Syringomatous tumor of the nipple: a case report.

Syringomatous tumor of the nipple is a benign, locally infiltrative tumor. There are reports in the literature of tumor recurrence in cases of incomplete excision. Clinical and mammographic findings in syringomatous tumors are like those of breast carcinoma and the pathologist has a fundamental role in final tumor diagnosis. Therefore, the aim of this study was to report a case of syringoma located in the areolar region. A 33-year-old woman reported that she had noticed a nodule in her left areolar region 4 years previously (February 2019). A breast ultrasound was performed, detecting intraparenchymatous breast cysts. Surgical resection of the nodule was indicated although it was not performed. Two years later, in August 2021, the patient underwent a mastopexy with prosthesis inclusion. Histopathology study of the surgical specimen revealed a syringomatous tumor with positive margins. Thirteen (13) months after diagnosis (September 3, 2021 - October 16, 2022), the patient is doing well and receives clinical follow-up.

Value of Immunohistochemistry to Differentiate Digital Papillary Adenocarcinoma From Acral Hidradenoma With Papillary Structures.

ABSTRACT: Digital papillary adenocarcinoma is a malignant adnexal tumor with a predilection for acral sites. Hidradenoma is a benign solid and cystic sweat gland neoplasm with focal ductal and glandular differentiation and good outcomes. Hidradenomas can occur at acral sites and show papillary structures; for this reason, they are included in the differential diagnosis of digital papillary adenocarcinoma, and immunohistochemistry is a valuable tool in this scenario. We described a case of a 43-year-old man with an epithelial tumor showing papillary structures in the intermediate phalanx of the fourth finger. There was diffuse positivity for p63 and negativity for S100 protein, suggesting that this tumor was an acral hidradenoma with papillary structures.

Breast carcinoma or sweat gland carcinoma? A report of two cases and a comparison with the literature / ¿Carcinoma de mama o carcinoma de glándula sudorípara? Presentación de dos casos y análisis de la literatura.

Primary apocrine carcinoma of the sweat gland is a neoplasm with a very low incidence that may represent a clinical and histological diagnostic challenge, as well as for adequate local, adjuvant, and advanced disease management. The average age of patients is around 67 years with no gender preference. This cancer develops primarily at the axillary and scalp levels and is clinically characterized by slow growth, but can progress aggressively with local, nodal, and metastatic involvement (primarily lung, liver, and bone). The recommended management, once the histology is established, consists of a wide local resection with a clear margin of 1 to 2 cm and regional lymphadenectomy if clinically positive nodes are detected. The adjuvant treatment (radiotherapy or chemotherapy) and for the advanced disease is not established. We report here the cases of two female patients initially diagnosed with breast cancer who were finally diagnosed with apocrine carcinoma of the sweat gland.

El carcinoma apocrino primario de glándula sudorípara es una neoplasia con una muy baja incidencia, que puede representar un reto diagnóstico, clínico e histológico, y un reto terapéutico local, adyuvante y de la enfermedad avanzada. La edad media de los pacientes es de alrededor de 67 años, y no se ha observado preferencia según el sexo. Se presenta con mayor frecuencia en las axilas y en el cuero cabelludo. Se caracteriza clínicamente por un lento crecimiento, aunque puede progresar agresivamente, con compromiso local, ganglionar y metastásico, principalmente, pulmonar, hepático y óseo. El tratamiento recomendado -una vez establecida la histología- consiste en una resección local amplia con un margen claro de 1 a 2 cm y linfadenectomía regional si se detectan ganglios clínicamente positivos. El tratamiento adyuvante (radioterapia o quimioterapia) y de la enfermedad avanzada no está claramente establecido. Se presentan dos pacientes de sexo femenino con sospecha inicial de cáncer de mama, en quienes se diagnosticó finalmente un carcinoma apocrino de glándula sudorípara.

Multiple eruptive and vulvar syringomas and the incipient role of dermoscopy in diagnosis / Siringomas eruptivos múltiples y vulvares y el papel incipiente de la dermatoscopia en el diagnóstico

INTRODUCCIÓN: Los siringomas son tumores benignos derivados de la porción intraepidérmica de los conductos sudoríparos ecrinos. Ocasionalmente pueden iniciar de forma súbita como siringomas eruptivos o localizarse en sitios atípicos que retrasan el diagnóstico por años. La dermatoscopía tiene un rol incipiente en diferenciar siringomas de su extenso diagnóstico diferencial. MÉTODOS: Estudio retrospectivo descriptivo de serie de casos de siringomas de localización atípica. Los datos fueron extraídos de fichas clínicas electrónicas. Todos incluyen dermatoscopía y correlación histopatológica. RESULTADOS: Cinco pacientes. Cuatro hombres y una mujer entre 40 y 79 años de edad con siringomas atípicos: cuatro casos eruptivos y un caso de siringomas vulvares. DISCUSIÓN: Proponemos la dermatoscopía basada en nuestros hallazgos como una herramienta útil con estructuras ovales amarillas y una pseudo-red café clara difusa en su superficie. Estas estructuras amarillas se pueden correlacionar con la proliferación ductal ecrina y el denso estroma en la histopatología. CONCLUSIÓN: Enfatizamos que se debe considerar esta entidad en el diagnóstico diferencial de dermatosis papulares y conocer sus manifestaciones clínicas para optimizar la sospecha diagnóstica.

INTRODUCCTION: Syringomas are common benign tumors, probably of origin derived from the intraepidermal portion of the eccrine sweat ducts. Occasionally they may develop suddenly and extensively as eruptive syringomas or be located in atypical sites delaying the diagnosis for years. Dermoscopy has an incipient role in differentiating syringomas from their extensive differential diagnosis. METHODS: Retrospective descriptive case-series study of atypical location syringomas. Data extraction from clinical history from electronic files. They all include dermoscopy and histopathological correlation. RESULTS: Five patients: Four men and one woman between 40 and 79 years old, with atypical syringomas diagnosis: four eruptive and one vulvar syringomas. DISCUSSION: We propose dermoscopy, based on our findings, as a useful tool to this entity, with its oval yellow structures and a diffuse light-brown network-like structure on its surface. These yellow enlargements may be correlated with the ductal eccrine proliferation and the dense stroma seen in the histopathology. CONCLUSION: We emphasize that they should be considered in the differential diagnosis of papular dermatosis, as they tend to be underdiagnosed, and to know their clinical manifestations to optimize the diagnostic suspicion.

Poroma no pigmentado inusualmente grande: reporte de un caso y revisión de la literatura / Unusually large unpigmented poroma: a case report and literature review

El poroma es un tumor benigno infrecuente derivado de la porción intraepitelial del epitelio ductal de la glándula sudorípara. La presentación clínica más habitual es un pápula o nódulo de color rosado a rojo, de pequeño diámetro, localizado con mayor frecuencia en palmas y plantas, generalmente asintomático. Debido a su variabilidad morfológica, los poromas son usualmente difíciles de reconocer, siendo la dermatoscopía una herramienta útil en su diagnóstico. Presentamos el caso de un paciente masculino con un gran tumor en su pierna derecha de 40 mm de diámetro, con características dermatoscópicas compatibles con poroma no pigmentado. El diagnóstico confirmatorio se realizó con una biopsia incisional seguido de la extirpación quirúrgica completa del tumor

Poroma is a rare benign tumour derived from the intraepithelial portion of the sweat glands' ductal epithelium. The most common clinical presentation is a pink to red papule or nodule, small in diameter, predominantly in the palms and soles and generally asymptomatic. Due to its morphologic variability, poromas are usually difficult to recognize, thus dermatoscopy becomes a useful aid in its diagnosis. We present the case of a male patient with a large tumour on his right leg, 40 mm in diameter, with dermatoscopic features consistent with nonpigmented poroma. Confirmatory diagnosis was made by incisional biopsy followed by the total surgical excision of the tumor.

Hidrocistoma apocrino desafío dermatoscópico / Apocrine hydrocystoma dermoscopic challenge

Los hidrocistomas apocrinos son lesiones quísticas benignas. Clínicamente se caracterizan por ser nódulos quísticos claros o traslucidos, solitarios, en forma de cúpula, con superficie lisa. En la mayoría de los casos la localización suele ser alrededor del ojo, particularmente lateral al canto externo. Suelen ser asintomáticos, de crecimiento lento pudiendo llegar a 10 mm de diámetro o más. Presentamos un caso de un paciente con un hidrocistoma apocrino variante pigmentada.

Apocrine hydrocystomas are benign cystic lesions, clinically characterized by being clear or translucent cystic nodules, solitary, domeshaped, with a smooth surface. In most cases the location is usually around the eye, particularly lateral to the outer edge. They are usually asymptomatic, slow growing and can reach 10 mm in diameter or more. We present a case of a patient with a pigmented variant apocrine hydrocystom

Concomitant poroma and porocarcinoma.

Eccrine poromas and porocarcinomas are adnexal tumors derived from the sweat duct epithelium. However, eccrine poroma is benign in nature, whilst eccrine porocarcinoma is regarded as its malignant counterpart. We report the case of a man who presented simultaneously with an eccrine poroma and eccrine porocarcinoma. Both lesions had no clear distinctive features, enhancing the need of high-level clinical suspicion together with surgical excision and histopathology for prompt diagnosis confirmation.

Concomitant poroma and porocarcinoma

Abstract: Eccrine poromas and porocarcinomas are adnexal tumors derived from the sweat duct epithelium. However, eccrine poroma is benign in nature, whilst eccrine porocarcinoma is regarded as its malignant counterpart. We report the case of a man who presented simultaneously with an eccrine poroma and eccrine porocarcinoma. Both lesions had no clear distinctive features, enhancing the need of high-level clinical suspicion together with surgical excision and histopathology for prompt diagnosis confirmation.

Ductal eccrine carcinoma of the axilla: a diagnostic pitfall.

Ductal eccrine carcinoma (DEC) is a rare sweat gland carcinoma with ductular differentiation. Clinically, it is characterized by a slowly growing, hardened plaque or nodule predominantly located on the head and neck. Histologically, DEC shares similar features to invasive breast carcinoma, thus causing great diagnostic challenges. We report a 69-year-old woman who presented with a hardened plaque on the axilla. A skin biopsy was performed and metastatic invasive breast carcinoma could not be ruled out. Complete excision and further workup were subsequently conducted, leading to the diagnosis of estrogen receptor positive DEC with associated axillary lymph node metastases. The patient received adjuvant radiotherapy to the left axilla and was started on oral letrozole. She is disease-free 14 months after initial diagnosis.

Ductal eccrine carcinoma of the axilla: a diagnostic pitfall

Abstract: Ductal eccrine carcinoma (DEC) is a rare sweat gland carcinoma with ductular differentiation. Clinically, it is characterized by a slowly growing, hardened plaque or nodule predominantly located on the head and neck. Histologically, DEC shares similar features to invasive breast carcinoma, thus causing great diagnostic challenges. We report a 69-year-old woman who presented with a hardened plaque on the axilla. A skin biopsy was performed and metastatic invasive breast carcinoma could not be ruled out. Complete excision and further workup were subsequently conducted, leading to the diagnosis of estrogen receptor positive DEC with associated axillary lymph node metastases. The patient received adjuvant radiotherapy to the left axilla and was started on oral letrozole. She is disease-free 14 months after initial diagnosis.

[Apocrine hidrocystoma associated with gouty tophi of the pinna]. / Hidrocistoma apocrino asociado con tofo gotoso en pabellón auricular.

BACKGROUND: Apocrine hidrocystomas, also known as apocrine cystadenomas, are bening cystic tumours derived from the secretory portion of apocrine sweat glands. CLINICAL CASE: A 78-year old female was referred to our division for assesment an asymptomatic translucent, well-defined cystic lesion located on the upper helix. The histological features were consistent with apocrine hidrocystoma coexisting with gouty tophi. CONCLUSIONS: We report the second case of apocrine hydrocystoma located in the pinna, outside of ear canal and the first case of its association with gouty tophi described. It is difficult to know which lesion was first established and if the tophi may lead to ductal obstruction and subsequent cystic retention.

Introducción: los hidrocistomas apocrinos, también conocidos como cistoadenomas apocrinos, son lesiones quísticas benignas derivadas de la porción secretora de las glándulas apocrinas. Caso clínico: remiten al servicio de Dermatología a una mujer de 78 años para valorar una lesión asintomática translúcida, bien definida, localizada en la porción superior del hélix. Los hallazgos histológicos fueron compatibles con un hidrocistoma apocrino asociado a tofo gotoso. Conclusiones: reportamos el segundo caso de hidrocistoma apocrino localizado en el pabellón auricular externo y el primer caso asociado a tofo gotoso. Es difícil de conocer qué lesión se estableció primero, y si el tofo gotoso pudo provocar una obstrucción ductal con la subsiguiente retención quística.

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.

Características clínicas y dermatoscópicas del poroma ecrino / Clinical and dermatoscopic characteristics of the eccrine poroma

El poroma ecrino (PE) corresponde a una neoplasia anexial benigna originada de la porción intraepidérmica del conducto de las glándulas sudoríparas. Habitualmente se describe como nódulos eritematosos o color piel ubicados en región palmoplantar, pero sus características clínicas son muy variables. Se decriben los hallazgos clínicos y dermatoscópicos de una serie de casos de pacientes con PE. La dermatoscopia puede facilitar significativamente el diagnóstico clínico de la mayoría de los tumores de la piel, sin embargo el PE se caracteriza por un alto grado de variabilidad a la dermatoscopía. Habitualmente se describe un patrón vascula polimorfo con vasos glomerulares, en horquilla, y lineales irregulares con terminaciones semicirculares rodeados de un halo blanco a rosa en forma de caliz o flor de cerezo asociado a áreas rosadas blanquecinas sin estructuras similares a lo observado en melanoma amelanótico, carcinoma espinocelular entre otros tumores benignos y malignos. El PE puede exhibir características clínicas y dermatoscópicas polimórficas que pueden hacer difícil el diagnóstico y su diferenciación de otras neoplasias cutáneas.

Eccrine poroma (PE) is a benign adnexal neoplasm of the intraepidermal portion of the sweat gland duct. PE typically present as erythematous or flesh-colored nodules on the palms and soles, but the clinical features of PE are highly variable. Dermatoscopic and clinical findings of patients with EP are described. Dermoscopy may significantly improves the clinical diagnosis of most skin tumours, but PE is characterised by a high degree of dermoscopic variability. The main dermoscopic features described are polymorphous vascular pattern, whiteto- pink halo surrounding the vessels, pink-white structureless areas, vascular structures of glomerular, hairpin vessels, and linear irregular vessels, mimicking amelanotic melanoma, keratinising tumours and several benign and malignant tumors. Eccrine poroma may exhibit polymorphic clinical and dermoscopic features making it difficult to diagnosis and differentiate from other skin cancers.

Periungual eccrine poroma.

BACKGROUND: Eccrine poroma is a rare, benign skin appendage tumor originating from the intraepidermal portion of the eccrine sweat duct, which typically occurs on the sides and soles of the feet. Nonetheless, eccrine poroma may be found in any skin area bearing sweat glands. OBJECTIVE: Herein we report a case of an eccrine poroma in an unusual location, the surgical management of the condition, and follow-up processes.

Porocarcinoma ecrino: reporte de un caso / Eccrine porocarcinoma: a case report

Los tumores anexiales de la piel representan un grupo heterogéneo de entidades de baja frecuencia de presentación. Su origen es controvertido, quizás a partir de células madres pluripotenciales, aceptándose principalmente dos líneas embriológicas: pilosebáceo-apócrina y ecrina. Se los clasifica en cuatro grupos principales: tumores del folículo piloso, tumores sebáceos, tumores apócrinos y tumores ecrinos. Su distribución anatómica refleja áreas con mayor densidad de anexos cutáneos, presentándose en forma única o múltiple.

Skin adnexal tumors are represented as a heterogeneous group of entities of low frequency range. Its origin is controversial, perhaps from pluripotent stem cells, mainly from two embryological lines: pilosebaceous-apocrine and eccrine. They are classified as four main groups: tumors of the hair follicle, sebaceous tumors, apocrine tumors and eccrine tumors. Their anatomic distribution reflects areas with the highest density of cutaneous adnexal, arising in either single or multiple forms.