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1.
Eur J Endocrinol ; 189(6): 575-583, 2023 Dec 06.
Artigo em Inglês | MEDLINE | ID: mdl-38039101

RESUMO

OBJECTIVE: Glucagonoma is a very rare functional pancreatic neuroendocrine tumor (PanNET). We aimed to provide data on the diagnosis, prognosis, and management of patients with glucagonoma. DESIGN AND METHODS: In this retrospective national cohort, we included all patients with glucagonoma, defined by at least 1 major criterion (necrolytic migratory erythema [NME] and/or recent-onset diabetes, and/or weight loss ≥ 5 kg) associated with either glucagonemia > 2 × upper limit of normal or positive glucagon immunostaining. Antisecretory efficacy was defined as partial/complete resolution of glucagonoma symptoms. Antitumor efficacy was assessed according to the time to next treatment (TTNT). RESULTS: Thirty-eight patients were included with median age 58.7 yo, primary PanNET located in the tail (68.4%), synchronous metastases (63.2%). Median Ki-67 index was 3%. Most frequent glucagonoma symptoms at diagnosis were NME (86.8%), weight loss (68.4%), and diabetes (50%). Surgery of the primary PanNET was performed in 76.3% of cases, mainly with curative intent (61.5%). After surgery, complete resolution of NME was seen in 93.8% (n = 15/16). The secretory response rates were 85.7%, 85.7%, 75%, and 60% with surgery of metastases (n = 6/7), chemotherapy (n = 6/7), liver-directed therapy (n = 6/8), and somatostatin analogs (n = 6/10), respectively. All lines combined, longer TTNT was reported with chemotherapy (20.2 months). Median overall survival (OS) was 17.3 years. The Ki-67 index > 3% was associated with shorter OS (hazard ratio 5.27, 95% CI [1.11-24.96], P = .036). CONCLUSION: Patients with glucagonoma had prolonged survival, even in the presence of metastases at diagnosis. Curative-intent surgery should always be considered. Chemotherapy, peptide receptor radionuclide therapy, or liver-directed therapy seems to provide both substantial antitumor and antisecretory efficacies.


Assuntos
Diabetes Mellitus , Neoplasias das Glândulas Endócrinas , Glucagonoma , Eritema Migratório Necrolítico , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Humanos , Pessoa de Meia-Idade , Glucagonoma/diagnóstico , Glucagonoma/terapia , Glucagonoma/complicações , Estudos Retrospectivos , Antígeno Ki-67 , Eritema Migratório Necrolítico/complicações , Eritema Migratório Necrolítico/diagnóstico , Eritema Migratório Necrolítico/tratamento farmacológico , Neoplasias Pancreáticas/diagnóstico , Tumores Neuroendócrinos/complicações , Redução de Peso
3.
J Dermatol ; 47(2): 178-180, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31829458

RESUMO

Autoimmune progesterone dermatitis (APD) is a rare cutaneous disorder with cyclic skin eruptions during the luteal phase of the menstrual cycle. Patients can present with various clinical manifestations, including urticaria and angioedema, erythema multiforme, eczema, fixed drug eruption and centrifugal erythema annulare. In our case, however, the patient's skin lesions mimic necrotic migratory erythema (NME) which is most commonly associated with glucagonoma and rarely with liver disease, inflammatory bowel disease, malnutrition and other tumors. To our knowledge, this is the first case of NME-like APD and is successfully controlled by danazol. This also sheds lights on the etiologic diversity of NME.


Assuntos
Doenças Autoimunes/diagnóstico , Danazol/uso terapêutico , Dermatite/diagnóstico , Antagonistas de Estrogênios/uso terapêutico , Eritema Migratório Necrolítico/diagnóstico , Progesterona/efeitos adversos , Adulto , Doenças Autoimunes/complicações , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Dermatite/complicações , Dermatite/tratamento farmacológico , Dermatite/imunologia , Diagnóstico Diferencial , Feminino , Glucagonoma/complicações , Glucagonoma/diagnóstico , Humanos , Eritema Migratório Necrolítico/tratamento farmacológico , Eritema Migratório Necrolítico/imunologia , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/diagnóstico , Progesterona/imunologia , Pele/imunologia , Pele/patologia , Testes Cutâneos , Resultado do Tratamento
4.
BMJ Case Rep ; 12(8)2019 Aug 10.
Artigo em Inglês | MEDLINE | ID: mdl-31401568

RESUMO

A 70-year-old man reported progressive weight loss, fatigue and a generalised rash. The rash was consistent with necrolytic migratory erythema, further investigations were performed and the patient was diagnosed with a mass in the tail of the pancreas, in keeping with a localised glucagonoma. Somatostatin analogue therapy was started for symptom control, leading to complete resolution of the skin rash and an improvement in constitutional symptoms. Subsequently, the pancreatic lesion was excised, and pathology assessment confirmed the diagnosis of well-differentiated neuroendocrine tumour with high expression of glucagon compatible with glucagonoma.


Assuntos
Glucagonoma/diagnóstico , Eritema Migratório Necrolítico/tratamento farmacológico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Somatostatina/análogos & derivados , Idoso , Glucagonoma/complicações , Glucagonoma/cirurgia , Humanos , Injeções , Masculino , Eritema Migratório Necrolítico/etiologia , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/complicações , Neoplasias Pancreáticas/cirurgia , Somatostatina/administração & dosagem
5.
Int J Dermatol ; 57(6): 642-645, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29450880

RESUMO

Necrolytic migratory erythema (NME) is a rare skin disorder that is a cutaneous manifestation of the glucagonoma syndrome. It presents with annular eruptions of migrating erythematous papules and plaques with superficial epidermal necrosis, central flaccid bullae, and crusted erosions located primarily in the intertriginous areas. Treatment with the long-acting somatostatin analog Octreotide is a potential therapy to help ameliorate skin symptoms. We present a case of a patient with a 1-year history of a pancreatic glucagonoma that developed an ulcerated, plaque-like, weeping rash over multiple areas of their body despite current treatment with Octreotide and stable pancreatic tumor staging. The patient had a similar rash when initially diagnosed with a glucagonoma, and it quickly improved after Octreotide treatment. Clinical examination and biopsy were consistent with necrolytic migratory erythema due to an underlying glucagonoma. This rare case adds to our understanding of the clinical presentation of NME, as well as highlights the relapsing and remitting course, even if the underlying pancreatic tumor is stable and the patient is undergoing treatment.


Assuntos
Glucagonoma/complicações , Eritema Migratório Necrolítico/etiologia , Neoplasias Pancreáticas/complicações , Fármacos Dermatológicos/uso terapêutico , Feminino , Seguimentos , Glucagonoma/diagnóstico , Glucagonoma/terapia , Humanos , Pessoa de Meia-Idade , Monitorização Fisiológica/métodos , Eritema Migratório Necrolítico/tratamento farmacológico , Eritema Migratório Necrolítico/fisiopatologia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/terapia , Medição de Risco
8.
Clin J Gastroenterol ; 7(3): 255-9, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-26183746

RESUMO

Necrolytic migratory erythema (NME) is a classical paraneoplastic symptom observed in patients with pancreatic glucagonoma. We report a 46-year-old Japanese woman with glucagonoma who presented with mucocutaneous manifestations 1 year prior to the diagnosis of the pancreatic neoplasm with multiple liver metastases. She was treated with octreotide long-acting release, a somatostatin analog, which resulted in a dramatic improvement of NME within 2 weeks after the start of treatment. Increased awareness of NME may avoid unnecessary delay in the diagnosis of pancreatic glucagonoma.


Assuntos
Antineoplásicos Hormonais/uso terapêutico , Glucagonoma/complicações , Eritema Migratório Necrolítico/tratamento farmacológico , Eritema Migratório Necrolítico/etiologia , Octreotida/uso terapêutico , Neoplasias Pancreáticas/etiologia , Feminino , Humanos , Pessoa de Meia-Idade , Indução de Remissão , Fatores de Tempo
9.
Can Vet J ; 54(9): 873-5, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24155493

RESUMO

A 9-year-old castrated male Shetland sheepdog was diagnosed with necrolytic migratory erythema and hepatocutaneous syndrome. Necrolytic migratory erythema was treated with intermittent intravenous amino acids as needed to control cutaneous lesions. The addition of lipid infusions extended the treatment interval. The patient had a favorable response for 24 months.


Cas d'érythème migratoire nécrolytique géré pendant 24 mois à l'aide d'infusions intraveineuses d'amino-acides et de lipides. Un chien berger Shetland mâle castré âgé de 9 ans a été diagnostiqué avec un érythème migratoire nécrolytique et un syndrome hépatocutané. L'érythème migratoire nécrolytique a été traité par des amino-acides intraveineux intermittents au besoin pour contrôler les lésions cutanées. L'ajout d'infusions de lipides a prolongé l'intervalle de traitement. Le patient a manifesté une réponse favorable pendant 24 mois.(Traduit par Isabelle Vallières).


Assuntos
Aminoácidos/uso terapêutico , Doenças do Cão/terapia , Emulsões Gordurosas Intravenosas/uso terapêutico , Eritema Migratório Necrolítico/veterinária , Aminoácidos/administração & dosagem , Animais , Neoplasias Ósseas/patologia , Neoplasias Ósseas/veterinária , Cães , Emulsões Gordurosas Intravenosas/administração & dosagem , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/veterinária , Masculino , Eritema Migratório Necrolítico/tratamento farmacológico , Sarcoma/patologia , Sarcoma/veterinária
12.
Vet Dermatol ; 21(5): 510-6, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20500495

RESUMO

Necrolytic migratory erythema (NME; also known as superficial necrolytic dermatitis) is a syndrome most often associated with certain chronic liver diseases or pancreatic glucagonomas. In humans with glucagonoma-associated NME, skin lesions usually respond to octreotide, a somatostatin analogue that inhibits glucagon release. In this report an 11-year-old golden retriever dog with pancreatic glucagonoma and metastasis to the regional lymph nodes, spleen and liver was diagnosed with NME. The dog exhibited erosions, ulcers and crusts on the paws, pressure points, muzzle, periocular area and prepuce. The dog was also anorexic and had difficulty walking. Because metastasis precluded surgery, treatment was initiated with subcutaneous octreotide (2 µg/kg twice daily). Skin lesions and systemic clinical signs improved markedly within 5 days. The dosage was increased to nearly 3 µg/kg twice daily and signs almost completely resolved within 10 days. Anorexia was the major adverse effect observed. During the following month, both dosage (1-3.7 µg/kg) and frequency (two to four times daily) of the octreotide injections were adjusted to permit control of clinical signs while maintaining adequate appetite. Temporary cessation of octreotide administration resulted in the rapid recurrence of skin lesions. Resuming injections led to improvement of clinical signs within 48 h. The dog was later euthanized because of progressive metastatic disease. In conclusion, subcutaneous octreotide injections were beneficial in this dog with glucagonoma-associated NME. This somatostatin analogue could be a valuable option to treat canine patients with non-resectable or relapsing pancreatic glucagonoma-associated NME.


Assuntos
Doenças do Cão/tratamento farmacológico , Glucagonoma/veterinária , Eritema Migratório Necrolítico/veterinária , Octreotida/uso terapêutico , Neoplasias Pancreáticas/veterinária , Animais , Anorexia/induzido quimicamente , Anorexia/veterinária , Antineoplásicos Hormonais/administração & dosagem , Antineoplásicos Hormonais/efeitos adversos , Antineoplásicos Hormonais/uso terapêutico , Cães , Relação Dose-Resposta a Droga , Glucagonoma/complicações , Glucagonoma/patologia , Linfonodos/patologia , Masculino , Eritema Migratório Necrolítico/tratamento farmacológico , Octreotida/administração & dosagem , Octreotida/efeitos adversos , Neoplasias Pancreáticas/complicações , Síndromes Paraneoplásicas/patologia , Síndromes Paraneoplásicas/veterinária
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