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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.
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@#Among the three subtypes of neurofibromatosis are type 1 and 2 neurofibromatosis and schwannomatosis, von Recklinghausen disease also known as type 1 neurofibromatosis has an autosomal dominant inheritance. It is the commonest form as and presents with numerous café-au-lait macules and neurofibromas. Giant congenital melanocytic nevus (CGMN) on the other hand is characterized by a melanocytic proliferation that present at birth. CGMN develops due to a defective embryonic pigment cell (melanocyte) precursors development and are often present at birth. Giant congenital melanocytic nevus (CGMN) and type 1 neurofibromatosis may occur together rarely. Clinicians should be aware of the rare presentation of both CGMN and type 1 neurofibromatosis in a patient.
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Objective:To investigate the efficacy of tumescent anesthesia combined with skin and soft tissue expansion for the repair of congenital giant melanocytic nevi.Methods:From July 2015 to December 2019, 41 patients with congenital giant melanocytic nevi, including 24 males and 17 females aged 7 - 45 years, were collected from the Department of Dermatology, Xijing Hospital, the Fourth Military Medical University. Skin lesions ranged from 5 cm × 12 cm to 12 cm × 18 cm in size, and were located on the scalp in 13 cases, on the face in 18 cases, as well as on the trunk in 10 cases. Before surgery, the composition of tumescent solution was adjusted according to the body weight, operation duration, skin lesion area, etc., and the total dose and peak plasma concentration of lidocaine should be below 35 mg/kg and 4 mg/L respectively. All the patients received tissue expander placement and second-stage flap transfer under tumescent anesthesia.Results:During surgery, satisfactory effect of tumescent anesthesia was achieved in all the 41 patients, the pain score assessed by a numerical rating scale was 1.82 ± 0.54. In addition, the surgical field and dissection levels were clear with little bleeding and no related complications. Follow-up of 3 - 36 months showed that the skin flaps matched the surrounding skin tissues well, with relatively concealed incision lines and soft flat scars.Conclusion:For the treatment of congenital giant melanocytic nevi, tumescent anesthesia is effective and safe, which combined with skin and soft tissue expansion can effectively reduce the incidence of postoperative complications, and this strategy is worthy of clinical promotion.
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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.
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Humans , Skin Neoplasms/diagnosis , Dermatology , Melanoma/diagnosis , Nevus, Pigmented/diagnosis , Brazil , Follow-Up Studies , Dermoscopy , Diagnosis, DifferentialABSTRACT
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
Subject(s)
Humans , Male , Female , Child , Adult , Skin Neoplasms/congenital , Skin Neoplasms/diagnosis , Dermoscopy , Nevus, Pigmented/congenital , Nevus, Pigmented/diagnosisABSTRACT
Objective To analyze special histopathological characteristics of melanocytic nevi and their associations with age,gender,anatomical locations and pathological subtypes.Methods Clinical and histopathological data were collected from 1 011 patients with melanocytic nevi,who visited Beijing Hospital from January 2005 to January 2019,and analyzed retrospectively.Statistical analysis was carried out by using chi-square test for comparing enumeration data,and t test for comparing measurement data.Results Among the 1 011 patients with melanocytic nevi,the age at the clinic visit was 40.90 + 19.19years,and there were 289 males and 722 females.Lesional (biopsy) sites included the trunk (402 cases,39.8％),face and neck (268 cases,26.5％),extremities (138 cases,13.6％),hands and feet (133 cases,13.2％),scalp (53 cases,5.2％) and vulva (17 cases,1.7％).Pathological subtypes included intradermal nevus (580 cases,57.4％),compound nevus (333 cases,32.9％) and junctional nevus (98 cases,9.7％).Among special histopathological characteristics,neuralization and adipose cell hyperplasia were observed in 172 (17.0％) and 155 (15.3％) cases respectively,and the prevalence of neuralization and adipose cell hyperplasia was significantly higher in female patients than in male patients,higher in elderly patients than in young patients,and higher on the scalp than on the other sites (all P ＜ 0.05);vascular proliferation was observed in 313 (31.0％) cases,and more commonly occurred on the scalp than on the other sites (P＜0.05);nevus cells distributed along the hair follicles/sebaceous glands were observed in 502 (49.7％)cases,and more commonly seen on the face and neck than on the other sites (P ＜ 0.05);nevus cell lysis occurred in 203 (20.1％) cases,and fissures were observed in 384 (38.0％).All the above histopathological characteristics were more frequently observed in the intradermal nevus subtype than in the compound nevus subtype (all P＜0.05).Nevus cells distributed along the blood vessels were observed in 20 (2.0％) cases,and more commonly seen on the extremities than on the trunk,hands and feet (P ＜ 0.05),as well as in the compound nevus subtype than in the intradermal nevus subtype (P ＜ 0.05).Conclusions There are many special histopathological characteristics in melanocytic nevi,such as neuralization,adipose cell hyperplasia,vascular proliferation,and nevus cells distributed along the hair follicles/sebaceous glands,which are associated with patients' age,gender,lesional locations and histopathological subtypes.
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Here we report a case of a focal atypical proliferative nodule (PN) arising from a congenital melanocytic nevus (CMN). Diagnosis was challenging because it had both benign and malignant clinical features. Unusual histopathology, immunohistochemistry, and intraoperative findings of this atypical PN are discussed. A 5-year-old girl was admitted for a congenital 5× 5 cm sized scalp mass. This hemangioma-like soft mass showed biphasic characteristics such as a slow, gradual, and benign increase in size but worrisome dural invasion with cranial bone defect. We removed the scalp mass with clear resection margins. Interoperatively, we found that the cranial bone defect had already filled. Histopathologic examination showed CMN with focal atypical PN. The nodule showed sharp demarcation and cellular pleomorphism. However, in immunohistochemical study, Ki-67 proliferation index and expression levels of protein S-100 and Melan-A were very low. These were unusual findings of atypical PNs. Despite her worrisome preoperative radiologic features, she showed an indolent clinical course compatible with previously reported biologic behavior. The patient underwent follow-up inspection with magnetic resonance imaging every 6 months for up to 3 years. The nodule appeared to be stationary at the last visit.
Subject(s)
Child, Preschool , Female , Humans , Diagnosis , Dura Mater , Follow-Up Studies , Immunohistochemistry , Magnetic Resonance Imaging , MART-1 Antigen , Neoplasm Invasiveness , Nevus, Pigmented , Scalp , Tissue Expansion DevicesABSTRACT
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.
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Although the management of benign pigmented skin tumors are mainly conducted by dermatologists, some Korean traditional doctors provide care. We aimed to evaluate the appropriateness of the medicinal knowledge of pigmented benign skin tumors that is recorded in the Korean traditional medicinal literature, in the context of modern medicine. We defined benign pigmented skin tumors as macules, papules, or nodules with homogeneous surfaces and coloration patterns, that are round or oval in shape, exhibit regular outlines, and have relatively sharp borders. We investigated textbooks and articles in the Korean traditional medicinal field to analyze descriptions of clinical classification, pathophysiology, histologic knowledge, and treatment method. We compared them with modern medicinal facts. In Korean traditional medicine, clinical classification of pigmented skin tumors is simple and did not include histologic natures. Unique theories, such as Yin-Yang and Qi, were applied to pathophysiologic understanding of these diseases. Interestingly, oral medications were used beside surgical methods. We could not find any comment about skin tumors with worrisome clinical features that warrant excision in Korean traditional medicinal literature. There is still a gap between traditional medicine and modern medicine regarding pigmented skin tumors. Traditional Korean medicinal knowledge about benign pigmented skin tumors seemed to be insufficient in the context of modern medicinal standards.
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Classification , Diagnosis , History, Modern 1601- , Medicine, Korean Traditional , Medicine, Traditional , Melanoma , Methods , Nevus, Pigmented , Qi , Skin , Yin-YangABSTRACT
BACKGROUND: An awareness of the prevalence and morphologic patterns of acral melanocytic nevi (AMN) is crucial in the diagnosis of AMN and for its differentiation from acral melanoma. Although studies regarding prevalence and dermoscopic patterns of AMN in other ethnic groups have been reported, studies in Korean populations are scarce. OBJECTIVE: To investigate the prevalence, number and dermoscopic patterns of AMN cases in a Korean population. METHODS: We investigated the prevalence and dermoscopic patterns of AMN in a Korean population. RESULTS: Six hundred Korean patients were enrolled in this study. AMNs were observed on the hands in 53.2% of patients and on the feet in 30.5% of patients. The prevalence of AMN according to anatomical location was as follows: dorsum of hands (43.8%), palms (15.7%), both palms and dorsum of hands (6.3%), dorsum of feet (24.8%), soles (9.2%), and dorsa of feet and soles (3.5%). On volar skin, the common dermoscopic patterns were as follows: parallel-furrow (51.7%), lattice-like (12.1%), globular (9.2%), fibrillar (8.6%), reticular (7.5%), and homogeneous (5.7%). On dorsal skin, reticular (41.0%), homogeneous (27.0%), globular (21.6%), and reticular-homogeneous (7.7%) patterns were commonly observed. The prevalence of AMN increased with patient age until patients reached their forties and decreased according to age once patients reached their sixties. CONCLUSION: The results of our study show that AMN on hands and feet are common in Korean patients and that characteristic dermoscopic patterns of AMN exist. This study provides information regarding the prevalence and dermoscopic patterns of AMN in a Korean population.
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Humans , Dermoscopy , Diagnosis , Ethnicity , Foot , Hand , Melanoma , Nevus, Pigmented , Prevalence , SkinABSTRACT
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.
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Aged, 80 and over , Female , Humans , Herpes Zoster , Melanoma , Neoplasm Metastasis , Nevus, Pigmented , SkinABSTRACT
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.
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Humans , Male , Skin Neoplasms/congenital , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Nevus, Pigmented/congenital , Nevus, Pigmented/diagnosis , Nevus, Pigmented/pathology , Skin Neoplasms/surgery , Nevus, Pigmented/surgeryABSTRACT
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.
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Humans , Male , Adult , Recurrence , Conjunctival Neoplasms/pathology , Melanoma/pathology , Nevus, Pigmented/pathology , Ophthalmologic Surgical Procedures , Biological Dressings , Biopsy , Conjunctival Neoplasms/surgery , Conjunctival Neoplasms/diagnosis , Amnion/transplantation , Melanoma/surgery , Melanoma/diagnosis , Nevus, Pigmented/complicationsABSTRACT
BACKGROUND: Melanomas need to be differentiated from benign melanocytic lesions during diagnosis. However, it is difficult to differentiate them using histopathology alone, since both neoplasms have broad morphological spectrums and subtle differentiating features. OBJECTIVE: To evaluate the usefulness of Ki-67/Melan-A double staining for differentiating melanoma from benign melanocytic nevi. METHODS: We selected 20 cases of intradermal nevi, 20 cases of compound nevi, 5 cases of dysplastic nevi, and 25 cases of melanoma from clinicopathologically proven cases reviewed by the Department of Dermatology at our medical center. Ki-67/Melan-A double staining was performed, and the Melan-A verified Ki-67 index (Ki-67-M index) and Ki-67 index were measured. The immunopositivity was measured in the deepest third of the lesions. RESULTS: The Ki-67-M index of intradermal nevi, compound nevi, dysplastic nevi, and melanoma were 0.4+/-0.9%, 1.0+/-1.1%, 4.3+/-1.7%, and 24.1+/-10.9%, respectively. The best Ki-67/Melan-A cut-off point to distinguish melanomas from benign melanocytic nevi was 5%; the sensitivity and specificity were 100% and 97.7%, respectively. Immunopositivity in the deepest third of the intradermal nevi, compound nevi, and melanoma, were 10.5%, 20%, and 100%, respectively; the sensitivity and specificity for diagnosing melanoma were 100% and 84.6%, respectively. The sensitivity and specificity of combined Ki-67-M and immunopositivity in the deepest third for diagnosing melanoma were 100% and 97.7%, respectively. CONCLUSION: The Ki-67-M index and immunopositivity in the deepest third of melanoma were significantly higher than that of benign melanocytic nevi. Therefore, Ki-67/Melan-A double staining is a potentially valuable diagnostic tool for differentiating melanoma from benign melanocytic nevi.
Subject(s)
Dermatology , Diagnosis , Dysplastic Nevus Syndrome , MART-1 Antigen , Melanoma , Nevus , Nevus, Intradermal , Nevus, Pigmented , Sensitivity and SpecificityABSTRACT
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.
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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.
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Female , Humans , Infant, Newborn , Axis, Cervical Vertebra , Extremities , Hair , Intellectual Disability , Lower Extremity , Melanosis , Neurocutaneous Syndromes , Nevus , Nevus, Pigmented , ParturitionABSTRACT
A nevus with cyst is defined as a single lesion of a melanocytic nevus, and this is commonly associated with an epidermal cyst. There have been rare reported cases of adnexal origin. The combination of nevus and cyst could possibly be misdiagnosed clinically as a malignant melanoma at initial examination. A 51-year-old woman presented with a painful, solitary, dome-shaped, erythemaous nodule on the forehead which had been present for several years. The histopathologic specimen of the nodule showed a solitary cyst with a melaocytic nevus. There were nevus cells without junctional activities in the upper dermis and melanin pigments in the upper dermal nevus nest. It contained lamellated keratin materials and vellus hair shafts. We report here a rare case of melanocytic nevus combined with an vellus hair cyst in a 51-year-old woman.