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.

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.

Pilomatricoma Unmasked on Fine Needle Aspiration Cytology.

Pilomatrixoma is an uncommon adnexal tumor with heterogeneous features can mimic various malignancies and contributes to false positive diagnosis on cytology. It is an uncommon, slow growing, benign skin adnexal neoplasm of hair matrix origin initially described by Malherbe and Chenantais. This tumor is predominantly found in the head and neck region, and presents as a small, asymptomatic, slow-growing dermal, subcutaneous, solitary lesion. More than 50% of these tumors are known to occur in the second decade of life and is often misdiagnosed clinically. Cytological features pose a diagnostic challenge although the histological features are very well delineated. This paper presents the cytological features in two cases outlining the importance of the clinicopathological features that need to be considered for an accurate diagnosis. Awareness of its varied cytologic features and clinical presentation can avoid misdiagnosis.

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.

Skin Tumours – Histopathological Review of 125 Cases.

The skin is the largest organ in the body. A wide variety of hyperplastic growths and tumours, both benign and malignant are encountered in the clinical practice. Any lesion, for which the diagnosis is uncertain, based on the history and clinical examination should be biopsied for histopathological examination to rule out malignancy. Objective: To analyze retrospectively tumours of skin with respect to age, sex, clinical features and histopathological features in a tertiary referral centre in Maharashtra, India. Material & Methods: The present study consisted of analysis of tumours of skin received in the histopathology section of department of pathology over a period of 5 years that is from August 2005 to July 2010. The material comprised of biopsies and excision specimens. The clinical and histopathological details were noted. The findings were compared with those reported by other authors. Results: One twenty five (125) tumours of skin were observed. The benign tumours were slightly more common (51.2%) than malignant tumours (48.8%). The maximum number of tumours was found in 7th decade (25.6%). Maximum number of tumours were found in third decade in benign tumours (20.3%) and seventh decade in malignant tumours (37.7%). Both benign and malignant tumours of skin were common in males than females. The equal numbers of skin tumours were seen in both the head and neck region (44.8%) and the extremities (44.8%). Face was the commonest site for skin tumours (35.2%). The keratinocytic tumours, both benign and malignant were common tumours of skin (62.4%) while neural tumours were rarely observed (1.6%). The Squamous Cell Carcinoma (SCC) was the commonest malignant tumour (45.9%) followed by Basal Cell Carcinoma (BCC) (34.4%). Verrucas (32.8%) were the commonest benign tumours followed by pyogenic granuloma (21.9%). Conclusion: SCC is the most common malignant skin tumour in India, unlike the Western countries. Histopathological study is a very important step in the diagnosis of skin tumours.

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.

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.

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

Quiste triquilemal proliferante: presentación de un caso gigante y otro maligno / Proliferating trichilemmal cyst: presentation of a giant and a malign case

Se presenta el caso de un paciente de sexo masculino con un quiste triquilemal proliferante maligno y otro de una mujer con un quiste triquilemal proliferante gigante. Se discute la histogénesis de estos tumores, así como las características clínicas, histopatológicas y estudios complementarios que permiten detectar el comportamiento maligno de dichas lesiones para efectuar la terapéutica adecuada. Se resalta la utilidad del marcador de proliferación nuclear Ki 67 como indicador de estos tumores