case of gllucagonoma and a review the literature

Glucagonoma is a rare islet cell tumor [alpha cell tumor] of the pancreas, when active it produces a syndrome characterized by necrolytic migratory erythema, diabetes mellitus, weight loss, anemia, glossitis, thromboembolism, neuropsychatric disturbances and hyperglucagonaemia. We present a case of a large pancreatic head tumor with diabetes mellitus [DM], anemia, weight loss and muscle wasting. After complete resection of the pancreatic tumor, the hyperglycaemia subsided. Peviewing 120 cases of glucagonoma in the literature the average tumor diameter was 3.6 cm. Most of the tumors [90%] occurred in the body and the tail. Two thirds of the reported glucagonomas were malignant and 53.3% metastasizes to other organs. Necrolytic migratory erythema [NME] is not a prerequisite for the diagnosis of Glucagonoma syndrome, where anemia, hyperglycemia, muscle wasting and weight loss may be the presenting features

Eritema necrolítico migratorio: a propósito de un caso / Necrolytic migratory erythema: a case report

Presentamos el caso de una paciente de 62 años con un cuadro de tres años de evolución, caracterizado por placas y pápulas eritematosas arciformes que comenzaron en cara, extendiéndose luego al resto del cuerpo, asociado a baja de peso y depresión. Después de un completo estudio y dos biopsias de piel se diagnóstica eritema necrolítico migratorio (ENM). El ENM junto con estomatitis/glositis, baja de peso, diarrea, diabetes y anemia forman el síndrome paraneoplásico asociado al tumor de células a pancreáticas, llamado síndrome del glucagonoma, El ENM corresponde a lesiones maculopapulares, coalescentes, de borde serpiginoso, acompañadas de una bula central que se erosiona y forma costras. La biopsia cutánea muestra hiperplasia psoriasiforme y espongiótica, paraqueratosis y separación de las capas superficiales de la epidermis. La resección del tumor conduce a la resolución del ENM. Presentamos este caso y revisión del tema por la baja frecuencia de esta enfermedad y para reforzar lo importante de su sospecha temprana.

We report the case of o 62 year old woman with a 3 year history of erythematous arciform plaques and papules that began in the face and spread to the rest of the body. These lesions were associated with depression and weight loss. After a comprehensive study and two skin biopsies, necrolytic migratory erythema (NME) was diagnosed together with glossitis/stomatitis, weight loss, diarrhea, diabetes and anemia, NME is part of the paraneoplastic syndrome associated with a cell pancreatic tumor, known as glucagonoma syndrome NME lesions are characterized by a coalescent maculopapular rash with a serpiginous edge and a central bulla that erodes and become crusted. Histological studies show a psoriasiform and spongiotic hyperplasia, porakeratosis, and detachment of the superficial layers of the epidermis. ENM usually resolves after tumor resection. We present this case and a review of the literature because of the low frequency of this disease and to reinforce the importance of its early suspicion.

Glucagonoma syndrome

Glucagonoma syndrome is a rare disease in which necrolytic migratory erythema is often one of the early symptoms. Weight loss and diabetes mellitus are two other characteristics of this syndrome. Necrolytic migratory erythema is typically characterized on skin biopsies by necrolysis of upper epidermis with vacuolated keratinocytes. Persistent hyperglucagonemia in excessive stimulation of basic metabolic pathway which in turn in diabetes mellitus at the expense of tissue glycogen stores. Deficiencies of various essential nutrients and vitamin B lead to dermatosis. Due to rarity of glucagonoma, it is quite difficult to diagnose this syndrome at early stages

Síndrome do glucagonoma: relato de caso / Glucagonoma syndrome: a case report

Relato de caso de síndrome do glucagonoma, cartacterizada por lesöes de pele tipo eritema necrolítico migratório, emagrecimento e piora de diabetes tipo II (preexistente) em um homem de 69 anos. Na suspeita diagnóstica, foi realizada investigaçäo laboratorial e de imagem. Evidenciou-se processo expansivo no pâncreas e foi realizada cirurgia abdominal, sendo detectadas metástases disseminadas. Optou-se por tratamento sintomático, e o paciente evoluiu para óbito em outro serviço. Näo foi realizada necrópsia. Os glucagonoma säo tumores na maioria solitários, sendo o tratamento cirúrgico precoce curativo; entretanto, a maioria é invasiva na ocasiäo do diagnóstico, tornando o prognóstico ruim. Ressaltam-se a raridade da síndrome e a importância do diagnóstico precoce