Transformación maligna de linfedema crónico en miembro inferior en linfangiosarcoma / Malignant transformation of lower limb chronic lymphodema into lymphangiosarcoma

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Stewart-Treves syndrome: pathogenesis and management.

Stewart-Treves syndrome is a malignancy that arises within chronic lymphedema. Although classically described as a consequence of radical mastectomy, this lymphangiosarcoma has been documented to occur in cases of congenital and other causes of chronic secondary lymphedema. The development of this aggressive lymphangiosarcoma at sites of chronic lymphedema renders it a possible model for Kaposi sarcoma. Because of the increase in conservative treatment for breast carcinoma and improvement of operative and radiation therapy techniques, the prevalence of Stewart-Treves syndrome has decreased. Regardless, this malignancy significantly worsens patients' outcomes and needs to be diagnosed and treated early. Chemotherapy and radiation therapy have not improved survivorship significantly. Early amputation or wide local excision offers the best chance for long-term survival. Yet, overall prognosis remains dismal. Untreated patients usually live 5 to 8 months after diagnosis.

Linfangiosarcoma en paciente mastectomizada (síndrome de Stewart-Treves) / No disponible

El linfangiosarcoma postmastectomía (LPM) es un tumorvascular muy infrecuente y agresivo, que asienta habitualmenteen brazos portadores de un linfedema de larga evolución,tras una mastectomía radical por cáncer. Su incidencia es del0,45% en pacientes que sobreviven 5 años tras la mastectomíaradical. La etiología de estos tumores es aún completamentedesconocida. En la actualidad no existe un tratamientoestandarizado. Las opciones terapéuticas incluyen la exéresisquirúrgica, amputación de la extremidad afectada, desarticulación,radioterapia y quimioterapia. El pronóstico es malo. Lasupervivencia tras el diagnóstico oscila entre 8 y 15 meses.Presentamos el caso de una paciente con linfangiosarcomapostmastectomía radical y radioterapía(AU)

Lymphangiosarcoma postmastectomy is an uncommonvascular tumor, arising in the area of chronically lymphoedematousextremity, after radical mastectomy and radiotherapyin patients with breast cancer. It shows an incidence of 0,45%among patients that survive more than five years after radicalmastectomy. The etiology of this enigmatic tumor is not yetcompletely understood. There is no standard treatment. Thetreatment options include radical ablative surgery, amputation,radiation therapy and chemotherapy. The prognosis is poor.Survival after diagnosis ranged from 8 to 15 months. We reportthe case of patients with LPM and RT(AU)

Linfangiosarcoma sobre un linfedema crónico postmastectomía: síndrome de Stewart-Treves / No disponible

En 1948, Stewart y Treves comunicaron la aparición de unlinfangiosarcoma en el brazo edematoso de 6 pacientes sometidasa mastectomía radical por cáncer de mama. El síndromede Stewart-Treves describe la presentación de un linfangiosarcomao de un hemangiosarcoma sobre un linfedema crónico.Comunicamos el caso de una mujer de 74 años que fue sometidaa mastectomía radical derecha con radioterapia 23 añosantes y que consultó por la aparición de lesiones cutáneas enforma de placas violáceas en el brazo homolateral que llegarona hacerse nodulares. Tras la biopsia, el análisis histopatológicoconfirmó el diagnóstico de linfangiosarcoma. El estudio de extensiónfue negativo para metástasis. Se decidió la amputaciónde la extremidad. Seis meses más tarde, se diagnosticóuna metástasis cerebral y la paciente falleció poco tiempo después.Las opciones terapéuticas en estos casos (amputación,radioterapia y quimioterapia) son muy agresivas y ofrecen pobresresultados. Sólo un diagnóstico muy precoz, basado en lasospecha clínica, puede mejorar el pronóstico de esta complicaciónde la linfadenectomía axilar. La biopsia es obligatoriaante cualquier lesión sospechosa(AU)

In 1948, Stewart and Treves reported a lymphangiosarcomain 6 patient’s edematous arm after radical mastectomy forbreast cancer. Stewart-Treves syndrome describes a cutaneouslymphangiosarcoma or hemangiosarcoma that develops inlong-standing chronic lymphedema. We report a 74-year-oldwoman who was submitted to a right mastectomy and radiotherapy23 years before. She developed a chronic lymphedemain the same limb and fast-growing purplish lesions on thearm. These lesions became nodular. After the biopsy examinationthey were diagnosed as lymphangiosarcoma. The studyfor metastatic extension was negative. It was decided the amputationof the limb. Six months after the treatment, a brainmetastasis was diagnosed and the patient died. The therapeuticpossibilities (amputation, radiotherapy, chemotherapy) arevery aggresive and offer poor results. The physician mustmaintain a high index of suspicion in diagnosing this complicationof lymphadenectomy. If there is any doubt, biopsy is mandatory(AU)

Síndrome de Stewar-Treves / Stewart-Treves syndrome

El síndrome de Stewart-Treves es uno de los tumores vasculares más agresivos, siendo su diagnóstico muchas veces tardío. Se define como un tumor maligno de origen lingfático (linfangiosarcoma) que se origina en la extremidad superior afecta de linfedema crónico secundario a mastectomía por neoplasia de mama. Su incidencia del 0,5% de los pacientes con linfedema postmastectomía, apareciendo en una media de 9 años después del inicio del linfedema. Se caracteriza por la presencia de una o múltiples máculas de color rojizo o rojo-púrpura en el miembro superior que tienden a crecer y multiplicarse convirtiéndose en nódulos duros, con tendencia a la ulceración y hemorragias espontáneas. Cursan con metástasis precoces a nivel torácico. Creemos importante que los cirujanos vasculares conozcan esta enfermedad, siendo el diagnóstico precoz y el tratamiento multidisciplinar la única esperanza para estos pacientes

Stewart-Treves Síndrome is one of the most aggresive vascular neoplasm. Sometimes the diagnosis is late. This neoplasm is a lymphatic tumor (lymphangiosarcoma) which appears in an upper limb with chronic lymphedema postmastectomy, arising 9 years after the appearance of lymphedema´s symptoms. The clinical features are one or multiple rose or red-purple macular lesions in the upper limb. The natural evolution of the lesions are growing and becoming a hard nodule with tendency of ulceration and spontaneous hemorrhage. The thoracic metastasis is early. We think is important that the vascular surgeons recognize this pathology, being the early diagnosis and the multidisciplinary treatment the unique opportunity for these patients

Lymphangiogenesis and lymphologic syndromes.

A unifying concept linking disorders of lymphatic dysplasia, hyperplasia and neoplasia is presented. The central role of disturbed lymphangiogenesis is illustrated in a wide variety of lymphologic syndromes characterized by lymphedema, lymphangiectasia, lymphangioma, and lymphangiosarcoma.

Stewart-Treves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency.

Lymphangiosarcoma is a fatal complication of postmastectomy lymphedema. The pathogenesis of lymphangiosarcoma in chronic lymphedema is a combination of two factors. First, the edematous region responds in a manner similar to "immunologically privileged sites." Second, because of its anatomic and physiologic properties, it is a favorable site for the development of mutant cell populations for reasons that are not fully understood. As a result, these mutant cells, with their genetically nonidentical antigens, escape recognition by the host's impaired immune surveillance mechanism. The failure to promote a sufficient immune response allows unrestricted tumor growth to take place, resulting in the ultimate death of the patient. Available therapeutic measures are equally unsatisfactory. Emphasis is placed on periodic examination of the lymphedematous extremity, aggressive treatment of established lymphedema and infections, and surgical preservation of lymphatic channels during breast cancer surgery.