RESUMO
El linfangiosarcoma es un tumor vascular infrecuente que asienta habitualmente sobre linfedema de larga evolución. Hemos recogido los casos observados de linfangioma en un hospital intentando analizar sus características: la edad, el sexo, la ubicación, tratamiento y los datos de seguimiento. Hemos estudiado 5 casos: 3 casos de síndrome de Steward-Treves tras mastectomía y radioterapia, y 2 que asientan en pacientes afectados de linfedema congénito (forma tardía). Cuatro eran mujeres y 1 varón. Se realizó cirugía radical en 4 pacientes. Las técnicas empleadas fueron: amputación supragoneal: 1; desarticulación de cadera: 1; desarticulación escapulohumeral: 2, y 1 tratado con gemcitabina y radioterapia ante la existencia de enfermedad metastásica en el momento del diagnóstico. Dentro de los primeros 14 meses de seguimiento fallecieron 3 pacientes y 2 se encuentran libres de enfermedad pasados 46 y 86 meses, respectivamente. Este estudio confirma el mal pronóstico de estos pacientes (AU)
Lymphangiosarcoma is an uncommon vascular tumor that usually develops in longstanding lymphedema. We gathered the cases of lymphangioma observed in a hospital and attempted to analyze their characteristics: age, sex, localization, treatment and follow-up data. We studied five cases: three cases of Stewart-Treves syndrome after mastectomy and radiotherapy and two cases that developed in patients with late-onset congenital lymphedema. There were four women and one man. Radical surgery was performed in four patients. The techniques employed were: above-knee amputation (one patient), hip disarticulation (one patient), scapulo-humeral disarticulation (two patients) and gemcitabine and radiotherapy in one patient with metastatic disease at diagnosis. Three patients died in the first 14 months of follow-up, while two are disease free after 46 and 86 months respectively. This study confirms the poor prognosis of patients with Steward-Treves syndrome (AU)
Assuntos
Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Humanos , Linfangiossarcoma/diagnóstico , Linfangiossarcoma/cirurgia , Linfedema/diagnóstico , Linfedema/cirurgia , Síndrome de Meige/diagnóstico , Síndrome de Meige/cirurgia , Mastectomia/métodos , Tomografia Computadorizada de Emissão/métodos , Neoplasias da Mama/tratamento farmacológico , Neoplasias da Mama/radioterapia , Prognóstico , Amputação Cirúrgica/métodos , Linfangioma/complicações , Linfangioma/diagnóstico , Hemangiossarcoma/complicações , Hemangiossarcoma/diagnóstico , Neoplasias da Mama/cirurgia , Neoplasias da Mama/complicaçõesRESUMO
Lymphangiosarcoma is an uncommon vascular tumor that usually develops in longstanding lymphedema. We gathered the cases of lymphangioma observed in a hospital and attempted to analyze their characteristics: age, sex, localization, treatment and follow-up data. We studied five cases: three cases of Stewart-Treves syndrome after mastectomy and radiotherapy and two cases that developed in patients with late-onset congenital lymphedema. There were four women and one man. Radical surgery was performed in four patients. The techniques employed were: above-knee amputation (one patient), hip disarticulation (one patient), scapulo-humeral disarticulation (two patients) and gemcitabine and radiotherapy in one patient with metastatic disease at diagnosis. Three patients died in the first 14 months of follow-up, while two are disease free after 46 and 86 months respectively. This study confirms the poor prognosis of patients with Steward-Treves syndrome.
Assuntos
Linfangiossarcoma , Adulto , Idoso , Feminino , Seguimentos , Humanos , Linfangiossarcoma/diagnóstico , Linfangiossarcoma/terapia , Masculino , Pessoa de Meia-Idade , SíndromeRESUMO
CONTEXT: Glucagonoma syndrome may present either associated with a pancreatic neoplasm which secretes glucagon or as a pseudo-glucagonoma associated with other diseases. It is extremely infrequent but well-known with a current prevalence estimated at 1/20,000,000. DESIGN: A retrospective review of glucagonoma and pseudoglucagonoma cases observed between January 1998 and December 2003 in three hospitals. PATIENTS: Five cases: 3 with a demonstrable glucagon-secreting tumor and 2 cases without an associated neoplasm. MAIN OUTCOME MEASURES: Age, sex, initial diagnosis, associated symptoms, and pathology were analyzed as were procedures employed in diagnosis, imaging studies, laboratory data, surgery and follow-up. RESULTS: Hyperglycemia and elevated plasma glucagon levels were found in all cases. In 3 cases, hypo-aminoacidemia and a descrease in fatty acids were found. No changes of zinc levels were observed. Abdominal ultrasound studies were of no value except in evaluating pancreatitis. A CT-scan was conclusive when a pancreatic neoplasm existed and 3 patients were operated on a curative basis. DISCUSSION: Necrolytic migratory erythema was the key diagnosis in all cases. Surgery was intended to be curative. The follow-up was of 8, 37 and 57 months in the cases of true glucagonoma syndrome. CONCLUSIONS: A real prevalence of glucagonoma syndrome could be greater than currently estimated. In our series, it was 13.5/20,000,000. Pseudoglucagonoma syndrome remains a rarity.