RESUMEN
PURPOSE: To determine the occurrence, clinical course and outcome of our patients with natural killer/T-cell lymphoma (NKTCL), nasal type. The nasal type of natural killer/T-cell lymphoma (NKTCL), is rare in the European population but is more frequent in Asia, Central and South America where it accounts for 15-20% of all non-Hodgkin lymphomas (NHL). This type of lymphoma is characterized by tissue necrosis and destruction of the nasal cavity and upper aero-digestive tract. The disease is closely associated with Epstein-Barr virus (EBV) infection and has an aggressive clinical course, poor clinical outcome and high resistance to chemotherapy. PATIENTS: 63 patients with NHL were treated in our department between 2006 and 2011. Eleven patients were diagnosed with extranodal lymphoma, 6 in the area of head and neck. Three of them belonged to NKTCL, nasal type group (4.7%), 1 was diffuse large B cell lymphoma and 2 were marginal zone lymphomas. CONCLUSION: Two out of 3 patients with nasal type NKTCL, had extremely aggressive disease resistant to applied chemotherapy (anthracycline containing regimen), while 1 patient remains in complete remission 6 years after diagnosis.
Asunto(s)
Neoplasias de Cabeza y Cuello/cirugía , Linfoma Extranodal de Células NK-T/cirugía , Adulto , Quimioterapia Adyuvante , Femenino , Estudios de Seguimiento , Humanos , Linfoma de Células B de la Zona Marginal/cirugía , Linfoma de Células B Grandes Difuso/cirugía , Masculino , Persona de Mediana Edad , Neoplasias Nasofaríngeas/cirugía , Terapia Neoadyuvante , Neoplasias Nasales/cirugía , Neoplasias Orbitales/cirugía , Neoplasias de los Senos Paranasales/cirugía , Radioterapia Adyuvante , Resultado del TratamientoRESUMEN
Primary cutaneus apocrine carcinoma is a rare malignancy with about 50 cases reported in the literature. Axilla is the most common site of occurrence, but locations like scalp, anogenital region, ear canal, chest, wrist, finger and eyelid have been described. The neoplasm presents itself as an asymptomatic, slow-growing, solid or cystic mass that varies in color. Most patients have a history of a long-standing neoplasm before the diagnosis is made. The disease is considered to have an indolent clinical course with favorable outcome although more than half of reported patients had regional lymph node metastases at the time of diagnosis. Systemic dissemination to lung, bones, liver and brain is extremely rare with only 14 cases documented in the literature. Wide surgical excision with lymph node dissection upon confirmation of the lymph node metastases remains the only curable treatment. Care and management of the disseminated disease is still challenging. We report a case of a 65-year-old woman with a very aggressive apocrine carcinoma of the scalp prone to local recurrence and distant metastases to lung and bones.