ABSTRACT
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.
ABSTRACT
A variety of malignancies have been documented to arise within congenital melanocytic nevi (CMNs).Although the most frequent malignancy arising within a CMN is melanoma, the association betweenrhabdomyosarcoma and CMN has rarely been documented. We report a 4-month-old girl presentedwith ulcerated nodule overlying a giant CMN at the posterior back that exhibited rapid growth.Biopsy of the nodule revealed embryonal rhabdomyosarcoma in association with CMN. She receivedchemotherapy with vincristine and actinomycin D. This the first case of rhabdomyosarcoma associatedwith giant CMN reported in our local setting. Clinicians must consider rhabdomyosarcoma as one ofthe differential diagnosis in patients presenting with ulcerated nodules on giant CMN.
ABSTRACT
Congenital nevi are hyperpigmented macular lesions that are derivatives of the melanoblasts. Crude incidence estimates suggest that approximately 1 in 20 000 are born with a large CMN and 1 in 500 000 are born with a very large (giant) CMN. They occur in less than 1% of the neonates in any site of the body. The giant congenital nevus is greater than 20 cm in size, pigmented and often hairy. We report a classical case of large congenital melanocytic nevus present over face. The case report is being presented because of its rarity.
ABSTRACT
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.
Subject(s)
Female , Humans , Infant , Melanosis/pathology , Neurocutaneous Syndromes/pathologyABSTRACT
Melanoma may arise from a very giant congenital melanocytic nevus even in the first several years of life. Therefore, excision of a giant congenital melanocytic nevus should be considered as early as possible. However considering surgical and anesthetic risks, the surgery can wait until after the first 6 months of life. Unfortunately, surgical treatment does not completely prevent occurrence of melanoma from a giant congenital melanocytic nevus. Besides, several cases of melanoma occurring after blunt trauma or laser therapy have been reported. We report a case of malignant melanoma associated with a giant congenital melanocytic nevus in a 4-year-old female. The malignant melanoma occurred at the boundaries of the previous surgical excision and laser therapy sites for a giant congenital melanocytic nevus. This is the youngest case of malignant melanoma developed from a congenital melanocytic nevus in the Korean dermatological literature.
Subject(s)
Female , Humans , Laser Therapy , Melanoma , Nevus, Pigmented , Child, PreschoolABSTRACT
ObjectivePresent treatment in plastic surgery on giant congenital melanocytic nevus has always been a tough practice because it is difficult to achieve balance between effects and costs of treatment.This paper aimed to explore the concrete procedure of tangential excision and dermabrasion in treatment of adult giant congenital melanocytic nevus. Methods Taking into consideration pathological examination results before surgery,diseased regions,psychological expectancy and other factors,we used a humby knife or globe grinding head to remove giant congenital relanocytic nevus by wiping off the surface of it in 10 cases.After operation,the operated area of the skin underwent a process of healing in a moisturized state.In each case,surgical procedure was carried out by 1 2 sta ges,with the interval period ranges from 3 months to 6 months.ResultsOne to 3 years follow-ups showed that among those cases,5 cases obtained good results in which skin color of surgical area turned to normal and pathological examination showed that nevus cells disappeared,4 cases achieved improvement,and 1 case was relapsed.ConclusionsThe two alternative methods for treatment of giant congenital melanocytic nevus,either tangential excision or dermabrasion,with combination of pathological examination results,diseased regions,and psychological expectancy should be taken into consideration,which can remain a maximum balance between effects and costs of treatments.Tangential excision and dermabrasion are effective in some cases of giant congenital nevus where traditional methods do not work,or in order to reduce the cost of body appearance in treatment.Therefore,these two methods deserve to be adopted extensively in clinical therapy.But it still needs further accumulation of experience in practice and longer period of follow-up after operation.
ABSTRACT
In the spectrum of neurofibroma, pigmented neurofibroma is a rare variant observed in patients with or without neurofibromatosis. It is histologically characterized by the presence of scattered melanin-laden pigmented cells within the neurofibromatous lesion. Herein, we describe a case of a 30-year-old man who presented with a large pigmented patch showing focal hypertrichosis on the upper back. The lesion had been present at birth and there was no other cutaneous features to support a diagnosis of neurofibromatosis. Histologically, a proliferation of melanocytes was evident in the epidermis and upper dermis and within underlying neurofibromatous tissue. This abnormality resulted in diagnostic difficulty in the differentiation between pigmented neurofibroma and large congenital melanocytic nevus with complete neurotization because of the clinical and histological similarities. In support of the use of immunohistochemical markers, the diagnosis of pigmented neurofibroma could be made.
Subject(s)
Adult , Humans , Dermis , Epidermis , Hypertrichosis , Melanocytes , Nerve Transfer , Neurofibroma , Neurofibromatoses , Nevus, Pigmented , ParturitionABSTRACT
Malignant melanoma is a very rare disease in children. There is an increased risk for the development of malignant melanoma in patient with giant congenital melanocytic nevus. The manifestations of distant metastases in malignant melanoma commonly indicate a poor prognosis. First line treatment of malignant melanoma is excision, but when associated with giant congenital melanocytic nevus, excision is very difficult due to the site and the size of the lesions. However, malignant melanoma is not sensitive to chemotherapy, but a child is more sensitive than an adult. We report a case of unresectable childhood malignant melanoma associated with a giant congenital melanocytic nevus in a 3 year-old female treated with chemotherapy.