RESUMEN
We report a 28 years old woman consulting for weight gain, progressive muscle weakness and appearance of facial plethora. Laboratory showed high cortisol and ACTH levels. Magnetic resonance of sella turcica was normal and an abdominal magnetic resonance showed a pancreatic mass, suggestive of a neuroendocrine tumor. A PET/CT with somatostatin analogues demonstrated an intense over-expression of somatostatin receptors in the tumor. The patient was subjected to a pancreatectomy and the day after surgery, ACTH levels decreased to less than 5 pg/ml. The patological study of the surgical piece showed a neuroendocrine carcinoma. The patient had a good postoperative evolution.
Asunto(s)
Humanos , Adulto , Femenino , Compuestos Organometálicos , Neoplasias Pancreáticas , Síndrome de Cushing/etiología , Somatostatina/análogos & derivados , Somatostatina , Tumores Neuroendocrinos , Laparoscopía , Neoplasias Pancreáticas/cirugía , Pancreatectomía , Tomografía de Emisión de Positrones , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Tumores Neuroendocrinos/cirugíaRESUMEN
We report a 59 years old female with a history of nephrolithiasis and progressive worsening of her bone mineral density. High serum PTH levels were detected, with normal serum calcium. Causes of secondary hyperparathyroidism were discarded. The patient was followed during six years, period in which she maintained elevated serum PTH and normal serum calcium. During the second year of follow up, hydrochlorothiazide was indicated. Serum calcium raised progressively and after six years, it became abnormally high. The patient was subjected to a total left lobe and subtotal right lobe thyroidectomy. The surgeon found a 1.6 mm diameter left parathyroid nodule. After surgery the patient is asymptomatic and is receiving levothyroxine supplementation.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Adenoma/sangre , Calcio/sangre , Hiperparatiroidismo Primario/sangre , Neoplasias de las Paratiroides/sangre , Adenoma , Adenoma/cirugía , Evolución Clínica , Hidroclorotiazida/uso terapéutico , Hiperparatiroidismo Primario/tratamiento farmacológico , Hormona Paratiroidea/sangre , Neoplasias de las Paratiroides , Neoplasias de las Paratiroides/cirugía , Radiofármacos , TiroidectomíaRESUMEN
Von Hippel Lindau disease is a hereditary syndrome characterized by the appearance of benign and malignant tumors in different organs. Its incidence is 1 case per 36000 born alive. We report a family with the disease. The index case was a male with a bilateral pheochromocytoma and cerebelar and retinal hemagioblastomas that had a sudden death due to a cerebrovascular accident at the age of 52 years. One sibling had central nervous system and retinal hemangioblastomas and other was operated for an unilateral pheochromocytoma. Both siblings had the R167Q VHL mutation of the syndrome. Other family members did not have the mutation.