[Ectopic Cushing's syndrome caused by a functioning pancreatic neuroendocrine tumour in a patient with von Hippel-Lindau disease]. / Síndrome de Cushing ectópico, producido por un tumor neuroendocrino pancreático funcionante, en una paciente con enfermedad de von Hippel-Lindau.

We present a 36-year-old woman diagnosed with von Hippel-Lindau disease. A haemangioblastoma of the central nervous system was successfully excised ten years ago and a left nephrectomy had been performed because of clear cell carcinoma. For the last six months, the patient has suffered from amenorrhoea, asthenia, muscular debility and capillary fragility. Physical examination showed hirsutism and bruising. Laboratory findings supported ectopic Cushing's syndrome. A solid pancreatic mass was identified on computed tomography. 111-In-DPTA-pentetreotide scintigraphy demonstrated marked uptake at this location, suggesting a neuroendocrine tumour. Duodenopancreatectomy was performed. After surgery, the cortisol levels returned to normal with complete resolution of the syndrome. This is the first case of Cushing's syndrome caused by an ectopic ACTH-secreting neuroendocrine tumour of the pancreas associated with von Hippel-Lindau disease.

Síndrome de Cushing ectópico, producido por un tumor neuroendocrino pancreático funcionante, en una paciente con enfermedad de von Hippel-Lindau / Ectopic Cushing’s syndrome caused by a functioning pancreatic neuroendocrine tumour ina patient with von Hippel-Lindau disease

Presentamos el caso de una mujer de 36 años con enfermedad de von Hippel-Lindau. Entre sus antecedentes personales destacamos la resección de un hemangioblastoma del sistema nervioso central, y una nefrectomía izquierda por carcinoma de células claras. En el momento actual la paciente refiere amenorrea, astenia, debilidad muscular y fragilidad capilar de 6 meses de evolución. El examen físico muestra hirsutismo y enrojecimiento facial. Los hallazgos de laboratorio son compatibles con síndrome de Cushing ectópico. La tomografía computarizada muestra una masa sólida en el páncreas. La gammagrafía con 111In-DTPA-pentetreotide evidencia una marcada captación del trazador en esta misma localización. La paciente es sometida a una duodenopancreatectomía tras la cual los valores de cortisol retornan a la normalidad, con resolución completa del síndrome. No hay descritos en la literatura otros casos de síndrome de Cushing ectópico producido por un tumor neuroendocrino pancreático en el seno de una enfermedad de von Hippel-Lindau

We present a 36-year-old woman diagnosed with von Hippel-Lindau disease. A haemangioblastoma of the central nervous system was successfully excised ten years ago and a left nephrectomy had been performed because of clear cell carcinoma. For the last six months, the patient has suffered from amenorrhoea, asthenia, muscular debility and capillary fragility. Physical examination showed hirsutism and bruising. Laboratory findings supported ectopic Cushing's syndrome. A solid pancreatic mass was identified on computed tomography. 111-In-DPTA-pentetreotide scintigraphy demonstrated marked uptake at this location, suggesting a neuroendocrine tumour. Duodenopancreatectomy was performed. After surgery, the cortisol levels returned to normal with complete resolution of the syndrome. This is the first case of Cushing's syndrome caused by an ectopic ACTH-secreting neuroendocrine tumour of the pancreas associated with von Hippel-Lindau disease

[Usefulness of thyroid scintigraphy in the therapeutic management of amiodarone-induced hyperthyroidism]. / Utilidad de la gammagrafía tiroidea en el manejo terapéutico del hipertiroidismo inducido por amiodarona.

UNLABELLED: Amiodarone-induced hyperthyroidism is relatively common in iodine-deficient regions. Two types have been described: type I, caused by increased synthesis and release of thyroid hormone in individuals with underlying thyroid disease, can be treated with antithyroid drugs or radioiodine; and type II, a destructive thyroiditis responsive to corticoid therapy but not to antithyroid drugs. It can be difficult to distinguish between the two types, and cases of mixed types have been reported. OBJECTIVE: to assess the usefulness of thyroid scintigraphy in amiodarone-induced hyperthyroidism. MATERIALS AND METHODS: 27 consecutive patients (13 females) with amiodarone-induced hyperthyroidism. Mean age was 65 years (range: 39-89). All patients underwent 99mTc-pertechnectate thyroid scintigraphy and were classified according to the qualitative estimation of radiotracer uptake: type I (increased / normal uptake): 9 patients, all of whom responded to antithyroid drugs or radioiodine, except one patient with sub-clinical hyperthyroidism who received no therapy; type II (very low or undetectable uptake): 13 patients, 11 of whom responded to discontinuation of amiodarone or prednisone therapy (2 patients). Hyperthyroidism was resistant in 2 patients and required antithyroid drugs or potassium perchlorate; mixed type (low uptake but with underlying thyroid pathology): 5 patients, with variable evolution; all needed antithyroid drugs, one required subtotal thyroidectomy, and another radioiodine treatment. CONCLUSION: thyroid scintigraphy can establish the correct therapeutic approach in most cases of amiodarone-induced hyperthyroidism, making it essential in the clinical management of these patients.

Utilidad de la gammagrafía tiroidea en el manejo terapéutico del hipertiroidismo inducido por amiodarona / Usefulness of thyroid scintigraphy in the therapeutic management of amiodarone-induced hyperthyroidism

El hipertiroidismo por amiodarona (HA) es una entidad relativamente frecuente en zonas dietéticamente deficitarias de yodo. Se describen dos entidades: la tipo I, causada por excesiva síntesis de hormona tiroidea en pacientes con patología tiroidea subyacente que responde a antitiroideos o al radioyodo, y la tipo II, producida por tiroiditis destructiva inflamatoriaque responde a los corticoides, pero no a los antitiroideos. La diferencia entre ambos tipos puede ser difícil, y se han descrito formas intermedias. Objetivo. Valorar la utilidad de la gammagrafía tiroidea en pacientes con HA. Material y métodos. Veintisiete pacientes consecutivos (13 mujeres) con 39-89 años e HA. Se realizó gammagrafía tiroidea con 99mTc-pertecnectato, clasificando al paciente en subgrupos, en función de una valoración cualitativa de la captación gammagráfica. Resultados: patrón tipo I (captación tiroidea normal o incrementada): 9 pacientes. Todos respondieron a la terapia con antitiroideos o radioyodo, excepto un paciente con hipertiroidismo subclínico que no recibió tratamiento; patrón tipo II (captación inhibida): 13 pacientes. La mayoría (11 pacientes) remite al retirar la amiodarona (9) o con corticoides (2). Sólo en dos pacientes el hipertiroidismo fue resistente y obligó a la terapia con antitiroideos o perclorato potásico; patrón intermedio (5 pacientes) con captación empobrecida y patología tiroidea subyacente. Tuvieron una evolución variable, necesitando antitiroideos en todos los casos; además uno de ellos precisó tiroidectomía subtotal y otro tratamiento con radioyodo. Conclusiones. La gammagrafía tiroidea permite establecerla terapia a administrar en la mayoría de pacientes con HA, por lo que resulta una herramienta diagnóstica imprescindible en el manejo clínico de estos pacientes

Amiodarone-induced hyperthyroidism is relatively common in iodine-deficient regions. Two types have been described: type I, caused by increased synthesis and release of thyroid hormone in individuals with underlying thyroid disease, can be treated with antithyroid drugs or radioiodine; and type II, a destructive thyroiditis responsive to corticoid therapybut not to antithyroid drugs. It can be difficult to distinguish between the two types, and cases of mixed types have been reported.Objective. to assess the usefulness of thyroid scintigraphy in amiodarone-induced hyperthyroidism. Materials and methods. 27 consecutive patients (13 females) with amiodarone-induced hyperthyroidism. Mean age was 65 years (range: 39-89). All patients underwent 99mTcpertechnectate thyroid scintigraphy and were classified according to the qualitative estimation of radiotracer uptake: type I (increased / normal uptake): 9 patients, all of whom responded to antithyroid drugs or radioiodine, except one patient with sub-clinical hyperthyroidism who received no therapy; type II (very low or undetectable uptake): 13 patients, 11 of whom responded to discontinuation of amiodarone or prednisone therapy (2 patients). Hyperthyroidism was resistant in 2 patients and required antithyroid drugs or potassium perchlorate; mixed type (low uptake but with underlying thyroid pathology): 5 patients, with variable evolution; all needed antithyroid drugs, one required subtotal thyroidectomy, and another radioiodine treatment. Conclusion. thyroid scintigraphy can establish the correct therapeutic approach in most cases of amiodarone-induced hyperthyroidism, making it essential in the clinical management of these patients

[Bone scintigraphy with 99mTc-MDP in a patient with acute lymphoblastic leukemia initially diagnosed of Still's disease]. / Gammagrafía ósea con 99mTc-MDP en un paciente con leucemia aguda linfoblástica diagnosticado inicialmente de enfermedad de Still.

We present a 43-year-old male, who was admitted with the diagnosis of Adult-onset Still's disease, after several months of arthralgias, febricula and loss of weight. Chest x-ray, abdominal ultrasonography, chest, abdomen and pelvic CT scan and bone scintigraphy were performed. Scintigraphic findings oriented to the performance of a bone marrow biopsy with diagnosis of acute lymphoblastic leukemia.

Gammagrafía ósea con 99mTc-MDP en un paciente con leucemia aguda linfoblástica diagnosticado inicialmente de enfermedad de Still / Bone scintigraphy with 99mTc-MDP in a patient with acute lymphoblastic leukemia initially diagnosed of Still’s disease

Presentamos el caso de un varón de 43 años, que ingresa con el diagnóstico de enfermedad de Still, tras varios meses aquejado de artralgias, febrícula y pérdida de peso. Se realizan radiografía de tórax, ecografía abdominal, TC de tórax, abdomen y pelvis con contraste y gammagrafía ósea. Los hallazgos gammagráficos orientan hacia la realización de punción de médula ósea con el diagnóstico de leucemia linfoblástica aguda

We present a 43-year-old male, who was admitted with the diagnosis of Adult-onset Still's disease, after several months of arthralgias, febricula and loss of weight. Chest x-ray, abdominal ultrasonography, chest, abdomen and pelvic CT scan and bone scintigraphy were performed. Scintigraphic findings oriented to the performance of a bone marrow biopsy with diagnosis of acute lymphoblastic leukemia