Nevus apocrino puro: una entidad raramente sospechada / Pure apocrine nevus: a rarely suspected entity

Los nevus apocrinos puros son hamartomas de las unidades pilosebáceas caracterizadas por proliferaciones benignas de glándulas apocrinas maduras, la cual es una descripción microscópica realizada en los reportes de patología sin que se nombre el diagnóstico exacto. Considerando además, los diagnósticos diferenciales clínicos y la baja frecuencia de este diagnóstico, presentamos un caso clínico y una revisión del tema

Pure apocrine nevi are hamartomas of the pilosebaceous units characterized by benign proliferations of mature apocrine glands, which is a microscopic description made in pathology reports without the exact diagnosis being named. Considering the clinical differential diagnoses and its low frequency, we present a case report and a review of the literature on this topic

Cistomatose de glândula apócrina em onça-pintada (Panthera onca) / Apocrine cystomatosis in a jaguar (Panthera onca)

A cistomatose de glândula apócrina é uma condição rara e não-neoplásica, que tem sido relatada em cães e gatos, e caracterizada por vários grupos de glândulas sudoríparas dilatadas, cuja distribuição generalizada raramente é observada. Uma fêmea de onça-pintada melânica (Panthera onca), com idade estimada de 16 anos e mantida em cativeiro, apresentou aumento de volume abdominal. Ao exame físico foram observados vários nódulos de consistência macia e flutuante, não aderidos, com tamanho de 2 a 15cm de diâmetro, na região ventral abdominal. Outras massas de menor diâmetro (0,5-1,0cm) estavam presentes na região cervical e no dorso do animal. Estas massas foram removidas em um procedimento cirúrgico. Dentro deste cisto havia conteúdo líquido, inodoro e translúcido. Microscopicamente, observou-se entre a derme média e profunda a formação de múltiplos cistos de tamanho variável. Esses cistos eram revestidos por uma única camada de células predominantemente cubóides. Em alguns focos eram observadas raras projeções papiliformes de epitélio glandular. O citoplasma estava levemente eosinofílico e com raras imagens de decapitação do ápice. O diagnóstico de cistomatose de glândulas apócrinas foi realizado através dos achados macroscópicos e microscópicos.(AU)

Cystomatosis of the apocrine gland is a rare condition reported in dogs and cats. It is a non-neoplastic condition, characterized by several groups of dilated cystic sweat glands. Rarely a more widespread distribution can be observed. A captive female about 16 year-old melanic jaguar (Panthera onca) presented increased abdominal volume. Physical examination showed multiple floating nodular masses ranging from to 2 to 15cm in diameter located in the ventral abdomen. Other masses of smaller diameter (0.5-1.0cm) were present in the cervical region and in the back of the jaguar. These masses were surgically removed. Within this cyst had a odorless translucent content. Microscopically, between the mid and deep dermis there existed multiple cysts of different sizes, coated with a single layer of cuboidal cells. In some foci, there were rare papilliform projections of glandular epithelium. The cytoplasm was slightly eosinophilic with rare images of decapitation of the apex. The diagnostic of apocrine cystomatosis was performed through macroscopic and microscopic findings.(AU)

Orbital apocrine hidrocystoma with immunohistochemical confirmation / Hidrocistoma apócrino orbitário com confirmação imunohistoquímica

ABSTRACT The authors report the first case in Brazilian literature of orbital apocrine hidrocystoma with immunohistochemistry confirmation. The tumor had been growing slowly and progressively, there was no history of impaired vision, diplopia, watering, discharge, or prior trauma. There was no proptosis and extraocular mobility was normal. The radiologic study by orbital computerized tomography revealed an extraconal nodule, with partially defined limits, with cystic and solid areas in the superomedial right orbit. An anterior orbitotomy, with full excision of the tumor was performed. A histopathology examination revealed apocrine hidrocystoma and immunohistochemistry confirmed the diagnosis. After surgery, the patient had total remission of symptoms.

RESUMO Os autores relatam o primeiro caso de hidrocistoma apócrino orbitário com confirmação imunohistoquímica. O tumor apresentou crescimento lento e progressivo, sem relato de baixa de acuidade visual, diplopia, epífora, secreção ou trauma prévio. Não houve proptose ou alteração da motilidade extraocular. O exame de imagem por tomografia computadorizada da órbita revelou uma lesão nodular, extraconal, de limites imprecisos, com áreas císticas e sólidas no seu interior, na região súpero-medial de órbita direita. O paciente foi submetido à orbitotomia por via anterior, com exérese integral da tumoração. O exame histopatológico revelou o diagnóstico de hidrocistoma apócrino e a imunohistoquímica confirmou o diagnóstico. Após a cirurgia, o paciente teve regressão total dos sintomas.

Tumor mixto cutáneo apocrino, un desafío diagnóstico a considerar / A mixed cutaneous apocrine tumors, a diagnostic challenge to to consider

El tumor mixto cutáneo es una neoplasia se aparición inusual al que se ha denominado también siringoma condroide. Se reporta un caso de Tumor mixto benigno apocrino en una paciente de 63 años. Paciente mujer de 63 años, con lesión tumoral de un año de evolución en zona frontal, asintomática. La ecografía de partes blandas fue compatible con lesión quística y el estudio histológico mostró la presencia de una neoplasia mixta benigna epitelial estromal, correspondiente a un tumor mixto benigno apocrino. Los tumores mixtos benignos apocrinos son neoplasias poco frecuentes, localizadas en las glándulas sudoríparas. Se caracterizan por presentarse como un nódulo subcutáneo o intradérmico, solitario, que puede alcanzar hasta 3 cm. de diámetro. Son asintomáticos y de crecimiento lento. Su principal ubicación corresponde a la cabeza y cuello y predominan en pacientes de sexo masculino, de edad media. El diagnóstico de certeza es histopatológico, encontrándose a la microscopía un patrón epitelial compuesto por estructuras tubulares, ductales, túbulo-alveolares o áreas sólidas con diferenciación pilo-sebácea focal, en un estroma que puede ser mixoide o condroide Se presenta este caso por ser un tumor de baja frecuencia, cuyo diagnóstico es esencialmente histopatológico y cuya terapia resolutiva es la extirpación quirúrgica completa.

Apocrine mixed tumor of the skin: a diagnosis challenge to consider. Cutaneous mixed tumor is an uncommon, benign adnexal neoplasm arising from apocrine or eccrine glands of the dermis. Apocrine mixed tumors often exhibit decapitation secretion, a feature of apocrine epithelium, but they may exhibit a wide range of metaplastic changes and differentiation in the epithelial, the myoepithelial, and the stromal components. We present two clinical cases of Apocrine mixed tumour of the skin in two different patients who have seen in our Department.

First case of congenital idiopathic hypohidrosis in China

AbstractA 43-year-old Chinese man presented with generalized hypohidrosis, which he had had since birth, without obvious abnormalities of other skin appendages except a sparse beard and axillary hairs. The sweat test revealed localized sweating on the face, axillae and palms. Histopathologic examination showed that the sweat glands were absent in the forearm and thigh, but some eccrine and apocrine sweat glands were present in the right axilla. S-100 was expressed in the nerve terminals surrounding the acini and ducts of the eccrine sweat glands, while PGP9.5 was positive in the acini of apocrine glands and the nerve terminals surrounding the eccrine glands in the axilla. To our knowledge, this is the first case of congenital idiopathic hypohidrosis in China.

Hidradenite supurativa (acne inversa): revisão da literatura e relato de caso sobre o tratamento cirúrgico de lesão pré-esternal / Hidradenitis suppurativa (acne inversa): review of the literature and case report on the surgical treatment of a presternal lesion

A Hidradenite Supurativa é uma doença crônica debilitante, estigmatizante e de difícil tratamento. A doença apresenta várias características clínicas, podendo ocorrer isolada ou simultaneamente em diversas localizações, geralmente simétricas, distribuídas na "linha do leite". Afeta a pele onde há maior quantidade de glândulas apócrinas intertriginosas, em ordem decrescente: axilas, região ano-genital, aréolas e sulco inframamário. Seu curso insidioso inicia com nódulos subcutâneos que se rompem e/ou coalescem, formando abscessos na derme profunda, extremamente doloridos. As lesões frequentemente drenam exudato purulento fétido, com importante prejuízo à qualidade de vida. Com a progressão da doença, ocorre formação de fistulas, comedões, fibrose, contraturas dérmicas e endurecimento da pele. Suas maiores chances de cura estão no diagnóstico precoce e tratamento individualizado, que abrange medidas farmacológicas, comportamentais e cirúrgicas. O tratamento cirúrgico tem sido considerado a medida curativa mais efetiva. A decisão entre as diversas modalidades vai depender do estágio, apresentação e comprometimento local e incluem incisão e drenagem dos abscessos, deroofing, marsupialização, eletrocirurgia, laser Nd:YAG, laser de CO2 e excisão cirúrgica extensa. As opções de reconstrução incluem cicatrização por segunda intenção, enxerto de pele total imediato ou tardio, fechamento primário e retalhos. O caso relatado de lesões préesternais apresentava características clínicas e histológicas compatíveis com HS, sendo esta uma localização incomum na Literatura. O resultado pós-operatório da ressecção de toda a lesão com fechamento primário mostrou-se resolutivo após longo tempo de seguimento. Mais ensaios clínicos randomizados são necessários para estipular o melhor manejo na HS.

Hidradenitis suppurativa is a chronic debilitating and stigmatizing disease that is difficult to treat. The disease presents several clinical characteristics, which may occur alone or simultaneously in various locations, generally symmetrical and distributed in the "milk line". It affects the following areas of the skin where intertriginous apocrine glands are numerous, in the descending order: axilla, anogenital region, areolas, and inframammary crease. Its insidious progression begins with formation of subcutaneous nodules that rupture and/or coalesce, forming extremely painful abscesses in the deep dermis. The lesions often drain foul purulent exudate, with significant damage to quality of life. As the disease progresses, formation of fistulas, comedones, fibrosis, dermal contractures, and hardening of the skin occur. The highest chances of cure are lie in early diagnosis and individualized treatment, which covers pharmacological, behavioral, and surgical measures. Surgical treatment has been considered a more effective curative measure. The decision between the different modalities will depend on the stage, presentation, and local commitment and include incision and drainage of abscesses, deroofing, marsupialization, electrosurgery, Nd:YAG laser, CO2 laser, and extensive surgical excision. The reconstruction options include healing by second intention, immediate or delayed full-thickness skin graft, primary closure, and flaps. The reported case of presternal injuries presented clinical and histological characteristics compatible with hidradenitis suppurativa; this location has been rarely reported in the literature. The postoperative results of complete resection of the lesion with primary closure indicated resolution over a long follow-up period. More randomized clinical trials are needed to determine the best management strategy for hidradenitis suppurativa.

Sonography of Invasive Apocrine Carcinoma of the Breast in Five Cases

OBJECTIVE: To evaluate the sonographic features of invasive apocrine carcinoma (IAC) of the breast. MATERIALS AND METHODS: This study included five pathologically proven cases of IAC, and their sonographic features were retrospectively analyzed according to the Breast Imaging Reporting and Data System (BI-RADS) lexicon. RESULTS: All five lesions involved the left breast and were seen as irregularly shaped masses. All lesions, except one, had a parallel orientation to the chest wall. All five lesions showed noncircumscribed margins and heterogeneous echotexture; however, they showed various posterior features. One lesion had edema as an associated feature. Sonographic assessments were classified as BI-RADS category 4 in all five cases. CONCLUSION: Invasive apocrine carcinoma sonographic findings are difficult to differentiate from those of invasive ductal carcinoma of no special type.

Carcinoma apócrino de mama: relato de caso e revisão de literatura / Apocrine breast carcinoma: a case report and literature review

O carcinoma apócrino de mama é raro e, por esse motivo, permanece com origem e definição controversas. Há quem acredite que se trate de uma variante do carcinoma invasivo. Apresenta-se em cerca de 0,5 a 8% dos casos de carcinoma mamário, não sendo possível sua diferenciação em relação aos carcinomas usuais da mama por meio de exames radiológicos. O diagnóstico pode ser realizado pela citologia, que é típica desse tumor. Geralmente é positivo para a GCDFP-15(gross cystic disease fluid protein-15), marcador de diferenciação apócrina mais confiável do que amorfologia. A imunoistoquímica costuma ser negativa para estrógeno e progesterona e positiva para o receptor de andrógeno. A taxa de positividade para a p53, HER-2 e BCL-2 em carcinomas apócrinos é quase a mesma que para os carcinomas não apócrinos. O tratamento é cirúrgico; a adjuvância costuma ser a mesma do carcinoma ductal invasor e, ao que tudo indica, também tem o mesmo prognóstico.

Apocrine carcinoma of the breast is a rare condition, and for this reason its origin and definition remains controversial. Some believe that is a variant of invasive carcinoma. The incidence of this disease is about 0.5 to 8% of the new diagnosed cases of breast carcinoma. Since it is not possible to differentiate the apocrine carcinoma from the usual breast carcinomas by using radiological images, diagnosis can be made by cytology showing typical alterations of this tumor. It is usually positive for GCDFP-15 (gross cystic disease fluid protein-15), a marker of apocrine differentiation more reliable than morphology. This type of tumor is often negative for estrogen and progesterone and positive for the androgen receptor. The rate of positivity for p53, HER-2 and BCL-2 in apocrine carcinomas is almost the same as for non-apocrine carcinomas. The surgical and adjuvant treatment is normally the same as invasive ductal carcinoma, and both seem also to have the same prognosis.

Occurrence of apocrine secretion in the larval gut epithelial cells of Aedes aegypti L., Anopheles albitarsis Lynch-Arribálzaga and Culex quinquefasciatus say (Diptera: Culicidae): a defense strategy against infection by Bacillus sphaericus Neide? / Ocorrência de secreção apócrina nas células epiteliais do intestino de larvas de Aedes aegypti L., Anopheles albitarsis Lynch-Arribálzaga e Culex quinquefasciatus say (Diptera: Culicidae): um mecanismo de defesa contra infecção causada por Bacillus sphaericus Neide?

Cell hypertrophy was the first reaction of the gut epithelial cells of Aedes aegypti (L.), Anopheles albitarsis (Lynch-Arribálzaga) and Culex quinquefasciatus (Say) to the toxins of strains 2362 and S1116 of Bacillus sphaericus, as cells had an increase of intracellular secretory activity. Soon after the cell hypertrophy developed, vesicles were formed at the cell apical portion, which detached with the plasma membrane, characterizing a type of apocrine secretion like. The first pathway of contamination of the mosquito larvae by these bacteria is through the gut, by feeding. Depending on the species of Culicidae and on the bacterial strain used, the hypertrophy of the gut epithelium occurred between 5 and 15 min after exposure to the contaminated environment. The second aspect observed after hypertrophy was the increase in apocrine secretion. The basophilic vesicles that detached from the cells remained between the peritrophic membrane and the gut lumen, such vesicles were filled with material of unknown nature. The gut posterior region showed secretory activity in both control and treated larvae, being much more intense in bacteria-exposed larvae. There were remarkable differences in the epithelial cell reaction according to the toxins of the two bacterial strains, but C.quinquefasciatus was the most susceptible. Differences in the gut cell reactions to the toxins produced by the two bacterial strains are discussed.

Observou-se hipertrofia das células do epitélio intestinal de Aedes aegypti (L.), Anopheles albitarsis (Lynch-Arribálzaga) e Culex quinquefasciatus (Say) devido ao aumento da atividade secretora intracelular como primeira reação às toxinas das linhagens 2362 e S1116 de Bacillus sphaericus (Neide). Após a hipertrofia epitelial, formaram-se vesículas na porção apical das células, as quais eram compostas de fragmentos de membrana plasmática contendo material de natureza desconhecida em seu interior, caracterizando um tipo de secreção apócrina. A via de contaminação das larvas dos mosquitos por essas bactérias é pelo intestino, através da alimentação. Dependendo da espécie de Culicidade e da linhagem bacteriana utilizada, a hipertrofia do epitélio ocorreu entre 5 a 15 min após a exposição das larvas ao meio contaminado. O segundo aspecto observado no processo de contaminação foi o aumento da atividade de secreção apócrina. As vesículas basófilas que se desprendiam das células permaneciam entre a membrana peritrófica e o lúmen intestinal. Observou-se atividade secretora tanto no grupo controle como no experimental, porém muito mais intensa no grupo experimental. Os Culicidae estudados apresentaram diferenças marcantes nas respostas às toxinas das bactérias utilizadas, sendo C. quinquefasciatus a espécie mais suscetível. As diferenças de reações das células em relação às toxinas produzidas pelas duas linhagens bacterianas são discutidas.

Apocrine hidrocystoma in external auditory canal

The present report describes clinical, radiographic, and pathological features of an external auditory canal giant apocrine hidrocystoma in a 36-year-old Iranian woman. To the best of our knowledge, this tumor has not previously been reported in this area. There was no evidence of local recurrence one year after surgery

Bilateral axillary apocrine nevi--a case report.

Apocrine glands are tubular glands, which function as scent glands. These are found at only a few sites like the axillae and anogenital region. They are present as modified glands in the external ear canal (ceruminous glands), in the eyelid (Moll's glands) and in the breast (mammary glands). Occasionally, apocrine glands are found on the face, in the scalp and on the abdomen where they are usually small and non-functional. Apocrine glands develop their secretory portion and become functional only at puberty. Tumours of these glands involving only apocrine tissue are very rare. We present an uncommon case of benign apocrine nevi of the axilla, which occurred bilaterally. These nevi are uncommon and only occasional reports are available in literature.

Apocrine carcinoma of the breast presenting as a solitary cyst: cytological and histopathological study of a case.

Apocrine carcinoma is a rare, unique and morphologically distinct type of invasive breast carcinoma. This tumor has predilection for elderly females (6th and 7th decade). This report is of apocrine carcinoma of the breast arising in a 27 year old female, a rare occurrence in this age group, with only a few cases on record.

Adenoma tubular apocrino: cuatro observaciones / Tubular apocrine adenoma: four cases

El adenoma tubular apocrino es una neoplasia anexial cutánea benigna (veinticinco referencias en la bibliografía). Referimos cuatro nuevos casos de adenoma tubular apocrino, dos de ellos localizados en la vulva, uno en el cuero cabelludo y otro en el tórax. El rango etario está entre los 40-70 años de edad. Las manifestaciones clínicas fueron nódulos de 1 a 2 cm de diámetro, tres de los cuales tenían áreas quísticas. En la microscopía las lesiones muestran estructuras ductales con una doble capa de células (epiteliales y mioepiteliales), con evidencia de secreción por decapitación. El estudio inmunohistoquímico confirma el origen apocrino de las lesiones. (EMA, CEA, S 100, citoqueratina). La resección quirúrgica fue definitiva en todos los casos. No se presentaron recidivas hasta la fecha. Constituyen los primeros casos referidos en nuestro país