Unusual presentation of phaeochromocytoma. 2016

D C D Hope, and J M Palan
St George's Hospital, London, UK.

A 44-year-old woman, with a background of heart, lung and renal transplantation secondary to cystic fibrosis and type 1 diabetes, presented with tachycardia, hyperglycaemia, nausea and vomiting. She was initially managed for diabetic ketoacidosis with severe dehydration. However, persistent episodic hypertension and tachycardia led the investigating team to identify significantly raised urinary metanephrines and a left-sided adrenal mass; Iodine-123-meta-iodobenzylguanidine single photon emission computer tomography scan (MIBG SPECT/CT) showed avid uptake of tracer, confirming a left-sided phaeochromocytoma. She was started on medical management and is awaiting an elective laparoscopic procedure. This case describes the presentation of a unilateral phaeochromocytoma as ketoacidosis in a patient with type 1 diabetes with no other apparent precipitant. This highlights the metabolic counter-regulatory effect of excess catecholamines in addition to the inotropic/chronotropic effects that are associated with this adrenal tumour. Recognition of atypical signs and symptoms may point towards an atypical precipitant of diabetic ketoacidosis-a medical emergency presenting to front-line clinicians.

UI MeSH Term Description Entries
D010535 Laparoscopy A procedure in which a laparoscope (LAPAROSCOPES) is inserted through a small incision near the navel to examine the abdominal and pelvic organs in the PERITONEAL CAVITY. If appropriate, biopsy or surgery can be performed during laparoscopy. Celioscopy,Laparoscopic Surgical Procedures,Peritoneoscopy,Surgical Procedures, Laparoscopic,Laparoscopic Assisted Surgery,Laparoscopic Surgery,Laparoscopic Surgical Procedure,Procedure, Laparoscopic Surgical,Procedures, Laparoscopic Surgical,Surgery, Laparoscopic,Surgical Procedure, Laparoscopic,Celioscopies,Laparoscopic Assisted Surgeries,Laparoscopic Surgeries,Laparoscopies,Peritoneoscopies,Surgeries, Laparoscopic,Surgeries, Laparoscopic Assisted,Surgery, Laparoscopic Assisted
D010673 Pheochromocytoma A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the ADRENAL MEDULLA or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of EPINEPHRINE and NOREPINEPHRINE, is HYPERTENSION, which may be persistent or intermittent. During severe attacks, there may be HEADACHE; SWEATING, palpitation, apprehension, TREMOR; PALLOR or FLUSHING of the face, NAUSEA and VOMITING, pain in the CHEST and ABDOMEN, and paresthesias of the extremities. The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1298) Pheochromocytoma, Extra-Adrenal,Extra-Adrenal Pheochromocytoma,Extra-Adrenal Pheochromocytomas,Pheochromocytoma, Extra Adrenal,Pheochromocytomas,Pheochromocytomas, Extra-Adrenal
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000310 Adrenal Gland Neoplasms Tumors or cancer of the ADRENAL GLANDS. Adrenal Cancer,Adrenal Gland Cancer,Adrenal Neoplasm,Cancer of the Adrenal Gland,Neoplasms, Adrenal Gland,Adrenal Cancers,Adrenal Gland Cancers,Adrenal Gland Neoplasm,Adrenal Neoplasms,Cancer, Adrenal,Cancer, Adrenal Gland,Cancers, Adrenal,Cancers, Adrenal Gland,Neoplasm, Adrenal,Neoplasm, Adrenal Gland,Neoplasms, Adrenal
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D015899 Tomography, Emission-Computed, Single-Photon A method of computed tomography that uses radionuclides which emit a single photon of a given energy. The camera is rotated 180 or 360 degrees around the patient to capture images at multiple positions along the arc. The computer is then used to reconstruct the transaxial, sagittal, and coronal images from the 3-dimensional distribution of radionuclides in the organ. The advantages of SPECT are that it can be used to observe biochemical and physiological processes as well as size and volume of the organ. The disadvantage is that, unlike positron-emission tomography where the positron-electron annihilation results in the emission of 2 photons at 180 degrees from each other, SPECT requires physical collimation to line up the photons, which results in the loss of many available photons and hence degrades the image. CAT Scan, Single-Photon Emission,CT Scan, Single-Photon Emission,Radionuclide Tomography, Single-Photon Emission-Computed,SPECT,Single-Photon Emission-Computed Tomography,Tomography, Single-Photon, Emission-Computed,Single-Photon Emission CT Scan,Single-Photon Emission Computer-Assisted Tomography,Single-Photon Emission Computerized Tomography,CAT Scan, Single Photon Emission,CT Scan, Single Photon Emission,Emission-Computed Tomography, Single-Photon,Radionuclide Tomography, Single Photon Emission Computed,Single Photon Emission CT Scan,Single Photon Emission Computed Tomography,Single Photon Emission Computer Assisted Tomography,Single Photon Emission Computerized Tomography,Tomography, Single-Photon Emission-Computed
D017558 Elective Surgical Procedures Surgery which could be postponed or not done at all without danger to the patient. Elective surgery includes procedures to correct non-life-threatening medical problems as well as to alleviate conditions causing psychological stress or other potential risk to patients, e.g., cosmetic or contraceptive surgery. Surgical Procedures, Elective,Elective Surgical Procedure,Procedure, Elective Surgical,Procedures, Elective Surgical,Surgical Procedure, Elective

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