Glucagonoma syndrome associated with necrolytic migratory erythema

Summary Introduction: glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with area...

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Main Authors: Florentino de Araújo Cardoso Filho, Roney Gonçalves Fechine Feitosa, Carolina Oliveira Costa Fechine, Carlos Márcio Melo de Matos, Amanda Linhares Cardoso, Daniel Linhares Cardoso
Format: Article
Language:English
Published: Associação Médica Brasileira 2015-06-01
Series:Revista da Associação Médica Brasileira
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0104-42302015000300203&lng=en&tlng=en
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spelling doaj-8e5d386e8b0c4f4c989364c9ccb3a5772020-11-24T22:04:10ZengAssociação Médica BrasileiraRevista da Associação Médica Brasileira1806-92822015-06-0161320320610.1590/1806-9282.61.03.203S0104-42302015000300203Glucagonoma syndrome associated with necrolytic migratory erythemaFlorentino de Araújo Cardoso FilhoRoney Gonçalves Fechine FeitosaCarolina Oliveira Costa FechineCarlos Márcio Melo de MatosAmanda Linhares CardosoDaniel Linhares CardosoSummary Introduction: glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with areas of necrosis and sloughing. This is a rare disease with worldwide incidence estimated at 1 case per 20 million people. Case report: we report a case of glucagonoma associated necrolytic migratory erythema in a male patient, 56 years, with signs of skin lesions mainly on his legs and groin, hyperglycemia and weight loss. Biopsies of the skin lesions were performed and imaging of the abdomen showed a mass of 10 x 9 cm, at the pancreatic region. The patient was subjected to body-caudal pancreatectomy and splenectomy with autotransplant of the spleen in the greater omentum. The histopathologic report indicated a tumor in the pancreatic alpha cells. Immunohistochemistry showed expression of glucagon and chromogranin A in most tumor cells, consistent with the diagnosis of glucagonoma. The patient presented 3 years of outpatient follow-up with no complications. Conclusion: the necrolytic migratory erythema is important for the clinical recognition of glucagonoma, and its early diagnosis is essential for a successful curative therapy.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0104-42302015000300203&lng=en&tlng=eneritema migratório necrolíticoglucagonomaneoplasias pancreáticastumores neuroendócrinos
collection DOAJ
language English
format Article
sources DOAJ
author Florentino de Araújo Cardoso Filho
Roney Gonçalves Fechine Feitosa
Carolina Oliveira Costa Fechine
Carlos Márcio Melo de Matos
Amanda Linhares Cardoso
Daniel Linhares Cardoso
spellingShingle Florentino de Araújo Cardoso Filho
Roney Gonçalves Fechine Feitosa
Carolina Oliveira Costa Fechine
Carlos Márcio Melo de Matos
Amanda Linhares Cardoso
Daniel Linhares Cardoso
Glucagonoma syndrome associated with necrolytic migratory erythema
Revista da Associação Médica Brasileira
eritema migratório necrolítico
glucagonoma
neoplasias pancreáticas
tumores neuroendócrinos
author_facet Florentino de Araújo Cardoso Filho
Roney Gonçalves Fechine Feitosa
Carolina Oliveira Costa Fechine
Carlos Márcio Melo de Matos
Amanda Linhares Cardoso
Daniel Linhares Cardoso
author_sort Florentino de Araújo Cardoso Filho
title Glucagonoma syndrome associated with necrolytic migratory erythema
title_short Glucagonoma syndrome associated with necrolytic migratory erythema
title_full Glucagonoma syndrome associated with necrolytic migratory erythema
title_fullStr Glucagonoma syndrome associated with necrolytic migratory erythema
title_full_unstemmed Glucagonoma syndrome associated with necrolytic migratory erythema
title_sort glucagonoma syndrome associated with necrolytic migratory erythema
publisher Associação Médica Brasileira
series Revista da Associação Médica Brasileira
issn 1806-9282
publishDate 2015-06-01
description Summary Introduction: glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with areas of necrosis and sloughing. This is a rare disease with worldwide incidence estimated at 1 case per 20 million people. Case report: we report a case of glucagonoma associated necrolytic migratory erythema in a male patient, 56 years, with signs of skin lesions mainly on his legs and groin, hyperglycemia and weight loss. Biopsies of the skin lesions were performed and imaging of the abdomen showed a mass of 10 x 9 cm, at the pancreatic region. The patient was subjected to body-caudal pancreatectomy and splenectomy with autotransplant of the spleen in the greater omentum. The histopathologic report indicated a tumor in the pancreatic alpha cells. Immunohistochemistry showed expression of glucagon and chromogranin A in most tumor cells, consistent with the diagnosis of glucagonoma. The patient presented 3 years of outpatient follow-up with no complications. Conclusion: the necrolytic migratory erythema is important for the clinical recognition of glucagonoma, and its early diagnosis is essential for a successful curative therapy.
topic eritema migratório necrolítico
glucagonoma
neoplasias pancreáticas
tumores neuroendócrinos
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0104-42302015000300203&lng=en&tlng=en
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