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
Zosteriform metastasis from malignant melanoma is a rare type of skin metastasis that shows cutaneous lesions including patches, plaques, and nodules along with dermatomes, and thus needs to be distinguished from herpes zoster skin infection. Although some authors have explained the mechanism of zosteriform metastasis, its pathogenesis remains unknown. Herein, we describe an 85-year-old woman with zosteriform metastasis of malignant melanoma arising in a medium-sized congenital melanocytic nevus.
Subject(s)
Aged, 80 and over , Female , Humans , Herpes Zoster , Melanoma , Neoplasm Metastasis , Nevus, Pigmented , SkinABSTRACT
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
Giant congenital melanocytic nevi (GCMN) are defined as melanocytic nevi that are greater than 20 cm in largest dimension at birth. GCMN is a relatively rare condition, affecting approximately 1 in 20,000 newborns. Patients with GCMN on the posterior axis and in the presence of many satellite nevi are associated with neurocutaneous melanosis. A 37-year-old female with mental retardation had dark brown nevi with hair on her back (paraspinal) and on her lower extremities. In addition, she has multiple satellite lesions distributed over her trunk and extremities. Herein, we present a case that demonstrates a relationship between CNS abnormality and multiple satellite nevus in GCMN patients with a review of the relevant literature.
Subject(s)
Female , Humans , Infant, Newborn , Axis, Cervical Vertebra , Extremities , Hair , Intellectual Disability , Lower Extremity , Melanosis , Neurocutaneous Syndromes , Nevus , Nevus, Pigmented , ParturitionABSTRACT
Congenital melanocytic nevi (CMN) are benign pigmented lesions that are defined as a tissue malformation of the neuroectoderm. Giant melanocytic nevi with multifocal involvement show significantly greater risk of developing malignant melanomas and neurocutaneous melanocytosis, particularly those in a posterior axial location. Neurocutaneous melanosis is a rare, congenital, non-inherited disorder characterized by the presence of large, multiple congenital melanocytic nevi with proliferation of melanocytes in the central nervous system. Asymptomatic neurocutaneous melanosis can be detectable only by MRI. The patients who have clinical manifestations have an extremely poor prognosis. We present a patient with giant congenital melanocytic nevi involving a major portion of the back with multiple satellite nevi scattered over the whole body associated with asymptomatic neurocutaneous melanosis. We emphasize the importance of imaging study for detection of early neurological symptoms or melanomas. To our knowledge, this is the first case of giant congenital melanocytic nevi associated with asymptomatic neurocutaneous melanosis in Korean dermatologic literatures.
Subject(s)
Humans , Central Nervous System , Magnetic Resonance Imaging , Melanocytes , Melanoma , Melanosis , Neural Plate , Neurocutaneous Syndromes , Nevus , Nevus, Pigmented , PrognosisABSTRACT
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.
ABSTRACT
The proliferative nodules within a giant congenital melanocytic nevus (GCMN) are generally benign and may regress. However, atypical features cause great interest in dermatopathology. Especially, in neonate proliferative nodules with bleeding, ulceration on GCMN makes it difficult to differentiate the nodules from congenital malignant melanoma. Herein, we describe an interesting case of proliferative nodules in a GCMN of a newborn, which has not been previously reported in Korea.
Subject(s)
Humans , Infant, Newborn , Hemorrhage , Korea , Melanoma , Nevus, Pigmented , UlcerABSTRACT
Congenital melanocytic nevi (CMN) are benign pigmented lesions found in about 1% of all newborns or shortly after birth. Giant melanocytic nevi, with multifocal involvement, show significantly greater risk of developing malignant melanomas and neurocutaneous melanosis (NCM), particularly in a posterior axial location. NCM is a rare congenital disease characterized by multiple (> or =3) small nevi, or at least one large congenital melanocytic nevus in combination with cerebral and/or leptomeningeal melanin deposits or melanoma. Dandy-Walker malformation (DWM) consists of a cystic dilatation of the fourth ventricle, hypoplasia or aplasia of the cerebellar vermis, and enlarged posterior fossa with or without hydrocephalus. The association of DWM and NCM has rarely been reported in the literature. A 3 month-old girl presented with increased head circumference and multiple various sized black plaques on her whole body. She underwent a ventriculoperitoneal shunt operation when she was 2 months-old. A skin biopsy was taken from the largest and darkest plaque of the trunk and showed hyperpigmentation of the basal layer of the epidermis. The dermis contained nevus cells in nests and sheets throughout the dermis, but no cellular atypia was noted. Magnetic resonance image (MRI) of the brain revealed severe hydrocephalus with hypoplasia of cerebellar vermis and agenesis of cerebellar tonsil which are consistent with Dandy-Walker malformation. We recommended a spinal MRI for check up the presence of leptomeningeal melanosis, but could not evaluate the result because of her being adapted. Herein, we present a rare case of multiple congenital melanocytic nevi in association with DWM in a neonate.
Subject(s)
Humans , Infant, Newborn , Biopsy , Brain , Dandy-Walker Syndrome , Dermis , Dilatation , Epidermis , Fourth Ventricle , Head , Hydrocephalus , Hyperpigmentation , Magnetic Resonance Spectroscopy , Melanins , Melanoma , Melanosis , Neurocutaneous Syndromes , Nevus , Nevus, Pigmented , Palatine Tonsil , Parturition , Skin , Ventriculoperitoneal ShuntABSTRACT
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
Congenital melanocytic nevus (CMN) may rarely regress which may also be associated with a halo or vitiligo. We describe a 10-year-old girl who presented with CMN on the left leg since birth, which recently started to regress spontaneously with associated depigmentation in the lesion and at a distant site. Dermoscopy performed at different sites of the regressing lesion demonstrated loss of epidermal pigments first followed by loss of dermal pigments. Histopathology and Masson-Fontana stain demonstrated lymphocytic infiltration and loss of pigment production in the regressing area. Immunohistochemistry staining (S100 and HMB-45), however, showed that nevus cells were present in the regressing areas.
ABSTRACT
Large congenital melanocytic nevi (>20 cm in greatest diameter) are very rare and are seen in approximately 1 in 20,000 newborns. The major risk these patients face is the development of neurocutaneous melanosis or malignant melanoma. We report a rare case of large congenital melanocytic nevus with metastatic melanoma in a 40-year-old woman. In this case, though the primary was not established with certainty, on the basis of clinical course and radiological evaluation of various organs, we presume that the primary could be in the lung.
ABSTRACT
Completely neurotized melanocytic nevi are often clinically and histologically indistinguishable from neurofibromas. We report a 23-year-old female who presented with a dark brown plaque with an underlying soft blue subcutaneous nodule in the left infra-axillary area. The skin biopsy revealed markedly increased melanocytic proliferation and nests of nevus cells within dermo-epidermal junction and upper dermis. Circumscribed, non-encapsulated tumor of the dermis was composed of loosely-spaced spindle cells and wavy stroma. Spindle cells in the lower dermis showed staining for S-100, but failed to express glial fibrillary acidic protein and Leu-7. We present an interesting case because the lesion presented as a soft blue subcutaneous nodule suggested an underlying hemangioma or neurofibroma initially, but was shown to be a neurotized melanocytic nevus based on immunohistochemical examination.
Subject(s)
Female , Humans , Young Adult , Biopsy , Dermis , Glial Fibrillary Acidic Protein , Hemangioma , Neurofibroma , Nevus , Nevus, Pigmented , SkinABSTRACT
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
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
A congenital melanocytic nevus (CMN) is present at birth in approximately 1% of newborn infants. Treating CMN with a single laser without complications is very difficult because of the deep nature of the pigments in this lesion. A 5-year-old girl presented with a single 3x6 cm sized dark brown plaque on the right lower leg; some black hairs were also evident inside the pigmented lesion. She received ten treatments with intense pulsed light and then four treatments with Q-switched Nd:YAG laser. In order to achieve the chemoexfoliation, a topically modified Kligman formula (Trilusta(R)) was applied to the pigmented lesion twice a day for 6 months. Complete pigment clearance and hair removal were obtained and no side effects were observed. These results have been maintained for up to 18 months follow-up. We suggest that this combined treatment will be a good option for treating CMN with minimal scarring.
Subject(s)
Humans , Infant, Newborn , Cicatrix , Follow-Up Studies , Hair , Hair Removal , Light , Nevus, Pigmented , Parturition , Child, PreschoolABSTRACT
Nevus with cyst is defined as a single lesion in which there is the coexistence of an epidermoid cyst and a melanocytic nevus. Similar clinical and histopathologic changes can be observed when a hair follicle ruptures and subsequent folliculitis with supprative granulomatous reaction occurs beneath a melanocytic nevus. This cystic change due to inflammation is a different pathologic phenomenon from the formation of an epidermal cyst. Hence, it is necessary to differentiate between these two conditions. We report here on a case of congenital melanocytic nevus combined with cystic change due to inflammation in a 39-year-old man.