RESUMEN
We report a case of a young hypertensive male who was first seen in 1998 with a right thalamic haemorrhage and uncontrolled hypertension. CT abdomen showed a right adrenal tumour and a hyperplastic left adrenal gland. Laparoscopic adrenalectomy performed followed by histopathological examination confirmed the diagnosis of adrenal adenoma. He subsequently presented to us again a year later with persistent hyperkalaemia and asymptomatic hyponatraemia. Further investigations strongly suggested the presence of isolated mineralocorticoid deficiency with normal cortisol levels. This was confirmed to be due to partial or late-onset congenital adrenal hyperplasia (CAH). We discuss the association of partial CAH and adrenal tumours and the unmasking of the mineralocorticoid deficiency following adrenalectomy.
Asunto(s)
Adenoma/etiología , Neoplasias de las Glándulas Suprarrenales/etiología , Hiperplasia Suprarrenal Congénita/complicaciones , Adenoma/cirugía , Neoplasias de las Glándulas Suprarrenales/cirugía , Adrenalectomía , Humanos , Masculino , Persona de Mediana Edad , Mineralocorticoides/deficiencia , Renina/sangreRESUMEN
Pheochromocytoma is a rare catecholamine-secreting tumour typically arising within the adrenal medulla. It may occur sporadically or be associated as part of a tumour syndrome including Von Hippel Lindau (VHL), Multiple Endocrine Neoplasia (MEN) 2 and Neurofibromatosis Type 1. VHL is associated with multi-organ involvement of benign and malignant tumours characterized by the presence of retinal angiomas, hemangioblastomas of the cerebellum and spinal cord, renal cell carcinomas, pheochromocytomas and other cystic lesions in the kidneys, pancreas, and epididymis. It is a rare disorder with prevalence estimated at 2-3 per 100,000. This case report describes a 37 years old Chinese gentleman who presented to our institution for further management of bilateral pheochromocytoma and retinal angioblastoma with problems of duodenal ulcer and anaemia. There was no family history of similar problems. With these features the criteria for the diagnosis of von Hippel Lindau disease was established.
Asunto(s)
Enfermedad de von Hippel-Lindau/diagnóstico , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Adrenalectomía , Adulto , Diagnóstico Diferencial , Humanos , Masculino , Feocromocitoma/diagnóstico , Tomografía Computarizada por Rayos X , Enfermedad de von Hippel-Lindau/cirugíaRESUMEN
We report a case of a 65 year old Malay lady with long-standing diabetes mellitus, who presented to our institution with a one month history of worsening neck pain and progressive upper and lower limb weakness. She was stable despite severe hyponatraemia which was initially treated as syndrome of inappropriate anti-diuretic hormone (SIADH). This was consistent with her underlying illness which was concluded as cervical tuberculosis (TB) with spinal cord compression. She underwent decompression and bone grafting. Despite continuous treatment her serum sodium levels remained low. There were no other problems with her adrenals or thyroid. A water loading and hypertonic saline perfusion test was performed and supported the diagnosis of reset osmostat. Her serum sodium remained below the normal range and she was discharged well.