Tumor de células granulares. Estudio clínico de 81 pacientes / Granular Cell Tumor: A Clinical Study of 81 Patients

Introducción: El tumor de células granulares (TCG) suele desarrollarse en la piel o en la mucosa oral, pero se ha descrito en muchos otros órganos. Suele ser único, pero puede ser múltiple y asociarse a otras enfermedades. Objetivos: Analizar las características clínicas de nuestros pacientes con TCG en la piel y la mucosa oral y su posible asociación con otras afecciones. Material y métodos: Estudio retrospectivo de los pacientes con TCG diagnosticados entre 1995-2019. Se revisaron las historias clínicas para obtener los siguientes datos: edad, sexo, localización, número de lesiones, diámetro, tiempo de evolución, diagnóstico clínico de sospecha, estado de los márgenes de resección, desarrollo de recidiva, tiempo de seguimiento y enfermedades asociadas. Resultados: Ochenta y un pacientes presentaron 89 TCG en la piel y la mucosa oral (43 mujeres/38 varones, edad media 40,21años). El diámetro medio fue de 1,34cm. Contabilizando los tumores extracutáneos 5 de los 81 pacientes presentaban TCG múltiple (6,2%). La edad media de los pacientes con TCG múltiple fue significativamente inferior a la de los pacientes con un único tumor (p=0,004). Solamente un paciente presentó recidiva local y ninguno desarrolló metástasis a distancia. No hemos detectado ningún caso asociado a otras enfermedades. Conclusiones: La mayoría de TCG son benignos, y a pesar de tener los márgenes afectados no suelen presentar recidivas locales. Sin embargo, hay que tener en cuenta la posibilidad de presentar TCG múltiples, tanto en la piel y en la mucosa oral como en órganos internos, especialmente en pacientes jóvenes (AU)

Introduction: Although a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. Objective: To describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. Material and methods: Retrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients’ medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. Results: We detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P=.004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. Conclusions: Most GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients (AU)

Granular Cell Tumor: A Clinical Study of 81 Patients. / Tumor de células granulares. Estudio clínico de 81 pacientes.

INTRODUCTION: Although a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases. OBJECTIVES: To describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases. MATERIAL AND METHODS: Retrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients' medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases. RESULTS: We detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P=.004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases. CONCLUSIONS: Most GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients.

Neurofibromatosis segmentaria bilateral en extremidades inferiores / Bilateral Segmental Neurofibromatosis on the Lower Limbs

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Cutaneous sebaceous tumours and Lynch syndrome: long-term follow-up of 60 patients.

BACKGROUND: Sebaceous neoplasms (SN) may appear sporadically in the general population but may also be part of the Muir-Torre variant of Lynch syndrome (MT-LS). There are few studies in southern Europe on the incidence of MT-LS in the population of patients with SN. AIM: To retrospectively review patients with SN and to analyse their clinical features and the incidence of MT-LS. METHODS: Patients with SN diagnosed between 1995 and 2015 were enrolled in the study. The diagnosis of MT-LS was made according to established clinical criteria and, whenever possible, was confirmed by germline mutation analysis. RESULTS: In 60 patients (32 men, 28 women, mean age 69.22 years), 96 SN were diagnosed: 65 adenomas (67.7%), 16 sebaceomas (16.7%) and 15 carcinomas (15.6%). Of the 60 patients, 50 (83.3%) had a single SN and 10 (16.7%) had multiple lesions. Patients diagnosed with MT-LS (12 patients, 20%) were younger (63.25 years vs. 70.71 years), and had a higher incidence of extrafacial SN (4/12 patients, 33.3%), and were significantly (P < 0.001) more likely to have multiple SNs (8/12, 75%) and keratoacanthomas (KAs) (6/12, 50%). CONCLUSION: Our study confirms that all patients with SN should be investigated, as 20% of our patients were diagnosed with MT-LS. The most specific features of SN associated with MT-LS in our study were the presence of multiple lesions and association with KAs.

Melanoma subungueal: estudio descriptivo de 34 pacientes / Subungual Melanoma: A Descriptive Study of 34 Patients

INTRODUCCIÓN Y OBJETIVOS: El melanoma subungueal constituye un reto diagnóstico por su presentación clínica frecuentemente atípica. El objetivo del estudio fue revisar las características clínico-patológicas de los pacientes con melanoma subungueal diagnosticados en un hospital universitario de tercer nivel y analizar los factores posiblemente asociados al retraso del diagnóstico. MATERIAL Y MÉTODOS: Fueron analizados los datos de 34 pacientes diagnosticados de melanoma subungueal durante 20 años en nuestro centro. RESULTADOS: Del total de pacientes, 18 eran mujeres y 16 eran varones, con una edad mediana al diagnóstico de 66 años. Únicamente 5 de los pacientes presentaron melanoniquia longitudinal al ser visitados en nuestro Servicio de Dermatología. De los 34 pacientes, 19 presentaron melanoma invasivo al diagnóstico, con una mediana de índice de Breslow de 3,70 mm; 16 presentaron ulceración y 8 invasión ganglionar regional al diagnóstico. Cinco pacientes fueron diagnosticados en fase de melanoma in situ. La mediana del tiempo de evolución de las lesiones desde su aparición hasta la consulta al Centro de Asistencia Primaria fue de 15 meses, y desde la consulta al Centro de Asistencia Primaria hasta el diagnóstico en nuestro hospital fue de 5,5 meses. Las lesiones localizadas en los dedos de los pies presentaron con mayor frecuencia ulceración (p = 0,017) y una mayor probabilidad de invasión ganglionar regional al diagnóstico (p = 0,012). CONCLUSIONES: Los factores que en nuestro estudio se asociaron a un mayor retraso del diagnóstico del melanoma subungueal fueron la ausencia de melanoniquia como presentación clínica inicial y la localización de las lesiones en los dedos de los pies

INTRODUCTION AND OBJECTIVES: Subungual melanoma constitutes a diagnostic challenge because it often has an atypical clinical presentation. The aims of this study were to revise the clinical and pathologic characteristics of patients with subungual melanoma diagnosed at a tertiary care university hospital and analyze the factors potentially associated with a delayed diagnosis. MATERIAL AND METHODS: We analyzed data for 34 patients diagnosed with subungual melanoma at our hospital over a period of 20 years. RESULTS: The study population comprised 18 women and 16 men with a median age at diagnosis of 66 years. Only 5 of the patients had longitudinal melanonychia when examined at the dermatology department. At the time of diagnosis, 19 of the 34 patients had invasive melanoma (median Breslow thickness, 3.70 mm); 16 had ulceration and 8 had regional lymph node involvement. Five patients had subungual melanoma in situ at diagnosis. The median time from appearance of the lesions to consultation at a primary care center was 15 months; the corresponding time from primary care consultation to diagnosis at our hospital was 5.5 months. Lesions located on the toes were more likely to be ulcerated (P = .017) and to be accompanied by regional lymph node involvement at diagnosis (P = .012). CONCLUSIONS: The factors associated with a longer diagnostic delay in patients with subungual melanoma were absence of melanonychia as a presenting feature and involvement of the toes

Subungual Melanoma: A Descriptive Study of 34 Patients. / Melanoma subungueal: estudio descriptivo de 34 pacientes.

INTRODUCTION AND OBJECTIVES: Subungual melanoma constitutes a diagnostic challenge because it often has an atypical clinical presentation. The aims of this study were to revise the clinical and pathologic characteristics of patients with subungual melanoma diagnosed at a tertiary care university hospital and analyze the factors potentially associated with a delayed diagnosis. MATERIAL AND METHODS: We analyzed data for 34 patients diagnosed with subungual melanoma at our hospital over a period of 20 years. RESULTS: The study population comprised 18 women and 16 men with a median age at diagnosis of 66 years. Only 5 of the patients had longitudinal melanonychia when examined at the dermatology department. At the time of diagnosis, 19 of the 34 patients had invasive melanoma (median Breslow thickness, 3.70mm); 16 had ulceration and 8 had regional lymph node involvement. Five patients had subungual melanoma in situ at diagnosis. The median time from appearance of the lesions to consultation at a primary care center was 15 months; the corresponding time from primary care consultation to diagnosis at our hospital was 5.5 months. Lesions located on the toes were more likely to be ulcerated (P=.017) and to be accompanied by regional lymph node involvement at diagnosis (P=.012). CONCLUSIONS: The factors associated with a longer diagnostic delay in patients with subungual melanoma were absence of melanonychia as a presenting feature and involvement of the toes.