RESUMEN
Abstract Background: Cutaneous leiomyosarcoma is a rare malignant neoplasm with muscular origin, representing 2%-3% of all cutaneous soft tissue sarcomas. Objectives: The aim of this study was to characterize clinicopathological features of patients diagnosed with cutaneous leiomyosarcoma in our center over the last 20-years. Methods: A retrospective study of patients with a histopathological diagnosis of leiomyosarcoma between 1999 and 2018 was conducted. Results: Eleven patients were diagnosed with cutaneous leiomyosarcoma during this period. Most cases occurred in men (n = 7). Age at presentation ranged from 47 to 92 years (mean 64.9 years). Head and neck were the most frequently involved locations (n = 5). Ten leiomyosarcoma were dermal, with one cutaneous metastasis. Immunohistochemical staining was available for 7 patients, demonstrating positivity for smooth muscle actin in all of them. All neoplasms were treated surgically. Mean survival was 32.2-months. Study limitations: This was a retrospective study based on medical and pathological records. Conclusions: Histopathology is essential for the diagnosis of leiomyosarcoma, usually revealing a dermal or subcutaneous lesion composed of intertwined fascicles of smooth muscle fibers. Immunohistochemistry is then used to adequately differentiate leiomyosarcoma from other spindle cell tumors. When dealing with cutaneous leiomyosarcoma, it is advisable to carefully evaluate the depth of subcutaneous extension, since even minimal subcutaneous involvement may be associated with a poorer prognosis.
Asunto(s)
Humanos , Masculino , Anciano , Anciano de 80 o más Años , Neoplasias Cutáneas , Leiomiosarcoma/diagnóstico , Inmunohistoquímica , Estudios Retrospectivos , Tejido Subcutáneo , Persona de Mediana EdadRESUMEN
BACKGROUND: Cutaneous leiomyosarcoma is a rare malignant neoplasm with muscular origin, representing 2%-3% of all cutaneous soft tissue sarcomas. OBJECTIVES: The aim of this study was to characterize clinicopathological features of patients diagnosed with cutaneous leiomyosarcoma in our center over the last 20-years. METHODS: A retrospective study of patients with a histopathological diagnosis of leiomyosarcoma between 1999 and 2018 was conducted. RESULTS: Eleven patients were diagnosed with cutaneous leiomyosarcoma during this period. Most cases occurred in men (nâ¯=â¯7). Age at presentation ranged from 47 to 92 years (mean 64.9 years). Head and neck were the most frequently involved locations (nâ¯=â¯5). Ten leiomyosarcoma were dermal, with one cutaneous metastasis. Immunohistochemical staining was available for 7 patients, demonstrating positivity for smooth muscle actin in all of them. All neoplasms were treated surgically. Mean survival was 32.2-months. STUDY LIMITATIONS: This was a retrospective study based on medical and pathological records. CONCLUSIONS: Histopathology is essential for the diagnosis of leiomyosarcoma, usually revealing a dermal or subcutaneous lesion composed of intertwined fascicles of smooth muscle fibers. Immunohistochemistry is then used to adequately differentiate leiomyosarcoma from other spindle cell tumors. When dealing with cutaneous leiomyosarcoma, it is advisable to carefully evaluate the depth of subcutaneous extension, since even minimal subcutaneous involvement may be associated with a poorer prognosis.
Asunto(s)
Leiomiosarcoma , Neoplasias Cutáneas , Anciano , Anciano de 80 o más Años , Humanos , Inmunohistoquímica , Leiomiosarcoma/diagnóstico , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Tejido SubcutáneoRESUMEN
Abstract Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.
Asunto(s)
Humanos , Femenino , Anciano , Neoplasias de la Vulva/patología , Enfermedad de Paget Extramamaria/patología , Vulvectomía , Imiquimod/uso terapéutico , Recurrencia Local de Neoplasia/patología , Antineoplásicos/uso terapéutico , Neoplasias de la Vulva/terapia , Resultado del Tratamiento , Enfermedad de Paget Extramamaria/terapia , Procedimientos de Cirugía PlásticaRESUMEN
Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.