Pancreatic glucagonoma with metastasis followed up for 15 years: A case report / 中华内分泌代谢杂志

To explore the clinical characteristics, diagnosis, and management of pancreatic glucagonoma, a retrospective analysis of the clinical data and diagnostic algorithm of a patient with pancreatic glucagonoma was conducted, along with literature review. Pancreatic glucagonoma is a rare neuroendocrine tumor that originates from the pancreatic alpha cells. The main manifestations of glucagonoma syndrome(GS) include necrolytic migratory erythema, diabetes, anemia, and other systemic involvement. Early diagnosis of GS is challenging and crucial. Early identification and recognition of skin lesions contribute to timely diagnosis and treatment of the disease. Surgical resection is an effective treatment modality for glucagonoma.

Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion / 罕见病研究

Glucagonoma is a rare neuroendocrine tumor of α cells of the pancreas. The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema (NME). We reported a patient of glucagonoma syndrome who was presented to the dermatology outpatient clinic with a 2-year-history of recurrent erythema and scaling on the skin migrating throughout the body. A skin biopsy was performed and resulting features matched with NME, whilst imaging examinations suggested a soft tissue density tumor present in the tail of the pancreas with somatostatin receptor expression and laboratory tests found an elevated levels of serum glucagon. After the diagnosis was confirmed, the patient was treated with surgical resection of the glucagonoma and the skin eruptions resolved rapidly in 4 days. Meanwhile, we reviewed relevant literature published in recent years and summarized its clinical characteristics in order to improve its understanding by clinicians, including clinical manifestations, laboratory and imaging examinations, diagnosis and treatments.

Dermatosis paraneoplásica asociada a tumor pancreático neuroendócrino / Paraneoplastic dermatosis associated with pancreatic neuroendocrine tumor

RESUMEN 19- Las dermatosis paraneoplásicas son un grupo heterogéneo de manifestaciones cutáneas que tienen fuerte asociación con patología maligna interna. Su patogenia es poco clara y no se conoce su prevalencia exacta. El tratamiento consiste en el abordaje terapéutico de la enfermedad subyacente. La importancia del conocimiento de las mismas radica en la posibilidad de realizar un diagnóstico temprano de una neoplasia. 20- Se presenta el caso clínico de un paciente con dermatosis paraneoplásica asociada a tumor neuroendócrino de páncreas.

ABSTRACT 24- Paraneoplastic dermatoses are an heterogeneous group of cutaneous manifestations that have a strong association with internal malignancy. Their pathogenesis is unclear and prevalence is unknown. Treatment consists of a therapeutic approach to the underlying disease. The importance of recognizingthem lies in the possibility of making an early diagnosis oftheneoplasm. 25- We report theclinical case of a patient with paraneoplastic dermatosis associated with a pancreatic neuroendocrine tumor.

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.

A retrospective study of 14 cases of glucagonoma with literature review / 中华内分泌代谢杂志

Objective To improve the clinical understanding of glucagonoma. Methods A total of 14 cases of glucagonoma were admitted to our hospital, and the clinical features of these cases were retrospectively analyzed. Results (1) The female/ male case ratio was 1 : 1. (2) The median age of onset was 47 years (range 33-61), while the median age at diagnosis was 50 years (range 33-64). (3) The most common initial presentation was necrolytic migratory erythema (7/ 14, 50％). (4) Hyperglycemia was the most common presentation at the time of diagnosis (14/ 14, 100％), followed by necrolytic migratory erythema (13/ 14, 92.9％). (5) The misdiagnosis rate before admission to our hospital was as high as 85.7％ (12/ 14), and the most common misdiagnosis was eczema (7/ 12, 58.3％). (6) The median time from onset of probable symptoms to diagnosis was 4 years. (7) The median plasma glucagon level at diagnosis was 798 pg/ ml (range 200-2853). (8) The median length of the longest tumor diameter of the pancreatic tumors at diagnosis was 4. 0cm ( range 2. 2-8. 0). ( 9) 57. 1％ of primary pancreatic glucagonomas localized in the tail of the pancreas. (10) 57.1％ of patients (8/ 14) had metastases at the time of diagnosis. Conclusions (1)Necrolytic migratory erythema is a relatively sensitive and specific symptom for diagnosis of glucagonoma. (2) The diagnosis for glucagonoma was often delayed, and misdiagnosis was common. ( 3) Most glucagonoma patients had metastases at the time of diagnosis.

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 / 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. .

Diagnosis and treatment of glucagonoma / 中华消化外科杂志

Glucagonoma is a kind of neuroendocrine tumor of the pancreas,which is rarely seen in clinical practice.Glucagonoma is characterized by necrolytic migratou erythema (NME),diabetes,anemia and body weight loss,and NME is the most characteristic clinical manifestation.The most important laboratory basis of glucagonoma is the levels of fasting plasma glucagon.Various imaging examinations are helpful for the localization of the tumor.Operation is the main method for the treatment of glucagonoma.A patient with glucagonoma was admitted to the Affiliated Hospital of Binzhou Medical College in December 2013,and the experiences in the diagnosis and treatment of glucagonoma were shared based on the data of this patient.

Clinical, pathologic and endoscopic ultrasound characteristics of pancreatic neuroendocrine tumors / 中华胰腺病杂志

Objective To investigate the clinical,pathologic and endoscopic ultrasound characteristics of pancreatic neuroendocrine tumors (PNETs).Methods Clinical data of 24 consecutive patients of PNETs who were admitted between January 2002 and January 2011 were reviewed.Results Among these 24 patients,19 were diagnosed to have insulinomas,1 was malignant insulinoma,2 were gastrinomas,and 2 were glucagonomas.Eighteen (75％) cases of PNETs were functional PNETs,and 6 (25％) were nonfunctional PNETs.The mean age of the patients was (42 ± 14) years old ranging from 19 ～ 64 years old,and the percentage of male patient was 33.3％.The main symptoms of insulinomas were intermittent abdominal pain or discomfort,and the main manifestations of glucagonomas were weight loss,skin migratory erythema; and the main symptoms of gastrinomas were diarrhea,vomiting with large amounts of water-like liquid.The detection rate of CT was 86.7％ (13/15),and the detection rate of EUS was 100％ (15/15),but PET-CT detected only 40％ of tumors (2/5).The endoscopic ultrasound characteristics of PNETs were circular or oval hypoechoic mass,and the volume was small with clear boundary and homogeneous echo.There was no enlarged lymph node,and liquid was detected in big tumor.Twenty-two patients received operation and 2 patients did not.PNETs expressed CgA and Syn protein.All patients of PNETs were alive with 7 to 80 months follow-up.Conclusions The clinical characteristics of PNETs were unique.EUS has a high accuracy for detecting and localizing PNETs.The surgical method is similar to that of pancreatic cancer,and the prognosis is relatively good.

Prolonged Neutropenia after Sunitinib Treatment in a Patient with a Glucagonoma and Multiple Hepatic Metastases / 대한내과학회지

Pancreatic neuroendocrine tumors (PNET) are rare, with approximately 2.2 in 1,000,000 people affected annually. In the classification of neuroendocrine tumors, glucagonomas are a functional PNET and comprise 1.6% of PNET. Glucagonoma syndrome is a paraneoplastic syndrome that is characterized by necrolytic migratory erythema, weight loss, anemia, and diabetes mellitus. Metastatic disease at presentation is common, but is often limited to the liver and regional lymph nodes. Sunitinib malate improves the progression-free and overall survival of PNET. This report presents a 45-year-old Asian woman with prolonged neutropenia after sunitinib treatment of a glucagonoma with multiple hepatic metastases. The severity of the neutropenia after the sunitinib treatment fluctuated from grade 1 to 4 repeatedly, with a non-febrile pattern. Ultimately, the patient did not recover from the neutropenia, even after stopping the sunitinib.

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.

Síndrome do gucagonoma em cão / Glucagonoma syndrome in a dog

O eritema necrolítico migratório é rara dermatose caracterizada por lesões erodo-ulcerativas e crostosas em áreas intertriginosas, junções mucocutâneas, superfícies articulares e de apoio crônico, que tem sido descrita em seres humanos, cães, gatos e rinocerontes. Sua etiologia tem sido relacionada a neoplasias pancreáticas, mormente das células alfa produtoras de glucagon, e a graves doenças do parênquima hepático ou gastroentérico. Relata-se o caso de um cão com necrose epidérmica metabólica secundária a glucagonoma, que apresenta fortes similaridades com a síndrome do glucagonoma em humanos.

Nail fold Necrolytic migratory erythema is a rare skin dermatosis characterized by erosive and crusted lesions in intertriginous areas, mucocutaneous junctions, articular areas and pressure points. It has been described in humans, dogs, cats and rhinoceros. The cause of this condition is related to glucagon-secreting alpha cell tumor of the pancreas, severe hepatopathy or gastrointestinal disease. Here, we report the case of a dog with necrolytic migratory erythema due to glucagonoma, closely resembling the glucagonoma syndrome observed in humans.

A Case of Glucagonoma Syndrome Diagnosed as Necrolytic Migratory Erythema / 대한피부과학회지

Necrolytic migratory erythema (NME) is a typical cutaneous manifestation of glucagonoma syndrome. The entire syndrome consists of NME, glucose intolerance, weight loss, anemia, glossitis, diarrhea, and increased glucagon levels. We herein report a patient with glucagonoma syndrome who was diagnosed as having NME. A 48-year-old male presented with a 2-month history of painful erythematous, desquamative, erosive papules and plaques on both lower extremities. Histological examination revealed an intraepidermal cleft, the presence of vacuolated, pale epidermal cells, and necrosis in the upper epidermis. His glucagon level was 2650 pg/ml, with the upper limit of a normal range being 250 pg/ml. The patient was treated with octreotide, and showed an improvement of the skin eruption with normalization of the glucagon level within 4 weeks.

Glucagonoma: Caso clínico

Objetivos. A propósito de un caso de glucagonoma, describir las principales características clínico-patológicas. Métodos. Se resume la historia clínica de una paciente a quien se le diagnóstico por inmunohistoquímica glucagonoma. Se revisa la literatura. Resultados. Paciente femenina de 54 años de edad, quien consulta por presentar dolor abdominal de moderada intensidad, mal definido, localizado a nivel de epigástrico con irradiación a mesogastrio; concomitantemente presenta pérdida de peso, poliuria, nicturia, estreñimiento severo y cifras de glucemias elevadas. Antecedentes de colecistectomía por litiasis biliar hace 3 años. En el examen físico refirió dolor a la palpación profunda a nivel de epigastrio y mesogastrio, resto del examen físico dentro de la normalidad. El estudio de laboratorio reveló una glucemia en ayunas de 325 mg/dL y postprandial de 531 mg/dL. Se inició tratamiento con insulina sin mejoría del control metabólico, por lo cual se aumento la dosis de insulina asociándola con un sensibilizador de la misma. Se realizó tomografía axial computarizada del abdomen observándose una tumoración ecomixta, bien delimitada, de 55.3 x 54.8 x 51.7 mm, localizada en el abdomen posterior, sin compromiso de la cabeza del páncreas. Debido al mal control metabólico y ante la sospecha de tumor funcionante del páncreas, se planteó la utilización de análogos de somatostatina, sin que pudiera ser utilizado por el costo del medicamento. La paciente fue llevada a quirófano realizándose recesión total de la tumoración. El control metabólico mejoró posterior a la cirugía. Los hallazgos histológicos fueron compatibles con células insulares pancreáticas con inmunohistoquímica positiva para glucagon. Conclusiones. El Glucagonoma es una entidad clínico-patológica poco frecuente pudiéndose presentar como una diabetes. La dificultad para un buen control metabólico de la diabetes y la pérdida incontrolada de peso pueden ser la clave para el diagnóstico.

Objectives. Based on a patient with a glucagonome, the clinical and histopathological characteristics of this tumor are described. Methods. A brief clinical history of a patient who was diagnosed to have a glucagonome, based on immunohistochemical analyses, is presented. Medical literature is reviewed about this entity. Results. A 54 years-old female patient, was admitted because of moderate, abdominal poorly localized pain, at the epigastric level, and referred to the mesogastric area. Concomitantly body-weight loss, polyuria, severe constipation, and hyperglycemia, were present. Cholecistectomy was performed because of cholelithiasis three years prior to admission. On physical examination, she presented pain with deep palpatory maneuvers at the epigastric and mesogastric areas. Without other particular findings. She presented fasting-blood glucose of 325 mg/dL, and 531 mg/dL in postprandial conditions. Insulin treatment was started, without improvement of the metabolic alteration. Consequently, insulin was administered associated with a sensitized, improving the glycemic control. An abdominal TAC was performed, revealing a mixed tumor, located in the posterior abdomen, well delimited, and measuring 55.3 x 54.8 x 51.7 mm. The head of the pancreas was not compromised. Due to difficulties in the control of the metabolic alterations, and under the suspicion of a pancreas functional tumor, it was considered the use of a somatostin analogous, which was never applied because of its high cost. Laparotomy was performed, and a total tumor resection was accomplished. The metabolic conditions improved after surgery. The histopathological findings were compatible with pancreatic insular cells, and immunohistochemically positive for glucagon. Conclusions. The glucagonome is rare clinical and pathological entity. It could be expressed as a diabetes. The difficulties to achieve a good metabolic control of the diabetes, and the continued weight loss, could be the clue for the diagnosis.

Glucagonoma presenting with obstructive jaundice : A case report.

A case report of a 2-cm glucagonoma at head of pancreas in 48-year old woman presented with obstructive jaundice. She had no manifestation of the glucahonoma syndrome. She underwent bylorus preserving pancreaticoduodenectomy (PPPP). Immunohistochemical studies revealed that this tumor was a malignant glucagonoma. She made an uneventful recovery after the operation and was discharged from the hospital without complication. Six months later, she was found to have multiple liver metastases which did not response to chemotherapy and died 11 months after the operation with liver failure.

Malignant Glucagonoma of the Pancreas: A case report / 대한내분비외과학회지

Glucagonomas are rare pancreatic tumors of islet ahpha-2 cells. Less than 430 cases have been reported worldwide and 210 cases are malignant tumors. In generally, the tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema of the skin, weight loss, non-insulin-dependent diabetes mellitus, anemia, cheliosis, stomatitis, and an increased thrombotic tendency. Since pancreatic glucagonomas are predominantly located in the tail and findings of radiographic or sonographic examination can remain unspecific, patients often present already metastasis when diagnosis is first established, and can be difficult to differentiate from the other pancreatic tumors. We report the case of a 59-year-old woman with an malignant glucagonoma of the pancreas infiltrating already the spleen and presenting metastatic lesion in perirenal lymph nodes, and that the tumor was not assocated with the characteristic skin rash. The pateint with a past history of a diabetes mellitus and hypertension for 9 years was admitted with cramp-like left lower abdominal pain, watery diarrhea, and nausea. A solid tumor of tail of the pancreas revealed by ultrasonography and abdominal computed tomography and distal pancreatectomy, radical nephrectomy, and splenectomy were performed. Immunohistochemial examination of the tumor did show glucagon-reactive tissue and electron microscopy revealed many secretory granules, 180 to 300 nm in diameter in granulated cells. After pancreatic tumor resection, the patient had normalization of plasma glucagon and blood sugar.

Islet Cell Tumors of the Pancreas

PURPOSE: Islet cell tumors are a rare disease that can be cured by surgical management if they are early diagnosed. However, diagnosis and localization are difficult due to their small size and varied clinical manifestations. We analyzed the clinicopathologic features, the diagnosis and the surgical management of islet cell tumors. METHODS: We retrospectively analyzed the case histories of 30 patients had undergone pancreatic surgery for islet cell tumors between April 1990 and December 1999. RESULTS: The islet-cell tumors included 16 insulinomas, 4 gastrinomas, 1 glucagonoma, one insulin-gastrin secreting tumor, and 8 nonfunctioning tumors. The major clinical manifestations were neuroglycopenic (94%) and adrenergic (75%) symptoms in cases of an insulinoma, abdominal ulcer symptoms (100%) in the cases of a gastrinoma, diabetis mellitus (100%) in the cases of a glucagonoma, and abdominal pain (63%) and a mass (25%) in nonfunctioning tumor. The preoperative tumor localization tools were angiography, transhepatic portal vein sampling, endoscopic ultrasonography, computed tomography, and octreotide scans which had sensitivities of 56%, 71%, 55.5%, 43.3%, and, 25% respectively. The surgical treatments were enucleation (38%) or segmental resection (25%) for insulinomas, pancreaticoduodenectomy with total gastrectomy (25%) or total pancreatectomy (25%) for gastrinomas, and pylorus preserving pancre aticoduodenectomy (38%) or regional pancreatectomy (26%) for nonfunctioning tumors. Malignant islet cell tumors were presenting cases (30%). Two patients died with postoperative complications on post operative day 3 and 35; the others survived during the follow-up period (1 month-10 years). Islet cell tumors with multiple endocrine neoplasm type I occurred in five (17%) cases; in three cases, the tumors were malignant. CONCLUSION: The early diagnosis and vigorous attempt to resect the lesion in islet cell tumors of the pancreas should be carried out for the long-term survival.

Glucagonoma with multiple hepatic metastasis and necroltic migratory erythema / 대한내과학회지

Glucagoma is rare disease and has been reported only 100 cases so far worldwidely. The experience of this disease in our country is also lacking. The associated symptoms and signs can be characterized by necrolytic migratory erythematous lesion of the skin, hypoaminoacidemia, diabetes, weight loss, anemia, diarrhea, thrombocytopenia, glossitis. Among these, necrolytic migratory erythematous lesion of the skin is particulary considered as specific in this disease entity. In some cases, glucagonoma is diagnosed after metastasis to liver or bone marrow. Our patient initially diagnosed as having multiple metastatic adenocarcinoma of the liver. But subsequent development of the dermatologic manifestation enabled us to consider this rare disease. The constitutional symptoms and skin lesion abates rapidly at receiving Octreotide, somatostatin analogue, whereas no remarkable change observed after administration of the Zinc and aminoacid.