Espiradenoma écrino en muslo de mujer: reporte de un caso

Introducción: El espiradenoma ecrino es un tumor anexial benigno raro que surge de las glándulas sudoríparas écrinas. Comúnmente se presenta como un nódulo de crecimiento lento en la parte superior del tronco y la región de la cabeza y el cuello, principalmente en el rango de edad de 15 a 35 años, sin predominio de género. Por lo general, se presentan como pequeños nódulos solitarios que pueden crecer varios centímetros, a menudo muy dolorosos. El diagnóstico de esta entidad es de suma importancia ya que puede albergar un componente maligno con desenlaces catastróficos, ya que pueden pasar desapercibidos por su gran parecido con lesiones benignas. Caso Clínico: Aquí presentamos el caso de una mujer 41 años que presentó una lesión cutánea de crecimiento similar a un papiloma en la cara externa de tercio superior del muslo, con el diagnóstico de espiradenoma écrino posterior a la extirpación.

Introduction: Eccrine spiradenoma is a rare, benign adnexal tumor arising from the eccrine sweat glands. It commonly presents as a slow-growing nodule on the upper trunk, and head and neck region, mostly in the age bracket of 15 - 35 years, with no gender preference. Typically, they present as small solitary nodules that can grow to several centimeters, often they are strikingly painful. The diagnosis of this entity is extremely important as it can harbour a malignant component with catastrophic outcomes wich may be missed due to its strong resemblance to benign lesions. Case Report: We present the case of a 41-year-old woman who presented with a papilloma-like growth on the upper lateral aspect of the thigh which was diagnosed as eccrine spiradenoma upon excision.

Giant Eyelid Eccrine Hidrocystoma: A Case Report

Aims: To report a case of a rare giant eyelid eccrine hidrocystoma confirmed on histopathological examination and successful surgical excision with no recurrence.Presentation of Case: A young male presented with a mass over his eyelid which was gradually increasing in size, leading to drooping of the upper eyelid with no other associated symptoms. The mass 20x15x10 mm was present over the upper lid, non-mobile, non-tender, and firm. On the CT scan, a well-defined round to oval hypodense cystic mass was seen to be originating from the left upper eyelid. The cyst was excised and sent for histopathological analysis. The histological appearance of cystic spaces lined by cuboidal epithelium and containing mucinous fluid was suggestive of eccrine hidrocystoma. There was no recurrence till 2 months of follow-up.Conclusion: Eccrine hidrocystomas are 1-6 mm in size, generally 4 mm, and cysts larger than 10 mm are rare. Larger cysts have been described as “giant eccrine hidrocystoma.” Giant eyelid eccrine hidrocystoma is an unusual condition of the eyelid that must be considered in the differential diagnosis of eyelid tumours. They can be managed by complete surgical excision although recurrences are found to be common.

Siringofibroadenoma écrino em paciente tetraplégico por mielite transversa / Eccrine syringofibroadenoma in a patient with quadriplegia following transverse myelitis

O siringofibroadenoma écrino (SFAE) é um tumor benigno raro que se origina das glândulas sudoríparas écrinas. Apresenta predileção pelas extremidades de indivíduos idosos e pode surgir em associação com várias doenças adquiridas ou hereditárias e com afecções cutâneas neoplásicas. Relatamos caso de homem de 48 anos, tetraplégico por mielite transversa há 30 anos, com tumoração plantar rapidamente progressiva de difícil diagnóstico

Eccrine syringofibroadenoma (EFAS) is a rare benign tumor that originates from the eccrine sweat glands. It has a predilection for the extremities of elderly individuals and may arise in association with various acquired or hereditary pathologies and neoplastic skin disorders. We report the case of a 48-year-old man who had been quadriplegic due to transverse myelitis for 30 years, with a rapidly progressive plantar tumor that was difficult to diagnose.

Modified Mohs micrographic surgery for the treatment of five cases of eccrine porocarcinoma / 中华皮肤科杂志

Objective:To investigate the efficacy of modified Mohs micrographic surgery in the treatment of eccrine porocarcinoma.Methods:Clinical data were collected from 5 patients with eccrine porocarcinoma, who treated with modified Mohs micrographic surgery in Department of Dermatology, Wuhan No.1 Hospital from 2016 to 2020, and retrospectively analyzed.Results:Among the 5 patients, 2 were males and 3 were females, their age ranged from 52 to 80 years, and the course of disease ranged from 1 to 50 years. Skin lesions were located on the scalp, face, abdomen, thighs and feet, with diameters ranging from 1 to 6 cm. Tumor excision was performed with 1-cm margins, and was deep to the superficial fascial layers. For histopathologically negative surgical margins, the defects were closed with skin flaps or grafts; for histopathologically positive margins, the excision margin continued to be widened until it was clean. The 5 patients were followed up for 1 - 5 years without recurrence, and 1 died 2 years later due to liver cancer metastasis.Conclusion:Modified Mohs micrographic surgery can ensure clean resection margins and reduce the loss of normal tissues in the treatment of eccrine porocarcinoma.

Malignant chondroid syringoma: An unusual scalp tumor

Chondroid syringomas are similar to mixed parotid tumors and present in both benign and malignant forms. Malignant chondroid syringoma (MCS) is a rare skin tumor that has a predilection for extremities, particularly in young women. It is even rarer to present as a scalp tumor with very few reported cases in the literature. We present a middle-aged woman, with a history of increasing fatigability of her right arm for the past 3 months who, on examination, was found to have scalp swelling and matted right posterior triangle lymph nodes. The working diagnosis on her was a large sebaceous cyst with secondary in the neck from an occult primary/non-Hodgkin抯 lymphoma. Preliminary fine-needle aspiration was inconclusive. Imaging followed by wide excision of the tumor and the nearby occipital node was done. The final histopathological diagnosis was MCS with secondary in the lymph node. These tumors are aggressive and metastasize early. Radical surgery is the only hope of cure as adjuvant treatment is yet to be standardized

ECCRINE SPIRADENOMA : CASE REPORT WITH AN UNUSUAL CLININAL PRESENTATION

Eccrine spiradenoma is one of the rare, benign dermal neoplasm showing eccrine sweat gland differentiation. The literature search accounts for less than 100 reported cases of eccrine spiradenoma. Clinically, the lesion presents as solitary, intradermal, circumscribed, round or oval, firm, painful or tender nodule measuring from 0.3 - 5 cm in diameter. Rarely if ES, presents as multiple lesions, in combination with other types of adnexal tumors such as cylindromas & trichoepitheliomas, it can be considered as a part of the Brooke-Spiegler syndrome. Majority of these tumours appear on the head/face & upper trunk region of the body. Spiradenomas arise in early adulthood in most instances with few reports also of congenital cases and no sex predilection seen. The definitive diagnosis is made by excisional biopsy of the tumour. Complete tumor excision with clear surgical margins is considered the primary treatment for these cases. Here, we present a rare case report of a 58 years old male presenting with a soft, cystic lesion in the left lumbar region of the abdomen for past 2 years duration. Surgical excision of the tumour was done with clear margins and the specimen was sent for histopathological examination. Due to the overlapping histomorphological features, this tumour is often confused with cylindromas and trichoepitheliomas along with other vascular tumours. Eccrine Spiradenoma, although a benign tumour, but malignant transformation has been described especially in long-standing cases or multiple lesions. So, early diagnosis is very important to prevent recurrence and to identify any onset of malignant transformation

A SURGEON'S SURPRISE TO VERRUCOUS GROWTH OVER CHEEK - CASE REPORT ON MALIGNANT ECCRINE POROMA

Malignant eccrine poroma is a rare malignancy of eccrine glands , whose diagnosis is an index of high suspicion and challenging to a clinician. A case of 67 year old female with swelling over left cheek since 20 years with no radiation exposure and no comorbidities , was investigated and wide local excision with 5mm margins from the tumor was performed , and specimen sent for biopsy - which revealed tumor cells arranged in lobules with peripheral palisading , ductal and squamous differentiation - suggestive of Porocarcinoma.Wide surgical excision is the mainstay of treatment, resulting in cure rates of 70–80% when the margins are clear. Some studies also reported good outcomes with Mohs micrographic surgery, and this technique could also be considered 1. Other treatments such as chemotherapy and radiotherapy have also been reported in the literature without clear standard guidelines

Hidradenoma nodular maligno / Malignant Nodular Hidradenoma

Introducción: El hidradenoma nodular maligno es un tumor maligno de glándula sudorípara ecrinas, poco común, considerada una lesión de diferenciación anexial ecrinas, que generalmente surge de nuevo, aunque se han descrito unos pocos casos surgidos sobre un hidradenoma nodular. Es decir, representa la contrapartida maligna del hidradenoma nodular. Objetivo: Dar a conocer la presentación de un caso, dada la inusual aparición de esta entidad, con revisión de los criterios para su diagnóstico. Caso clínico: Se informa el caso de un hombre de 74 años de edad con una neo formación en la región parietal derecha del cuero cabelludo. Conclusiones: Debemos pensar en un hidradenoma nodular maligno ante un tumor solitario, firme o fluctuante, infrecuente en el cuero cabelludo, con curso agresivo, recurrencias y metástasis ganglionares y confirmar su diagnóstico con el estudio inmunohistoquímico(AU)

Introduction: Malignant nodular hidradenoma is a rare malignant eccrine sweat gland tumor considered a lesion of eccrine adnexal differentiation, which usually arises again, although a few arising cases on nodular hidradenoma have been described. In other words, it represents the malignant counterpart of nodular hidradenoma. Objective: To report a case, given the unusual occurrence of this entity, with a review of the criteria for its diagnosis. Case report: We report the case of a 74-year-old man with a neoformation in the right parietal region of the scalp. Conclusions: We should consider a malignant nodular hidradenoma when faced with a solitary, firm or fluctuant tumor, rare in the scalp, with aggressive evolution, recurrences and lymph node metastasis, and confirm its diagnosis with immunohistochemical study(AU)

Tratamento de siringomas periorbitários: revisão da literatura científica nos últimos 5 anos / Treatment of periorbital syringomas: review of scientific literature in the last 5 years

Introdução: O siringomas são tumores anexais benignos com caraterísticas histopatológicas decorrentes dos ductos écrinos, em forma de pápulas amareladas ou cor da pele, de 1-3 mm, comumente na região periorbitária inferior, podendo causar problemas cosméticos importantes. O objetivo do tratamento é melhorar a aparência, através da destruição completa do tumor usando métodos minimamente invasivos e inclusa cirurgia. Existem na literatura múltiplas opções de tratamento com vários graus de sucesso, porém pouco se conhece sobre a eficácia. Em geral, a remoção completa não é bem-sucedida, e têm sido descritos efeitos colaterais, sendo a recorrência o mais frequente. Métodos: Trata-se de uma revisão narrativa de literatura, de publicações científicas no período de 2014-2019. Resultados: Após revisar 45 artigos, e identificar os publicados nos últimos cinco anos que tiveram registro de número de pacientes, descrição de tratamento, escalas de avaliação dos resultados e acompanhamento, foram selecionados seis artigos. Do número total de seis artigos, foram encontrados: uma revisão sistemática, e cinco estudos retrospectivos, sendo um comparativo. Foi designado um número para cada artigo analisado, e coletados o número de pacientes incluídos, tratamento realizado, escalas de avaliação e resultados, complicações e conclusões. Conclusões: Os siringomas periorbitários ainda são um desafio terapêutico, e até agora nenhum tratamento demostrou ser consistentemente eficaz. O laser CO2 continua sendo a primeira escolha de tratamento quando usado fracionado, e a eletrocoagulação intralesional representa uma segunda alternativa com resultados moderados e menor risco de complicações. Novos tratamentos como Laser Erbium Laser Erbium Yttrium Aluminum Garnet, Neodymium-Doped Yttrium Aluminum Garnet e monoterapia com toxina botulínica A poderiam ser boas alternativas. Estudos prospetivos comparativos são necessários.

Introduction: Syringomas are benign adnexal tumors with histopathological characteristics arising from the eccrine ducts, in yellowish or skin-colored papules, 1-3 mm, commonly in the lower periorbital region, which can cause important cosmetic problems. The goal of treatment is to improve appearance by destroying the tumor using minimally invasive methods and including surgery. There are multiple treatment options in the literature with varying degrees of success, but little is known about their effectiveness. Complete removal is unsuccessful, and side effects have been described, recurrence being the most frequent. Methods: This is a narrative review of the literature of scientific publications in the period 2014-2019. Results: After reviewing 45 articles and identifying those published in the last five years that had a record of the number of patients, treatment description, scales of evaluation of results and follow-up, six articles were selected. Of the total number of six articles, we found: a systematic review and five retrospective studies, one being a comparative one. A number was assigned to each article analyzed, and the number of patients included, treatment performed, assessment scales and results, complications and conclusions were collected. Conclusions: Periorbital syringomas are still a therapeutic challenge, and so far, no treatment is consistently effective. The CO2 laser remains the first choice of treatment when used fractionally, and intralesional electrocoagulation represents a second alternative with moderate results and a lower risk of complications. New treatments such as Laser Erbium Laser Erbium Yttrium Aluminum Garnet, Neodymium-Doped Yttrium Aluminum Garnet and botulinum toxin A monotherapy could be good alternatives. Comparative prospective studies are needed.

Siringoma condroide: apresentação incomum de tumor raro / Chondroid syringoma: unusual presentation of a rare tumor

O siringoma condroide, também conhecido como tumor misto cutâneo, é uma neoplasia benigna rara, originada das glândulas sudoríparas, composta por estruturas epiteliais imersas em um estroma mixocondroide. Geralmente, apresenta-se como tumor sólido, único, localizado em face ou pescoço, com evolução crônica e assintomática. Relata-se caso de mulher, 75 anos, com lesão discretamente elevada na fronte, cujo diagnóstico foi definido pela análise histopatológica.

Chondroid syringoma, also known as a cutaneous mixed tumor, is a rare benign neoplasm originating from the sweat glands, composed of epithelial structures immersed in a myxochondroid stroma. It usually presents as a solid, single tumor located on the face or neck with a chronic and asymptomatic course. We report the case of a 75-year-old woman with a slightly elevated lesion on the forehead, whose diagnosis was defined by histopathological analysis.

Study on ultrasonic characteristics of eccrine spiradenomas and diagnostic efficacy / 中华超声影像学杂志

Objective:To investigate the ultrasonic characteristics of eccrine spiradenoma (ES) and to analyze the diagnostic value.Methods:Nineteen ES patients with 24 lesions confirmed by pathology in 3 grade-A tertiary hospitals from October 2011 to October 2021 were enrolled as study group, and in the same time, 46 patients with 46 masses in the skin and muscular tissues with clinical features of automatic pain and/or tenderness were selected as control group. The ultrasonographic characteristics of the two groups were analyzed retrospectively, including anatomical location, shape, boundary, internal echogenicity, echogenic distribution, calcification, posterior acoustic effect, and vascularity. The ultrasonographic characteristics of the two groups were compared, and the risk sonographic characteristics of ES were obtained by multivariate logistic regression analysis. The sensitivity and specificity of ultrasound characteristics for the diagnosis of ES were calculated.Results:The ultrasonic characteristics of the two groups had significant differences in lobulated shape, boundary, internal echogenicity and posterior acoustic effect (χ 2=32.65, 15.65, 5.77, 13.63; all P<0.01). Multivariate logistic regression analysis showed that lobulated shape and posterior acoustic enhancement were the risk ultrasonic characteristics of ES. The sensitivity and specificity of lobulated shape and posterior acoustic enhancement characteristics in the diagnosis of ES were 79.17%, 89.13%, 95.83% and 47.83%, respectively; and the sensitivity and specificity of the combination of lobulated shape and posterior acoustic enhancement characteristics in the diagnosis of ES were 79.17% and 97.83%, respectively. Conclusions:The lobulated shape and posterior acoustic enhancement characteristics are important for the identification of ES, which have higher diagnostic efficacy for ES.

Foxa1 mediates eccrine sweat gland development through transcriptional regulation of Na-K-ATPase expression

Eccrine sweat glands (ESGs) perform critical functions in temperature regulation in humans. Foxa1 plays an important role in ESG maturation and sweat secretion. Its molecular mechanism, however, remains unknown. This study investigated the expression of Foxa1 and Na-K-ATPase (NKA) in rat footpads at different development stages using immunofluorescence staining, qRT-PCR, and immunoblotting. Also, bioinformatics analysis and Foxa1 overexpression and silencing were employed to evaluate Foxa1 regulation of NKA. The results demonstrated that Foxa1 was consistently expressed during the late stages of ESGs and had a significant role in secretory coil maturation during sweat secretion. Furthermore, the mRNA abundance and protein expression of NKA had similar accumulation trends to those of Foxa1, confirming their underlying connections. Bioinformatics analysis revealed that Foxa1 may interact with these two proteins via binding to conserved motifs in their promoter regions. Foxa1 gain-of-function and loss-of-function experiments in Foxa1-modified cells demonstrated that the activities of NKA were dependent on the presence of Foxa1. Collectively, these data provided evidence that Foxa1 may influence ESG development through transcriptional regulation of NKA expression.

A case of recurrent porocarcinoma of the scalp requiring scalp reconstruction

Eccrine porocarcinoma (EPC) is a rare malignant tumour, first reported by Pinkus and Mehregan in 1963. It can develop from the eccrine duct epithelial component or transform from an underlying eccrine poroma. The tumor usually presents as an exophytic growth and has a female predilection. Rarely it can occur in scalp, face, ear, genitalia and eye lids. Treatment modalities have included standard excision, Mohs micrographic surgery, chemotherapy, and radiation therapy. Therapy of choice is surgical excision with clear margins with 70-80% cure rates. Chemotherapy is of no known significance and radiation might help in selective cases. Majority of cases are managed with excision and closure of defects but in selective cases there may be need to reconstruct the scalp defects. The methods of reconstruction may be local flaps, free tissue transfer and partial/full thickness skin grafts. Regional flaps are very useful in patients requiring cover of large defects.

Nodulos plantares dolorosos tras ejercicio intenso en paciente pediátrica: un reto diagnóstico / Painful nodules on the soles in a pediatric patient: a diagnostic challenge

Resumen: Introducción: La Hidradenitis palmoplantar ecrina idiopática (HPPI) es una dermatosis neutrofílica infrecuente, que cursa con nódulos eritematosos dolorosos de comienzo brusco en regiones plantares o palmoplantares, en niños que no tienen otra enfermedad subyacente. Objetivo: Presentar un caso que ilustra las principales características clínicas e histológicas de la HPPI. Caso Clínico: Niña de 11 años evaluada por nódulos eritematovioláceos dolorosos en planta de pie derecho de 48 horas de evo lución y fiebre de hasta 38,2 °C, sin antecedentes de interés salvo hiperhidrosis y práctica intensa de ejercicio en los días previos. Ante la sospecha clínica de HPPI se realizó biopsia cutánea, que mostró infiltrado inflamatorio neutrofílico alrededor de glándulas sudoríparas ecrinas y abscesos de neutrófilos, confirmando el diagnóstico. Se indicaron antiinflamatorios no esteroidales orales y reposo, con resolución de las lesiones en 7 días. Conclusiones: Este caso demuestra los aspectos más importantes de la HPPI. Esta entidad en muchos casos es infradiagnosticada, dado que puede confundirse con otras patologías que también cursan con nódulos acrales dolorosos, pero tienen distintas implicacio nes patogénicas y terapéuticas. Identificar apropiadamente la HPPI permite evitar la alarma innece saria, tanto en pacientes y sus padres, como en los propios dermatólogos y pediatras.

Abstract: Introduction: Idiopathic Palmoplantar Eccrine Hidradenitis (IPPH) is a rare neutrophilic derma tosis, with painful erythematous nodules of sudden onset in the plantar or palmoplantar region, in children without other underlying diseases. Objective: To present a case that shows the main clinical and histological characteristics of this entity. Clinical Case: 11-year-old girl with a 48-hours history of painful erythematous-violaceous nodules on the right foot plant associated with fever of up to 38.2 °C, with no history of interest except hyperhidrosis and intense exercising on previous days. Given the clinical suspicion of IPPH, a skin biopsy was performed, which showed inflammatory neutrophil infiltration around eccrine sweat glands and neutrophilic abscesses, confirming the diagnosis. Oral NSAIDs and rest were prescribed, with resolution of the lesions in 7 days. Conclusions: This case demonstrates the most important aspects of this entity, in many cases underdiagnosed, since it can be confused with other pathologies that occur with painful acral nodules, but have different pathogenic and therapeutic implications. To properly identify the IPPH allows preventing an unnecessary alarm, both patients and their parents, as in dermatologists and pediatricians themselves.

Hidradenocarcinoma en miembro inferior: reporte de caso y revision de literatura / Hydradenocarcinoma in lower member: case report and review of literature

RESUMEN Objetivo : Presentación de un caso, consistente en una lesión tumoral, compatible con hidradenocarcinoma, desarrollado en un paciente joven, y revisión de la literatura referida a la entidad mencionada. Métodos : Se valora en el servicio de dermatología un paciente de 17 años con una lesión de crecimiento progresivo, localizada en tercio medio de la pierna derecha, la cual a la inspección muestra secreción hialina y sangrado, manifestando prurito como síntoma ocasional, al inicio de la lesión. Se decide tomar biopsia de la lesión para confirmar diagnóstico. Resultados : Se hace diagnóstico clínico e histopatológico de hidradenocarcinoma siendo este un tumor raro maligno, originado en glándulas sudoríparas que se localiza cualquier parte del cuerpo, el cual en la mayoría de los casos se ha descrito como de origen ecrino. Conclusiones : Aunque no es una patología frecuente tiene altas tasas de recurrencia y riesgo de metástasis. Por lo tanto, es importante considerarla siempre como uno de los diagnósticos diferenciales, y de esa manera ofrecer un oportuno tratamiento quirúrgico, con márgenes amplios de resección.

SUMMARY Objective : Presentation of a case report, consisting of a tumor lesion, compatible with hydradenocarcinoma, developed in a young patient, and review of the literature referring to the mentioned entity. Methods : A 17-year-old patient with a progressive growth lesion, located in the middle third of the right leg, was evaluated in the dermatology service, which upon inspection showed hyaline secretion and bleeding, manifesting pruritus as an occasional symptom. A skin biopsy was performed to confirm the diagnosis. Results : A clinical and histopathological diagnosis of hydradenocarcinoma is made, this being a rare and malignant tumor originating in sweat glands that is located anywhere in the body, which in most cases has been described as of eccrine origin. Conclusions : Although it is not a frequent pathology, it has high recurrence rates and risk of metastasis. Therefore, it is important to always consider it as one of the differential diagnoses, and thus offer timely surgical treatment, with wide resection margins.

Eccrine porocarcinoma from the abdominal wall in an elderly female: a case report

Eccrine porocarcinoma (EPC) is a rare malignancy arising from the sweat gland. It is commonly seen in elderly female patients. There is no characteristic appearance for this malignancy and so making a clinical diagnosis is difficult. The diagnosis is confirmed by histopathological examination (HPE). Authors present a case of a 53-year-old female who presented with an ulceroproliferative lesion on the left side of the abdominal wall. After the lesion was radiologically ascertained to be localized and having a diagnosis of porocarcinoma from wedge biopsy, a wide local excision was done.  The HPE confirmed the diagnosis of EPC.

Clinical characteristics and associations of palmoplantar pustulosis: an observational study

Abstract Background: Palmoplantar pustulosis is a chronic and relapsing disease of the palms and soles, which is characterized by scattered clusters of pinhead-sized, sterile pustules. Objective: The aim of the present study was to determine demographic features, co-morbidities, and relation of palmoplantar pustulosis with psoriasis. Methods: A total of 48 patients (M/F: 15/33) were enrolled in the present study. A detailed history regarding age of onset, palmoplantar pustulosis duration, number of recurrences, personal and family history of psoriasis, accompanying arthritis, sternoclavicular tenderness, dental fillings, smoking status, and autoimmune disease was obtained; thorough dermatological examination was carried out. Patch testing results and laboratory investigations for thyroid autoimmunity were recorded. Results: Thirty-five of 48 patients (72.9%) were current smokers. Twenty of the 48 patients (41.7%) had dental fillings. There was not any significant correlation between palmoplantar pustulosis duration and dental filling duration (p = 0.170). Psoriasis was not detected in any patients either in history or in dermatological examination. Nail involvement and joint complaints were observed in seven of 48 patients (14%) and in nine of 48 patients (18%), respectively. Autoimmune thyroiditis was observed in four of 48 patients (12%). Patients with patch testing positivity (12.5% of patients, M/F: 1/5) had no considerable association for history of external contact with these materials. Study limitations: Retrospective analysis. Conclusion: Palmoplantar pustulosis appears to be a distinct entity from psoriasis. Routine thyroid functions test could be analyzed, but patch testing is not required in patients with palmoplantar pustulosis. Also, patients with palmoplantar pustulosis must be evaluated for musculoskeletal symptoms and signs.

Eccrine Syringofibroadenoma Associated with Bowen's Disease: A Case Report and Review of the Literature

Porocarcinoma ecrino metastásico / Metastatic eccrine porocarcinoma

Presentamos el caso de un paciente varón de 69 años, con lesión tumoral en región inguinal izquierda, asintomática. El estudio histopatológico e inmunohistoquímico fue consistente con porocarcinoma ecrino. A la realización de imágenes se evidenció metástasis ganglionares en región iliaca femoral, retroperitoneal y supraclavicular izquierda. Se confirmó infiltración de porocarcinoma ecrino en ganglio supraclavicular al estudio histopatológico. El porocarcinoma ecrino metastásico es de muy rara presentación. El tratamiento quirúrgico no es posible en estos casos. No existe un tratamiento estándar sistémico, solo series de casos con respuestas variables. Nuestro paciente recibió tratamiento sistémico con docetaxel con buena tolerancia.

We present the case of a 69-year-old male patient with tumor lesion in the left inguinal region, asymptomatic. The histopathological and immunohistochemical study was consistent with ecrine porocarcinoma. Upon imaging, nodal metastases in the iliac femoral, retroperitoneal and supraclavicular left region are evident. Ecrine porocarcinoma infiltration into supraclavicular ganglion is confirmed at histopathological study. Metastatic eccrine porocarcinoma is very rare. Surgical treatment is not possible in these cases. There is no standard systemic treatment, only case series with variable responses. Our patient received chemotherapy treatment with doxetacel with good clinical response to follow-up.

Carcinoma mucinoso primario de piel / Primary mucinous skin carcinoma

El adenocarcinoma mucinoso primario de piel es una neoplasia anexial, maligna e infrecuente, que afecta principalmente a hombres en una relación 2:1 con respecto a las mujeres. Presenta mayor incidencia entre la sexta y séptima década de la vida, y se manifiesta como un tumor único, asintomático, de tamaño variable. La localización más frecuente es la región periorbitaria y el cuero cabelludo. Se comunica el caso de una paciente de 92 años, con diagnóstico de carcinoma mucinoso primario de piel, sin evidencia de enfermedad sistémica ni recurrencia local al año de la resección por cirugía micrográfica de Mohs. Describimos sus principales características clínicas, dermatoscópicas y hallazgos histopatológicos. (AU)

Primary mucinous adenocarcinoma of the skin is an adnexal, malignant, and infrequent neoplasm that mainly affects men with a 2: 1 ratio, with respect to women. It presents higher incidence between the sixth and seventh decade of life, and manifests as a single tumor, asymptomatic, of variable size. The most frequent location is the periorbital region and scalp. We report the case of a 92-year-old patient with a diagnosis of primary mucinous skin carcinoma, without evidence of systemic disease or local recurrence one year after resection by Mohs micrographic surgery. We describe its main clinical features, dermatoscopic and histopathological findings. (AU)