Melanoma arising in a child with a medium-sized congenital melanocytic nevus.

Pediatric melanomas are rare and some of them may arise on giant congenital melanocytic nevi. The risk of developing melanoma on a medium-sized nevus is not clear but is thought to be very rare. Proliferative cellular nodules which mimic malignant melanoma may pose significant diagnostic challenges. We report the case of a 9-year-old patient who developed a melanoma on a medium-sized congenital melanocytic nevus on the tip of the nose, requiring a complex surgery with excellent aesthetic results.

Los melanomas en pediatría son raros y algunos de ellos pueden surgir sobre nevos melanocíticos congénitos gigantes. El riesgo de desarrollar melanoma en un nevo de tamaño mediano no está claro, pero se cree que es muy raro. Los nódulos proliferativos celulares, que imitan al melanoma, pueden plantear importantes desafíos diagnósticos. Presentamos el caso de una paciente de 9 años que desarrolló un melanoma sobre un nevo melanocítico congénito de tamaño mediano en la punta de la nariz, que requirió un procedimiento quirúrgico complejo con excelentes resultados estéticos.

Melanocytic nevus of the anal canal and granular cell tumor of the cecum: a case report and literature review of 2 coincidentally co-occurring neurocristopathies.

Granular cell tumors are predominantly benign soft tissue tumors originating from Schwann cells, whereas melanocytic nevi are benign proliferations of melanocytes. We present the case of a patient with the presence of both entities located in the cecum and anal canal, respectively, constituting an extremely rare coincidental finding. A 43-year-old woman was evaluated by colonoscopy for iron-deficiency microcytic anemia that had lasted for 1 year. Colonoscopy demonstrated a macular lesion of 0.3 cm with a melanocytic appearance in the anal canal; at the cecum level, a subepithelial, yellowish, and partially mobile firm nodular lesion measuring 1.3 cm was observed. A histopathological study showed a melanocytic nevus in the anal canal and a granular cell tumor in the cecum. This is the first reported case of a patient with the extremely rare coincidental-incidental finding of these 2 entities at the same time.

Focal melanocytic lesions of the oral mucosa: An epidemiological and morphological study.

OBJECTIVE: This study aimed to analyse the clinical and histopathological characteristics of focal oral melanocytic lesions in a Brazilian reference service in Oral and Maxillofacial Pathology. MATERIALS AND METHODS: A cross-sectional study was conducted over an 18-year period. Demographic data and clinical features were collected from the archives, and all biopsy specimens diagnosed as oral melanocytic lesions were retrieved and reviewed. RESULTS: We identified 339 melanocytic lesions. Of these, 191 were melanotic macules, 112 melanocytic nevi, 14 mucosal lentigo simplex, 12 melanomas, 9 solar lentigos, and 1 melanoacanthoma. Lesions occurred mostly in white-skinned (74.2%) women (65.2%). The main reported clinical aspect was the macule (67.4%), and the most affected site was the lip vermilion (25.4%), followed by the palate (22.9%). Melanomas were larger in size and were observed in older patients with an overall shorter time of onset. The most frequent subtypes of melanocytic nevi were intramucosal (44.6%), compound (24.1%), and blue nevus (20.5%). They showed a heterogeneous architectural pattern with the presence of the three cell types. CONCLUSION: The most frequent lesions are melanotic macule and nevus, especially the intramucosal subtype. Patients are usually white-skinned women presenting a small, long-lasting, macular lesion on the lip vermilion or palate.

Nevo Melanocítico Congénito Gigante con hemangioma hepático asociado. Reporte de caso clínico. Revisión bibliográfica

El nevo melanocítico congénito gigante es una lesión pigmentada de gran tamaño presente al nacimiento. Su incidencia es de 1/1.000-500.000 recién nacidos. La localización más frecuente es el tronco posterior, la cara, el cuero cabelludo y las extremidades. El objetivo que buscamos con la presentación de este caso clínico es ofrecer una revisión actualizada sobre la evaluación al nacimiento, conducta y tratamiento a seguir por los neonatólogos y pediatra de atención primaria ante la inesperada presencia de las dermatopatías no tan frecuentes como la que nos ocupa. Se trata de un recién nacido que nace con un "nevo melanocítico congénito gigante" y que además se asocia con un hemangioma hepático diagnosticado en el ingreso. Valoramos la importancia de esta patología que radica en los problemas impactantes desde el punto de vista estético, quirúrgico y emocional que pueden originar en el paciente y sus familiares, además de la posible asociación con otras malformaciones del sistema nervioso central y en algunos de ellos, el riesgo de ser el origen de un melanoma.

The giant congenital melanocytic nevus is a large pigmented lesion present at birth. Its incidence is 1 / 1,000-500,000 newborns. The most frequent location is the posterior trunk, the face, the scalp and the extremities. We decided with the presentation of this clinical case to offer an updated review on the evaluation at birth, behavior and treatment to be followed by neonatologists, primary care pediatrician before the unexpected presence of dermatopathies not as frequent as the one we are dealing with today, the "nevus" giant congenital melanocytic ". The importance of this pathology lies in the impactful problems from the aesthetic, surgical and emotional point of view that can originate in the patient and their relatives, in addition to the possible association with other malformations of the central nervous system and in some of them, the risk of being the origin of a melanoma.

O nevo melanocítico congênito gigante é uma grande lesão pigmentada presente ao nascimento. Sua incidência é de 1/1.000-500.000 recém-nascidos. A localização mais frequente é o tronco posterior, face, couro cabeludo e extremidades. O objetivo que buscamos com a apresentação deste caso clínico é oferecer uma revisão atualizada sobre a avaliação ao nascimento, comportamento e tratamento a ser acompanhado por neonatologistas e pediatras da atenção primária na presença inesperada de dermatopatias não tão frequentes quanto a em questão. É um recém-nascido nascido com um "nevo melanocítico congênito gigante" e também está associado a um hemangioma hepático diagnosticado na admissão. Valorizamos a importância dessa patologia que reside nos problemas chocantes do ponto de vista estético, cirúrgico e emocional que podem se originar no paciente e em seus familiares, além da possível associação com outras malformações do sistema nervoso central e, em algumas de las, o risco de ser a origem do melanoma.

Total body mapping in the follow-up of melanocytic lesions: recommendations of the Brazilian Society of Dermatology

Abstract Total body mapping comprises photographic documentation of the entire body surface followed by digital dermatoscopy of selected melanocytic lesions, aiming to compare their evolution over time and identify new lesions. As this is an exam based on comparative analysis of serial dermoscopic body images, standardization of the technique for performing total body mapping is essential. Prepared by specialists from the Brazilian Society of Dermatology, using the modified Delphi method, this article provides recommendations for carrying out total body mapping in Brazil, regarding its indications, technical aspects, and the issuing of the report.

Total body mapping in the follow-up of melanocytic lesions: recommendations of the Brazilian Society of Dermatology.

Total body mapping comprises photographic documentation of the entire body surface followed by digital dermatoscopy of selected melanocytic lesions, aiming to compare their evolution over time and identify new lesions. As this is an exam based on comparative analysis of serial dermoscopic body images, standardization of the technique for performing total body mapping is essential. Prepared by specialists from the Brazilian Society of Dermatology, using the modified Delphi method, this article provides recommendations for carrying out total body mapping in Brazil, regarding its indications, technical aspects, and the issuing of the report.

Intraoral atypical lentiginous melanocytic lesion in a pediatric patient.

We present a rare case of intraoral atypical lentiginous melanocytic lesion affecting a pediatric patient, in which the diagnosis of lentiginous junctional melanocytic nevus with cytologic atypia was favored. The main differential diagnosis is lentiginous melanoma, which is a slowly progressing lesion, affecting mainly older adults, and microscopically presenting lentiginous growth pattern of moderately atypical melanocytes, with focal nesting and pagetoid spread. It is strongly recommended that melanocytic lesions showing features of atypical lentiginous growth pattern should be treated with wide excision; however, the impact of these guidelines on pediatric patients needs to be better defined with the report of further cases.

Dermatoscopía de nevos melanocíticos congénitos acrales: puesta al día / Dermoscopy of melanocytic nevi acral congenitals: update

Los nevos melanocíticos congénitos (NMC) son aquellos presentes al nacer o que se desarrollan durante los primeros dos años de vida. Hay muy pocos casos documentados de melanoma lentiginoso acral en asociación con nevos preexistentes en comparación a otros subtipos de melanoma. De éstos, la mayoría serían asociados a nevos melanocíticos adquiridos acrales (NMAA) y muy excepcionalmente asociados a nevos melanocíticos congénitos acrales (NMCA). Sin embargo, la extirpación de lesiones pigmentadas acrales congénitas es practicada con frecuencia y se desconocen los patrones dermatoscópicos más característicos, así como tampoco se disponen de algoritmos de seguimiento. A continuación, presentamos dos casos de NMCA con patrones dermatoscópicos característicos y realizamos una revisión de los patrones dermatoscópicos más comunes de NMCA descritos en la literatura.

Congenital melanocytic nevi (CMN) are those present at birth or that develop during the first two years of life. There are very few documented cases of acral lentiginous melanoma in association with pre-existing nevi compared to other subtypes of melanoma. Of these, the majority would be associated with acral acquired melanocytic nevi (NMAA) and very exceptionally associated with acral congenital melanocytic nevi (NMCA). However, the excision of congenital acral pigmented lesions is frequently practiced and the most characteristic dermoscopic patterns are unknown, nor are there any follow-up algorithms. In the following, we present two cases of NMCA with characteristic dermoscopic patterns and a review of the most common dermoscopic patterns of NMCA described in the literature

Collision Tumor between Trichofolliculoma and Melanocytic Nevus.

Trichofolliculoma (TF) is a hamartomatous hair follicle-related tumor, clinically described as a dome-shaped papule with a central pore crossed by one or more silky white hairs. Histologically, it described as a cystic cavity containing keratinous debris, hair shaft fragments, and numerous hair follicles arising from its linings. Collision or compound tumors are a coexistence of two or more identifiable tumors in the same lesion. We present a case of a 47-year-old man with a lesion on his left cheek clinically characterized as a TF. However, the histopathological study reveals a collision tumor involving a TF and a melanocytic nevus. Collision tumors involving melanocytic nevi and hair follicle-related tumors have been previously reported, such as desmoplastic trichoepithelioma, epidermoid cyst, folliculosebaceous cystic hamartoma, and trichoadenoma.

Atypical Location and Morphology of a Proximal Nail Fold Nevus Mimicking Other Diseases.

Melanocytic nevi result from the proliferation of melanocytes at the dermal-epidermal junction and/or in the dermis and very rarely affect the nail matrix and bed. Intradermal nevi are tumors often diagnosed in routine clinical dermatological practice, with typical clinical and histopathologic aspects, and found in a wide variety of skin sites. We report a case of intradermal melanocytic nevus of the proximal nail fold with uncommon intraoperative gross findings and without involvement of the nail bed or matrix, showing intradermal component only, which draws our attention to the possible different presentation of melanocytic lesions. No description of intradermal nevi at this site has been reported in the literature so far.

Oral Congenital Melanocytic Nevus: A Rare Case Report and Review of the Literature.

Melanocytic nevi are congenital or acquired benign proliferations of cells of melanocytic origin. Oral congenital melanocytic nevi are rare, and only a few cases have been reported in the literature. The purpose of this study is to present the clinical, histological and immunohistochemical features of an oral congenital melanocytic nevus in a 16-year-old female with an 11-year follow-up and to review the pertinent literature. The reported case is the fifth well-documented case report of oral congenital melanocytic nevus in the English literature and the first with a long period of follow-up, thereby making it an important contribution to the knowledge regarding this uncommon oral mucosa lesion.

Complete regression of a melanocytic nevus after epilation with diode laser therapy.

The use of lasers and intense pulsed light (IPL) technology has become an established practice in dermatology and aesthetic medicine. The use of laser therapy and IPL in the treatment of pigmented melanocytic lesions is a controversial issue. We report clinical, dermoscopic and histological changes of a completely regressed pigmented melanocytic nevus after hair removal treatment with the LightSheer™ Diode Laser (Lumenis Ltd, Yokneam, Israel).

Nevus melanocítico congénito, a propósito de un caso / Congenital melanocytic nevi, case report

INTRODUCCIÓN: Los nevus melanocíticos son proliferaciones benignas de células névicas. Los nevus melanocíticos congénitos (NMC) representan el 1 por ciento del total y según su tamaño se clasifican en pequeños, medianos o gigantes. PRESENTACIÓN DEL CASO: Paciente de sexo masculino, 6 años de edad, con NMC gigante en forma de traje de baño. Controlado en policlínico de Dermatología desde su nacimiento, se mantuvo en observación realizándose exámenes imagenológicos, biopsias de piel y nódulos. En reunión multidisciplinaria respecto al caso, se plantea tratamiento quirúrgico incisional por etapas. DISCUSIÓN: El manejo de los NMC es controversial, se describen múltiples terapias que incluyen escisión, dermoabrasión, ablación con láser, etc. Pero para disminuir el riesgo de malignización la única efectiva es la escisión, no siempre posible en los NMC gigantes. Para los NMC de pequeño o mediano tamaño se recomienda un manejo individualizado con evaluaciones periódicas con dermatoscopía. En el caso de los NMC gigantes la mayoría de los autores concuerda en una extirpación temprana agresiva para disminuir el riesgo de malignización. En el caso clínico expuesto, el paciente presenta factores de riesgo para las dos principales complicaciones, por lo que se plantea el tratamiento quirúrgico. La remoción completa frecuentemente necesita de escisión por partes, usando expansores de piel e injertos dérmicos. Independiente de la terapia que se elija hay que considerar la necesidad del apoyo psicológico en este tipo de lesiones.

INTRODUCTION: Melanocytic nevi are benign proliferations of nevus cells. Represent 1 percent of all melanocytic nevi and are classified by size into small, medium or giant. CASE REPORT: Male patient, 6-year-old with giant congenital melanocytic nevi (CMN) as swimsuit. Controlled in the Department of Dermatology at birth, was kept under observation imaging tests, and skin biopsies performed nodules. In multidisciplinary meeting on the case, incisional surgical treatment arises in stages. DISCUSSION: The management of NMC is controversial; multiple therapies are described, including excision, dermabrasion, laser ablation, etc. But excision is the only way to reduce the risk of malignancy, not always possible in the giant NMC. NMC for small to medium size individualized management with periodic evaluations with dermoscopy is recommended. In the case of the giant NMC most authors agree on an aggressive early removal to reduce the risk of malignancy. In the case report, the patient had risk factors for the two major complications, so that surgical treatment is considered. Complete removal often requires cleavage by parts, using skin expanders and skin grafts. Independent of therapy you choose must consider the need for psychological support in this type of injury.

Melanoma conjuntival multifocal recidivado originado de nevus pigmentado preexistente / Recurrent multifocal conjunctival melanoma originated from preexisting pigmented nevus

O melanoma conjuntival multifocal recidivado originado de nevus preexistente é extremamente raro, ocorrendo em uma pessoa para cinco milhões de habitantes. Seu estudo é de extrema relevância, devido sua potencial letalidade. Este estudo objetiva descrever um caso de melanoma conjuntival multifocal recidivado proveniente de nevus pigmentado preexistente ocorrido em Patos de Minas, MG. Este é um estudo de caso com revisão de literatura. O diagnóstico histopatológico e o estadiamento precoce da lesão conjuntival é de fundamental importância para designar a conduta frente ao paciente. O procedimento terapêutico mais utilizado nos dias atuais é a excisão cirúrgica com crioterapia adjuvante associada à mitomicina C. O prognóstico do melanoma conjuntival multifocal recidivado originado de nevus preexistente é o pior dentre todos os melanomas oculares, apresentando alta taxa de mortalidade, 12% a 20% em 5 anos e 30% em 10 anos de desenvolvimento patológico.

Recurrent multifocal conjunctival melanoma originated from preexisting nevus is extremely rare: it occurs in one out of five million individuals. The investigation of this disease is extremely important due to its potential lethality. Thus, this study aims to describe a case of recurrent multifocal conjunctival melanoma originated from preexisting pigmented nevus, which occurred in the city of Patos de Minas, state of Minas Gerais. This is a case study and literature review. Histopathological diagnosis and early staging of the conjunctival lesion is a key element on how to approach the patient. The treatment procedure most commonly used today is surgical excision with adjuvant cryotherapy and mitomycin C. The prognosis of recurrent multifocal conjunctival melanoma originated from preexisting nevus is the worst of all ocular melanomas, with high mortality rate: 12% to 20% within 5 years and 30% within 10 years of pathological development.

Nevo melanocítico congénito gigante: presentación de tres casos / Giants congenital melanocytic nevus: Report of three cases

Los Nevos Melanocíticos Congénitos (NMC) son lesiones pigmentarias, que pueden aparecer en el nacimiento, aunque hay casos comunicados hasta el primer año de vida (Nevo Melanocítico Congénito Tardío). Presentan ciertas características clínicas y evolutivas de gran importancia: el compromiso estético de las formas gigantes, la asociación con melanocitosis meníngea y su transformación maligna de la lesión cutánea o meníngea. Presentamos tres casos de nevos melanocíticos congénitos gigantes. Cabe destacar, que el objetivo de este artículo es realizar una revisión del tema, orientar al seguimiento y control de los mismos y destacar la dificultad para su tratamiento debido a su gran tamaño.

The Congenital Melanocytic Nevus are lesions melanocytic pigmented. Already apparent at birth but there are cases reported to the first year of life (Late Congenital Melanocytic Nevus). Certain features and evolution clinics that are important: the great compromise of aesthetic forms giant, meningeal melanocitosis association and its malignant transformation. We present three cases of Giants Congenital Melanocytic Nevus. It should be noted, that the purpose of this article is a review of the issue and guide the monitoring and control of them and point out the difficult treatment.

Nevo melanocítico congénito gigante: presentación de tres casos / Giants congenital melanocytic nevus: Report of three cases

Los Nevos Melanocíticos Congénitos (NMC) son lesiones pigmentarias, que pueden aparecer en el nacimiento, aunque hay casos comunicados hasta el primer año de vida (Nevo Melanocítico Congénito Tardío). Presentan ciertas características clínicas y evolutivas de gran importancia: el compromiso estético de las formas gigantes, la asociación con melanocitosis meníngea y su transformación maligna de la lesión cutánea o meníngea. Presentamos tres casos de nevos melanocíticos congénitos gigantes. Cabe destacar, que el objetivo de este artículo es realizar una revisión del tema, orientar al seguimiento y control de los mismos y destacar la dificultad para su tratamiento debido a su gran tamaño.(AU)

The Congenital Melanocytic Nevus are lesions melanocytic pigmented. Already apparent at birth but there are cases reported to the first year of life (Late Congenital Melanocytic Nevus). Certain features and evolution clinics that are important: the great compromise of aesthetic forms giant, meningeal melanocitosis association and its malignant transformation. We present three cases of Giants Congenital Melanocytic Nevus. It should be noted, that the purpose of this article is a review of the issue and guide the monitoring and control of them and point out the difficult treatment.(AU)

Melanosis neurocutánea: Comunicación de un caso y revisión de la bibliografía / Neurocutaneous melanosis: Case report and literature review

La melanosis neurocutánea se caracteriza por la proliferación de melanocitos y depósitos de melanina en el sistema nervioso central asociada con nevos melanocíticos gigantes. Los pacientes con nevos melanocíticos congénitos gigantes localizados en el eje axial posterior (dorso, nuca o cabeza) o múltiples nevos melanocíticos congénitos pequeños son los que tienen mayor riesgo de presentarla. En la mayoría de los pacientes la melanosis neurocutánea es asintomática y se detecta como un hallazgo en los estudios por imágenes; sin embargo, los casos que desarrollan síntomas tienen mal pronóstico y el óbito se produce antes de los 3 años de iniciados. Se presenta una paciente con un nevo melanocítico congénito gigante y múltiples satelitosis, con compromiso asintomático del sistema nervioso central. Se destaca la importancia del seguimiento multidisciplinario de estos niños con el fn de detectar, en forma precoz, cualquier signo o síntoma neurológico que pudiesen desarrollar, como así también la presencia de melanoma.

Neurocutaneous melanosis is characterized by an increased number of melanocytes and melanin deposit in central nervous system associated with giant melanocytic congenital nevi. Patients with multiple satellite nevi or giant cutaneous melanocytic nevus in a midline location (overlying the back, neck or head) have more likelihood of having neurocutaneous melanosis. In most patients, the neurocutaneous melanosis is asymptomatic, only detectable by MRI; nevertheless, those patients with clinical manifestations have a poor prognosis, dying within 3 years of initial neurological manifestations. We present a patient with giant melanocytic congenital nevi and multiple satellite nevi associated with asymptomatic neurocutaneous melanosis. We emphazise the importance of multidisciplinary evaluation in order to detect early neurological symptoms and/or melanoma.

Tumores en colisión: quiste velloso eruptivo y nevus melanocítico congénito / Collision tumors: eruptive villous cyst and congenital melanocytic nevus

Presentamos dos casos de un tumor en colisión, de quistes vellosos con nevus melanocíticos congénitos, comentando en forma breve, aquellas características de asociación entre quistes y nevus melanocíticos, con diferentes patologías en coexistencia tumoral.

We presented two cases of a collision tumor, of a villous cyst within a congenital melanocytic nevus. We made short comments about those characteristics of the association of cysts and melanocytic nevus with different pathologies with tumoral coexistence.