Mycosis fungoides and Sézary syndrome: focus on the current treatment scenario

Abstract Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.

Colgajo bilobulado para reconstrucción de defectos por tumores nasales del tercio inferior / Bilobed flap for reconstruction of tumoral defects on the lower third of the nose

Introducción. Los defectos de cobertura en la región nasal secundarios a resecciones oncológicas deben ser cubiertos con tejido de las mismas características que el resecado, idealmente con tejido vascularizado adyacente. El colgajo bilobulado reúne las condiciones doble transposición, con una estructura y diseño que permite una adecuada distribución de las fuerzas de tensión a lo largo de su eje de rotación, evitando deformidades y excedente cutáneo generados por otros colgajos o cierre primario, respetando las unidades estéticas de la nariz. Método. Se presentan 48 casos clínicos en el periodo abril 2018 - abril 2020 describiendo la técnica de realización del colgajo bilobulado para la reconstrucción de secuelas de resección oncológica nasal por los diferentes tipos de tumores de piel. Para defectos de dorso nasal 26, punta nasal 14 y alares 8 casos. Resultados. La vitalidad de los colgajos fue de un 100%, 6 casos presentaron dehiscencia en extremo distal que requirió revisión. Adecuado resultados estéticos con similitud de textura y color de piel del colgajo. Cicatrices resultantes en área dadora y receptora ocultas en líneas de sombra. Disminución de complicaciones agregando resección triangular adicional. Conclusiones. Los defectos de tegumento en región nasal deben ser cubiertos por tejido vascularizado. Las resecciones de hasta 3 cm de diámetro pueden ser cubiertas con colgajos bilobulados adyacentes. El adecuado planeamiento de las cicatrices tanto en área dadora como receptora permite mejores resultados estéticos. Siempre deben tenerse en cuenta las regiones estéticas para obtener mejores resultados.

Background. Coverage defects in the nasal region secondary to oncological resections should be covered with tissue of the same characteristics as the resected, ideally with adjacent raised vascular tissue. The bilobed flap meets the conditions, is a double transposition flap, with a structure and design that allows an adequate distribution of the tension forces along its axis of rotation, avoiding deformities and excess skin generated by other flaps or primary closure. Respecting the aesthetic units of the nose. Methods. 48 clinical cases are presented in the period April 2010- April 2020 describing the technique of making the bilobed flap for the reconstruction of the sequelae of nasal oncological resection by the different types of skin tumors. For dorsal defects 26, nasal tip 14 and alares 8 cases. Results. The vitality of the flaps was 100%, 6 cases presented dehiscence in the distal end that required revision. Adequate aesthetic results with similarity of texture and skin color of the flap. Resulting scars in donor and recipient area hidden in shadow lines. Decrease complications by adding additional triangular resection. Conclusions. Integument defects in the nasal region should be covered by vascularized tissue. Resections up to 3 cm in diameter can be covered with adjacent bilobed flaps. The adequate planning of the scars in both the donor and recipient areas allows for better aesthetic results. Aesthetic regions should always be taken into account to obtain better results

HeberFERON en el carcinoma basocelular periocular. Serie de casos / HeberFERON in periocular basal cell carcinoma. Case series

RESUMEN Fundamento: El carcinoma basocelular periocular es una lesión tumoral que surge de las células basales de la epidermis y los folículos pilosos, con un alto potencial de destrucción local, pueden ser desfigurantes e invaden el tejido que los rodea dando lugar a deformidades o pérdida de la función del órgano afectado. En orden de aparición es más común en el párpado inferior, el canto medial, el párpado superior y el canto temporal. Objetivo: Describir los resultados de la aplicación del HeberFERON en una serie de casos con carcinoma basocelular periocular que acudieron a consulta de dermatología del Policlínico Centro, de enero de 2017 a diciembre del 2020. Metodología: Se realizó un estudio de serie de casos clínicos con carcinoma basocelular periocular que acudieron a la consulta de dermatología del Policlínico Centro. Se incluyeron 17 casos con diagnóstico clínico, dermatoscópico e histopatológico. Se realizó una evaluación inicial, durante y 16 semanas después del tratamiento; se administró 10.5 UI de HeberFERON 3 veces por semana perilesional e intradérmica hasta completar 9 dosis. Las variables principales fueron la respuesta al tratamiento y la presencia o no de eventos adversos. Resultados: Predominó el sexo masculino, el fototipocutáneo II, la localización en párpado inferior, el subtipo clínico nódulo ulcerativo y el histológico sólido, se logró respuesta completa en la mayoría de los pacientes. Como eventos adversos se presentaron dolor en el sitio de inyección, fiebre, mal estar general, edema y eritema perilesional. Conclusiones: La respuesta al tratamiento fue favorable en la mayoría de los pacientes tratados con HeberFERON.

ABSTRACT Background: Periocular basal cell carcinoma is a tumor lesion arising from the epidermis and hair follicles basal cells, with a high potential local destruction, can be disfiguring and invade the surrounding tissue leading to deformities or loss of function of the affected organ. In order of appearance it is most common in the lower eyelid, medial edge, upper eyelid and temporal edge. Objective: To describe the results of the application of HeberFERON in a case series with periocular basal cell carcinoma who attended dermatology appointment at the Policlínico Centro, from January 2017 to December 2020. Methodology: A series study of clinical cases with periocular basal cell carcinoma who attended the dermatology appointment at the Policlínico Centro was conducted. 17 cases with clinical, dermatoscopic and histopathological diagnosis were included. A baseline evaluation was conducted, during and 16 weeks after treatment; 10.5 IU of HeberFERON was administered 3 times a week perilesional and intradermally until completing 9 doses. The main variables were the treatment response and the presence or absence of adverse events. Results: Male sex, phototypocutaneous II, lower eyelid location, clinical subtype ulcerative nodule and solid histological subtype predominated, complete response was achieved in most patients. Adverse events were pain at the injection site, fever, general malaise, edema and perilesional erythema. Conclusions: Treatment response was favorable in most patients treated with HeberFERON.

Variedades clínicas de micosis fungoide en una cohorte / Clinical variants of mycosis fungoides in a cohort

Resumen Introducción: La micosis fungoide es el linfoma primario de células T en piel más frecuente, con expresividad clínica heterogénea. Objetivo: Reportar las variedades clínicas y las características sociodemográficas de pacientes con micosis fungoide tratados en un hospital dermatológico. Métodos: Se incluyeron 290 pacientes con diagnóstico clínico e histopatológico de micosis fungoide atendidos en el transcurso de 11 años. Se realizó descripción sociodemográfica de los pacientes, quienes se clasificaron conforme las variantes clínicas e histopatológicas. Resultados: 58 % de los casos de micosis fungoide se presentó en mujeres y 42 % en hombres. La variedad clínica más común fue la clásica en 46.2 %; la discrómica representó 35.2 %, del cual la hipopigmentada fue la más representativa (7.6 %); la poiquilodérmica constituyó 4.1 % y la foliculotrópica, 3.1 %. La variedad papular se presentó en seis pacientes (2.1 %), la de placa única en tres (1 %) y la ictiosiforme, siringotrópica y la piel laxa granulomatosa, en un paciente cada una. La variedad granulomatosa se encontró en 0.7 % y 1.4 % presentó eritrodermia. Conclusiones: La variedad clínica más frecuente de micosis fungoide fue la clásica en fase de placa, seguida de las variedades discrómicas. Otras variedades clínicas representaron 18.6 %.

Abstract Introduction: Mycosis fungoides (MF) is the most common primary skin T-cell lymphoma, which is characterized for a heterogeneous clinical expressivity. Objective: To report clinical variants and sociodemographic characteristics in patients with MF under the care of a dermatological hospital. Methods: 290 patients with MF clinical and histopathological diagnosis attended to over the course of 11 years were included. Sociodemographic description of patients was made, who were classified according to clinical and histopathological variants. Results: MF was recorded in 57.9 % of women and 42 % of men. The most common clinical variant was the classic type in 46.2 %; dyschromic variants accounted for 35.2 %, out of which hypopigmented MF was the most representative (17.6 %); poikilodermatous MF accounted for 4.1 %, and folliculotropic, for 3.1%. The papular variant occurred in six patients (2.1 %), the single-plaque variety in three (1%), and the ichthyosiform, syringotropic and granulomatous slack skin varieties occurred in one patient each. The granulomatous variant was found in 0.7 %, and 1.4 % had erythroderma. Conclusions: The most common MF clinical variant was classic plaque stage, followed by dyschromic variants. Other clinical variants accounted for 18.6 %.

Melanoma in Singapore: A 20-year review of disease and treatment outcomes

INTRODUCTION@#Melanomas in Asians have different clinicopathological characteristics and prognosis from melanomas in Caucasians. This study reviewed the epidemiology and treatment outcomes of cutaneous melanoma diagnosed at a tertiary referral dermatology centre in Singapore, which has a multiracial population. The study also determined whether Asians had comparable relapse-free and overall survival periods to Caucasians in Singapore.@*METHOD@#This is a retrospective review of cutaneous melanoma cases in our centre between 1996 and 2015.@*RESULTS@#Sixty-two cases of melanoma were diagnosed in 61 patients: 72.6% occurred in Chinese, 19.4% in Caucasians and 3.2% in Indians, with an over-representation of Caucasians. Superficial spreading melanoma, acral lentiginous melanoma and nodular melanoma comprised 37.1%, 35.5% and 22.6% of the cases, respectively. The median time interval to diagnosis was longer in Asians than Caucasians; median Breslow's thickness in Asians were significantly thicker than in Caucasians (2.6mm versus 0.9mm, @*CONCLUSION@#More physician and patient education on skin cancer awareness is needed in our Asian-predominant population for better outcomes.

Ulcera de Marjolin (carcinoma escamoso) sobre eventración abdominal / Marjolin ulcer (squamous carcinoma) over abdominal hernia

Introducción: Las úlceras de Marjolin son neoplasias cutáneas que asientan sobre pieles afectadas por úlceras crónicas, quemaduras, cicatrices, estasis venosa o heridas cutáneas. Generalmente se diagnostica por medio de biopsias, siendo los carcinomas escamosos la variedad más frecuente. Existe escasa información sobre su desarrollo en heridas quirúrgicas abdominales previas, y aún menos casos reportados de su aparición sobre un defecto de la pared abdominal. Objetivo: Reporte de un caso de un paciente con antecedente de múltiples cirugías abdominales que desarrolló un carcinoma escamoso sobre cicatriz de laparotomía previa. Pacientes y Métodos: Presentación de un de un paciente masculino de 61 años, con antecedente de laparotomía exploradora, que consultó por una tumoración, de 6 meses de evolución, vegetante sobre eventración de mediana suprainfraumbilical. Se realiza exéresis de lesión en bloque de pared con enterectomia, anastomosis primaria y eventroplastia con malla de reemplazo. Cursa internación prolongada y con mala evolución, falleciendo al 77 día postoperatorio. Conclusión: Los carcinomas de células escamosas que se desarrollan sobre lesiones cutáneas previas presentan una agresividad mayor a aquellos desarrollados espontáneamente, tienen alto porcentaje de recidiva y metástasis asociadas. Se propone abordajes quirúrgicos radicales para su tratamiento, aun asi presentando una morbimortalidad elevada

Background: Marjolin ulcers are skin malignancies that appear on skin affected by chronic ulcers, burns, scars, venous stasis or skin wounds. They are generally diagnosed through a biopsy and the most frequent type is the squamous cell carcinoma. There is little information on its development in existing abdominal surgical wounds, and there are even fewer cases reported in relation to its appearance upon an abdominal wall defect. Objective: To report a case of a patient with a history of multiple abdominal surgeries who developed a squamous cell carcinoma in an existing laparotomy scar in association with an incisional hernia. Patient and Methods: A 61-year-old male patient, with history of an exploratory laparotomy in 1986, presents with a 6-month-old vegetating tumor upon a supra-infraumbilical median eventration. An excision of the lesion that included the abdominal wall and an associated enterectomy, primary anastomosis, and eventroplasty with replacement mesh was performed. During his prolonged hospital stay, he underwent with many medical intercurrences and even an additional surgery was needed. Eventually, the patient dies 77 days after the surgery. Conclusion: Squamous cell carcinomas that develop in existing skin lesions tend to be more aggressive than those that develop spontaneously. They have a high percentage of recurrence and associated metastases. Radical surgical approaches are suggested for its treatment, although it has a high morbidity and mortality rate.

Guía de práctica para el manejo de melanoma cutáneo primario de la Sociedad Chilena de Dermatología / Practice guideline for the management of melanoma primary skin cancer of the Chilean Society of Dermatology

Introducción: Desde el año 2011 a la fecha se han realizado grandes avances en el diagnóstico y el tratamiento del melanoma cutáneo. Las diferentes sociedades dermatológicas internacionales han actualizado sus recomendaciones y guías de manejo, tomando en consideración los nuevos avances científicos y resultados de ensayos clínicos. Objetivo: El objetivo de la presente revisión es proponer recomendaciones basadas en la evidencia sobre del rol del dermatólogo en el manejo del melanoma cutáneo en el sistema de salud de Chile. Diseño: Revisión sistemática de la literatura en cinco bases de datos: Pubmed/Medline, Embase, Web of Science, Lilacs/SciELO y Cochrane Library; desde julio del 2016 a julio del 2021. Resultados: 1306 registros fueron identificados y 153 cumplieron los criterios de inclusión. Se exponen recomendaciones actualizadas con respecto al diagnóstico y manejo del melanoma cutáneo en Chile. El panel de expertos presenta un diagrama de flujo del proceso de atención de una lesión sospechosa de melanoma, la participación global del equipo médico encargado del cuidado paciente y el rol del dermatólogo. Conclusión: El rol del dermatólogo es central e indispensable en todas las etapas de atención del melanoma cutáneo.

Introduction: Since 2011 great advances have been made in the diagnosis and treatment of cutaneous melanoma. The different international dermatological societies have updated their recommendations and clinical guidelines taking into account new scientific advances and the results of clinical trials. Objective: The aim of this review is to propose evidence-based recommendations on the role of the dermatologist in the diagnosis and management of cutaneous melanoma in the Chilean healthcare system. Design. Systematic review of the literature in five databases: Pubmed/Medline, Embase, Web of Science, Lilacs/SciELO and Cochrane Library; from July 2016 to July 2021. Results: 1306 records were identified and 153 met the inclusion criteria. Updated recommendations regarding the diagnosis and management of cutaneous melanoma in Chile are presented. The expert panel presents a flowchart of the process of care of a suspected melanoma lesion, the overall involvement of the medical team in charge of patient care, and the role of the dermatologist. Conclusion: The role of the dermatologist is central and critical in all stages of cutaneous melanoma care

Reconstrução de couro cabeludo após ampla ressecção de angiossarcoma / Scalp reconstruction after wide resection of an angiosarcoma

Introdução: O couro cabeludo é uma área de difícil reconstrução devido à sua pouca elasticidade e por sobrepor-se a uma estrutura rígida e convexa. Existem diferentes técnicas cirúrgicas para reparação dos defeitos, que podem ser produto de diversas etiologias, como: traumas, deformidades e consequência de doenças, principalmente oncológicas, como é o caso do paciente apresentada neste trabalho. O angiossarcoma cutâneo é um tumor vascular maligno raro e extremamente agressivo, que afeta principalmente idosos. É caracterizado clinicamente pelo aparecimento de placas eritêmato-violáceas e de rápida evolução. O tratamento depende da extensão da doença. A maioria dos casos são tratados com ampla ressecção cirúrgica e reconstrução. O objetivo é relatar um caso de reconstrução de grande defeito do couro cabeludo depois de uma dissecção oncológica, realizado no Hospital Central do Exército (HCE) - RJ. Métodos: O caso foi tratado com enxerto autólogo e uso de matriz dérmica, em 2 tempos cirúrgicos, até a total cobertura da área lesionada. Resultados: Obteve-se resultado satisfatório após as cirurgias de enxertia de pele na área ressecada. Conclusão: O enxerto autólogo, juntamente a matriz dérmica mostrou-se uma opção viável na reconstrução do couro cabeludo.

Introduction: It is difficult to reconstruct the scalp due to its poor elasticity and presence of layers over a rigid convex structure. Different surgical techniques are used to repair defects that may develop due to several etiologies, such as trauma, deformities, and disease sequelae, especially cancer, as noted in the present case. Cutaneous angiosarcoma, a rare and extremely aggressive malignant vascular tumor that mainly develops in elderly individuals, is clinically characterized by the onset of rapidly evolving erythematous purple plaques. The treatment depends on disease extent. Most patients are treated with wide surgical resection and reconstruction. The objective is to report a case of reconstruction of a major scalp defect after an oncologic dissection performed at the Hospital Central do Exército in Rio de Janeiro. Methods: An autologous graft and dermal matrices were applied during two surgical periods till the damaged area was fully covered. Results: Satisfactory results were obtained after performing skin grafting surgery in the resected area. Conclusion: The autologous graft and dermal matrix proved to be a viable option for scalp reconstruction.

Dermatofibrosarcoma protuberans: lo diagnosticamos en la edad pediátrica / Dermatofibrosarcoma protuberans: we diagnose it in the pediatric age

El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo, de baja frecuencia, fibrohistiocítico, infiltrante, de lento crecimiento, de agresividad local, de malignidad intermedia; con escasas probabilidades de metástasis pero con alto índice de recurrencia local. El diagnóstico debe sospecharse y confirmarse con histología e inmunohistoquímica. El tratamiento de elección es con cirugía convencional y/o cirugía micrográfica de Mohs, con márgenes de 2-4 cm. Se considera que la prevalencia del DFSP en la edad pediátrica es baja, debido al escaso índice de sospecha. En el presente trabajo compartimos cinco casos de DFSP en la edad pediátrica, estudiados en el Hospital General de Niños Pedro de Elizalde. (AU)

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous, low frequency, fibrohistiocytic, infiltrating, slow growing, local aggressiveness, intermediate malignancy tumor; with little chance of metastasis but with a high rate of local recurrence. The diagnosis should be suspected and confirmed with histology and immunohistochemistry. The treatment of choice is with conventional surgery and / or Mohs micrographic surgery, with margins of 2-4 cm. The prevalence of DFSP in pediatric age is considered to be low, due to the low index of suspicion. In this paper we share five cases of DFSP in the pediatric age, studied at the Pedro de Elizalde Children's General Hospital. (AU)

Differences in the diagnosis of primary cutaneous melanoma in the public and private healthcare systems in Joinville, Santa Catarina State, Brazil

Abstract: Background: Cutaneous melanoma accounts for up to 80% of deaths caused by skin cancer. Diagnostic suspicion and access to medical care and early intervention in suspected cases is vital to the patient's prognosis. Objectives: To compare demographic and histopathological characteristics of primary cutaneous melanoma diagnosed in the public healthcare system (Sistema Único de Saúde SUS) and the private system in Joinville, Santa Catarina State, Brazil. Methods: This cross-sectional retrospective study analyzed primary cutaneous melanoma cases recorded from 2003 to 2014 in the resident population of Joinville. Ethical approval was obtained from the local Research Ethics Committee. Results: 893 cases of primary cutaneous melanoma were identified. Patients in the private system were mostly younger, while there were more elderly patients in the public healthcare system (p <0.001). There was no statistically significant association between type of care (public/private) and gender or presence of multiple primary cutaneous melanomas. Histological diagnosis of superficial spreading melanoma was more common in patients treated in private healthcare, while nodular melanoma was more frequent in patients in the public healthcare system (p <0.001). Mean Breslow depth in patients treated in private healthcare was 1.35mm, compared to 2.72mm in the public system (p <0.001). Study limitations: This was a retrospective study using secondary databases. Conclusions: thin cutaneous melanoma (in situ cutaneous melanoma and Breslow T1) showed the strongest association with the private healthcare system, while thick cutaneous melanoma was more frequent in the public system (Breslow category T3 and T4) (p <0.001).

The efficacy and safety of phototherapy in geriatric patients: a retrospective study

Abstract: Background: While phototherapy is a well-established treatment for many dermatoses, data from the literature regarding its use in elderly patients are quite limited. Objective: In this study, we aimed to determine the phototherapy indications in geriatric patients and to evaluate the effectiveness and reliability of phototherapy in this group. Methods: This study included 95 patients of 65 years of age and older who were treated in our phototherapy unit between 2006 and 2015. The data for this study were collected retrospectively from patient follow-up forms in the phototherapy unit. Results: Phototherapy was administered to 28 (29.5%) patients for mycosis fungoides, 25 (26.3%) patients foplaque type psoriasis, 12 (12.6%) patients for palmoplantar psoriasis, 12 (12.6%) patients for generalized pruritus, and 18 (19%) for other dermatoses. Of the patients, 64.2% had received a narrowband UVB (NB-UVB), 21.1% oral psoralen UVA (PUVA), and 14.7% local PUVA treatment. A complete response was achieved in 76.9-85.7% of the mycosis fungoides and in 73.71-100% of the psoriasis vulgaris patients treated with NB-UVB and PUVA, respectively. All the patients with generalized pruritus were treated with NB-UVB, and 80% of these patients achieved significant improvement. The erythema rate was found to be 0.43% per session for NB-UVB treatment and 0.46% per session for PUVA treatment as a side effect. Study limitations: The limitations of our study are that it was retrospective and the remission durations of the patients are not known. Conclusion: This study showed that phototherapy is effective and reliable in the elderly population with proper dose increases and close follow-up.

Expansão prévia no tratamento de dermatofibrossarcoma protuberante envolvendo o osso frontal / Preceding expansion in the treatment of dermatofibrosarcoma protuberans involving the frontal bone

Introdução: Dermatofibrossarcoma protuberante é um tumor cutâneo raro de malignidade intermediária e potencial metastático baixo, apresentando, entretanto, alta taxa de recorrência após tratamento cirúrgico. O tratamento cirúrgico clássico é a ressecção alargada com margens variadas. Métodos: Os pacientes foram submetidos à expansão prévia dos tecidos da região frontal utilizando dois expansores convencionais. Margens laterais de 3 cm de tecido de aparência normal foram tatuadas com tinta nanquim. Os expansores foram incluídos em uma cirurgia anterior, através de incisões cutâneas distantes dessas marcas, em um plano cirúrgico logo acima do periósteo, preservando a área de ressecção alargada previamente delimitada. Os tecidos laterais foram expandidos e, posteriormente, os pacientes foram submetidos à ressecção do tumor. A margem profunda incluiu da lâmina externa do osso frontal. O período médio de expansão foi de 45 dias. Resultados: Foram relatados três casos de dermatofibrossarcoma protuberante da região frontal com envolvimento da lâmina externa do osso frontal. Os retalhos expandidos fecharam facilmente os defeitos. O acompanhamento pós-operatório médio foi de 194 meses, sem nenhuma recorrência tumoral. Conclusão: A expansão tecidual prévia da região frontal no tratamento do dermatofibrossarcoma protuberante, invadindo o osso frontal, usando margens laterais de 3 cm com remoção da lâmina externa, permitiu facilmente o fechamento do defeito, em três pacientes. O tempo de acompanhamento médio de 194 meses sem nenhuma recorrência tumoral mostrou a eficiência do método.

Introduction: Dermatofibrosarcoma protuberans is a rare skin tumor with intermediate malignancy and low metastatic potential, but a high recurrence rate after surgical treatment. Treatment involves extended resection with variable margins. Methods: Patients were managed with preceding expansion of tissues of the forehead using two conventional expanders. The lateral margins of normal-appearing tissue 3-cm apart were tattooed with China ink. In a preceding operation, expanders were inserted through skin incisions at a distance from the marks, by undermining the surgical plane just above the periosteum, while preserving the area of wide resection previously delimited. After the lateral tissues were expanded, the patients underwent surgical resection. The deep margin included the outer table of the frontal bone. The expansion period averaged 45 days. Results: Three cases of dermatofibrosarcoma protuberans of the forehead with involvement of the outer table of the frontal bone are described. The expanded flaps easily closed the defects. The average follow-up was 194 months without tumor recurrence. Conclusion: Preceding expansion of forehead tissue in the treatment of dermatofibrosarcoma protuberans with invasion of frontal bone, using 3-cm lateral margins and removal of the outer table of the frontal bone, allowed easy closure of the defect in 3 different patients. Average follow-up of 194 months with no recurrence of the lesion showed the effectiveness of the method.

Carcinoma basocelular metastásico / Metastatic basal cell carcinoma

El carcinoma basocelular es la neoplasia con mayor incidencia a nivel mundial. Es de crecimiento lento y localmente agresiva pero posee un potencial metastásico extremadamente bajo, que se estima entre 0,0028% y 0,5%. El objetivo de este trabajo es mostrar la experiencia de una institución con el manejo de esta enfermedad metastásica. Estudio descriptivo retrospectivo de los pacientes con carcinoma basocelular metastásico tratados en el Instituto Nacional del Cáncer, entre julio 2004 y julio 2015. Se realizó una revisión de la literatura. Se evidenciaron 3 casos con esta enfermedad poco habitual en un plazo de 11 años. Un paciente falleció a los 42 meses de seguimiento. Hay 2 pacientes aún en control, uno de ellos lleva 29 meses de seguimiento y actualmente tiene enfermedad metastásica pulmonar, y el último paciente lleva 92 meses de seguimiento libre de enfermedad. El carcinoma basocelular metastásico, es una entidad poco frecuente por lo que todos los que nos vemos involucrado en su manejo debemos mantenernos alerta para una pesquisa oportuna. La cirugía asociada a la radioterapia siguen siendo los pilares del tratamiento. Esta patología debiera manejarse en centros de referencia a nivel nacional.

Basal cell carcinoma is the most frequent neoplasm worldwide. It's a slow growing and locally aggressive tumor, but it has a metastatic potential estimated between 0,0028 and 0,5%. The purpose of this study is to show the experience of a center in the management of this metastatic disease. Descriptive retrospective study of patients with metastatic basal cell carcinoma treated at the Instituto nacional del cancer from July2004 to July 2015. A revision of the literature was also made. 3 cases with this unusual disease during an 11 years period. The first one died at 42 months of follow up. There are 2 patients on follow up, 1 of them has lung metastatic disease after 29 months of follow up, and the last one is free of disease with 92 months of follow up. Metastatic basal cell carcinoma is an unusual entity, thus everyone that is involved in it's treatment, must be aware of this in order to do an early diagnosis. Surgery associated to radiotherapy are the mainstays of treatment. This disease should be managed on national referral centers.

Brazilian guidelines for diagnosis, treatment and follow-up of primary cutaneous melanoma - Part II

Abstract The last Brazilian guidelines on melanoma were published in 2002. Development in diagnosis and treatment made updating necessary. The coordinators elaborated ten clinical questions, based on PICO system. A Medline search, according to specific MeSH terms for each of the 10 questions was performed and articles selected were classified from A to D according to level of scientific evidence. Based on the results, recommendations were defined and classified according to scientific strength. The present Guidelines were divided in two parts for editorial and publication reasons. In this second part, the following clinical questions were answered: 1) which patients with primary cutaneous melanoma benefit from sentinel lymph node biopsy? 2) Follow-up with body mapping is indicated for which patients? 3) Is preventive excision of acral nevi beneficious to patients? 4) Is preventive excision of giant congenital nevi beneficious to patients? 5) How should stages 0 and I primary cutaneous melanoma patients be followed?.

Tratamento de hemangioma da infância com maleato de timolol tópico / Treatment of infantile hemangioma with topical timolol maleate

Introdução: o hemangioma da infância é uma proliferação neoplásica benigna de células endoteliais, decorrente de um desequilíbrio na angiogênese. Atinge 10 a 12% das crianças com menos de ano de vida, sendo, portanto, o tumor mais comum da infância. O tratamento, geralmente, é expectante, mas são utilizados, em alguns casos, propranolol oral, corticosteroides, interferon alfa-2a, laserterapia, embolização, imunomoduladores e cirurgia. Descrição do caso: criança do sexo masculino com hemangioma em couro cabeludo de 3 cm de diâmetro. Utilizou-se maleato de timolol 0,5%, solução oftálmica, três gotas duas vezes ao dia na superfície da lesão, durante dois anos. Após um ano de tratamento, a lesão apresentava-se praticamente plana, com involução quase completa e alopecia residual. Nenhum efeito colateral foi observado durante o período de tratamento. Discussão: o hemangioma da infância possui amplo espectro clínico e graus variados de gravidade, o que torna o seu manejo difícil e controverso. Diante do alto número de efeitos colaterais descritos para os tratamentos convencionais, o timolol tópico tem se tornado excelente alternativa para os casos não complicados. Conclusão: o timolol tópico vem sendo uma nova opção terapêutica eficaz e desprovida de efeitos colaterais para o tratamento do hemangioma da infância, sendo capaz de acelerar sua involução e prevenir complicações. Entretanto, é necessário que sejam realizados estudos que padronizem a dosagem terapêutica mais segura e avaliem o real risco x benefício do uso do fármaco no tratamento desse tipo de tumor.(AU)

Introduction: Infantile hemangioma is a benign neoplastic proliferation of endothelial cells, resulting from an imbalance in angiogenesis. Reaches 10-12% of children under one year old, therefore it is the most commom tumor of childhood. Treatment is usually expectant, but in some cases can be used oral propranolol, corticosteroids, interferon alpha-2a, laser therapy, embolization, immunomodulators and surgery. Case report: Male child with scalp hemangioma of 3 cm diameter. It was used eye drop solution of timolol maleate 0.5%, three drops, twice daily, on the surface of the lesion, during two years. After one year, it was almost plane and completely involuted with residual alopecia. No side effects were observed during the treatment. Discussion: Infantile hemangioma has a wide spectrum of clinical presentation and varying degrees of severity, which make it difficult and controversial management. Due to the high number of side effects reported for conventional treatments, topical timolol has become an excellent alternative for uncomplicated cases. Conclusion: Topical timolol has been an effective therapeutic option devoid of side effects for treatment of infantile hemangioma, being able to accelerate tumors involution and to prevent complications. Still, it is necessary studies to standardize the safest therapeutic dose and evaluate the actual risk-benefit ratio of timolol use in treatment of this tumor.(AU)

Cicatrização por segunda intenção no tratamento de carcinoma basocelular nasal / Second intention healing in the treatment of nasal basal cell carcinoma

INTRODUÇÃO: A busca por melhores alternativas para o tratamento do carcinoma basocelular (CBC) nasal é referida por diversos autores há anos. Os enxertos e os retalhos cutâneos apresentam problemas, como o risco de não integração ou de necroses; e ainda determinam a formação de cicatrizes que, muitas vezes, são extensas ou inestéticas. Além disso, eles demandam um tempo operatório maior, sendo realizados em centro cirúrgico. A exérese simples do CBC nasal, seguida da cicatrização por segunda intenção e de cuidados cosmiátricos simples no pós-operatório, ressurge como alternativa válida para o tratamento destes tumores. MÉTODOS: Os autores vêm apresentar sua experiência com a técnica, discutindo as vantagens do método. No período de 4 anos, todos os pacientes submetidos à técnica foram acompanhados prospectivamente. RESULTADOS: Dos 23 pacientes operados, 12 foram acompanhados por 2 anos e incluídos na avaliação. O procedimento cirúrgico teve duração média de 21 minutos (+6) e foi realizado sem dificuldades. A evolução pós-operatória se deu sem intercorrências ou recidivas tumorais. CONCLUSÃO: A técnica empregada coloca-se como uma alternativa simples, segura e com excelentes resultados para o tratamento dos CBC nasais.

INTRODUCTION: The search for better alternatives for the treatment of nasal basal cell carcinoma (BCC) has been reported by several authors. The complications associated with grafts and skin flaps include the risk of non-integration or necrosis, and the formation of scars that are often large or unaesthetic. In addition, these procedures require longer operative times, which implies the need for a surgical center. The simple removal of nasal BCC, followed by second intention healing and simple postoperative cosmetic care, has reemerged as a valid alternative for the treatment of these tumors. METHODS: In this study, the authors describe their experience with the technique, and report the advantages of the method. All patients submitted to this procedure were followed-up prospectively for a period of 4 years. RESULTS: Of 23 patients, 12 were monitored for 2 years and were included in the study. The surgical procedure was performed without difficulty in an average of 21 minutes (+6). Postoperative evolution was uncomplicated and without tumor recurrence. CONCLUSION: This technique is a simple and safe alternative, providing excellent results for the treatment of nasal BCC.

Versatility of the mid-forehead flap in facial reconstruction / Versatilidade do retalho médio-frontal nas reconstruções faciais

Introduction: Reconstruction of complex facial defects is a challenge to the plastic surgeon. Different missing anatomical units must be accessed in different ways and with individualized goals, always tailoring the options to the patient's needs. The objective is to examine the role of the mid-forehead flap in the reconstruction of different anatomical facial units. Methods: Retrospective analysis of patients who were operated on by the author from February 2010 to June 2015. Patients were evaluated according to age, sex, lesion etiology, defect location, number of operations performed per patient, and postoperative complications. Results: Fifteen patients (mean age, 69 years) underwent facial reconstruction with a mid-forehead flap. Thirteen patients required more than one operation for pedicle refinement and transection. There was one case of partial necrosis of the flap in the columella region, with satisfactory healing by second intention. There was no infection or hematoma. All secondary cartilage grafts showed integration into the recipient bed. Conclusions: The mid-forehead flap remains an important tool for the reconstruction of major facial defects. It allows the transfer of frontal tissue in an efficient and reliable way with minimal deformity in the donor area, resulting in an esthetically acceptable reconstruction.

Introdução: A reconstrução de defeitos complexos faciais é um desafio ao cirurgião plástico. Diferentes unidades anatômicas ausentes devem ser acessadas de maneiras distintas e com objetivos próprios, sempre adequando as possibilidades ao paciente em questão. O objetivo é mostrar o papel do retalho médio-frontal na reconstrução de diferentes unidades anatômicas faciais. Métodos: Análise retrospectiva de pacientes operadas pela autora, no período de fevereiro de 2010 a junho de 2015. Os pacientes foram avaliados em relação à idade, sexo, etiologia da lesão, localização do defeito, número de tempos cirúrgicos realizados por paciente e complicações pós-operatórias. Resultados: Quinze pacientes foram submetidos à reconstrução facial com retalho médiofrontal, com média de idade de 69 anos. Treze pacientes necessitaram mais de um tempo cirúrgico para refinamento e transecção do pedículo. Houve um caso de necrose parcial do retalho na região da columela, com cicatrização satisfatória por segunda intenção. Não houve infecção ou hematoma. Todos os enxertos cartilaginosos secundários se integraram ao leito receptor. Conclusões: O retalho médio-frontal permanece como importante ferramenta na reconstrução de grandes defeitos faciais. Permite a transferência de tecido frontal de forma eficiente e confiável com mínima deformidade na área doadora, possibilitando uma reconstrução esteticamente aceitável.

Clinical and laboratorial evaluation of dogs with cutaneous lymphoma treated with lomustine / Avaliação clínica e laboratorial de cães com linfoma cutâneo tratados com lomustina

The aim of this prospective study was to evaluate the clinical response of dogs with cutaneous lymphoma treated with lomustine (CCNU) and to identify possible adverse effects and toxicity during treatment. Fifteen dogs, seven females and eight males aged between five and 17 years old, diagnosed with cutaneous lymphoma by histopathological analysis were selected and treated with lomustine at 90 mg/m² every three weeks. Monitoring was carried out and consisted of the assessment of laboratory hematology and serum chemistry before and during treatment. Partial response was observed in 53.3% of the animals. None of the animals achieved a complete response and seven dogs (46.6%) had progressive disease. The median survival time was 22 days. The major hematological and biochemical changes found after therapy were leukopenia (73.3%), thrombocytopenia (60%) and anemia (46.1%). Renal and liver toxicity was observed in 40% and 73.3% of dogs, respectively. Hematocrit, total protein, leukocyte count, neutrophil count, serum creatinine, ALT, GGT, alkaline phosphatase and urine specific gravity were affected during therapy. The use of lomustine as a monotherapy in the treatment of canine cutaneous lymphoma was effective; however, adverse effects occurred and compromised the quality of life of the majority of dogs in this study. Therefore, lower doses of lomustine should be considered in future studies...

O objetivo deste estudo prospectivo foi avaliar a resposta clínica de cães com linfoma cutâneo tratados com lomustina (CCNU) e identificar possíveis efeitos adversos e toxicidade durante o tratamento. Quinze cães, sendo 7 fêmeas e 8 machos, com idades entre 5 e 17 anos diagnosticados com linfoma cutâneo por avaliação histopatológica foram selecionados e tratados com lomustina na dose de 90 mg/m2 a cada três semanas. Os cães foram monitorados por avaliação hematológica e bioquímica sérica antes e durante o tratamento. A resposta parcial foi observada em 53,3% dos animais. Nenhum dos animais apresentou resposta completa e sete animais (46,6%) apresentaram progressão da doença. O tempo médio de sobrevida foi de 22 dias. As principais alterações hematológicas e bioquímicas observadas após o tratamento foram leucopenia (73,3%), trombocitopenia (60%) e anemia (46,1%). Sinais de toxicidade renal e hepática foram observados em 40% e 73,3% dos cães, respectivamente. Durante o tratamento foram afetados os parâmetros hematócrito, proteínas séricas totais, contagem de leucócitos, contagem de neutrófilos, creatinina sérica, ALT, GGT, fosfatase alcalina e densidade urinária. O uso de lomustina como monoterapia no tratamento do linfoma cutâneo canino foi efetivo; entretanto, efeitos adversos ocorreram e comprometeram a qualidade de vida da maioria dos animais neste estudo. Assim, sugere-se que doses mais baixas de lomustina sejam consideradas em estudos futuros...

Guidelines of the Brazilian Dermatology Society for diagnosis, treatment and follow up of primary cutaneous melanoma - Part I

Abstract: The last Brazilian guidelines on melanoma were published in 2002. Development in diagnosis and treatment made updating necessary. The coordinators elaborated ten clinical questions, based on PICO system. A Medline search, according to specific MeSH terms for each of the 10 questions was performed and articles selected were classified from A to D according to level of scientific evidence. Based on the results, recommendations were defined and classified according to scientific strength. The present Guidelines were divided in two parts for editorial and publication reasons. In the first part, the following clinical questions were answered: 1) The use of dermoscopy for diagnosis of primary cutaneous melanoma brings benefits for patients when compared with clinical examination? 2) Does dermoscopy favor diagnosis of nail apparatus melanoma? 3) Is there a prognostic difference when incisional or excisional biopsies are used? 4) Does revision by a pathologist trained in melanoma contribute to diagnosis and treatment of primary cutaneous melanoma? What margins should be used to treat lentigo maligna melanoma and melanoma in situ?.

Tratamento cirúrgico do câncer de pele pelo cirurgião plástico / Surgical treatment of skin cancer by the plastic surgeon

Introdução: O câncer de pele é a neoplasia mais frequente no Brasil e corresponde a 25% de todos os tumores malignos. O melhor tratamento é a ressecção cirúrgica em fases iniciais. O cirurgião plástico, juntamente com uma equipe multiprofissional, participa no tratamento desta doença. O objetivo é analisar a eficácia dos procedimentos cirúrgicos realizados pelo cirurgião plástico no tratamento do câncer de pele. Método: Foram analisados 404 prontuários de pacientes operados no período de fevereiro de 2009 a dezembro de 2012 e analisados gênero, idade, diagnóstico, localização e evolução. Resultados: Faixa etária com média de 62 anos. 53% de casos de carcinoma basocelular, 25,5% melanoma e 15,1% carcinoma espinocelular. Houve predomínio de mulheres nos carcinomas basocelulares (56%) e no melanoma (54%) e de homens nos carcinomas espinocelulares (61%). Os carcinomas basocelulares (92,99%) e espinocelulares (72,13%) tiveram sua predominância na região de cabeça e pescoço; enquanto o melanoma predominou em região de tronco (36,89%) e membros inferiores (24,27%). Foram realizadas 67 cirurgias com pesquisa de linfonodo sentinela, com positividade em 14,93%. 7,76% dos pacientes de melanoma apresentaram metástases e 2,91% vieram a óbito. Conclusão: O cirurgião plástico é um dos profissionais importantes para o tratamento do câncer de pele, sendo o mais apto para realizar as reconstruções após as ressecções tumorais, pois tem em sua formação os conceitos de reparação, utilizando-se de enxertos e retalhos e considerando o aspecto estético dos pacientes. Cabe-lhe também a realização da cirurgia de pesquisa de linfonodo sentinela e o seguimento dos pacientes com câncer de pele.

Introduction: Skin cancer is the most common neoplasm in Brazil and it corresponds to 25% of all diagnosed malignant tumors. The best treatment is surgical resection in early stages. The disease is treated by a plastic surgeon along with a multidisciplinary team. The objective is to assess the effectiveness of surgical procedures performed by plastic surgeons to treat skin cancer. Methods: We analyzed medical records of 404 patients operated on between February 2009 and December 2012. Data analyzed included gender, age, diagnosis, localization and evolution. Results: Patients' mean age was 62 years. A total of 53% of patients had basal cell carcinoma, 25.5% melanoma and 15.1% squamous cell carcinoma. There was a predominance of women in basal cell carcinomas (56%) and melanoma (54%) and predominance of men in squamous cell carcinomas (61%). Basal cell carcinomas (92.99%) and squamous (72.13%) were predominant in the head and neck, melanoma predominated in the trunk region (36.89%) and in lower limbs (24.27%). We performed 67 surgeries with sentinel lymph node, with positivity in 14.93%. Patients with melanoma who had metastasis accounted for 7.76% and 2.91% patients died. Conclusion: Plastic surgeons are one of the important professionals for skin cancer treatment. These professionals are the most skilled one to perform reconstructions after tumor resections, because during education they learn repair concepts using grafts and flaps especially focused on aesthetic appearance of patients. They can also perform surgery for sentinel lymph node and offer follow-up to patients with skin cancer.