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Resumen Introduccion El sindrome del glucagonoma puede ser verdadero –asociado a un tumor pancreatico secretor de glucagon– o un seudoglucagonoma, asociado a otro tipo de enfermedad. Es una enfermedad extremadamente infrecuente con una prevalencia actual estimada de 1/20.000.000. Material y metodos Se ha realizado una revision retrospectiva de casos de glucagonoma y seudoglucagonoma entre enero de 1998 y diciembre de 2003 en 3 hospitales. Se han tratado 5 casos: 3 con tumoracion pancreatica demostrable y 2 sin neoplasia asociada. Se han analizado la edad, el sexo, el diagnostico inicial, los sintomas asociados, las enfermedades concomitantes, los procedimientos diagnosticos de imagen empleados, los datos analiticos existentes, los procedimientos terapeuticos empleados, la cirugia y el seguimiento. Resultados Se encontro hiperglucemia en todos los casos y elevacion de valores de glucagon plasmatico. En 3 casos habia hipoaminoacidemia y descenso de acidos grasos totales. No se observaron alteraciones de los valores de cinc. La ultrasonografia abdominal no ofrecio resultados concluyentes. La tomografia axial computarizada demostro tumoracion pancreatica en 3 pacientes que fueron intervenidos, por lo que fue posible la reseccion curativa. El eritema necrolitico migratorio ha sido la clave diagnostica en todos los casos. La hiperglucagonemia estaba presente en todos ellos. El tratamiento quirurgico ha sido curativo con seguimientos de 7, 36 y 56 meses en los casos de glucagonoma verdadero. Conclusiones La prevalencia real del sindrome del glucagonoma puede ser mayor que la estimada actualmente. En nuestra serie ha sido de 13,5/20.000.000 habitantes/ano.

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.

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.A retrospective review of glucagonoma and pseudoglucagonoma cases observed between January 1998 and December 2003 in three hospitals.Five cases: 3 with a demonstrable glucagon-secreting tumor and 2 cases without an associated neoplasm.Age, sex, initial diagnosis, associated symptoms, and pathology were analyzed as were procedures employed in diagnosis, imaging studies, laboratory data, surgery and follow-up.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.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.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.