Introduction When clinicians think of pheochromocytomas, diabetes might not be the first thing that comes to mind. Pheochromocytomas elicit deterioration in glucose tolerance in 20% to 40% of affected individuals. Size of the pheochromocytoma could be an independent risk factor for developing diabetes, as larger pheochromocytomas often present with diabetes mellitus in 23% to 33% of patients with symptomatic pheochromocytomas. Evidence suggests that pheochromocytoma proposes a risk for development of diabetes. There is no consensus or recommendations on which patients with new onset diabetes should be screened for pheochromocytoma or how to manage hyperglycemia in these patients. Clinical Case 42 YO F with PMH of HTN presented to primary care clinic c/o polyuria, fatigue, 25-pound weight loss, epigastric pain and worsening hypertension over 3 months. Initial work up showed a HbA1C of 9%. The patient was diagnosed with type 2 diabetes mellitus and started on Metformin 1g BID with no improvement of glucose levels or symptoms. As part of initial work up for suspicion of pancreatic malignancy patient had an abdominal CT scan showing a 5.6 x 4.8 cm well-circumscribed left adrenal mass. Patient was referred for endocrine evaluation. Initial workup showed negative GAD Ab, normal 1mg Dexamethasone suppression test, normal aldosterone/renin ratio, elevated Free Normetanephrine of 17,921 pg/mL and elevated chromogranin A of 3,427 ng/mL. Based on biochemical evidence of catecholamine excess and left adrenal mass on imaging, patient was diagnosed with Pheochromocytoma. MIBG showed no evidence of metastatic disease. She was started on insulin therapy, Doxazosin 1mg BID which was titrated to 2mg BID and Amlodipine 5mg was increased to 10mg daily. Pt was referred to surgery for adrenalectomy. On pre-op evaluation patient had significant improvement of glucose levels and symptoms. Propranolol 20mg BID was started for BP optimization. A laparoscopic left adrenalectomy was performed without complications. Patient did not require anti-hypertensive medications or insulin during hospitalization and was discharged on metformin 500mg daily with no anti-hypertensive medications. At one-month Post-adrenalectomy follow up patient had normal BP of 116/72 mmHg and A1C of 5.4% with normal glucose logs indicating resolution of diabetes and HTN post adrenalectomy. Conclusion Diabetes is a multifactorial disease that has direct impact on patient’s quality of life, morbidity and mortality. It is important to consider pheochromocytoma as a risk factor for development of diabetes, especially in young patients with atypical presentation, uncontrolled hypertension or without evidence of antibodies or insulin resistance. Screening and early diagnosis of pheochromocytoma could mean significant reduction on long term diabetes complications as diabetes seems to improve or even resolve after adrenalectomy.
Background: A pheochromocytoma diagnosed during pregnancy is an extremely rare condition with an incidence of 0.002% of all pregnancies. If untreated, consequences are devastating including a maternal and fetal mortality as high as 50%. There is no consensus in the literature regarding the management of this condition during pregnancy. Clinical Case: A 25-year-old woman G4P1021 presented at 15 weeks of gestation with shortness of breath, palpitations and chest pain. Vitals revealed hypertension and tachycardia. A CT angiogram of the chest was obtained which ruled out a pulmonary embolism but detected a 5.5-cm left adrenal mass. Biochemical work up revealed elevated plasma and urine normetanephrines. A dexamethasone suppression test was normal. A plasma aldosterone to renin ratio was normal. An MRI of the abdomen confirmed the presence of a large heterogeneous 6-cm left adrenal mass. A multi-disciplinary team discussion including an obstetrician, endocrinologist, endocrine surgeon and anesthesiologist led to the decision to perform an adrenalectomy during the second trimester. Doxazosin was started at a dose of 2 mg daily, which was later titrated up to 2 mg BID with excellent blood pressure control. Metoprolol was added 4 days prior to surgery for heart rate control. A laparoscopic left adrenalectomy was performed at 19 weeks of gestation. Intra-operatively, the patient had a brief period of hypotension which resolved with IV fluids and a short course of vasopressors. Patient did not have any post-operative complications and is currently normotensive and with a normal heart rate without the use of any anti-hypertensive medications. Repeat plasma metanephrines and normetanephrines were within the reference range. Her latest obstetric ultrasound at 20 weeks of gestation revealed a live fetus without anatomical abnormalities. The fetus is within the normal weight and size percentiles. Conclusion: A pheochromocytoma is a rare event during pregnancy and is associated with high maternal and fetal mortality rates. Timely diagnosis and proper treatment are of utmost importance to reduce mortality. The optimal time for surgical tumor removal has not been established but the second trimester appears to be safest period given the risk for spontaneous abortion during the first trimester and that the enlarged uterus diminishes tumor accessibility during the third trimester. Pre-operative medical management is crucial similar to non-pregnant patients. The treatment of these patients should include a dedicated team including an obstetrician, anesthesiologist, surgeon and endocrinologist.
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