Proliferating Pilar Tumors in the Scalp Over a Period of 23 Years in a General Hospital in Mexico City.

BACKGROUND: Proliferating pilar tumors are an unusual skin tumor, and they have a cystic component with trichilemmal keratinization and epithelial proliferation. These arise from the outer root sheath of hair follicles. It mainly affects women. The scalp is the most affected area. Diagnosis can be made by biopsy. Surgical excision is the best treatment option. OBJECTIVE: Report the frequency of proliferating pilar tumors in the scalp in general hospital in Mexico over a period of 23 years. METHODS: The authors reviewed the database of the dermatopathology service of the General Hospital "Dr Manuel Gea González" from 1999 to August 2022, selecting diagnosticated cases of proliferating pilar tumor, pilar cyst, trichilemmal cyst, or proliferating trichilemmal cyst in the scalp. RESULTS: The authors discovered 17 cases, 13 were women, the average age was 54.9 years, all the tumors affecting the scalp, and just 3 cases were reported as malignant. CONCLUSION: In comparison with the existing data, the authors can observe that most of their patients were women and the scalp is the most affected area. Most did not present associated symptoms. As the authors can see, most are benign and long-lasting: however, the authors cannot ignore that a small percentage can be malignant.

In Vivo Reflectance Confocal Microscopy of Adnexal Tumors: Evaluation of Trichoepithelioma, Sebaceoma, and Fibrofolliculoma.

ABSTRACT: Cutaneous adnexal tumors are benign and malignant neoplasms that undergo morphological differentiation into cutaneous adnexa, comprising pilosebaceous, eccrine, or apocrine units. Reflectance confocal microscopy is a noninvasive diagnostic method that enables in vivo visualization of tissues at a similar resolution as conventional histopathology. The use of this method in skin imaging over the past several years has improved dermatological diagnoses, potentiating its wide application, especially for benign and malignant skin tumors. We describe the use of reflectance confocal microscopy in cases of trichoepithelioma, sebaceoma, and fibrofolliculoma and correlate the resulting clinical, histopathological, and confocal microscopy images.

Approaches to Perineural, Lymphovascular, and Single-Cell Disease.

BACKGROUND: Mohs micrographic surgeons should be adept in identifying and managing perineural invasion (PNI), lymphovascular invasion (LVI), and single-cell spread (SCS), features denoting high-risk behavior of basal cell carcinoma (BCC), cutaneous squamous cell carcinoma (cSCC) and microcystic adnexal carcinoma (MAC). OBJECTIVE: The purpose of this article is to review the literature and guidelines regarding the diagnosis of PNI, LVI, and SCS in BCC, cSCC, and MAC and examine the role of advanced diagnostic studies, adjuvant therapy, and reconstructive techniques of these high-risk tumors. MATERIALS AND METHODS: We performed a literature search including the following terms: PNI, LVI, SCS, BCC, cSCC, keratinocyte carcinoma, MAC, sentinel lymph node biopsy, radiation, chemotherapy, and staging. Relevant studies, case reports, and review articles were included, as well as National Comprehensive Cancer Network guidelines. RESULTS: Pancytokeratin immunohistochemistry may aid in the diagnosis of high-risk features of BCC and cSCC. Reconstruction of the Mohs defect should be carefully considered to allow for thorough inspection. Radiation therapy should be considered as an adjuvant treatment option for high-risk cSCC and BCC. Close surveillance for recurrence is warranted. CONCLUSION: The Mohs surgeon should be competent in identification of high-risk tumors and to understand how best to manage, further treat, and follow these tumors.

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.

Cutaneous metastases in a patient with no previous diagnosis of cancer: diagnostic challenge.

On rare occasions, skin lesions are the first local of metastatic manifestation of internal malignancies. In case of no previous diagnosis of these tumors, the approach of suspicious skin lesions becomes a challenge, especially in differentiating cutaneous metastases and adnexal primary neoplasms. Currently, besides epidemiologic, dermoscopic and histopathologic aspects, the evaluation also integrates immunohistochemical exams and cell markers such as p40 and p63, highly specific for skin metastases. This article describes the case of cutaneous metastases as the sole obvious sign of breast cancer in a previously asymptomatic woman. The diagnosis was made by the finding of neoplastic cells in the dermis and immunohistochemistry compatible with ductal carcinoma.

Surgery and Adjuvant Radiation for High-risk Skin Adnexal Carcinoma of the Head and Neck.

OBJECTIVES: Skin adnexal carcinoma (SAC) is a rare cutaneous malignancy that arises from sebaceous and sweat glands. These carcinomas are believed to behave more aggressively than cutaneous squamous cell carcinomas (SCC) with a propensity for local recurrence. The role of adjuvant radiotherapy in SAC is undefined. METHODS: We retrospectively reviewed all cases of head and neck SAC treated with surgery and adjuvant radiation from 2000 to 2012 at a single institution. RESULTS: Nine cases were identified. Median age was 67 (range, 52 to 88) years. The histologies were: adnexal carcinoma (n=1), adnexal carcinoma with sebaceous differentiation (n=1), adnexal carcinoma with squamous differentiation (n=1), skin appendage carcinoma (n=1), sclerosing sweat duct carcinoma (n=1), mucinous carcinoma (n=1), ductal eccrine adenocarcinoma (n=1), porocarcinoma (n=1), and trichilemmal carcinoma (n=1). All tumors were reviewed by a dermatopathologist to confirm the SAC diagnosis.All patients had undergone surgery. Indications for adjuvant radiation included involved lymph nodes (n=4), perineural invasion (n=2), nodal extracapsular extension (n=2), positive margin (n=1), high-grade histology (n=6), multifocal disease (n=2), and/or recurrent disease (n=5). Radiation was delivered to the primary site alone (n=3), to the draining lymphatics alone (n=2), or to both (n=4). One patient received concurrent cisplatin. Median dose to the primary site was 60 Gy and to the neck was 50 Gy.Median follow-up was 4.0 years (range, 0.6 to 11.4 y). Locoregional control was 100%. Five-year progression-free survival was 89%. There was 1 acute grade 3 toxicity and no greater than or equal to grade 2 late toxicities were recorded. CONCLUSIONS: Surgery and adjuvant radiation for high-risk SAC offers excellent locoregional control with acceptable toxicity.

Tricoblastoma de localización acral, una ubicación infrecuente: reporte de un caso / Acral location trichoblastoma, a location infrequent: report of a case

Tricoblastoma es una neoplasia anexial benigna de la piel, de difícil diagnóstico clínico, por su baja prevalencia y por la ausencia de características clínicas patognomónicas. Por esta razón, es la biopsia la que hace el diagnóstico definitivo, ya que se suele confundir con otras neoplasias anexiales. Se presenta caso clínico de una mujer de 87 años con historia de cinco años de evolución de lesión nodular, sésil y simétrica ubicada en la falange proximal del primer ortejo del pie izquierdo, la cual había presentado un crecimiento progresivo en los últimos seis meses, refiriendo molestias leves con el calzado, sin mayores complicaciones. La biopsia excisional de la lesión, es informada como tricoblastoma. El escaso conocimiento del tricoblastoma lleva a la incorrecta interpretación de su forma clínica, lo que resulta relevante, pues su principal diagnóstico diferencial corresponde al carcinoma basocelular (CBC). Se presenta el caso clínico por lo infrecuente de su localización, lo que indujo a confusión del diagnóstico. No se debe olvidar que el tricoblastoma es una neoplasia anexial benigna, que puede presentarse en cualquier sitio en el que existan folículos pilosebáceos, por lo que hay que considerarlo dentro de los diagnósticos diferenciales de cualquier neoplasia anexial.

Trichoblastoma is a skin benign adnexal neoplasm, clinical diagnosis is difficult because presents a low prevalence, it has not any pathognomonic clinical characteristics and it's often mistaken with other adnexal tumors; so the biopsy makes the definitive diagnosis. We report the case of a woman of 87 years with a history of 5 years of evolution of a nodular, sessile and symmetrical lesion located in the proximal phalanx of the first left toe, which had presented a progressive growth in the last 6 months, she presented mild discomfort with footwear, without major complications. The excisional biopsy of the lesion, was reported as a trichoblastoma. Poor knowledge of trichoblastoma leads to a clinical misinterpretation, which is relevant, because its main differential diagnosis corresponds to basal cell carcinoma (BCC). A case located on an extremely rare location is presented, which led to confusion in clinical diagnosis. You must not forget that trichoblastoma is an adnexal benign tumor, which can occur at any site with pilosebaceous follicles, so we should consider in the differential diagnoses of any skin adnexal neoplasms.

Cutaneous metastases in a patient with no previous diagnosis of cancer: diagnostic challenge

Abstract: On rare occasions, skin lesions are the first local of metastatic manifestation of internal malignancies. In case of no previous diagnosis of these tumors, the approach of suspicious skin lesions becomes a challenge, especially in differentiating cutaneous metastases and adnexal primary neoplasms. Currently, besides epidemiologic, dermoscopic and histopathologic aspects, the evaluation also integrates immunohistochemical exams and cell markers such as p40 and p63, highly specific for skin metastases. This article describes the case of cutaneous metastases as the sole obvious sign of breast cancer in a previously asymptomatic woman. The diagnosis was made by the finding of neoplastic cells in the dermis and immunohistochemistry compatible with ductal carcinoma.

Carcinoma triquilemal, um tumor incomum: atualização sobre manejo e prognóstico / Trichilemmal carcinoma, an uncommon tumor: update on its management and prognosis

INTRODUÇÃO: O carcinoma triquilemal (CT) é uma neoplasia rara dos anexos cutâneos. Foi descrito pela primeira vez em 1968, como tricoleptocarcinoma, e tem incidência de 0,05% em pacientes submetidos a exame histopatológico após excisão de lesões cutâneas. Parece ser um tumor de baixa agressividade, porém, relatos na literatura colocam em dúvida tal comportamento indolente. OBJETIVOS: Oferecer uma atualização sobre manejo e prognóstico do CT. MÉTODOS: Pesquisa no PubMed e SciELO com os termos MeSH "trichilemmal carcinoma", "tricholemmal carcinoma", "adnexal skin tumor", "carcinoma triquilemal". RESULTADOS: Devido a sua raridade, a maior parte da literatura se baseia em relatos de caso, os quais na sua maioria corroboram a natureza indolente da doença. Excisão cirúrgica é o tratamento preconizado, com baixo risco de recidiva e baixa morbimortalidade. CONCLUSÃO: O comportamento da lesão e as condutas no tratamento do CT baseiam-se em casos isolados ou de pequenas séries de casos. Devido a sua baixa prevalência, uma colaboração multicêntrica agrupando um maior número de casos pode ajudar a definir melhor recomendações de tratamento, fisiopatologia e prognóstico. Excisão cirúrgica continua a ser o padrão-ouro de tratamento, com baixo risco de recidiva.

INTRODUCTION: Trichilemmal carcinoma (TC) is a rare neoplasm of skin appendages. It was first described in 1968 as tricoleptocarcinoma, and has an incidence of 0.05% in patients subjected to histopathological examination after excision of cutaneous lesions. TC has an indolent clinical course ; however, reports in the literature put in doubt this indolent behavior. OBJECTIVES: To provide an update on the management and prognosis of TC. METHODS:A search of the PubMed and SciELO databases by using with the MeSH terms "trichilemmal carcinoma", "tricholemmal carcinoma", "adnexal skin tumor", and "carcinoma triquilemal" was performed. RESULTS: Owing to the rarity of TC, most studies were case reports, which essentially corroborate the indolent nature of the disease. Surgical excision is the recommended treatment, and is associated with a low risk of recurrence and low morbidity and mortality. CONCLUSION: The behavior of the lesion and the procedure of treatment of TC are based on isolated cases or in a small series of cases. Because of its low prevalence, a multicenter collaboration of a greater number of cases can help define the best treatment recommendations, pathophysiology, and prognosis. Surgical excision remains the gold standard of treatment, and is associated with a low risk of recurrence.

Tumor of follicular infundibulum with unique features

Tumor of the follicular infundibulum is a rare benign cutaneous adnexal neoplasm with variable clinical presentation. In most cases the diagnosis is made with the help of histopathology, due to lack of a characteristic clinical presentation. The most common form is a solitary lesion, but it can be multiple or eruptive, then called infundibulomatose. This case report illustrates a patient with multiple lesions of tumor of the follicular infundibulum with bizarre aspect and atypical location.

Tumor of follicular infundibulum with unique features.

Tumor of the follicular infundibulum is a rare benign cutaneous adnexal neoplasm with variable clinical presentation. In most cases the diagnosis is made with the help of histopathology, due to lack of a characteristic clinical presentation. The most common form is a solitary lesion, but it can be multiple or eruptive, then called infundibulomatose. This case report illustrates a patient with multiple lesions of tumor of the follicular infundibulum with bizarre aspect and atypical location.

Reticulated acanthoma with apocrine differentiation.

Reticulated acanthoma with apocrine differentiation (RAAD) represents a rare variant of adnexal neoplasm first described by Ackerman et al. in 1998. It consists of cords and columns of adnexal keratinocytes that form a reticulated pattern. Variable signs of apocrine, sebaceous and follicular differentiation may be present. Since 1998, no further cases of this condition have been published. We report on a lesion excised from the leg of a 46-year-old man, which displayed histopathological features diagnostic of RAAD. Criteria for diagnosis and differential diagnoses are discussed.

Tricoepitelioma desmoplásico múltiple de mejillas: a propósito de un caso / Multiple desmoplastic trichoepithelioma: a case report

El tricoepitelioma es una neoformación benigna diferenciada hacia el folículo piloso. Clínicamente puede presentarse en forma múltiple, solitaria o desmoplásica. Histológicamente plantea algunas dificultades para diferenciarlos del carcinoma basocelular (CSC). Se comunica el caso de una mujer de 28 años de edad y se revisa brevemente la literatura.

Trichoepithelioma is a benign neoformation differentiated from the hair follicle, clinical manifestations can be solitary, multiple or desmoplastic lesions. Histologically, it is not easy to differentiate from basal cell carcinoma. We report the case of a 28-year-old woman, and short review.

Brooke-Spiegler syndrome - report of four families: treatment with CO2 laser.

BACKGROUND: Brooke-Spiegler syndrome is a well known but rare cutaneous disorder characterized by the presence of multiple trichoepitheliomas and cylindromas. METHODS: Four families with Brooke-Spiegler syndrome are described. RESULTS: Spiradenomas developed on the back in two patients. A CO(2) laser was used to treat facial trichoepitheliomas in two patients with good results. CONCLUSIONS: The large number of facial lesions in this syndrome often produce a significant emotional impact. We believe that it is important to provide patients with a good therapeutic option. CO(2) laser therapy may fill this role.

Dermatofibromas eruptivos múltiplos einfecção pelo HCV tratada com interferon / Multiple eruptive dermatofibromas and HCV infection treated by interferon

O dermatofibroma é um tumor fibrohistiocístico benigno comum. Os dermatofibromas eruptivos múltiplos são definidos arbitrariamente pela presença de 15 ou mais dermatofibromas ou pelo desenvolvimento de 5 a 8 destes em um período menor do que 4 meses. A patogênese dos dermatofibromas eruptivos múltiplos ainda permanece incerta, mas há estudos que relacionam seu aparecimento a determinadas doenças auto-imunes, doenças infecciosas e uso de imunomoduladores. É importante pesquisar e reconhecer o quadro descrito como dermatofibromas eruptivos múltiplos, pois ele pode ser indicativo de importante condição subjacente. Nosso relato descreve o caso de um homem portador do vírus da hepatite C que fez uso de alfa-interferon e que, posteriormente, desenvolveu múltiplos dermatofibromas. No contexto da infecção pelo HCV e do uso de interferon, o quadro de DEM poderia estar relacionado à infecção propriamente dita e/ou a um efeito idiossincrásico da droga

Dermatofibroma is a common benign fibrohistiocystic tumor. Multiple eruptive dermatofibromas are defined arbitrarily as more than 15 dermatofibromas or 5 to 8 dermatofibromas developing over a span of less than 4 months. A etiology is unknown, but there are studies that relate their appearance to determined self-immune illnesses, infectious illnesses and use of immunomodulators. It is important to search and to recognize the described picture as multiple eruptive dermatofibromas, therefore it can be indicative of important underlying condition. Our story describes the case of a carrying man of HCV that made alpha-interferon use and that, later developed multiples dermatofibromas. In the context of the infection for the HCV and the use of interferon, the multiple eruptive dermatofibromas picture could be related to the infection properly said and/or an idiosyncratic effect of the drug

Melanoma maligno nodular acral gigante / Giant nodular malignant melanoma

El Melanoma Maligno Acral es una entidad rara, asociado a un patrón nodular es menos frecuente. El melanoma es un tumor maligno de los melanocitos. La mayoría se localizan en piel y menos frecuentemente en mucosas, retina y meninges. El melanoma cutáneo constituye del 2-3 por ciento del total de neoplasias. El diagnóstico se basa en la sospecha clínica de una lesión cutánea con coloración heterogénea, asimetría, bordes irregulares y diámetro >5mm. Se presenta el caso clínico de paciente femenino de 47 años de edad, quien consultó por presentar tumoración exofítica gigante, de crecimiento rápido y progresivo, en talón derecho. Se realizó exéresis para estudio histopatológico, el cual reportó melanoma maligno nodular ulcerado, con bordes de resección quirúrgica positivos para invasión tumoral.

Epidermal nevus syndrome associated with adnexal tumors, spitz nevus, and hypophosphatemic vitamin D-resistant rickets.

The epidermal nevus syndrome is the association of epidermal nevi with abnormalities in other organ systems, most commonly the central nervous system, the skeletal system, and the eyes. We present a patient with epidermal nevus syndrome associated with hypophosphatemic vitamin D-resistant rickets and multiple adnexal and spindle cell tumors.

Incidencia de tumores cutáneos anexiales en el Hospital Barros Luco-Trudeau / Incidence of cutaneous adnexal tumor in Barros Luco-Trudeau Hospital

Los tumores anexiales, derivados de las glándulas sebáceas, sudoríparas o de los folículos pilosos, son poco frecuentes y de difícil diagnóstico en Dermatología, correspondiendo la mayoría de ellos a hallazgos histopatológicos. Con objeto de determinar la incidencia de este tipo de tumores en pacientes de edad adulta, se realizó una revisión total de protocolos de biopsias del Servicio de Anatomía Patológica del Hospital Barros Luco-Trudeau. De 3.589 enviadas por el Servicio de Dermatología, 124 de éstas correspondieron a tumores anexiales, lo que da una incidencia de alrededor del 3,5 por ciento. La mayoría de los casos con diagnósticos de tumor anexial correspondieron a pacientes del sexo femenino (65 por ciento). Respecto a la edad de presentación, la mayor parte de los pacientes en quienes se diagnosticó este tipo de tumor cutáneo sobrepasaba los 40 años de edad (68,5 por ciento). Los tumores anexiales más frecuentes son los de origen piloso, seguidos por los originados por las glándulas sudoríparas. De acuerdo a nuestros resultados, es posible plantear que existe una correlación entre edad de presentación y tipo de tumor y entre sexo y tipo de tumor. Un porcentaje importante de estos tumores correspondió a un hallazgo histopatológico (55 por ciento), lo que confirma la dificultad en el diagnóstico clínico, y a su vez explicaría el bajo número de antecedentes bibliográficos existentes a nivel de literatura mundial.

Síndrome de Birt-Hogg-Dubé: presentación de un caso y revisión de la literatura / Birt-Hogg-Dubé syndrome: case report and literature review

Se presenta una paciente de 59 años de edad con síndrome de Birt-Hogg-Dubé. Se describen las características clínicas e histopatológicas, así como las asociaciones que pueden observarse en esta infrecuente enfermedad (AU)