Reflectance confocal microscopy - Consensus terminology glossary in Brazilian Portuguese for normal skin, melanocytic and non-melanocytic lesions

Abstract Background: Currently, there is no uniform and official terminology in Portuguese for reflectance confocal microscopy analysis, despite the increasing number of Brazilian dermatologists using this new tool. Objective: To present the terminology in Brazilian Portuguese for the description of reflectance confocal microscopy and establish the first Brazilian consensus on terms related to normal skin and cutaneous tumors. Methods: 10 Brazilian specialists from different institutions and states of Brazil were selected to evaluate the best corresponding terms in Portuguese for normal skin, melanocytic and non-melanocytic tumors. The terms used were translated from international consensuses in the English language. The modified Delphi method was used to create the consensus in 3 steps. Results: The terms considered the most appropriate in the Portuguese language to describe the findings of normal skin, melanocytic and non-melanocytic lesions in the reflectance confocal microscopy analysis were presented. Study limitations: The limitations of the present study include the number of participants and limited regional representation (only two of the five Brazilian regions were represented). Conclusion: This Brazilian consensus represents an opportunity for dermatologists and physicians specializing in cutaneous oncology to become familiar with reflectance confocal microscopy, propagating the technique in clinical and research environments to stimulate national and international publications on this subject.

In vivo reflectance confocal microscopic findings in a case of trichofolliculoma

Abstract Trichofolliculoma is a rare follicular hamartoma whose dermoscopic features have been scarcely reported. On the other hand, reflectance confocal microscopy features have not been described yet. In the present study, the authors report reflectance confocal microscopy features in a case of trichofolliculoma as squamous hyperplasia forming irregular finger-like protrusions around the hair follicle and papillomatous hyperplasia of the hair follicle epithelium, which correlated with histopathology. This case suggests that reflectance confocal microscopy may help incorrect in vivo diagnosis of trichofolliculoma in cases difficult to diagnose by morphology and dermoscopy.

Cistoadenoma volumoso associado a teratoma ovariano maduro, com evolução para pseudomixoma peritoneal: relato de caso / Giant cystadenoma associated with mature ovarian teratoma with progression to pseudomyxoma peritonei: a case report

Massas anexiais representam grande desafio diagnóstico, devido a seu caráter geralmente assintomático¹. Traz-se caso peculiar de paciente que evoluiu com sintomas exuberantes de rápida evolução, situação característica de cistoadenomas, tumores benignos de origem epitelial²,³. Realizada laparotomia, com achados macroscópicos que revelaram conteúdo mucinoso e presença de pelos e neovascularização em seu interior. O anatomopatológico confirmou o diagnóstico de cistoadenoma associado a teratoma ovariano, sendo assim considerado um tumor de colisão ovariana.

Adnexal masses represent a major diagnostic challenge due to its usually asymptomatic characteritcs¹. We present a peculiar case of a patient who evolved with exuberant symptoms of rapid evolution, characteristic of cystadenomas, benign tumors of epithelial origin²,³. Laparotomy was performed, with macroscopic findings that revealed mucinous content and the presence of hairs and neovascularization inside. Pathology confirmed the diagnosis of cystadenoma associated with ovarian teratoma, thus being considered an ovarian collision tumor.

Pilomatrixomas en niños: a propósito de 149 casos. Estudio retrospectivo en dos centros pediátricos / Pilomatrixomas in children: Report of 149 cases. A retrospective study at two children's hospitals

El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.

Espiradenoma écrino gigante associado à síndrome de Brooke-Spiegler / Giant eccrine spiradenoma associated with Brooke-Spiegler syndrome

A síndrome de Brooke-Spiegler é doença genética autossômica dominante rara, com predisposição a diversos tumores anexiais, dentre eles tricoepitelioma, cilindroma e espiradenoma. Os tumores surgem na segunda década de vida, aumentam progressivamente com a idade e sua prevalência é maior em mulheres. É causada por mutação no gene CYLD, localizado no cromossomo 16q12-q13. Relatamos caso exuberante de espiradenoma écrino gigante associado a essa síndrome.

Brooke-Spiegler syndrome is a rare autosomal dominant genetic disease with predisposition to many adnexal tumors, including trichoepithelioma, cylindroma and spiroadenoma. Tumors appear in the second decade of life, progressively increase with age, and their prevalence is higher in women. It is caused by a mutation in the CYLD gene, localized in the chromosome 16q12-q13. We report a exuberant case of giant eccrine spiradenoma associated to this syndrome.

Locally aggressive trichilemmal carcinoma

Abstract: Trichilemmal carcinoma is a rare adnexal neoplasia with histologic features reminiscent of the outer root sheath of hair follicles. The clinical presentation of trichilemmal carcinoma is variable, as it may mimic basal cell carcinomas and squamous cell carcinomas, thus requiring histologic diagnosis. We report the case of a patient with aggressive trichilemmal carcinoma in the auricle.

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.

Case for diagnosis. A bluish nodule on the scalp

Abstract: Acrospiroma, also known as hidradenoma, is a rare cutaneous tumor that has several histological characteristics. As a consequence, a high index of suspicion is necessary for its diagnosis. Here we report a case that illustrates the importance of a good clinical-pathologic correlation in order to recognize this disease.

Syringocystadenoma papilliferum in an unusual location

Abstract: Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor of the apocrine or eccrine sweat glands. Most patients present a solitary lesion in the head and neck region. Presentation outside the head and neck region is even more uncommon. We present a case of Syringocystadenoma papilliferum with papulonodular lesion located on the vulva of an infant girl. This case illustrates the atypical location of this rare disease and adds to the differential diagnosis of lesions on the vulva.

Benign follicular tumors

Abstract: Benign follicular tumors comprise a large and heterogeneous group of neoplasms that share a common histogenesis and display morphological features resembling one or several portions of the normal hair follicle, or recapitulate part of its embryological development. Most cases present it as clinically nondescript single lesions and essentially of dermatological relevance. Occasionally, however, these lesions be multiple and represent a cutaneous marker of complex syndromes associated with an increased risk of visceral neoplasms. In this article, the authors present the microscopic structure of the normal hair follicle as a basis to understand the type and level of differentiation of the various follicular tumors. The main clinicopathological features and differential diagnosis of benign follicular tumors are then discussed, including dilated pore of Winer, pilar sheath acanthoma, trichoadenoma, trichilemmoma, infundibuloma, proliferating trichilemmal cyst/tumor, trichoblastoma and its variants, pilomatricoma, trichodiscoma/fibrofolliculoma, neurofollicular hamartoma and trichofolliculoma. In addition, the main syndromes presenting with multiple follicular tumors are also discussed, namely Cowden, Birt-Hogg-Dubé, Rombo and Bazex-Dupré-Christol syndromes, as well as multiple tumors of follicular infundibulum (infundibulomatosis) and multiple trichoepitheliomas. Although the diagnosis of follicular tumors relies on histological examination, we highlight the importance of their knowledge for the clinician, especially when in presence of patients with multiple lesions that may be the cutaneous marker of a cancer-prone syndrome. The dermatologist is therefore in a privileged position to recognize these lesions, which is extremely important to provide further propedeutic, appropriate referral and genetic counseling for these patients.

Cellular neurothekeoma: case report and its (un) relation with nerve sheath myxoma

Neurothekeoma is an uncommon, benign neoplasm presenting in young adults, primarily on the head and neck. It was initially related to nerve sheath myxoma but with the advent of immunohistochemistry, new insights into its cellular differentiation and origin have emerged, unlinking Neurothekeoma and nerve sheath myxoma. Herein we describe a 19-year-old male who had had a frontal, fl esh-colored, asymptomatic papule for 2 years. Histology showed a dermal fusocellular-spindle cell tumor, including an eosinophilic cytoplasm with mild cellular pleomorphism and moderately dense fi brous stroma. IHQ was positive for CD10 and negative for S100 and Claudin-1. These fi ndings were compatible with cellular Neurothekeoma. The lesion was completely extirpated and at the 6-month follow-up, the patient was asymptomatic and had experienced no recurrences.

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.

Syndrome In Question

Brooke-Spiegler syndrome is an autosomal dominant disorder with variable penetrance and expression. It is characterized by a genetic predisposition to develop multiple adnexal neoplasias: cylindromas, trichoepitheliomas, and trichoblastomas. We describe a 54-year-old male patient with cylindromas, trichoepitheliomas, and trichoblastoma.

Siringocistoadenoma papilífero localizado na coxa / Syringocystadenoma papilliferum located in the thigh

Siringocistadenoma papilífero é tumor benigno raro, mais comum em couro cabeludo e face. Pode apresentar-se como lesão independente ou associada ao nevo sebáceo de Jadassohn. Foram relatados na literatura raros casos desse tumor com localização na coxa. Descrevemos o caso de uma paciente com siringocistadenoma papilífero distinguido por seu componente apócrino intradérmico tubular papilífero.

Syringocystadenoma papilliferum is a rare, benign tumor, most common in the scalp and face. It can present as an independent lesion or be associated with nevus sebaceous of Jadassohn. Rare cases of this tumor located in the thigh have been reported in the literature. The present article reports the case of a patient with syringocystadenoma papilliferum, distinguished by its intradermal tubular apocrine papillary component.

Nodular fibrofolliculoma:a new entity of skin adnexal tumor / 中华皮肤科杂志

A 22-year-old patient was admitted to the hospital with a solitary,gradually growing and painless mass in the left shoulder for 2 years.Physical examination revealed no abnormality except for the skin lesion.Skin examination showed an elevated lesion measuring about 3.5 cm× 2.0 cm × 1.5 cm with smooth surface and normal color,which was located in the subcutaeous tissue,indurated and movable.Resection of the tumor was performed under local anesthesia.On visual observation during operation,the tumor was sited in the subcutaneous fat tissue,nodular-like and surrounded locally by fibrous pseudocapsules with a grey incisal surface and mild texture.Microscopicalty,the tumor was extremely similar to breast fibroadenoma with multiple lobuli,and each of the lobuli was composed of tubiform structures,basal cell-like epithelial cell trabs and fibromyxoid stroma abundant in fibroblast-like spindle cells.No hair bulb or primitive dermal papillae were observed in the lobuli,which were separated by compact collagen fibers infiltrated by a few scattered inflammatory cells.Fibromyxoid strotma was surrounded by basal cell-like epithelial cell strabs in most lobuli,and some tubiform structures were filled with a little thin lightly eosinophilic material in a concentric arrangement.Immunohistochemistry showed that intralobular epithelial cells were strongly positive for cytokeratin 5/6,but negative for CAM5.2 or carcinoembryonic antigen (CEA).In addition,the lightly eosinophilic material in lumens was negative for periodic acid-Schiff (PAS) staining.These results suggested that the tubiform structures were immature follicles,but not sweat ducts.The patient was diagnosed with nodular fibrofolliculoma (NFF) based on the clinical manifestations,morphological features,immunohistochemical and PAS staining results.No relapse was observed in more than 3 months of postoperative follow-up.As a benign trichogenic adnexal neoplasm with unique clinicopathological manifestations,NFF may be a new entity of cutaneous adnexal neoplasm.

Aspectos dermatoscópicos do siringocistoadenoma papilífero associado a nevo sebáceo / Dermoscopic aspects of syringocystadenoma papilliferum associated with nevus sebaceus

O siringocistoadenoma papilífero é uma neoplasia anexial benigna rara, com frequente diferenciação apócrina. Localiza-se preferencialmente no couro cabeludo e está associado ao nevo sebáceo em 40 por cento dos casos. Apesar da variabilidade clínica, a histologia é característica. Há relatos da dermatoscopia de tumores anexiais, como poroma écrino, hidradenoma e angio-histiocitoma; porém, até o momento, não há descrição da dermatoscopia do siringocistoadenoma. Apresentamos aspectos dermatoscópicos de um caso de siringocistoadenoma associado a nevo sebáceo, visualizando-se padrão vascular polimorfo e vasos em ferradura.

Syringocystadenoma papilliferum is a rare benign adnexal tumor that frequently shows apocrine differentiation. It usually develops on the scalp and is associated with a nevus sebaceus in 40 percent of cases. Although the clinical presentation may differ, its histology is characteristic. Reports have been made of dermoscopy used in cases of adnexal tumors such as eccrine poromas, hidradenomas and angiohistiocytomas; however, up to the present moment there have been no reports of dermoscopy in a case of syringocystadenoma. This paper describes the dermoscopic features found in a case of syringocystadenoma associated with a nevus sebaceus, revealing a polymorphous vascular pattern including a horseshoe-shaped arrangement of vessels.

Carcinoma triquilemal: relato de caso / Trichilemmal carcinoma: case report

O carcinoma triquilemal é um tumor raro, que ocorre, geralmente, na pele exposta ao sol, principalmente face, couro cabeludo, pescoço e dorso das mãos, em indivíduos idosos, entre a 4ª e 9ª décadas de vida, sem predilação por sexo. O presente estudo mostra um caso de carcinoma triquilemal, recidivado, de difícil tratamento, em mesma topografia de um carcinoma basocelular tratado previamente com cirurgia e radioterapia.

The trichilemmal carcinoma is a rare tumor that usually occurs on sun-exposed skin, especially on the face, scalp, neck and back of hands, mainly in elderly subjects but commonly between the 4th and 9th decades of life. It is not a gender-based illness. This study shows a difficult to treat case of recurrent trichilemmal carcinoma on the same location of a basal-cell carcinoma previously treated with surgery and radiotherapy.

Skin adnexal tumor with multipote ntial differentiation complicated by nevus spilus: a case report / 中华皮肤科杂志

A 41-year-old female patient developed round,bright yellow patches on the left calvarial region without obvious precipitating factors 40 years prior to the presentation,which gradually grew to form plaques with age.Two years prior to the presentation,nipple-like lesions appeared in the calvarial and temporal region with an erythematous and wet surface; concurrently,black masses developed in the left temporal region and gradually enlarged with central ulceration but no subjective symptoms.At about 1 year of age,pitchy macules developed on the light tan patches located on the left jaw,posterior and anterior neck,trunk and upper limbs,and gradually increased in quantity and size with the involvement of the homolateral dorsal hand and gradual appearance of papules.Skin examination revealed two well-marginated,indurated,bright red neoplasms sized 3 cm × 2 cm and 2 cm × 1 cm respectively,with erosive and cauliflower-like surface; black or pink papules were scattered between these neoplasms.There was a ring-shaped black mass sized 1.5 cm × 1.5 cm in the left temporal region with central ulceration.Pitchy tough macules and papules were observed on the light tan patches located in the left cheek,lower mandible,posterior and anterior neck,protothorax,shoulder and back,upper limbs and dorsal hand.Based on the histopathology of multiple lesions,the cauliflower-like lesions on the head were diagnosed as syringocystadenoma papilliferum,the yellow plaques as syringocystadenoma papilliferum complicated by sebaceous adenoma,the black proliferative lesions in the temporal region as trichoblastoma accompanied by basal cell epithelioma,the black papuloid lesions and brown maculopapuloid lesions on the lower mandible as nevus spilus.The patient was diagnosed with skin adnexal tumor with multipotential differentiation (syringocystadenoma papilliferum,sebaceous adenoma,trichoblastoma and basal cell epithelioma)accompanied by nevus spilus.

Adnexal Clear Cell Carcinoma with Comedonecrosis: A Case Report

Adnexal clear cell carcinoma with comedonecrosis (ACCCC) is a very rare malignancy of the skin with an aggressive clinical course and a predilection for the scalp. This is the first reported case of ACCCC in Korea. A 79-year-old male presented with left abdominal masses that proved to be two subcutaneous nodules. The tumors histologically consisted of epithelial nests that showed a distinctive zonal arrangement. The centrally located clear cell areas with comedonecroses were merged with the peripheral squamoid cells, often exhibiting retraction artifacts and an infiltrating border. Nuclear pleomorphism and frequent mitoses were prominent. The clear cells were immunopositive for carcinoembryonic antigen and epithelial membrane antigen. We report here on a case of ACCCC involving the abdominal skin, and this tumor should be distinguished from the more indolent squamous cell and tricholemmal carcinomas.

Steatocystoma Simplex of the Anterior Neck / 대한이비인후과학회지

As a benign adnexal tumor, steatocystoma usually occurs as an autosomal dominant condition known as steatocystoma multiplex. Steatocystoma simplex, the noninherited solitary counterpart of steatocystoma multiplex,was first described by Brownstein in 1982, and is exceedingly rare. We report a case of steatocystoma simplex in a 27-years-old female, who presented with an asymptomatic soft, oval shaped mass on the lower anterior neck. The lesion was completely excised and confirmed as steatocystoma simplex on histopathological examination.