, melanoma desmoplásico, melanoma dérmico primario, melanoma en mucosas y melanoma nevoide.

Several infrequent subtypes of malignant melanoma have been described in the medical literature. Although their low frequency ­ less than two percent in various series of melanoma ­ it is very important to early recognize them because late diagnosis is associated with a very poor prognosis. In this first manuscript we will include; animal melanoma, desmoplastic melanoma, dermal melanoma, mucous melanoma and nevoid melanoma.

Formas cutáneas y mucosas infrecuentes de melanoma: parte II / Skin and mucosal forms infrequent melanoma: part II

Se han descrito varios subtipos infrecuentes de melanoma maligno en la literatura médica. A pesar de su baja frecuencia ­ que no suele superar el dos por ciento en varias series de melanoma ­ es muy importante reconocerlos precozmente pues un diagnóstico tardío está asociado a un muy mal pronóstico. En esta segunda parte incluiremos: melanoma verrugoso, melanoma polipoideo, melanoma hipomelanótico y amelanótico, melanoma folicular, melanoma spitzoide, melanoma con diferenciación no melanocítica.

Several infrequent subtypes of malignant melanoma have been described in the medical literature. Although their low frequency ­ less than two percent in various series of melanoma ­ it is very important to early recognize them because late diagnosis is associated with a very poor prognosis. In this second part we will include: verrucous melanoma, polypoid melanoma, hypomelanotic and amelanotic melanoma, follicular melanoma, spitzoid melanoma and non-melanocytic differentiation melanoma.

Evaluación de reactividad inmunohistoquímica con método del Complejo Avidina­Biotina (ABC) / Evaluation of immunohistochemical reactivity with Avidin-Biotin Complex (ABC) method

Inmunohistoquímica es toda técnica que permite detectar in situ componentes celulares y extracelulares por medio de anticuerpos específicos, empleando sistemas de detección enzimáticos. Dentro de los métodos inmunohistoquímicos, la técnica del complejo avidina­biotina(ABC) es ampliamente utilizada debido a su alta sensibilidad. El objetivo del presente estudio fueevaluar la reactividad inmunohistoquímica del anticuerpo 4C4.9 para la detección de la proteínaS-100, utilizando el método ABC. Para la evaluación de la reactividad inmunohistoquímica se utilizaron 2 biopsias de piel humana con diagnóstico histopatológico de melanoma maligno nodular ulcerado y nevus melanocítico intradérmico, provenientes del Laboratorio de Investigación en Biotecnología Animal de la Universidad de La Frontera, Temuco, Chile. Se utilizó el Kit VECTASTAIN®como método de detección, la dilución del anticuerpo 4C4.9 fue 1/250 y la temperatura de incubación fue a 4 ºC ó 37 ºC por 18 horas. Para validar la técnica, se realizó un control positivo y otro negativo para 4C4.9. Los resultados de la tinción inmunohistoquímica por el método del complejo ABC mostraron tinción positiva para la proteína S-100, tanto en melanoma maligno nodular ulcerado, como en nevus melanocítico intradérmico, incubados durante 18 horas a 4 ºC ó 37 ºC. Sin embargo, la inmunotinción fue más intensa cuando el anticuerpo primario se incubó a 37 ºC. Para una correcta interpretación de los resultados, es necesario tener en consideración que la reacción antígeno-anticuerpo se ve influenciada por diversos factores, como la concentración del anticuerpo, el tiempo y la temperatura de incubación. En conclusión, nuestros resultados sugieren incubarlas muestras con el primer anticuerpo (4C4.9) en una dilución de 1/250 en agua destilada, incu-bando durante 18 h a 37 ºC. Se recomienda la utilización del anticuerpo 4C4.9 como apoyo al diagnóstico y diagnóstico diferencial.

Immunohistochemistry is anytechnique that can detect cellular and extracellular components in situ by means of specific antibodies,using enzymatic detection systems. Among immunohistochemical methods, the technique ofavidin - biotin complex (ABC) is widely used because of its high sensitivity. The aim of this study was to evaluate the immunohistochemical reactivity of the4C4.9 antibody for detection of S-100 protein using the ABC method. For the evaluation ofimmunohistochemical reactivity 2 biopsies of humanskin were used with histopathological diagnosis ofulcerated malignant melanoma and melanocyticintradermal nevi from the Research Laboratory onAnimal Biotechnology of the Universidad de La Fron-tera, Chile. The Kit VECTASTAIN® was used asdetection method, the dilution the 4C4.9 antibodywas 1/250 and incubation temperature was at 4 °Cor 37 °C for 18 hours. To validate the technique, apositive control and a negative for 4C4.9 was performed. The results of immunohistochemicalstaining by the method of ABC complex showed positive staining for protein S-100 both in ulcerated malignant melanoma and melanocytic intradermalnevi, incubated for 18 hours at 4 °C or 37 °C.However, immunostaining was more intense when the primary antibody was incubated at 37° C. For acorrect interpretation of the results, it is necessary to take into consideration that the antigen-antibody reaction is influenced by various factors such as the concentration of antibody, time and temperature ofincubation. In conclusion, our results suggest incubating the samples with the first antibody (4C4.9)at 1/250 dilution in distilled water, incubating for 18h at 37 ºC. However, immunostaining was moreintense when the primary antibody was incubated at37° C. For a correct interpretation of the results, it isnecessary to take into consideration that antigen-antibody reaction is influenced by various factors suchas the concentration of antibody, time and temperature of incubation. In conclusion, our results suggest incubating the samples with the first antibody(4C4.9) at 1/250 dilution in distilled water, incubating for 18 h at 37 ºC. The use of the antibody 4C4.9 is recommended to support the diagnosis and differential diagnosis.

Melanoma subungueal de la mano: reporte de un caso / Hand’s subungueal melanoma: A case report

El melanoma es considerado una neoplasia melanocítica maligna; la localización subungueal corresponde al 0.7-3.5% de todos los melanomas. Clínicamente se presenta como una melanoniquia en general asintomática. Comunicamos un paciente masculino de 67 años, que consulta por presentar melanoniquia estriada en el cuarto dedo de la mano izquierda. El estudio histopatológico de biopsia informa un melanoma acral, por lo que se procede a realizar resección completa de la lesión con amputación de la falange distal; el paciente evolucionó satisfactoriamente. Este melanoma se origina de la matriz ungular, aunque se ha propuesto la asociación con traumas, su etiología no está clara. Histológicamente, se caracteriza por una proliferación de melanocitos atípicos o fusiformes, con grados variables de invasión; algunas lesiones son pigmentadas y otras amelanóticas. Se debe establecer el diagnóstico diferencial con: hematomas, osteomielitis, onicomicosis, granuloma piógeno, paroniquia, enfermedad de Kaposi, carcinoma espinocelular, entre otras. Algunos factores pronósticos incluyen profundidad de invasión, índice mitótico, ulceración, índice de proliferación con Ki 67 para nombrar solo algunos factores del denominado “histopronóstico”. El retraso en el diagnóstico del melanoma subungueal condiciona un mal pronóstico. El tratamiento apunta hacia la intervención quirúrgica en estadios tempranos, con evaluación del ganglio centinela (esto varía según la escuela académica). Sin embargo, actualmente se hace referencia a nuevos fármacos, que modifican la respuesta inmune o que intervienen en el metabolismo celular, con lo que se dan los primeros pasos para modificar la historia natural de esta enfermedad.

Introduction: melanoma is considered a biologically aggressive neoplasm. It’s more common in the 6th decade. Subungual melanoma corresponding to 0.7-3.5% of all melanomas. The most common locations are hallux, thumb, index and ring. Clinically, it presents as an asymptomatic melanonychia. Case Report: male aged 67 who consulted for striated melanonychia fourth finger left hand. Biopsy histopathological study reports malignant melanoma. Complete resection of the lesion was performed with amputation of distal phalanx. Patient evolves satisfactorily. Discussion: this melanoma originates from the nail matrix. Although it has been proposed partnership with traumas, its etiology is unclear. Hasn’t been able to establish an association with race, skin type and sun exposure, as in other melanoma’s types. Histologically it is characterized by a proliferation of atypical melanocytes or fusiform with varying degrees of invasion. Some injuries are pigmented and others may be amelanotic. Should be established the differential diagnosis with hematomas, osteomyelitis, onychomycosis, pyogenic granuloma, paronychia, among other injuries. Some prognostic factors include depth of invasion, mitotic rat, ulceration, proliferation index among others. The delay in diagnosis of subungual melanoma determines a poor prognosis. The treatment aims to surgery in early stages, with evaluation of sentinel node. But now referred to new drugs that modify the immune response or that involve its cellular metabolism thus taking the first steps to modify the natural history of this disease.

Melanoma maligno mucoso de cavidad nasal / Mucosal malignant melanoma of the nasal cavity

El melanoma maligno de cavidad nasal y senos paranasales es una patología rara, representa alrededor del 1% de todos los melanomas. Su sintomatología es inespecífica lo que dificulta el diagnóstico, tiene muy mal pronóstico con bajas tasas de supervivencia. Presentamos el caso de un varón de 66 años de edad, fumador de 30-40 cigarrillos por día, trabajador de la huerta expuesto a productos fitosanitarios que consultó por epistaxis recurrentes por fosa nasal derecha y ligera obstrucción respiratoria, a la exploración se observó una masa de color negruzco que ocupaba la fosa nasal derecha dependiente del cornete inferior derecho, el diagnóstico histopatológico fue de melanoma maligno; recibió tratamiento quirúrgico y radioterápico con evolución favorable. Revisamos la literatura de este raro tumor nasosinusal.

Malignant melanoma of the nasal cavity and paranasal sinuses is a rare disease, represents less than 1% of all melanomas. The symptoms are nonspecificand therefore diagnosis difficult. Has very poor prognosis, with low survival rates. We report a 66 year old smoking 30-40 cigarettes per day, orchard worker exposed to pesticides consulted for recurrent epistaxis by right nostril and light airway obstruction, exploration was observed a mass blackish located in the lower right turbinate, histopathological diagnosis was malignant melanoma; He received surgical and radiotherapy with favorable outcome. We review the literature on this rare sinonasal tumor. inferior turbinate.

Corneal melanosis successfully treated using topical mitomycin-C and alcohol corneal epitheliectomy: a 3-year follow-up case report / Tratamento com êxito da melanose de córnea utilizando mitomicina-C tópica e epiteliectomia auxiliada por álcool: relato de caso com três anos de seguimento

ABSTRACTWe report a case of primary acquired corneal melanosis without atypia associated with corneal haze in a patient with a history of limbal malignant melanoma and the effect of mitomycin-C. A 75-year-old woman with a history of limbal malignant melanoma presented with loss of vision in right eye. Corneal examination showed a patchy melanotic pigmentation with a central haze. Topical mitomycin-C improved visual acuity and corneal haze. However, the pigmented lesions persisted, and they were removed with alcohol corneal epitheliectomy. Histopathological examination demonstrated primary acquired melanosis without atypia. The lesions were successfully removed, and there were no recurrences during the follow-up period of 36 months. The association of conjunctival and corneal melanosis without atypia is a rare condition. In addition, co-existence of central corneal haze and melanosis may decrease visual acuity. Topical mitomycin-C and alcohol corneal epitheliectomy can be useful treatments in this condition.

RESUMORelatar um caso de melanose adquirida primária de córnea sem atipia, associado a haze corneano em um paciente com história de melanoma maligno de limbo e o efeito da mitomicina-C. Uma mulher de 75 anos de idade, com história de melanoma maligno do limbo apresentado com diminuição de visão no olho direito. O exame de córnea mostrou uma pigmentação melânica irregular com um haze central. O uso de mitomicina-C tópica levou à melhora da acuidade visual e da opacidade corneana. No entanto, as lesões pigmentadas persistiram e foram removidas com epiteliectomia associada ao álcool. O exame histopatológico demonstrou melanose adquirida primária sem atipia. As lesões foram removidas com êxito, e não houve recidiva durante o período de acompanhamento de 36 meses. A associação melanose sem atipia da conjuntiva e da córnea é uma condição rara. Além disso, a coexistência de haze corneano central e melanose pode diminuir a acuidade visual. O uso de mitomicina-C tópica e epiteliectomia corneana auxiliada pelo álcool podem ser tratamentos úteis nessa situação.

Caracterización clínica e histopatológica de melanomas malignos de piel / Biopsy-based clinical and histopathological characterization of cutaneous malignant melanoma

Antecedentes: la incidencia del melanoma cutáneo ha venido en aumento en las últimas décadas, convirtiéndose en causa importante de muerte a nivel mundial. El objetivo es determinar la incidencia y caracterizar los melanomas con base en los resultados de las biopsias del Servicio de Patología del Hospital San Rafael de Alajuela. Métodos: se realizó la revisión histopatológica de cada una de las biopsias diagnosticadas como melanoma maligno de piel, con tinciones de hematoxilina y eosina, en el periodo comprendido de enero de 2009 a diciembre de 2012, para determinar sus características histopatológicas. Se obtuvieron frecuencias simples de todas las variables y medidas de tendencia central y dispersión para las variables cuantitativas...

Melanoma Cutâneo: aspectos clínicos, epidemiológicos e anatomopatológicos de um centro de formação em Belo Horizonte / Cutaneous melanoma: clinical, epidemiological, and anatomopathological aspects of a training center in Belo Horizonte

Introdução: O melanoma cutâneo maligno (MCM) constitui cerca de 5% dos tumores cutâneos malignos e apresenta crescente incidência e alta letalidade. O objetivo deste estudo é rever as características clínicas, epidemiológicas e anatomopatológicas do MCM em pacientes tratados nos serviços de Cirurgia Plástica e Anatomia Patológica de um hospital geral. Método: Dados de 45 pacientes, correspondendo a 47 lesões, tratados entre 2011 e 2013, foram revisados. Estudou-se: gênero e idade dos pacientes, localização e características histopatológicas das lesões. Resultados: Vinte e quatro pacientes eram do gênero masculino e 21 do feminino. A média de idade à consulta foi de 61,9 anos. Vinte e quatro neoplasias localizaramse nas extremidades, 14 acometeram o tronco e nove a face. Quanto ao diagnóstico histológico, 34,0% dos tumores consistiu-se em melanoma in situ e 66,0% em melanoma invasor. Neste grupo, 14 lesões correspondiam ao tipo nodular, 12 ao extensivo superficial, três ao acral lentiginoso e dois ao lentigo maligno. A biópsia de linfonodo sentinela foi realizada em 14 melanomas invasores, com positividade em quatro procedimentos. Dentre as linfadenectomias realizadas, quatro mostraram-se positivas para metástase. No momento do diagnóstico, quatro pacientes apresentavam metástase em trânsito e três com metástase linfonodal. Recidiva tumoral local foi verificada em dois casos. Em relação ao estadiamento, 14 pacientes encontravam-se no estádio 0, 11 no I, 10 no II e 10 no III. Conclusão: Os dados deste estudo corroboram os achados da literatura. O diagnóstico e tratamento cirúrgico precoce permanecem o melhor caminho para o aumento da sobrevida.

Introduction: Malignant cutaneous melanoma (MCM) comprises nearly 5% of all malignant cutaneous tumors and shows increasing incidence and a high mortality. The objective of this study is to review the clinical, epidemiological, and anatomopathological characteristics of MCM in patients treated at the plastic surgery and pathological anatomy services of a general hospital. Method: Data corresponding to 47 lesions from 45 patients treated between 2011 and 2013 were reviewed. We analyzed the sex and age of patients, as well as the site and histopathological characteristics of the lesions. Results: A total of 24 patients were men and 21 were women. Their average age at the medical appointment was 61.9 years. Twenty-four neoplasias were in the extremities, 14 in the torso, and 9 in the face. Concerning histological diagnosis, 34.0% of the tumors were in situ melanoma and 66.0% were invasive melanoma. In the latter group, 14 lesions corresponded to the nodular melanoma type, 12 to the superficial spreading, three to the acral lentiginous, and two to the malignant lentigo melanoma type. Sentinel node biopsy was performed in 14 invasive melanomas, with 4 being positive. Among the lymphadenectomies performed, four were positive for metastasis. At diagnosis, four patients showed in-transit metastasis, whereas three patients had lymph node metastasis. Local tumor relapse was observed in two cases. Concerning tumor staging, 14 patients were in stage 0, 11 in stage I, 10 in stage II, and 10 in stage III. Conclusion: The data from this study support the findings described in the literature. Early diagnosis and surgical treatment remain the best way to increase survival.

A case report: primary melanoma of the nasal cavity / Un reporte de caso: melanoma primario de la cavidad nasal

We report a rare case of sinonasal mucosal malignant melanoma in the nasal cavity. The patient had respiratory difficulty, continuous epistaxis and nasal pain. We identified a malignant tumour which is a rare pathology with detailed physical examination, anterior rhinoscopy, computed tomography (CT) scan, magnetic resonance imaging (MRI) and histopathologic examination. The patient did not accept surgical procedures and was referred for chemotherapy or immunotherapy. Continued follow-up of this is necessary.

Reportamos un caso raro de melanoma maligno de la mucosa del tracto nasosinusal. El paciente tenía dificultad respiratoria, epistaxis continua, y dolor nasal. Identificamos un tumor maligno que es una patología, con examen físico detallado, rinoscopia anterior, tomografía computarizada (TC), resonancia magnética (MRI) y examen histopatológico. El paciente no aceptaba los procedimientos quirúrgicos y fue remitido para quimioterapia o inmunoterapia. Es necesario realizar un seguimiento continuo.

Fotoproteção tópica na infância e na adolescência / Topical photoprotection in childhood and adolescence

OBJETIVO: A exposição à luz solar na infância ocorre, frequentemente, de forma mais intensa do que em muitos adultos. Dados da literatura comprovam de maneira inequívoca a associação desse comportamento social com o risco de desenvolvimento do melanoma maligno e do câncer cutâneo não melanoma mesmo na vida adulta. Além disso, o fotoenvelhecimento cutâneo é semeado já na infância com a exposição solar inadequada. Esta revisão tem como objetivo orientar os pediatras nas medidas adequadas de fotoproteção tópica nas crianças e adolescentes, o que irá alterar de maneira positiva o futuro desses pacientes. FONTES DOS DADOS: Realizou-se uma revisão da literatura indexada na base de dados MEDLINE/PubMed entre os anos de 1999 e 2012 sobre fotoproteção na infância, selecionando-se como fonte os artigos de revisão mais relevantes, do ponto de vista de abrangência do tema fotoproteção em crianças e adolescentes, fotoproteção e vitamina D, fototerapia na neonatologia e impacto no câncer cutâneo, bronzeamento artificial e câncer cutâneo. SÍNTESE DOS DADOS: Crianças e adolescentes devem adotar medidas adequadas de fotoproteção para diminuir o risco de câncer cutâneo melanoma e não melanoma. CONCLUSÕES: Há dados na literatura que suportam a associação de hábitos de exposição solar segura e uso de fotoprotetores tópicos em crianças e adolescentes com a redução da ocorrência do câncer cutâneo.

OBJECTIVE: Exposure to sunlight in childhood is often more intense than in adults. Literature data unequivocally show the association between this social behavior and the risk for developing malignant melanoma and non-melanoma skin cancer, even in adulthood. Furthermore, skin photoaging begins already in childhood through inadequate sun exposure. This review aims to guide pediatricians on appropriate measures of topical photoprotection in children and adolescents, which will positively change the future of these patients. SOURCES: A review of the literature indexed in MEDLINE/PubMed between the years 1999 and 2012 on photoprotection in childhood was conducted. The most relevant review articles on photoprotection in children and adolescents, photoprotection and vitamin D in neonatal phototherapy and impact on skin cancer, artificial tanning and skin cancer were selected as sources. SUMMARY OF THE FINDINGS: Children and adolescents should adopt appropriate measures of photoprotection in order to decrease the risk of melanoma and non-melanoma skin cancer. CONCLUSIONS: There are published data that support the association between sun exposure habits and safe use of topical sunscreens in children and adolescents on the one hand and a reduced occurrence of skin cancer on the other.

Metástasis gastroduodenales de melanoma maligno a propósito de un caso / Gastroduodenal metastasis of malignant melanoma: case study

Melanoma is a common malignancy whose prevalence has been increasing, accounting for between 1 to 3 percent of cancers in the United States (USA), and is one of the most common types of cancer that metastasize to the gastrointestinal tract in advanced stages. It is reported that malignant melanoma (MM) either skin or mucous, can metastasize to virtually every organ in the human body. Although these have frequently secondary location in the gastrointestinal tract (GIT), where it is known to have special predilection, gastro intestinal metastasis are rarely found in the follow-up of these patients. Late diagnosis is due to its generally silent evolution. Endoscopy is a valuable tool both to confirm histological diagnosis, and monitoring of systemic therapies and palliative care locally. MM compromising the GIT is generally already spread by the time of diagnosis, in which case survival is only of 6 – 8 months, despite aggressive treatment. Hence the importance of anamnesis, to have a history of melanoma surgery, in addition to diligent physical examination that can reveal a primary skin or mucosal melanoma. All patients diagnosed with malignant melanoma should undergo endoscopic GIT studies.

El melanoma es una neoplasia frecuente, cuya prevalencia ha ido en aumento, representando entre 1 a 3 por ciento de los cánceres en EE.UU. y es una de las neoplasias que con mayor frecuencia presenta metástasis al tracto gastrointestinal en etapas avanzadas. Se ha reportado que el melanoma maligno (MM), ya sea cutáneo o mucoso, es capaz de producir metástasis a prácticamente todos los órganos del cuerpo humano. Aunque es frecuente su ubicación secundaria en el tracto gastrointestinal (TGI) por el cual se sabe que tiene especial predilección, rara vez se pesquisan metástasis gastrointestinales en el seguimiento de estos pacientes. El diagnóstico tardío se debe a que su evolución es generalmente silente. La endoscopia es una valiosa herramienta tanto para confirmar el diagnóstico histológico, como para el seguimiento de terapias sistémicas y tratamiento paliativo local. El MM con compromiso del TGI generalmente se encuentra diseminado al momento del diagnóstico; en cuyo caso la sobrevida es sólo de 6 a 8 meses, pese a un tratamiento agresivo. De ahí la importancia de la anamnesis, para contar con el antecedente de melanoma operado, además del examen físico acucioso que nos pueda dar cuenta de un melanoma primario en piel o mucosas visibles. Todo paciente con diagnóstico de melanoma maligno debería ser sometido a estudios endoscópicos del TGI.

Conocimiento sobre melanoma y prácticas de protección frente al sol en pacientes del Hospital Cullen de Santa Fe, Argentina / Knowledge of melanoma and sun-protective practices in patients at José M. Cullen Hospital of Santa Fe, Argentine

Introducción. Melanoma es un cáncer de piel de mortalidad alta, cuya incidencia ha aumentado mundialmente en los últimos años. El siguiente estudio está destinado a detectar el conocimiento de la población sobre melanoma y las medidas de protección frente a la radiación UV (RUV), considerando que las dos terceras partes de los casos de melanoma se asocian al sol. Objetivo general. Detectar el conocimiento sobre melanoma y las prácticas de protección solar, en pacientes que consultan en el Servicio de Dermatología e internados en Clínica Médica del Hospital José M. Cullen. Materiales y métodos. Estudio observacional, prospectivo, longitudinal y descriptivo. La población abarcó 275 pacientes, entre 16 y 85 años, voluntarios del consultorio de Dermatología e internados en Clínica Médica del Hospital José M. Cullen. Mediante el programa Statistical Package for the Social Sciences 15.0, se realizó un análisis descriptivo y la prueba de Chi cuadrado, para determinar asociación estadística, entre variables cualitativas con significancia de p<0,05. Resultados. El conocimiento resultó escaso, sólo el 53% relacionó correctamente melanoma con cáncer de piel. El elemento de protección a la RUV más utilizado fue el sombrero y más frecuente en hombres (p<0,001). El uso de FPS es más común en mujeres (p= 0,003); de hecho, fueron las que en mayor frecuencia (p=0,011) lo utilizaron el verano pasado y en personas menores de 40 años (p=0,002). La utilización de ropas largas es más frecuente en hombres (p=0,006) y en personas mayores de 40 años (p=0,002). Se encontró un resultado significativo entre la utilización de FPS y su renovación periódica (p<0,001), entre la práctica de no utilizar ningún elemento de protección frente al sol y considerar que un lunar puede transformarse en cáncer de piel (p=0,014). Conclusión. Existe poco conocimiento, lo que se refleja en las prácticas inadecuadas de protección frente a RUV.

Background. Melanoma is one of the deadliest forms of skin cancer, showing an increasing incidence worldwide over the past years. The aim of this study was to assess people's knowledge about melanoma, and their protective measures against UV radiation, considering that two-thirds of melanoma cases are associated to sun exposure. General objective. To assess the knowledge about melanoma and the sun protective practices in patients who attended the Department of Dermatology and in those admitted to the General Internal Medicine Unit of José M. Cullen Hospital. Material and methods. It was an observational, prospective, longitudinal, descriptive study. A sample of 275 participants aged between 16-85 years was included, composed of voluntary patients from the Department of Dermatology and inpatients from the General Internal Medicine Unit of José M. Cullen Hospital. The Statistical Package for the Social Sciences 15.0 software was used to make a descriptive analysis; the chi-square test was performed with a significance level of p < 0.05 to determine the statistical association among qualitative variables. Results. The knowledge was poor, since only 53% of respondents associated melanoma to skin cancer correctly. Hats were the most used sun protection choice, specially in men (p < 0.001). The use of sunscreen was more common among women (p= 0.003) - in fact, they were the ones who used it more regularly (p= 0.011) last summer - and among people under 40 (p= 0.002). Using long clothing was more common in men (p= 0.006) and in people over 40 (p= 0.002). A significant result was found between the use of SPF and its daily reapplication (p < 0.001), between not using any sun protection element and considering that a mole can develop skin cancer (p= 0.014). Conclusion. There is little knowledge among people, which is reflected on the inadequate protective practices against UV radiation.

Intususcepción intestinal secundaria a metástasis de melanoma: caso clínico / Intestinal intussusception secondary to a metastatic melanoma: case report

Cutaneous melanoma is one of the extra-abdominal cancers that more commonly originates metastases in the gastrointestinal tract. We report a 63 years old male presenting with iron deficiency anemia and episodes of gastrointestinal bleeding, with a history of a melanoma of the lower eyelid excised 13 years ago, with negative surgical margins. During the follow up, the patient consulted in the emergency room for an intestinal obstruction. An abdominal CAT scan showed an ileal-ileal intussusception. The patient was operated and the involved intestinal segment was excised. The pathological study of the surgical piece disclosed a pigmented polypoid metastatic melanoma. The patient had an uneventful postoperative evolution.

El melanoma cutáneo es uno de los cánceres extra-abdominales que más frecuentemente originan metástasis viscerales en el tracto gastrointestinal. Su presentación clínica suele ser silente e insidiosa, generalmente desapercibida, atentando contra las posibilidades terapéuticas y paliativas de este grupo de pacientes. El objetivo de este trabajo es presentar el caso clínico de un paciente tratado trece años antes de un melanoma del párpado inferior izquierdo, resecado con márgenes negativos, sin evidencias de recurrencia hasta 3 meses previo a su ingreso, en que mientras estaba en estudio por una anemia ferropriva de origen digestivo, se presenta con un íleo mecánico de intestino delgado por una intususcepción íleo-ileal secundaria a una metástasis visceral pedunculada de su melanoma. Se describe el caso, se comunican los elementos clínicos y de imágenes necesarios para su diagnóstico y se realiza una revisión de la literatura pertinente.

BRAF mutations in cutaneous melanoma: no correlation with histological prognostic factors or overall survival / Mutações BRAF em melanomas cutâneos: nenhuma correlação com fatores prognósticos e sobrevida global

INTRODUCTION: Molecular biology techniques allow identification of molecular markers such as BRAF and c-Kit gene mutations in melanomas. Studies on genetic alterations in melanomas of South-American patients are sparse. OBJECTIVES: To identify the incidence of BRAF and c-Kit gene mutations in primary cutaneous melanomas in Brazilian patients and to evaluate pathogenetic and prognostic implications of these mutations correlating them with clinical and histopathological data. MATERIAL AND METHODS: Ninety-six surgical specimens of primary cutaneous melanoma and 15 corresponding metastasis were analyzed using TaqMan Real-Time polymerase chain reaction (PCR) assays. RESULTS: In comparison with the medical literature, a relatively low frequency of BRAF mutation in primary (39 percent) and metastatic (40 percent) melanomas and complete absence of c-Kit gene mutations were demonstrated. BRAF mutations arose at an early stage during melanoma progression and were not involved in the transition of thin (< 1 mm) to thick (> 1 mm) melanomas. BRAF mutations are related to patients' younger age and to the pattern of sun exposure, although there was no correlation with any histological prognostic factor or overall survival. CONCLUSION: The identification of both BRAF and c-Kit mutation is not a suitable prognostic indicator in the Brazilian population. Moreover, the relatively low frequency of BRAF mutations brings into question if it actually plays a key role in melanoma pathogenesis.

INTRODUÇÃO: Técnicas de biologia molecular permitem a identificação de marcadores moleculares como mutações dos genes BRAF e c-Kit em melanomas. Estudos de alterações genéticas em pacientes sul-americanos são escassos. OBJETIVOS: Identificar a incidência de mutações dos genes BRAF e c-Kit em melanomas cutâneos primários em uma série de pacientes brasileiros e avaliar as implicações patogenéticas e prognósticas dessas mutações, correlacionando-as com dados clínicos e histopatológicos. MATERIAL E MÉTODOS: Noventa e seis espécimes cirúrgicos de melanomas cutâneos e 15 metástases correspondentes foram analisados por meio da técnica TaqMan Real-Time polymerase chain reaction (PCR). RESULTADOS: Uma frequência relativamente baixa de mutações BRAF em melanomas cutâneos primarios (39 por cento) e metastáticos (40 por cento) em comparação com os dados da literatura e ausência de mutações c-Kit foi demonstrado. Mutações BRAF surgiram em um estágio inicial da progressão do melanoma e não foram envolvidas na transição de melanomas finos (< 1 mm) para grossos (> 1 mm). Essas mutações estavam presentes em pacientes mais jovens e se correlacionaram com o padrão de exposição solar dos pacientes estudados, mas não houve correlação com nenhum fator prognóstico histológico ou sobrevida global dos mesmos. CONCLUSÃO: A identificação de ambas as mutações (BRAF e c-Kit) não servem como indicadores de prognóstico na população brasileira. Além disso, a baixa frequência de mutações BRAF encontrada neste estudo nos faz questionar se essa mutação realmente tem papel-chave na patogênese do melanoma.