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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.

A 72-YEAR-OLD woman presented with an 11-year history of a waxing and waning scaling rash that principally involved her face (Fig 1) and extremities (Fig 2), along with glossitis. The diagnosis of migratory necrolytic erythema was made on biopsy of the skin. Routine blood tests were normal except for an elevated fasting blood sugar of 145 mg/dL. Basal plasma glucagon was 1100 pg/mL (normal, 55-177), and serum zinc was 97 μg/dL (normal, 60-150). An abdominal computed tomography scan showed a 4-cm mass in the body of the pancreas but no evidence of metastases. During somatostatin infusion, plasma glucagon levels dropped to 673 pg/mL. Distal pancreatectomy and splenectomy were performed. There were no metastases found. Postoperative plasma glucagon levels were 222 pg/mL. The skin rash cleared immediately, and the patient is free of recurrence at 36 months postoperatively.

A 63-year-old white male living in a rural area, with previously untreated congenital lymphoedema, was admitted to our clinic (figure 1). A histological analysis of a biopsy from the lesion showed squamous-cell carcinoma cells. Metastases were also found in the ulcerated inguinal-lymphnodes. The patient underwent lymphoedema-reducing surgery, with wide excision of the ulcerated area and inguinal lymphadenectomy. Unfortunately, he died of a massive pulmonary embolism 4 days after surgery even though adequate pharmacological and physical measures were taken. Lymphoedema 1 may be a primary or secondary occurrence following either disease or surgery. Primary lymphoedema is sometimes congenital (Milroy’s disease) but it may also occur at any time of life, particularly puberty. Secondary lymphoedema is the most common form however, and often the result of filariasis, which is particularly common in southeast Asia and Africa. In developed countries, postsurgical lymphoedema of the extremity is common, and complications of chronic limb-lymphoedema include recurrent cellulitis and lymphangiosarcoma. Although lymphangiosarcoma 2 is a recognised compli