ABSTRACT
Resumen Se describe el caso de un paciente de 75 años sin antecedentes de relevancia, que fue diagnosticado con insulinoma maligno en estado avanzado, con metástasis hepáticas, con síntomas por hipoglucemia hiperinsulinémica refractaria al tratamiento, y con diazóxido y octreotide de acción corta. El paciente presentó una respuesta clínica poco esperada a la embolización transarterial de metástasis hepáticas, pues a pesar de que persisten las lesiones tumorales, desarrolló hiperglucemia persistente y requirió manejo con insulina. Adicionalmente, se hace una breve revisión de la literatura sobre las opciones terapéuticas disponibles para el tratamiento sintomático de la hipoglucemia hiperinsulinémica.
Abstract The case is presented of a 75 year-old man who was diagnosed with malignant insulinoma in an advanced stage with diffuse liver metastases and symptoms due to hyperinsulinaemic hypoglycaemia refractory to treatment with diazoxide and short-acting octreotide. The patient had an unexpected clinical response to trans-arterial embolisation of liver metastases, since, despite still having the tumour, he developed persistent hyperglycaemia that required insulin treatment. A brief review of the literature is also presented on the treatment options for hyperinsulinaemic hypoglycaemia.
Subject(s)
Humans , Male , Aged , Hyperglycemia , Hypoglycemia , Insulinoma , Neoplasm MetastasisABSTRACT
Introducción: los insulinomas son tumores neuroendocrinos pancreáticos secretores de insulina, la incidencia en la población en general estimada es de 1 a 4 casos en un millón de habitantes por año. Los insulinomas constituyen el 60% de los tumores de páncreas, de los cuales el 90% son benignos y únicos; el 10% son múltiples, la mayoría asociados a MEN 1 (Neoplasia Endocrina Múltiple) y del 5 al 10% son malignas. La posibilidad de recidiva obliga a un diagnóstico y seguimiento a largo plazo. La característica clínica del insulinoma es la hipoglucemia en ayunas, con signos y síntomas de neuroglucopenia, que pueden estar precedidos o no por manifestaciones adrenérgicas. El tratamiento de elección es el quirúrgico; en etapas irresecables o avanzadas se proponen tratamientos paliativos o sintomáticos con quimioterapia o procedimientos mediante radiología intervencionista con pobres respuestas. Se ha demostrado que con nuevos tratamientos con inhibidores de la tirosina kinasa e inhibidores de la vía de la rapamicina en tumores neuroendocrinos pancráticos bien diferenciados las respuestas tanto en sobrevida libre de progresión así como sobrevida global mejoran importantemente. Caso: presentamos el caso de un hombre de 44 años, con diagnóstico de insulinoma y MEN 1, debutó con convulsiones tónico clónicas generalizadas tratado con pancreatectomia parcial, siete años más tarde, tras haber permanecido asintomático nuevo episodio de convulsiones, documentándose hipoglicemias severas y múltiples metástasis hepáticas, sometido a una pancreatoduodenectomia y como tratamiento sintomático recibió corticoides en altas dosis, diazóxido, octreotide mensual y dos líneas de quimioterapia, además de embolización de las metástasis hepáticas de mayor tamaño y por progresión de enfermedad se inició inhibidores de tirosin-kinasa por un período de tres meses y el paciente fallece.
Introduction: the Insulinomas are pancreatic neuroendocrine tumors secreting insulin. The incidence in the general population is estimated 1-4 per 1 000 000 yearly Insulinoma accounts for 60% of pancreatic tumors, of which 90% are benign and unique, 10% are multiple, mostly associated with MEN 1 (Multiple Endocrine Neoplasia), and 5-10% is malignant. The possibility of recurrence requires diagnosis and long-term monitoring. The clinical characteristic of insulinoma is fasting hypoglycemia, with signs and symptoms of neuroglucopenia, which may be preceded or not by adrenergic manifestations. Surgery is the treatment of choice, in irrsecable; and in advanced stages the proposed palliatives or symptomatic treatments are with chemotherapy or procedures through interventional radiologist with poor responses. It has been shown that new treatments with inhibitors of tyrosine kinase inhibitors and rapamycin pathway for pancreatic neuroendocrine well differentiated tumors, the response in both progression-free survival and overall survival signifcantly improved. Case study: we report the case of a 44 year old male of, diagnosed with insulinoma and MEN 1, debuted with tonic clonic seizures treated with partial pancreatectomy, 7 years later after a new episode of seizures documented severe hypoglycemia and multiple liver metastases, underwent a pancreatoduodenectomy and as symptomatic treatment received high-dose corticosteroids, diaxozido, octreotide monthly and 2 lines of chemotherapy, in addition to embolization of the larger liver metastases masses, and due to disease progression tyrosine kinase inhibitors was initiated for a period of 3 months and the patient dies.
Subject(s)
Humans , Male , Adult , Pancreatectomy , Multiple Endocrine Neoplasia , Neuroendocrine Tumors , Hypoglycemia , Insulinoma , Neoplasm Metastasis , Gastrinoma , Mortality , Adrenal Cortex HormonesABSTRACT
Se presenta el caso de una paciente de cuarenta años de edad, el cual es el segundo caso de insulinoma maligno en nuestro país. Llega al hospital con un predominio del cuadro clínico de las manifestaciones neuroglucopénicas debido a las hipoglucemias severas y simulaban crisis epilépticas y trastornos psiquiátricos. La prueba del ayuno prolongado, de la tolbutamida, del glucagón y el test intelectual fueron positivos. La ultrasonografría y la tomografía axial computarizada no revelaron el tumor. Se sometió a la paciente a una pancreatectomía corporocaudal con esplenectomía, observándose el insulinoma de 2.5cm y las metástasis esplénicas de 4 x 3cm. Después de la intervención quirúrgica se encuentra totalmente asintomática.
The second case of malignant insulinoma reported in our country is presented. A 41-years female patient is admitted with the predominant clinical picture of neuroglycopenic manifestations due to severe hypoglycemias mimicking seizures and psychiatric disorders. Positive results are obtained in tests of tolbutamide, glucagon, intellectual performance and prolonged fasting. In this patient, the insulinoma is not revealed through ultrasonography and CT scan. She is undergone a distal pancreatectomy with splenectomy, procedures that demonstrate the insulinoma and splenic metastasis. After surgery, the patient is free of neurologic symptoms.
Subject(s)
Humans , Blood Glucose , Neoplasm MetastasisABSTRACT
Malignant insulinomas are very rare endocrine tumours with a variable clinical course. Here, a case of a malignant insulinoma, resected from the tail of the pancreas 10 years previously, which was found to have hepatic metastasis, is reported. A pancreatic mass, without evidence of metastasis, has been found using an abdominal CT scan and intra-operative ultrasonography 10 years previously. Recently, the patient has suffered from dizziness, sweating and an altered mentality. Hyperinsulinemia was diagnosed from the biochemical laboratory finding. An abdominal CT scan and intra-operative abdominal sonography showed multiple hepatic metastasis, without local recurrence in pancreas. Therefore, a partial hepatic segmentectomy was performed. Immunohistochemical staining of the postoperative specimen was strongly positive for insulin. The postoperative biochemical response was normalized, and the patient experienced no further hypoglycemic symptom.
Subject(s)
Humans , Dizziness , Hyperinsulinism , Hypoglycemia , Insulin , Insulinoma , Liver , Mastectomy, Segmental , Neoplasm Metastasis , Pancreas , Pancreatectomy , Recurrence , Sweat , Sweating , Tomography, X-Ray Computed , UltrasonographyABSTRACT
Insulinoma is clinically characterized by fasting hypoglycemia, various neuropsychiatric symptoms, and these caused by secondary to hypoglycemia.Of patients with insulinoma, 80 percent have single benign tumors, 11 percent have multiple benign tumors, 6 percent have single malignant tumors, and the remainder have multiple malignant tumors or islet hyperplasia according to Service's statement.A 42 year-old male chemical engineer who had insulinoma associated with regional lymph node metastasis has been successfully performed with curative resection and consecutive postoperative therapy with streptozotocin.He entered to this hospital because of frequent occasion of bizarre behavior and language, transient unconsiousness and syncopal episode, and sweating associated with hypoglycemia especially in night four months prior to addmission in November 1992. Those symptoms and hypoglycemia were controlled by intravenous glucose injection and/or oral feeding of glucose riched stuffs.No physical abnormalities were found except neuropsychiatric symptoms. On admission the fasting blood glucose level was 10 mg/dl, plasma immunoreactive plasma insulin level was 125.23 uU/ml, and plasma C-peptide level was 10.24 ng/ml, respectively.Abdominal CT was suggestive of retroperitoneal tumor just behind the pancreas. Selective celiac axis angiography demonstrated hypervascular mass supplied by dorsal pancreatic artery which was compatible with insulinoma. Surgical intervention including distal pancreatectomy, as well as splenectomy and parital omental resection were performed successfully. Consecutive postoperative treatment of streptozotocin was done without any side effects.His condition is very good and enjoyed his life with full activity to date.