Glucagonoma del páncreas: la piel puede llevarnos al diagnóstico / Pancreatic glucagonoma: observing the skin can lead to diagnosis

Resumen Presentamos el caso de una paciente que tenía un tumor del páncreas -denominado glucagonoma- y cuyo diagnóstico se sospechó por las manifestaciones cutáneas, las cuales nos condujeron realizar una tomografía axial computarizada (TAC). En ella se halló una masa. La paciente se remitió a cirugía y presentó una buena evolución.

Abstract This is a case report of a patient with a pancreatic tumor, known as glucagonoma, whose diagnosis was suspected because of skin manifestations which led to performing a CT scan, finding the mass. She underwent surgery with satisfactory results.

Sweet´s syndrome and pancreatic neoplasm: an atypical association

El síndrome de Sweet es una dermatosis inflamatoria poco común, que se ha asociado a tumores malignos, principalmente de tipo hematológico. Presentamos un caso clínico de síndrome de Sweet asociado con una rara neoplasia pancreática, siendo uno de los pocos casos reportados en la literatura médica acerca de esta asociación.

Sweet's syndrome is an uncommon inflammatory dermatosis, which has been associated with malignant tumors, mainly of hematological type. We report a clinical case of Sweet syndrome associated with a rare pancreatic neoplasm, which is one of the few cases reported in the medical literature about this association.

Necrolytic migratory erythema in a cat with glucagonoma syndrome

Background: Glucagonomas are very rare neuroendocrine tumors of pancreatic endocrine islets alpha cells and they produced excessive amount of glucagon hormone. Necrolytic migratory erythema (NME) is a rare dermatosis that characterized by erosive, ulcerative and crusted lesions in different sites of the skin and the common cause of this situation related to glucagon secreted tumors. NME can occur commonly in man but some rare and recent reports available the occurrence of this situation in pet animals especially dogs. Both gross and histological findings in both human and animals are similar. This paper reports a NME case with glucagonoma and diabetes mellitus (DM) by clinical, histopathological and immunohistochemical examinations. Case: A 12-year-old, cat presented with complaints of skin lesions in neck region, hyperglycemia, weight loss and history of anorexia during the 2 months. Biochemical analysis results revealed high glucose, aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP) and creatinine levels but decreased blood urea nitrogen (BUN), total protein and potassium levels. After 2 weeks of anti-diabetic drug treatment, the blood glucose level became normal and skin lesions ameliorated but anorexia and weight loss continued. The cat exhibited general weakness and pain in abdominal area. Although the clinical sings ameliorated and skin lesions and serum biochemical findings returned the relatively normal levels compared the admission, the cat died after 2 weeks of treatment and necropsy performed. At necropsy, marked cachexia, loss of skin elasticity and decreased skin thickness were observed. During the examination of abdominal cavity of the cat, there was a mass 0.5x0.5 cm in diameter, hard and grayish color was diagnosed at the pancreas...

Necrolytic migratory erythema in a cat with glucagonoma syndrome

Background: Glucagonomas are very rare neuroendocrine tumors of pancreatic endocrine islets alpha cells and they produced excessive amount of glucagon hormone. Necrolytic migratory erythema (NME) is a rare dermatosis that characterized by erosive, ulcerative and crusted lesions in different sites of the skin and the common cause of this situation related to glucagon secreted tumors. NME can occur commonly in man but some rare and recent reports available the occurrence of this situation in pet animals especially dogs. Both gross and histological findings in both human and animals are similar. This paper reports a NME case with glucagonoma and diabetes mellitus (DM) by clinical, histopathological and immunohistochemical examinations. Case: A 12-year-old, cat presented with complaints of skin lesions in neck region, hyperglycemia, weight loss and history of anorexia during the 2 months. Biochemical analysis results revealed high glucose, aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP) and creatinine levels but decreased blood urea nitrogen (BUN), total protein and potassium levels. After 2 weeks of anti-diabetic drug treatment, the blood glucose level became normal and skin lesions ameliorated but anorexia and weight loss continued. The cat exhibited general weakness and pain in abdominal area. Although the clinical sings ameliorated and skin lesions and serum biochemical findings returned the relatively normal levels compared the admission, the cat died after 2 weeks of treatment and necropsy performed. At necropsy, marked cachexia, loss of skin elasticity and decreased skin thickness were observed. During the examination of abdominal cavity of the cat, there was a mass 0.5x0.5 cm in diameter, hard and grayish color was diagnosed at the pancreas...(AU)

Necrolytic migratory erythema and pancreatic glucagonoma.

Necrolytic migratory erythema is a rare paraneoplastic dermatosis that may be the first clinical manifestation of the glucagonoma syndrome, a disorder characterized by mucocutaneous rash, glucose intolerance, hypoaminoacidemia, hyperglucagonaemia and pancreatic glucagonoma. The clinical case of a 45-year-old woman is presented. She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. Erythematous, scaly and migratory plaques with 20 days of evolution were found on her trunk, perineum, elbows, hands, feet, inframammary and antecubital folds. The skin biopsy revealed noticeable vacuolar changes in high epidermal cells, extensive necrosis and thin orthokeratotic cornified layer. These findings pointed to a diagnosis of necrolytic migratory erythema. A suggestion was made to investigate a pancreatic glucagonoma. Laboratory tests showed moderate anemia, hyperglycemia and marked hyperglucagonaemia. Abdominal ultrasound revealed a mass in the tail of the pancreas measuring 6 x 5 x 5 cm which was resected. The histopathological findings were compatible with a diagnosis of glucagonoma, as confirmed by immunohistochemistry. Skin symptoms disappeared 10 days after the tumor resection. We can conclude that the histological changes defined may be clues that can lead the search for a distant skin disease and allow for its diagnosis. The histological pattern of vacuolation and epidermal necrosis should arouse suspicion of pancreatic glucagonoma.

Eritema necrolítico migratorio y glucagonoma pancreático / Necrolytic migratory erythema and pancreatic glucagonoma

El eritema necrolítico migratorio es una dermatosis paraneoplásica infrecuente que puede ser la primera manifestación clínica del glucagonoma, el cual se caracteriza por erupción mucocutánea, intolerancia a la glucosa, hipoaminoacidemia, hiperglucagonemia y glucagonoma pancreático. Se presenta el caso clínico de una mujer de 45 años que presentó pérdida de peso, polidipsia, polifagia, vómito posprandial, caída abundante del cabello y dolor abdominal de dos meses de evolución. Tenía, además, placas eritematosas, descamativas y migratorias en tronco, periné, codos, manos, pies, pliegues submamarios y antecubitales de 20 días de evolución. En la biopsia de piel se observaron células epidérmicas altas con cambio vacuolar notorio, extensa necrosis y delgada capa córnea ortoqueratósica, hallazgos interpretados como eritema necrolítico migratorio. Se sugirió investigar un glucagonoma pancreático. En los exámenes de laboratorio se encontró anemia moderada, hiperglucemia e importante hiperglucagonemia. La ecografía abdominal reveló una masa de 6 x 5 x 5 cm en la cola pancreática, la cual fue resecada. El diagnóstico histopatológico fue de glucagonoma confirmado por inmunohistoquímica. Los síntomas cutáneos desaparecieron a los 10 días de la resección tumoral. Se concluye que los cambios histológicos observados pueden ser claves en la búsqueda de una enfermedad distante de la piel y permiten hacer su diagnóstico. El patrón histológico de vacuolización y necrosis epidérmica subcórnea debe llevar a sospechar la presencia de un glucagonoma pancreático.

Necrolytic migratory erythema is a rare paraneoplastic dermatosis that may be the first clinical manifestation of the glucagonoma syndrome, a disorder characterized by mucocutaneous rash, glucose intolerance, hypoaminoacidemia, hyperglucagonaemia and pancreatic glucagonoma. The clinical case of a 45-year-old woman is presented. She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. Erythematous, scaly and migratory plaques with 20 days of evolution were found on her trunk, perineum, elbows, hands, feet, inframammary and antecubital folds. The skin biopsy revealed noticeable vacuolar changes in high epidermal cells, extensive necrosis and thin orthokeratotic cornified layer. These findings pointed to a diagnosis of necrolytic migratory erythema. A suggestion was made to investigate a pancreatic glucagonoma. Laboratory tests showed moderate anemia, hyperglycemia and marked hyperglucagonaemia. Abdominal ultrasound revealed a mass in the tail of the pancreas measuring 6 x 5 x 5 cm which was resected. The histopathological findings were compatible with a diagnosis of glucagonoma, as confirmed by immunohistochemistry. Skin symptoms disappeared 10 days after the tumor resection. We can conclude that the histological changes defined may be clues that can lead the search for a distant skin disease and allow for its diagnosis. The histological pattern of vacuolation and epidermal necrosis should arouse suspicion of pancreatic glucagonoma.

Glucagonoma syndrome associated with necrolytic migratory erythema.

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.

Glucagonoma syndrome associated with necrolytic migratory erythema / Síndrome do glucagonoma associado a eritema necrolítico migratório

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. .

Resumo Introdução: Introdução: o glucagonoma é um tumor neuroendócrino do pâncreas derivado das células alfa das ilhotas de Langerhans. É marcado pela produção tumoral autônoma de glucagon e caracterizado, dentre outros sintomas, por eritema necrolítico migratório (ENM), uma lesão eritematosa circinada com áreas de necrose e descamação. Trata-se de uma doença rara com incidência mundial estimada em 1 caso para cada 20 milhões pessoas. Relato de caso: apresentamos um caso de glucagonoma associado a ENM em um paciente de sexo masculino, 56 anos de idade, com quadro de lesões cutâneas, principalmente em membros inferiores e região inguinal, hiperglicemia e perda ponderal. Biópsias das lesões cutâneas foram realizadas e exames de imagem do abdome evidenciaram uma massa de 10 x 9 cm em região pancreática. O paciente foi submetido à pancreatectomia corpocaudal e esplenectomia total com autoimplante do baço em omento maior. O laudo histopatológico foi de tumor de células alfa pancreáticas. Imuno-histoquímica evidenciou expressão de glucagon e cromogranina A na maioria das células tumorais, compatível com diagnóstico de glucagonoma. O paciente apresentou seguimento de 3 anos em ambulatório sem intercorrências clínicas. Conclusão: o ENM é importante para o reconhecimento clínico do glucagonoma, sendo seu diagnóstico precoce fundamental para uma terapia curativa de sucesso. .

Tumores neuroendocrinos del páncreas / Pancreatic neuroendocrine tumors

Pancreatic neuroendocrine tumors are infrequent and slow-growing neoplasms. They are classified basedon their clinical presentation as functioning and nonfunctioning tumors. The most common functionaltumors are the insulinoma and gastrinoma. They can be sporadic or be part of hereditary forms as MEN-1.The diagnosis is based on the detection of the specific clinical syndrome in association of high levels of the substance secreted by the tumor and conventional imaging studies or others such as stimulation tests, somatostatin receptor scintigraphy and endoscopic ultrasound. In general, these tumors have a better prognosis than the pancreatic adenocarcinoma and they can show metastasis to the liver and infrequently, in bones. The treatment can be managed medically diminishing the inappropriate secretion of the substances by the tumor using, for example, the somatostatin analogs. Surgery should be always considered, especially in case of insulinomas, small non-functioning tumors, and small gastrinomas that can be managed with surgery enucleation. More advanced resective surgery, such as Whipple resection, are not routinely recommended and they should be limited to selected patients. In advanced tumors, there are other treatment alternatives, for example, hepatic resection, radiofrequency, chemotherapy and new agents such as sunitinib and everolimus.

Los tumores neuroendocrinos pancreáticos son infrecuentes y de crecimiento lento. Se clasifican en tumores funcionantes o no funcionantes (TNEP-NF), de acuerdo a la presentación clínica. Los tumores funcionantes más frecuentes son los insulinomas y los gastrinomas. Pueden ocurrir en forma esporádica o asociados a síndromes hereditarios como la NEM- 1, entre otros. El diagnóstico se basa en la detección del síndrome clínico específico asociado a la demostración de niveles elevados de la sustancia secretada y exámenes imagenológicos convencionales u otros más específicos como de estimulación, cintigrafía de receptores de somatostatina y endosonografía. En general, tienen mejor pronóstico que los adenocarcinomas pancreáticos y pueden dar metástasis hepáticas y con menor frecuencia, óseas. El tratamiento puede ser médico disminuyendo la secreción inapropiada de las sustancias producidas por el tumor como los análogos de somatostatina. La cirugía siempre debe ser considerada, especialmente en caso de insulinomas, pequeños TNEP-NF, y gastrinomas pequeños, que pueden ser tratados con enucleación quirúrgica. Las cirugías resectivas más avanzadas, como la operación de Whipple no están recomendadas en forma rutinaria y sólo deben ser utilizadas en pacientes seleccionados. En casos de tumores avanzados, existen alternativas de tratamiento, como la resección hepática, radiofrecuencia, quimioterapia, y terapia con nuevos agentes en estudio como el sunitinib y everolimus.

Eritema necrolítico migratório (dermatite necrolítica superficial)em uma cadela diabética: síndorme do glucagonoma? / Necrolitic migratory erithema (superficial necrolytic dermatitis) in a diabetic bitch: glucagonoma syndrome?

O eritema necrolítico migratório é uma síndrome cutânea rara em humanos e cães muitas vezes associadaà presença de tumores pancreáticos produtores de glucagon. Pacientes com esta síndrome apresentamfrequentemente, além das alterações dermatológicas associadas à necrose epidérmica, a perda de peso e o diabetes mellitus, como consequência da hiperglucagonemia. Apenas um caso de síndrome do glucagonoma canino foi relatado até o momento na literatura nacional e aproximadamente dez casosexistem na literatura mundial. Relatamos um caso de eritema necrolítico migratório em uma cadeladiabética da raça Cocker Spaniel com oito anos de idade e quadro clínico compatível com a síndromedo glucagonoma.

The necrolytic migratory erythema is a rare skin syndrome in humans and dogs often associated with the presence of glucagon-producing pancreatic tumors. Patients with this syndrome develop epidermal necrosis generally associated with weight loss and diabetes mellitus as a consequence of hyperglucagonemia.Only one case of canine glucagonoma syndrome has been reported so far in the nacional literature and there are about ten cases all over the world. We report a case of necrolytic migratory erythema in an eight year-old, female, diabetic Cocker Spaniel dog with a clinical history compatible withthe glucagonoma syndrome.

Eritema necrolítico migratório (dermatite necrolítica superficial)em uma cadela diabética: síndorme do glucagonoma? / Necrolitic migratory erithema (superficial necrolytic dermatitis) in a diabetic bitch: glucagonoma syndrome?

O eritema necrolítico migratório é uma síndrome cutânea rara em humanos e cães muitas vezes associadaà presença de tumores pancreáticos produtores de glucagon. Pacientes com esta síndrome apresentamfrequentemente, além das alterações dermatológicas associadas à necrose epidérmica, a perda de peso e o diabetes mellitus, como consequência da hiperglucagonemia. Apenas um caso de síndrome do glucagonoma canino foi relatado até o momento na literatura nacional e aproximadamente dez casosexistem na literatura mundial. Relatamos um caso de eritema necrolítico migratório em uma cadeladiabética da raça Cocker Spaniel com oito anos de idade e quadro clínico compatível com a síndromedo glucagonoma. (AU)

The necrolytic migratory erythema is a rare skin syndrome in humans and dogs often associated with the presence of glucagon-producing pancreatic tumors. Patients with this syndrome develop epidermal necrosis generally associated with weight loss and diabetes mellitus as a consequence of hyperglucagonemia.Only one case of canine glucagonoma syndrome has been reported so far in the nacional literature and there are about ten cases all over the world. We report a case of necrolytic migratory erythema in an eight year-old, female, diabetic Cocker Spaniel dog with a clinical history compatible withthe glucagonoma syndrome. (AU)

Pancreatic glucagonoma presenting as a pulmonary mass.

Glucagonoma is an uncommon disease, a neuroendocrine tumour that develops from glucagon-producing pancreatic cells. They are usually slow-growing, but generally advanced at diagnosis, and metastatic disease is virtually incurable. Liver is the most common site of metastatic disease. We present the case of a 48-year-old man with a glucagonoma being diagnosed from a pulmonary mass. This case had no liver affection in the whole evolution of the disease, and showed a particularly aggressive course, with very little response to all therapies administered, and a survival from diagnosis of just 16 months.

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.

Cervical metastases of glucagonoma in a patient with multiple endocrine neoplasia type 1: report of a case.

Multiple endocrine neoplasia type 1 (MEN 1) is a syndrome characterized by tumors of the parathyroid glands, pancreatic islet cells, duodenum, and pituitary gland. We report a case of cervical metastases of glucagonoma with MEN 1. The patient was a 34-year-old woman admitted to our hospital with epigastric pain. Her medical history included two resections of prolactinoma and two upper GI hemorrhages secondary to duodenal ulcers. Computed tomography (CT) showed two hypervascular lesions in the tail of the pancreas and cervical ultrasound showed multiple hypoechogenic ovoid images in the neck. A cervical CT scan confirmed two 15-mm lymph nodes in the left cervical region and 111In-DOTATOC imaging showed focal abnormal somatostatin expression in the pancreatic tail and the cervical nodes. The patient had asymptomatic hypoglycemic episodes, with blood sugar levels as low as 30 mg/dl, which raised our suspicion of MEN 1 associated with pancreatic insulinoma. Thus, we performed a distal pancreatectomy with bilateral cervical dissection and parathyroid gland resection. Histopathological examination revealed 12 pancreatic tumors as well as metastases in four cervical lymph nodes. The resected parathyroid glands had normal structure, suggesting parathyroid hyperplasia. A follow-up CT scan, 18 months after surgery, showed new tumors in the head of the pancreas and in the duodenal wall. A pancreatoduodenectomy was performed and histopathological examination revealed nine nonfunctioning endocrine tumors in the pancreas, one tumor in the duodenal wall, and metastases in two peripancreatic lymph nodes. The patient recovered well and remains asymptomatic.

Pancreatic neuroendocrine tumours.

INTRODUCTION: Pancreatic neuroendocrine tumours (PNT) are infrequent epithelial neoplasms associated with a better outcome than pancreatic adenocarcinoma. MATERIALS AND METHODS: We analysed our 22 years of experience in managing PNT. Forty-nine patients (27 women and 22 men) with a mean age of 49 years were studied. There were 28 insulinomas, eight glucagonomas, three gastrinomas, one VIPoma and one carcinoid. Eight patients presented with nonfunctional tumours. Enucleation was performed in 20 patients, distal pancreatectomy in 16, middle pancreatic resection in four, cephalic pancreatoduodenectomy in two and total pancreatoduodenectomy in one. In six patients, the tumour was not resected. RESULTS: Postoperative complication rate was 22%: six pancreatic fistulas, three intra-abdominal collections, one remnant pancreatitis and one pancreatic pseudocyst. There was no mortality. 39 cases showed benign histologic features and ten malignant ones. Symptomatic palliation was achieved in 94% of the cases. Five patients presented recurrences: three liver metastases and two pancreatic recurrences. Actuarial mean survival was 163 months and was longer in insulinomas, in those tumours completely resected and in tumours with benign histological features. CONCLUSION: Conservative surgery of the pancreas is preferred, but aggressive surgery is indicated when the primary tumour can be controlled. Despite of minimising pancreatic resection, there is a high complication rate, mainly pancreatic fistulas, though they can often be conservatively managed. Insulinomas are the PNT with better outcome; those completely resected also associate a better prognosis.

Manifestaciones anatomoclínicas de las enfermedades cutáneas metabólicas y sistémicas misceláneas: Parte II / Anatomoclinical manifestations of metabolic and systemic diseases: Part II

Numerosas enfermedades sistémicas y metabólicas tienen manifestaciones cutáneas, muchas de estas manifestaciones pueden favorecer su diagnóstico. Dado el gran número de estas patologías, esta revisión no pretende ser un análisis exhaustivo de todas ellas, sino que presenta un análisis clínico-patológico de algunas enfermedades metabólicas y sistémicas seleccionadas.

Numerous systemic and metabolic diseases have coetaneous manifestations, many of these manifestations can favor diagnosis Due to the great number of these conditions, this review does not try to be a comprehensive analysis of all of them, but present a clinicopathological analysis of some selected metabolic and systemic diseases.

Glucagonoma with two pancreatic masses and pulmonary metastases as debut of MEN-1.

This is a rare case of a patient with type 1 multiple endocrine neoplasia (MEN-1) syndrome. The case is further unusual in that the glucagonoma debuted with two synchronic pancreatic masses at the time of diagnosis and with pulmonary metastases as the primary site of metastasis and not the more usual site of the liver.