Cargando…

LBODP009 Resection Of An Adrenocorticotropic Hormone Secreting Pheochromocytoma In A Jehovah'S Witness Patient

Background: Pheochromocytomas are rare neuroendocrine tumors arising from chromaffincells in the adrenal medulla, and the excessive catecholamine secreted causes serious cardiovascular effects to the patient. Adrenalectomy is the definitive treatment with a morbidity rate of 40% and a mortality rate...

Descripción completa

Detalles Bibliográficos
Autor principal: Andalahao, Edzele Marie Fernandez
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9700281/
http://dx.doi.org/10.1210/jendso/bvac150.085
Descripción
Sumario:Background: Pheochromocytomas are rare neuroendocrine tumors arising from chromaffincells in the adrenal medulla, and the excessive catecholamine secreted causes serious cardiovascular effects to the patient. Adrenalectomy is the definitive treatment with a morbidity rate of 40% and a mortality rate of 2%. Intraoperative bleeding is a major concern with any surgical procedure, thus preparation of blood products for possible transfusion during the perioperative period is prudent. A religious group known as the Jehovah's witnesses recommend blood transfusion refusal to its members. We encountered a case of a Jehovah's witness with pheochromocytoma requiring adrenalectomy. CASE: A case of 54 year old, Filipino male, a Jehovah's witnesses, hypertensive for 14 years, complained of 1 week vague right upper quadrant abdominal pain and bouts of headache. A CT scan of the abdomen with contrast revealed an 11.7x 12.2×10.3cm mass on the right adrenal. Biochemical tests were consistent with pheochromocytoma that is co-secreting with ACTH, but with no physical features of hypercortisolism. An elevated levels of 24 hours urine Metanephrine (8.95 mg/ 24hrs),24 hours urine cortisol (173.98 mcg /24 hours), serum ACTH (57 pg/ml), 8am serumcortisol (22.44 mcg/dl), and 1 mg Dexamethasone suppression 8am cortisol (18.85mcg/dl). Patient consented to surgery but signed a waiver of refusal to any blood transfusion. Erythropoietin 5000 IU SC three times per week was then initiated 1month prior to the surgery. Terazosin 25 mg three times a day followed by Metoprolol25 mg twice daily, for adequate alpha and beta blockade, and hydration prior to surgery was done. The Estimated blood loss during surgery was 600 ml, patient's hemoglobin level dropped to 98 g/L on the 7th post-operative day from pre-operative level of 125 g/L, hence, the Erythropoietin was continued until was discharged. The Histopathology report showed round cell neoplasm, with positive immunohistochemical stain for synaptophysin and chromogranin, and a weak positive of NSE (Neuro-specific enolase) consistent with pheochromocytoma. References: 1. Narita et al, Surgery without Blood Transfusion for Giant Paraganglioma in a Jehovah's Witness Patient, Case Rep Oncol 2014;7: 233–238, DOI: 10.1159/0003608132. Bachman et al, Pheochromocytoma, Fulminant Heart Failure, and a Phenylephrine Challenge. The Perioperative Management of Adrenalectomy in a Jehovah's Witness Patient: A Case Report, The Journal of Critical Care Medicine 2022; 8(1): 55-60, DOI: https://doi.org/10.2478/jccm-2021-0038 Presentation: No date and time listed