Histiocitosis de Células de Langerhans en el área maxilofacial en adultos. Informe de tres casos / Langerhans cell histiocytosis in the maxillofacial area in adults. Report of three cases

La Histiocitosis de Células de Langerhans (HCL), es una enfermedad de etiología desconocida, que se caracteriza por la proliferación e infiltración anormal, de órganos, por células de Langerhans patológicas. Afecta predominantementea pacientes en edad pediátrica, siendo en adultos la incidencia de la enfermedad de uno a dos casos por millón de habitantes. Las manifestaciones en cabeza y cuello aparecen en casi un 90% de los casos. El diagnóstico se obtiene por medios anatomopatológicos, siendo necesarias una serie de pruebas, determinantes de extensión, en todos los pacientes diagnosticados de HCL. No existen estudios, controlados, que determinen un tratamiento óptimo para la HCL. El pronósticode esta enfermedad en adultos es generalmente bueno debido a la lenta evolución de la enfermedad y a su buena respuesta al tratamiento. Presentamos una revisión de tres casos de HCL, de 16, 24 y 28 años de edad, con manifestación primaria en el área Maxilofacial. Así mismo, realizamos una revisión de la literatura

Langerhans cell histiocytosis (LCH) is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. The head and neck are affected in almost 90% of cases. Diagnosis is made by means of histopathologicalanalysis, and imaging studies are necessary in order to determine extent of the disease. There are no controlled trials proposing an optimal treatment protocol for LCH. Prognosis in adults is generally good due to the slow evolution of the disease and its favourable response to treatment. In our report, we present three cases of LCH in patients aged 16, 24, and 28 years respectively, with primary manifestation in the maxillofacial area. A literature review was also conducted

Langerhans cell histiocytosis in the maxillofacial area in adults. Report of three cases.

Langerhans cell histiocytosis (LCH) is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million. The head and neck are affected in almost 90% of cases. Diagnosis is made by means of histopathological analysis, and imaging studies are necessary in order to determine extent of the disease. There are no controlled trials proposing an optimal treatment protocol for LCH. Prognosis in adults is generally good due to the slow evolution of the disease and its favourable response to treatment. In our report, we present three cases of LCH in patients aged 16, 24, and 28 years respectively, with primary manifestation in the maxillofacial area. A literature review was also conducted.

Inflammatory pseudotumor of the parotid gland.

Inflammatory pseudotumor is a term that refers to a reactive pseudoneoplastic disorder that can appear in different locations of the human body. The lung is the most frequently affected organ. The etiology is still unknown. It affects individuals of both sexes and of a wide range of ages. The diagnosis is still difficult and it is based on the histological examination of the lesions composed of four cell-types: histiocytes, myofibroblasts, plasma cells and lymphocytes. With regard to the treatment regimes there is no agreement. Treatment ranges from surgical excision to radiotherapy, chemotherapy or steroids. The purpose of this article is to report one case of inflammatory pseudotumor located in the parotid gland and to make a special point of the difficulty in arriving at a correct diagnosis in order to achieve the most adequate treatment.

Pseudotumor inflamatorio de glándula parótida / Inflammatory pseudotumor of the parotid gland

El término pseudotumor inflamatorio hace referencia a un proceso reactivo y pseudoneoplásico que puede aparecer en diferentes localizaciones del cuerpo humano. El pulmón es el órgano que se afecta con mayor frecuencia. Su etiología sigue siendo desconocida. Afecta a individuos de ambos sexos y con un amplio rango de edad. Su diagnóstico sigue siendo dificultoso y se basa en el examen histológico de las lesiones que están formadas por cuatro elementos fundamentalmente: histiocitos, miofibroblastos, células plasmáticas y linfocitos. A la hora de elegir el tratamiento no hay unanimidad y se incluye desde tratamiento quirúrgico de las lesiones hasta radioterapia, quimioterapia y esteroides. El propósito de este artículo es describir un caso de pseudotumor inflamatorio localizado en glándula parótida, su dificultad para llegar al diagnóstico correcto y realizar así el tratamiento más adecuado

Inflammatory pseudotumor is a term that refers to a reactive pseudoneoplastic disorder that can appear in different locations of the human body. The lung is the most frequently affected organ. The etiology is still unknown. It affects individuals of both sexes and of a wide range of ages. The diagnosis is still difficult and it is based on the histological examination of the lesions composed of four cell-types: histiocytes, myofibroblasts, plasma cells and lymphocytes. With regard to the treatment regimes there is no agreement. Treatment ranges from surgical excision to radiotherapy, chemotherapy or steroids. The purpose of this article is to report one case of inflammatory pseudotumor located in the parotid gland and to make a special point of the difficulty in arriving at a correct diagnosis in order to achieve the most adequate treatment