Characterization of Surgical Procedures in the Spanish Mohs Surgery Registry (REGESMOHS) for 2013-2015. / Descripción de las intervenciones quirúrgicas recogidas en el registro español de cirugía de Mohs (REGESMOHS) (2013-2015).

INTRODUCTION: The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. MATERIAL AND METHODS: Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications. RESULTS: Data were available for 1796 operations. The most common tumor treated by MMS was basal cell carcinoma (85.96%), followed by squamous cell carcinoma (6.18%), lentigo maligna (2.81%), and dermatofibrosarcoma protuberans (1.97%). Primary tumors accounted for 66.9% of all tumors operated on; 19.2% of tumors were recurrent and 13.9% were persistent. The most common previous treatment was surgical. MMS was mostly performed under local anesthesia (86.7% of cases) and as an outpatient procedure (71.8%). The frozen section technique was used in 89.5% of cases. One stage was needed to achieve tumor-free margins in 56.45% of patients; 2 stages were required in 32.1% of patients, 3 in 7.1%%, 4 in 2.7%, and 5 or more in 1.8%. The defect was reconstructed by the dermatologist in 98% of patients and the most common technique was flap closure (47.2%). Intraoperative complications were recorded for just 1.62% of patients and the median (interquartile range) duration of surgery was 75 (60-100) minutes. CONCLUSION: The characteristics of the patients and tumors treated by MMS are similar to those reported for similar studies in other geographic areas. Lentigo maligna and dermatofibrosarcoma protuberans accounted for a higher proportion of cases in our series, and repair of the surgical defect by a dermatologist was also more common. Operating times in MMS are not much longer than those reported for other procedures and the rate of intraoperative complications is very low.

Description of Patients Undergoing Mohs Surgery in Spain: Initial Report on Data From the Spanish Registry of Mohs Surgery (REGESMOHS).

INTRODUCTION: The Spanish registry of Mohs micrographic surgery started collecting data in July 2013. The aim of the registry is to report on the use of this technique in Spain and the outcomes achieved. In the present article, we describe the characteristics of patients and the tumors treated. MATERIAL AND METHODS: This is a prospective cohort study of patients treated with Mohs micrographic surgery. The participating centers are hospitals where at least one intervention of this type is performed each week. All patients considered for Mohs micrographic surgery in participating centers are included in the registry except those who have been declared legally incompetent. RESULTS: Between July 2013 and October 2014, data from 655 patients were included in the registry. The most common tumor involved was basal cell carcinoma, and the most common histological subtype was infiltrative basal cell carcinoma. Most of the tumors treated were located on the face or scalp, and the most common site was the nose. Almost 40% of the tumors treated were recurrent or persistent, and preoperative tumor size was similar to that reported in other European studies and in Australia. In total, 45.5% of patients had received previous surgical treatment. CONCLUSION: The findings are similar to those reported in other studies, and the data collected are useful for assessing whether the results of studies carried out elsewhere are applicable in Spain.

Spanish adaptation of the European guidelines for the evaluation and treatment of actinic keratosis.

Current trends in our setting indicate that the prevalence of actinic keratosis and similar diseases will increase in coming years and impose a greater burden on health care resources. A long list of clinical features must be taken into account when approaching the treatment of actinic keratosis. Until recently, therapeutic approaches focused solely on ablative procedures and the treatment of individual lesions and did not take into account areas of field cancerization. Now that the therapeutic arsenal has grown, standardized criteria are needed to guide the optimal choice of treatment for each patient. The elaboration of evidence-based consensus recommendations for the diagnosis and treatment of actinic keratosis generates knowledge that will help clinicians to deliver the highest level of care possible, standardizing decision-making processes and enhancing awareness among all the health professionals involved in the care pathway.

Cutaneous squamous cell carcinoma: defining the high-risk variant.

With a lifetime incidence of approximately 10% in the general population, cutaneous squamous cell carcinoma (CSCC) is the second most common type of nonmelanoma skin cancer. Most CSCCs are benign and can be completely eradicated by surgery or other dermatological procedures. There is, however, a subgroup associated with an increased likelihood of lymph node metastases and, therefore, with high morbidity and mortality. This article analyzes the various factors that define aggressive CSCC. We propose a method for defining high-risk SCC on the basis of a series of major and minor criteria. This method will allow better prognostic evaluation and enable personalized management of patients with high-risk SCC, possibly leading to improved overall survival.

Biosynthetic porcine collagen dressings as an adjunct or definitive tool for the closure of scalp defects without periosteum.

BACKGROUND: Aggressive carcinomas of the scalp usually occur in elderly patients with multiple comorbidities. Complete excision of this type of tumor often involves the removal of periosteum, and the resulting defects can be difficult to reconstruct. OBJECTIVE: To evaluate the usefulness of porcine type I collagen dressings as adjunct or definitive treatment in the surgical closure of scalp defects without periosteum. MATERIALS AND METHODS: We performed a prospective study between January 2009 and November 2011 of patients with scalp defects larger than 5cm resulting from surgery that required the removal of periosteum to obtain tumor-free margins. RESULTS: The most prevalent type of tumor was recurrent cutaneous squamous cell carcinoma. The surgical defects ranged in diameter from 5 to 7cm. In 100% of the patients who received a graft after dressing removal (n=4), the graft took well. In the patients in whom the biosynthetic dressing was definitive (n=6), granulation tissue filled the defect and complete closure was achieved in approximately 3.5 months. CONCLUSIONS: The use of porcine type I collagen dressings as an adjunct or definitive tool for the closure of surgical defects on the scalp measuring more than 5cm in which periosteum has been removed proved to be simple, inexpensive, and effective.

Biosynthetic Porcine Collagen Dressings as an Adjunct or Definitive Tool for the Closure of Scalp Defects Without Periosteum. / Parche biosintético de colágeno porcino como herramienta coadyuvante o definitiva en el cierre de defectos del cuero cabelludo sin periostio.

BACKGROUND: Aggressive carcinomas of the scalp usually occur in elderly patients with multiple comorbidities. Complete excision of this type of tumor often involves the removal of periosteum, and the resulting defects can be difficult to reconstruct. OBJECTIVE: To evaluate the usefulness of porcine type I collagen dressings as adjunct or definitive treatment in the surgical closure of scalp defects without periosteum. MATERIALS AND METHODS: We performed a prospective study between January 2009 and November 2011 of patients with scalp defects larger than 5cm resulting from surgery that required the removal of periosteum to obtain tumor-free margins. RESULTS: The most prevalent type of tumor was recurrent cutaneous squamous cell carcinoma. The surgical defects ranged in diameter from 5 to 7cm. In 100% of the patients who received a graft after dressing removal (n=4), the graft took well. In the patients in whom the biosynthetic dressing was definitive (n=6), granulation tissue filled the defect and complete closure was achieved in approximately 3.5 months. CONCLUSIONS: The use of porcine type I collagen dressings as an adjunct or definitive tool for the closure of surgical defects on the scalp measuring more than 5cm in which periosteum has been removed proved to be simple, inexpensive, and effective.

[Intralesional infusion of methotrexate as neoadjuvant therapy improves the cosmetic and functional results of surgery to treat keratoacanthoma: results of a randomized trial]. / Ensayo clínico: la infiltración intralesional con metotrexato de forma neoadyuvante en la cirugía del queratoacantoma permite obtener mejores resultados estéticos y funcionales.

BACKGROUND: Keratoacanthoma is currently considered to be an in situ squamous cell carcinoma that mainly affects patients over 70 years of age. The tumor has a good prognosis and, in some cases, can resolve spontaneously. Treatment involves simple excision. However, since the tumors generally occur on the face or extremities and display rapid growth, aggressive surgery may be required and the cosmetic results may be poor. OBJECTIVE: The primary study objective was assessment of the efficacy of presurgical intralesional methotrexate infiltration to reduce the size of the tumor and the corresponding surgical defect. MATERIAL AND METHODS: A prospective, randomized study was undertaken in patients with a diagnosis of keratoacanthoma of at least 1.5 cm who were seen in our service between January 2009 and January 2010. Two groups were established: one receiving a single infiltration of methotrexate prior to surgery and another that did not receive methotrexate. RESULTS: Of the 25 patients included in the study, 10 received neoadjuvant intralesional methotrexate (group A) and 15 underwent surgery without prior infiltration of methotrexate (group B). The patients in group A displayed a reduction of between 50% and 80% in the size of the lesion prior to surgery. No complications were observed either in relation to methotrexate infusion or surgery. In group B, only 1 patient had a slight reduction in the dimensions of the lesion prior to surgery. In the remaining cases, the lesions remained similar (4 cases, 26%) or had increased in size (10 cases, 66%) at the time of surgery. Five patients in this group required hospital admission following surgery. CONCLUSIONS: Neoadjuvant intralesional methotrexate is well tolerated and reduces the need for aggressive surgery in elderly patients with keratoacanthoma measuring more than 1.5 cm on the face or extremities.

[Acquired progressive lymphangioma (benign lymphangioendothelioma)]. / Linfangioma progresivo adquirido (linfangioendotelioma benigno).

Acquired progressive lymphangioma is a rare vascular tumor with a locally aggressive behavior. Histologically it is characterized by a proliferation of numerous, dilated, thin-walled vessels lined by flat endothelial cells with no nuclear atypia. The vessels appear to dissect between the collagen fibers. It usually presents as an asymptomatic, slow-growing, reddish-brown plaque. We present the case of a 32-year-old man with acquired progressive lymphangioma. The tumor was in the hypogastric region and had arisen on a congenital vascular lesion previously diagnosed as multifocal cutaneous angiomatosis. It was very painful and impeded walking, for which reason it was decided to perform excision. Dermatologists and pathologists must be aware of acquired progressive lymphangioma as early surgical treatment, while the lesion is still small, is curative and prevents subsequent complications due to growth.

Description of patients excluded for Mohs surgery after pre-surgical evaluation: data from the Regesmohs Spanish registry. / Descripción de pacientes no aptos para ser sometidos a cirugía de Mohs tras valoración prequirúrgica: datos del registro español Regesmohs.

BACKGROUND: Regesmohs registry is a nationwide registry including patients evaluated for Mohs surgery in 17 Spanish centres since July 2013. Given that Mohs surgery is the therapy with best results for high risk basal cell carcinoma (BCC) and other skin tumours, we wanted to describe the reasons that lead to some patients being excluded from this therapy and the alternative treatments that they received. These data may be useful to avoid excluding patients for Mohs surgery use, to estimate the healthcare demand of these patients and the demand for Hedgehog inhibitors therapy in this group. OBJECTIVE: To describe patients excluded for Mohs surgery after pre-surgical assessment, and the treatments that they received. METHODS: Regesmohs includes all consecutive patients assessed for Mohs surgery in the participating centres, collecting data on patient characteristics, intervention, and short and long-term results. Patients excluded for Mohs surgery after pre-surgical evaluation were described. RESULTS: 3011 patients were included in Regesmohs from July 2013 to October 2016. In 85, Mohs surgery was not performed as they were considered inadequate candidates. 67 had BCC. Reasons for exclusion were: medical contraindication (27.1%, n=23) low-risk tumour in (18.8%, n=16) and giant tumour and bone invasion (15.3%, n=13). Only 1 patient (1.2%) showed lymph node involvement and no patients had visceral metastases. Of the 85 excluded patients, 29 (34.1%) were treated with conventional surgery, 24 (28.3%) with radiotherapy, 4 (4.7%) with inhibitors of the Hedgehog pathway (only indicated for BCC), and 2 (2.4%) received palliative care. We had no follow-up data on 14 patients (16.5%). CONCLUSION: Medical comorbidities were the most common reason for withholding Mohs surgery. Withholding therapy on the basis of distant extension is uncommon. Most excluded patients received simpler therapies: conventional surgery or radiotherapy, with hedgehog inhibitors being a new option.

[Kaposi sarcoma and Hodgkin disease]. / Sarcoma de Kaposi y enfermedad de Hodgkin.

Kaposi's sarcoma is frequently associated to other primary neoplasias, especially those arising from mononuclear-phagocytic system. A case of Kaposi's sarcoma associated to Hodgkin's disease is presented.

[Multiple, painful, treatment-resistant leg ulcers associated with dermatomyositis-like lesions over the interphalangeal joints induced by hydroxyurea]. / Ulceras dolorosas múltiples en la pierna resistentes al tratamiento asociadas a lesiones dermatomiositis-like en las articulaciones interfalángicas de las manos: hidroxiurea como agente causal.

The onset of a dermatomyositis-like rash and persistent skin ulcers during long-term treatment with hydroxyurea is a very rare event that has not been fully described in the literature. The fact that these lesions have a typical clinical presentation and course, and that complete resolution can only be achieved by immediate withdrawal of the drug, means that dermatologists should be aware of this condition in order to avoid a delay in diagnosis. We present the case of a 76-year-old woman who developed a dermatomyositis-like eruption associated with chronic ulcers on the lower limbs during long-term treatment with hydroxyurea.

[Oral leukoplakia: clinical, histopathologic, and molecular features and therapeutic approach]. / La leucoplasia oral: definición de parámetros clínicos, histopatológicos y moleculares y actitud terapéutica.

Dermatology departments are currently seeing an increase in the number of cases of oral diseases. Of note among the range of lesions covered by this broad area of knowledge is oral leukoplakia-the most common precancerous lesion and the most problematic in terms of both diagnosis and therapeutic management. In addition to defining leukoplakia, this review also establishes a differential diagnosis with the other most important oral diseases and analyzes the different clinical, histologic, and molecular features that can provide an indication of the risk of malignant transformation. Finally, a therapeutic algorithm is presented to help us optimize clinical management of the disease.

[Microcystic adnexal carcinoma: Mohs micrographic surgery as the treatment of choice]. / Carcinoma anexial microquístico: la cirugía micrográfica de Mohs como tratamiento de elección.

INTRODUCTION AND OBJECTIVES: Microcystic adnexal carcinoma is a rare and aggressive tumor that manifests clinically as a subcutaneous nodule located on the head or neck. The tumor can be confused clinically and histologically with other benign and malignant skin lesions, often leading to inappropriate initial treatment. The chief concern with microcystic adnexal carcinoma is the elevated morbidity and the high rate of recurrence after wide local excision. Recent preliminary studies point to higher cure rates with Mohs micrographic surgery. MATERIAL AND METHODS: We reviewed the medical histories of 6 consecutive patients with microcystic adnexal carcinoma who underwent Mohs micrographic surgery in our dermatology department between 1995 and 2007. RESULTS: In all cases, lesions were located on the head and were primary tumors. Seventy percent of the tumors were wrongly diagnosed initially as basal cell carcinoma. Perineural invasion was not detected in any patient, and all were free of recurrence after between 1 and 12 years of postoperative follow-up. CONCLUSIONS: The absence of perineural involvement and substantial cell atypia can be attributed to the lesions being primary tumors. This would provide a rationale for definitive radical treatment of the primary tumor from the outset to avoid the complications associated with recurrence. The site and the absence of recurrence in all our patients who underwent Mohs micrographic surgery support the use of this technique as the treatment of choice in microcystic adnexal carcinoma.

Descripción de pacientes no aptos para ser sometidos a cirugía de Mohs tras valoración prequirúrgica: datos del registro español Regesmohs / Description of patients excluded for Mohs surgery after pre-surgical evaluation: data from the Regesmohs Spanish registry

ANTECEDENTES: El registro Regesmohs es un registro de ámbito nacional, de pacientes evaluados y sometidos a una cirugía de Mohs, en 17 centros españoles, desde julio de 2013. Como la cirugía de Mohs es el tratamiento que mejores resultados da para el manejo del carcinoma de células basales (CCB) de alto riesgo y otros tumores de la piel, queríamos describir los motivos por los que algunos pacientes fueron considerados no aptos para ser sometidos a este tratamiento y qué tratamientos alternativos recibieron. Estos datos pueden ser útiles para evitar excluir a pacientes aptos para ser sometidos a una cirugía de Mohs, para calcular la demanda que estos pacientes generan a nivel sanitario, así como la demanda que hay de tratamientos de inhibidores de la vía de Hedgehog en dicho grupo de pacientes. OBJETIVO: Describir a aquellos pacientes que fueron considerados no aptos para ser sometidos a una cirugía de Mohs tras valoración prequirúrgica y los tratamientos que recibieron. MÉTODOS: Regesmohs incluye a todos los pacientes consecutivos para ser sometidos a una cirugía de Mohs en los centros participantes, recogiendo datos sobre las características de los pacientes, las intervenciones y los resultados a corto y largo plazo. Se hizo una descripción de los pacientes considerados no aptos para ser sometidos a una cirugía de Mohs tras valoración prequirúrgica. RESULTADOS: Tres mil once pacientes fueron incluidos en el registro Regesmohs entre julio de 2013 y octubre de 2016. En 85 pacientes no se realizó cirugía de Mohs porque se consideraron candidatos inadecuados. Sesenta y siete pacientes presentaban CCB. Las razones para ser considerado paciente no apto fueron: contraindicaciones médicas (27,1%, n = 23), tumores de bajo riesgo (18,8%, n = 16) y tumores gigantes e invasión ósea (15,3%, n = 13). Solo un paciente (1,2%) reveló compromiso de ganglios linfáticos y ningún paciente metástasis visceral. De los 85 pacientes considerados no aptos 29 (34,1%) fueron sometidos a cirugía convencional, 24 (28,3%) a radioterapia, 4 (4,7%) a inhibidores de la vía de Hedgehog (solo indicado para el CCB) y 2 (2,4%) a tratamiento paliativo. No hubo datos de seguimiento de 14 pacientes (16,5%). CONCLUSIÓN: Las comorbilidades médicas fueron la razón más habitual para retener la cirugía de Mohs. Retener un tratamiento en función de una propagación a lugares distantes no es algo habitual. La mayoría de los pacientes considerados no aptos recibieron tratamientos más sencillos: cirugía convencional o radioterapia, siendo los inhibidores de la vía de Hedgehog una opción novedosa

BACKGROUND: Regesmohs registry is a nationwide registry including patients evaluated for Mohs surgery in 17 Spanish centres since July 2013. Given that Mohs surgery is the therapy with best results for high risk basal cell carcinoma (BCC) and other skin tumours, we wanted to describe the reasons that lead to some patients being excluded from this therapy and the alternative treatments that they received. These data may be useful to avoid excluding patients for Mohs surgery use, to estimate the healthcare demand of these patients and the demand for Hedgehog inhibitors therapy in this group. OBJECTIVE: To describe patients excluded for Mohs surgery after pre-surgical assessment, and the treatments that they received. METHODS: Regesmohs includes all consecutive patients assessed for Mohs surgery in the participating centres, collecting data on patient characteristics, intervention, and short and long-term results. Patients excluded for Mohs surgery after pre-surgical evaluation were described. RESULTS: 3011 patients were included in Regesmohs from July 2013 to October 2016. In 85, Mohs surgery was not performed as they were considered inadequate candidates. 67 had BCC. Reasons for exclusion were: medical contraindication (27.1%, n = 23) low-risk tumour in (18.8%, n = 16) and giant tumour and bone invasion (15.3%, n = 13). Only 1 patient (1.2%) showed lymph node involvement and no patients had visceral metastases. Of the 85 excluded patients, 29 (34.1%) were treated with conventional surgery, 24 (28.3%) with radiotherapy, 4 (4.7%) with inhibitors of the Hedgehog pathway (only indicated for BCC), and 2 (2.4%) received palliative care. We had no follow-up data on 14 patients (16.5%). CONCLUSION: Medical comorbidities were the most common reason for withholding Mohs surgery. Withholding therapy on the basis of distant extension is uncommon. Most excluded patients received simpler therapies: conventional surgery or radiotherapy, with hedgehog inhibitors being a new option

Descripción de las intervenciones quirúrgicas recogidas en el registro español de cirugía de Mohs (REGESMOHS) (2013-2015) / Characterization of Surgical Procedures in the Spanish Mohs Surgery Registry (REGESMOHS) for 2013-2015

Introducción: El Registro Español de Cirugía de Mohs recoge los datos de aplicación y resultados de esta técnica en España. Se describen los datos de las intervenciones realizadas desde el inicio del Registro en julio de 2013 a enero de 2016. Se analizan los datos de las cirugías tanto perioperatorios como intraoperatorios. Material y métodos: Estudio de cohortes prospectivo en el que participan 18 centros. Se recogen los datos de las intervenciones quirúrgicas como tipo de anestesia, técnica quirúrgica, ingreso hospitalario, número de estadios, manejo de factores de riesgo preoperatorios, tratamientos complementarios, tratamientos previos, tipo de tumor, tiempo empleado en la cirugía y complicaciones. Resultados: Se analizan 1.796 intervenciones quirúrgicas. El tumor intervenido con más frecuencia es el carcinoma basocelular (85,96%), seguido del carcinoma epidermoide (6,18%), lentigo maligno (2,81%) y dermatofibrosarcoma protuberans (1,97%). El 66,9% de los tumores eran primarios, el 19,2% recurrentes y el 13,9% persistentes. El tratamiento previo más frecuente fue quirúrgico. La cirugía de Mohs se realizó con más frecuencia bajo anestesia local (86,7%) y de forma ambulatoria (71,8%). En el 89,5% de los casos se utilizó la técnica de Mohs en congelación. El número de etapas requerido para alcanzar márgenes libres de tumor fue una en 56,45% de los pacientes, 2 en 32,1%, 3 en 7,1%, 4 en 2,7% y 5 o más en 1,8%. El propio dermatólogo reconstruyó el defecto en el 98% de los pacientes y la técnica reconstructiva más utilizada fue el colgajo (47,2%). Solo el 1,62% de los pacientes presentó alguna complicación intraoperatoria y la mediana de la duración de la cirugía fue 75 (p25:60-p75:100). Conclusión: Las características de los pacientes y tumores tratados son similares a las descritas en estudios de las mismas características en otras áreas geográficas. Existe un porcentaje mayor de lentigo maligno y dermatofibrosarcoma protuberans. La reconstrucción la realiza el dermatólogo con más frecuencia que en otras series. El tiempo de utilización de quirófano no es mucho mayor que para otras técnicas y la tasa de complicaciones intraoperatorias es muy reducida (AU)

Introduction: The Spanish Mohs Surgery Registry is used to collect data on the use and outcomes of Mohs micrographic surgery (MMS) in Spain. The aim of this study was to describe perioperative and intraoperative data recorded for MMS procedures performed between July 2013 (when the registry started) and January 2016. Material and methods: Prospective cohort study of data from 18 hospitals. The data collected included type of anesthesia, surgical technique, hospital admission, number of Mohs stages, management of preoperative risk factors, additional treatments, previous treatments, type of tumor, operating time, and complications. Results: Data were available for 1796 operations. The most common tumor treated by MMS was basal cell carcinoma (85.96%), followed by squamous cell carcinoma (6.18%), lentigo maligna (2.81%), and dermatofibrosarcoma protuberans (1.97%). Primary tumors accounted for 66.9% of all tumors operated on; 19.2% of tumors were recurrent and 13.9% were persistent. The most common previous treatment was surgical. MMS was mostly performed under local anesthesia (86.7% of cases) and as an outpatient procedure (71.8%). The frozen section technique was used in 89.5% of cases. One stage was needed to achieve tumor-free margins in 56.45% of patients; 2 stages were required in 32.1% of patients, 3 in 7.1%%, 4 in 2.7%, and 5 or more in 1.8%. The defect was reconstructed by the dermatologist in 98% of patients and the most common technique was flap closure (47.2%). Intraoperative complications were recorded for just 1.62% of patients and the median (interquartile range) duration of surgery was 75 (60-100) minutes. Conclusion: The characteristics of the patients and tumors treated by MMS are similar to those reported for similar studies in other geographic areas. Lentigo maligna and dermatofibrosarcoma protuberans accounted for a higher proportion of cases in our series, and repair of the surgical defect by a dermatologist was also more common. Operating times in MMS are not much longer than those reported for other procedures and the rate of intraoperative complications is very low (AU)

[Adenoid cystic carcinoma]. / Carcinoma adenoide quístico.

Adenoid cystic carcinoma is an uncommon tumor of the head and neck. Although it is mainly located in the salivary gland, a skin location has also been described. Metastases are rare, but 50 % of the cases relapse. A 65-year-old male patient had a lesion in the upper lip. After resection, the histological diagnosis was adenoid cystic carcinoma. Treatment was completed with radiotherapy. Ten years later, a nodule was detected in the neck. Its histological diagnosis was lymphatic metastasis due to adenoid cystic carcinoma. Primary cutaneous adenoid cystic carcinoma is a very uncommon tumor in which treatment consists in extensive local excision with free margins. Radiotherapy is not curative and should be reserved for palliative treatments. Multicenter, prospective studies are necessary to determine the best treatment and especially the adjuvant treatment for adenoid cystic carcinoma.