Sebaceous nevus of Jadassohn: review and clinical-surgical approach

Abstract Background: Nevus sebaceous of Jadassohn is defined as a rare congenital malformation characterized as a non-hereditary hamartoma of the adnexal structures of the skin. Its etiology is not yet well understood, but it is believed to be related to post-zygotic mutations in the HRAS, NRAS and KRAS genes. Objective: To describe the clinical manifestation of nevus sebaceous, as well as the main management techniques addressed in the medical literature. Moreover, the present study discusses a case report of a congenital linear nevus in the left retroauricular region found in a male patient, without extracutaneous manifestations. Method: A narrative review of the literature was carried out. Discussion: Nevus sebaceous occurs as lesions with a linear or oval appearance, with a smooth or verrucous texture, generally alopecic and with very variable color. Moreover, nevus sebaceous is one of the components of the so-called linear nevus syndrome or Schimmelpenning-Feuerstein-Mims syndrome, which is associated with multisystemic complications. The treatment of the lesions is still controversial; however, most experts indicate surgical excision as the most frequently adopted treatment method, in addition to multidisciplinary follow-up when the diagnosis of Schimmelpenning-Feuerstein-Mims syndrome is established. Conclusion: The linear nevus syndrome constitutes a rare manifestation; however, its diagnosis should be considered in children born with nevus sebaceous. There is no consensus yet on the best therapy, but surgical removal has shown to be a viable option.

Nevo sebáceo com transformação maligna tratado com cirurgia micrográfica de Mohs: relato de caso / Nevus sebaceous with malignant transformation treated with Mohs surgery: A case report

Nevo sebáceo de Jadassohn é um hamartoma congênito da pele que se localiza mais frequentemente em face e couro cabeludo. Uma pequena porcentagem desses tumores evolui para lesões malignas, dentre elas o carcinoma basocelular. Relata-se um caso de paciente idoso com nevo sebáceo desde a primeira infância, que evoluiu com transformação maligna da lesão para carcinoma basocelular. O tratamento realizado foi a excisão cirúrgica por meio da cirurgia micrográfica de Mohs.

Nevus sebaceous of Jadassohn is a congenital skin hamartoma often located on the face and scalp. A small percentage of these tumors evolve into malignant lesions, including basal cell carcinoma. We report a case of an elderly patient with nevus sebaceous since early childhood that evolved with a malignant transformation from the lesion to basal cell carcinoma. The treatment performed was surgical excision using Mohs surgery

Do you know this syndrome? Schimmelpenning-Feuerstein-Mims syndrome

Abstract Nevus sebaceous is the most common type of organoid epidermal nevus, often located on the face, following the Blaschko's lines and with alterations in the ipsilateral central nervous system. Distinct disorders can be distinguished by the type of association with epidermal nevus. Schimmelpenning-Feuerstein-Mims syndrome is a rare multisystem disorder characterized by sebaceous nevus associated with extracutaneous abnormalities affecting the brain, eyes and bones. We report the case of an 8-year-old female patient with a yellowish verrucous plaque on the left temporal area extending ipsilaterally to the cervical region, combined with cicatricial alopecia, periocular nodule, and epibulbar tumors.

Carcinoma basocelular, siringocistoadenoma papilífero, adenoma apócrino e triquilemoma sobre nevo sebáceo de Jadassohn / Basal cell carcinoma, papillary syringocystadenoma, apocrine adenoma and trichilemmoma on nevus sebaceous of Jadassohn

Descrito por Jadassohn e também conhecido como nevo organoide, o nevo sebáceo é considerado hamartoma que exibe má-formação folicular, sebácea, écrina e apócrina de graus variados. Durante a idade adulta, de dez a 30% dos pacientes com nevo sebáceo de Jadassohn têm risco de desenvolver neoplasia cutânea ou anexial. Relatamos caso de paciente com nevo sebáceo de Jadassohn associado a múltiplas neoplasias (benignas e malignas) de diferentes linhagens e ressaltamos a importância do conhecimento dessa entidade e do exame do couro cabeludo por parte do dermatologista.

Described by Jadassohn and also known as organoid nevus, nevus sebaceous is considered a hamartoma that exhibits follicular, sebaceous, eccrine and apocrine malformations of varying degrees. Between 10% and 30% of patients with sebaceous nevi of Jadassohn are at risk of developing cutaneous or adnexal neoplasia during adulthood. The authors describe the case of a patient with nevus sebaceous of Jadassohn associated with multiple tumors (benign and malignant) of different strains, highlighting the importance of the dermatologist physician's knowing this entity and how to perform an examination of the scalp.

Siringocistoadenoma papilífero: reporte de 11 casos del Hospital Clínico de la Universidad de Chile / Papilliferum Syringocystoadenoma: report of 11 cases of Clinical Hospital of the University of Chile

Introducción: El siringocistoadenoma papilífero (SP) es un tumor anexial benigno inusual, de etiología desconocida. Por lo general, se presenta desde el nacimiento, o en la primera infancia como una lesión aislada, frecuentemente asociada a un nevo sebáceo de Jadassohn. Objetivo: Describir y analizar las características demográficas, clínicas e histopatológicas del SP. Materiales y métodos: Revisión retrospectiva de informes histopatológicos del Departamento de Anatomía Patológica del Hospital Clínico de la Universidad de Chile entre los años 2005 y 2016. Se incluyeron todas las muestras que describieron dentro del diagnóstico histológico las palabras "siringocistoadenoma papilífero". Resultados: La muestra está compuesta por 11 pacientes; 9 mujeres y 2 hombres. En 5 pacientes, se desarrolló un SP a partir de un nevo sebáceo y en 6 surgió de novo. El total de la muestra desarrolló el SP durante o posterior a la pubertad. Tanto los pacientes con SP solitario, como aquellos asociados a un nevo sebáceo, presentaron clínica similar con una placa o pápula verrucosa aislada. Los principales diagnósticos diferenciales fueron el nevo sebáceo, nevo verrucoso y verruga. Conclusiones: Este estudio constituye la primera serie de casos de SP en un hospital en Santiago de Chile. La forma clínica más frecuente fue como placa verrucosa solitaria asintomática, con casos aislados como pápula verrucosa y sólo un caso con aspecto de cuerno cutáneo. Se destaca el rol de la extirpación quirúrgica completa, tanto para establecer el diagnóstico, como para ofrecer un tratamiento definitivo, disminuyendo el riesgo de transformación maligna a siringocistoadenocarcinoma papilífero.

Introduction: Syringocystoadenoma papilliferum (SCAP) is an unusual benign adnexal tumor of unknown etiology. Usually it is presented at birth or in early childhood as an isolated lesion associated with nevus sebaceous of Jadassohn. Objective: To describe and analyze the demographic, clinical and histopathological characteristics of SCAP. Materials and methods: A retrospective review of histopathological reports was performed at the Clinical Hospital´s Department of Pathological Anatomy from the University of Chile including biopsies from 2005 to 2016. All the samples that described the histological diagnosis of "syringocystoadenoma papilliferum" were included. Results: The sample consists of 11 patients; 9 women and 2 men. In 5 patients, an SCAP was developed from a sebaceous nevus and in 6 it developed de novo. The total sample developed SCAP during or after puberty. Both the patients with solitary SCAP, and those associated with nevo sebaceous, presented similar clinical signs with an isolated verrucous plaque or papule. The main differential diagnoses were the nevo sebaceous, warty nevus and wart. Conclusions: This study is the first SCAP case series in Santiago, Chile. The most frequent clinical form was an asymptomatic solitary verrucous plaque, with isolated cases as a verrucous papule and a single case as a cutaneous horn. The role of complete surgical resection is highlighted, both to establish the diagnosis and to offer a definitive treatment, reducing the risk of malignant transformation to syringocystadenocarcinoma papilliferum.

Syringocystadenoma papilliferum combined with a tubular apocrine adenoma

Abstract: Syringocystadenoma papilliferum and tubular apocrine adenoma are rare benign sweat gland tumors. Syringocystadenoma papilliferum occurs alone or in association with other tumors. Although it is rare, the association of tubular apocrine adenoma with syringocystadenoma papilliferum developing in a sebaceous nevus on the scalp is well documented. However, the co-existence of these two tumors without the background of a sebaceous nevus has not been frequently reported. Syringocystadenoma papilliferum and tubular apocrine adenoma may have a histopathological overlap, but a few cases of a syringocystadenoma papilliferum combined with a tubular apocrine adenoma have been reported. Herein we describe an unusual case of syringocystadenoma papilliferum co-existing with a tubular apocrine adenoma located on the back of a 14-year-old patient in the absence of a pre-existing sebaceous nevus.

Carcinoma basocelular desenvolvido sobre nevo sebáceo: tratamento com terapia fotodinâmica abordando campo de cancerização / Basal cell carcinoma growth over a nevus sebaceous: treatment of the field cancerization with photodynamic therapy

Nevo sebáceo de Jadassohn é hamartoma congênito que pode apresentar evolução para neoplasia cutânea maligna. A terapia fotodinâmica é utilizada para tratamento de ceratoses actínicas e carcinomas basocelulares superficiais ou nodulares, podendo-se observar o campo de cancerização cutâneo através da lâmpada de Wood, durante a realização da técnica. Relata-se um caso do uso da terapia fotodinâmica para o tratamento de um carcinoma basocelular, que se desenvolveu sobre nevo sebáceo, demonstrando-se o campo cancerizável através do uso da lâmpada de Wood. O procedimento consistiu em alternativa de tratamento não cirúrgico para o carcinoma basocelular, com excelente resultado estético. A paciente encontra-se em seguimento clínico, não apresentando recidiva da neoplasia 18 meses após o tratamento.

The sebaceous nevus of Jadassohn is a congenital hamartoma that may develop into a malignant cutaneous neoplasia. Photodynamic therapy is used to treat actinic keratoses and superficial or nodular basal cell carcinomas, and the cutaneous field cancerization can be observed using the Wood's lamp during the performance of the technique. This article describes a case of photodynamic therapy used in the treatment of a basal cell carcinoma, which developed on a sebaceous nevus, where the field cancerization was demonstrated through the use of Wood's lamp. The procedure is a non-surgical alternative for the treatment of the basal cell carcinoma, with excellent aesthetic outcome. The patient is on clinical follow-up, with absence of recurrence of the neoplasia 18 months after the treatment.

Siringocistoadenoma papilífero y carcinoma basocelular en nevo sebáceo: reporte de caso / Papilliferous sarcomatocystoma and basal cell carcinoma in nevus sebaceous: case report

El nevo sebáceo o nevo de Jadassohn es un hamartoma cutáneo congénito poco frecuente formado por múltiples estructuras cutáneas. Presenta potencial de transformación a una variedad de neoplasias epidérmicas benignas y malignas, que suelen presentarse de manera individual. Si bien esta descrito en la literatura, es inusual el desarrollo de más de una neoplasia sobre un nevo sebáceo. En esta publicación se presenta el caso de un hombre de 62 años con una lesión de 3 años de evolución, cuyo estudio histopatológico demostró la presencia de un carcinoma basocelular y un siringocistoadenoma papilífero sobre un nevo sebáceo de Jadassohn.

Nevus sebaceous of Jadassohn is an infrequent cutaneous congenital hamartoma, formed by multiple cutaneous structures. It possesses a transformation potential to benign and malignant epidermic neoplasms, that usually present individually. Even though a few cases have been published, the coexistence of two or more tumors is rare. We hereby present the case of a 62 years old male, with a nevus sebaceous of Jadassohn history, that after excisional biopsy showed the presence of a basal cell carcinoma associated to a syringocystoadenoma papilliferum.

Nevus sebáceo cerebriforme: uma rara apresentação

A 13-year-old boy presented congenital, raised, yellowish colored plaques with fibroelastic consistency, which were velvety in appearance, alopecic, with clearly defined limits and sulci or well-marked meandering invaginations, a cerebriform appearance on the upper part of the right ear, accompanied by alopecia in the temporomandibular region-parietal. The lesion was asymptomatic. Histopathologic examination revealed acanthosis, hyperkeratosis, papillomatosis and follicular plugging in the epidermis. The upper dermis showed underdeveloped hair follicles with sebaceous glands, consistent with sebaceous nevus. There were no skeletal, cardiological, ophthalmological or neuropsychomotor changes; nor were there any records of relevant pathologies in the family history. The patient complained of his appearance, experiencing aesthetic and social issues. Following a diagnosis of cerebriform nevus sebaceous, complete excision was performed with excellent aesthetic results.

Cerebriform sebaceous nevus: a rare presentation

Abstract A 13-year-old boy presented congenital, raised, yellowish colored plaques with fibroelastic consistency, which were velvety in appearance, alopecic, with clearly defined limits and sulci or well-marked meandering invaginations, a cerebriform appearance on the upper part of the right ear, accompanied by alopecia in the temporomandibular region-parietal. The lesion was asymptomatic. Histopathologic examination revealed acanthosis, hyperkeratosis, papillomatosis and follicular plugging in the epidermis. The upper dermis showed underdeveloped hair follicles with sebaceous glands, consistent with sebaceous nevus. There were no skeletal, cardiological, ophthalmological or neuropsychomotor changes; nor were there any records of relevant pathologies in the family history. The patient complained of his appearance, experiencing aesthetic and social issues. Following a diagnosis of cerebriform nevus sebaceous, complete excision was performed with excellent aesthetic results.

Development of Six Tumors in a Sebaceus Nevus of Jadassohn: Report of a Case

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprised of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual for more than four tumors to arise simultaneously within a single sebaceus nevus. Here in, we report a case of a 70-year-old woman with six neoplastic proliferations including a syringocystadenoma papilliferum, pigmented trichoblastoma, tubular apocrine adenoma, sebaceoma, tumors of follicular infundibulum and superficial epithelioma with sebaceus differentiation arising in a long standing nevus sebaceus on the scalp. Our case is extraordinary because a single nevus sebaceus contained six neoplastic proliferations with differentiation toward the folliculosebaceous-apocrine unit.

Basal cell carcinoma appearing in a facial nevus sebaceous of Jadassohn: dermoscopic features / Carcinoma basocelular aparecendo em um nevo sebáceo de Jadassohn: características dermatoscópicas

The nevus sebaceous of Jadassohn usually affects the face or scalp. It tends to evolve in three stages, and the final stage is characterized by the appearance of tumours. We present the case of a facial nevus sebaceous of Jadasshon in which a basal cell carcinoma developed. We also explore the diagnosis of this disease, which was established through dermoscopy, and propose using this technique in the clinical follow-up of this type of hamartoma, thereby allowing the early detection of cancer development.

O nevo sebáceo de Jadassohn geralmente afeta a face ou o couro cabeludo. A sua tendência natural é evoluir em três estágios, sendo que o estágio final é caracterizado pelo aparecimento de tumores. Apresentamos o caso de um nevo sebáceo de Jadassohn na face a partir do qual um carcinoma basocelular se desenvolveu. Também abordamos o diagnóstico dessa doença, estabelecido por meio da dermatoscopia. Sugerimos a utilização dessa técnica no acompanhamento clínico desse hamartoma, permitindo assim a detecção precoce de um câncer.