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.
A 25-year-old female with a two-month history of left lower quadrant pain, found to have an ovarian cyst and recently started on OCPs, presented to an outside medical center after a witnessed seizure with fever, tachycardia, and altered mental status. Her husband reported that she had been lethargic, complained of headaches, and had brief intermittent episodes of staring into space or stopping mid-task four days prior to the current event. On physical exam, she was hypertensive to 170/90s, tachycardic to 120s, febrile to 101, with hyperreflexia and altered mental status without focal deficits. Burch Wartofsky scale was calculated to be 50, suggestive of thyroid storm. Initial labs showed a suppressed TSH (<0.01 uIU/mL) and an elevated free T4 (7.27 ng/dL). Blood cultures and a lumbar puncture demonstrated no evidence of infection. A non-contrast brain CT showed no acute intracranial findings. She was started on propylthiouracil (PTU), propranolol, hydrocortisone, and Lugol’s solution. Thyrotropin Receptor Antibody and Thyroid Stimulating Immunoglobulin levels were elevated, confirming the diagnosis of Grave’s disease. After the initial management, her vital signs normalized and free T4 decreased to 4.2 (ng/dL), but she had no mental status improvement. Although she had no further seizure activity, she continued to have waxing and waning levels of consciousness. She then developed left sided focal deficits and was transferred to our hospital for the management of a possible cerebrovascular event. MRI and MRV of the brain were performed and demonstrated a thrombus within the superior sagittal sinus. She was started on heparin and bridged on warfarin prior to discharge. Her anti-thyroid regimen was down titrated, steroids tapered and discontinued, PTU changed to methimazole, and propranolol changed to metoprolol. Her mental status and focal deficits improved prior to discharge. Patients who present with hyperthyroidism and new-onset neurological symptoms, especially in the setting of OCP use or other risk factors for a hypercoagulable state, should be evaluated for possible cerebral venous thrombosis. The association between overt hyperthyroidism and acute venous thrombosis, especially at cerebral sites, has been documented in numerous case reports.
Background: Insulinomas are exceptionally uncommon pancreatic islet cell neuroendocrine tumors. Typically, insulinoma induced hypoglycemia occurs exclusively in the fasting state in 73 percent, reported in a retrospective analysis of 237 patients, whereas 6 percent reported only postprandial symptoms. Clinical Case: A 53-year-old female with a history of rheumatoid arthritis, obesity, and prediabetes initially admitted for new onset seizures and recurrent spontaneous hypoglycemic episodes. She experienced recurrent, symptomatic, post-prandial, hypoglycemia daily for the past 8 years. Each episode was closely associated with a high carbohydrate meal, inducing a post-prandial hypoglycemia more consistently and more profoundly than intermittent fasting. Symptoms of lightheadedness, shakiness, and seizure were exacerbated by each carbohydrate meal. Initial labs revealed serum glucose of 35 mg/dl. After recovery with dextrose infusion, a brief fasting trial less than 24 hours was performed with no recurrence of hypoglycemia. However, a mixed meal study utilizing watermelon resulted in a postprandial serum hypoglycemia of 28 mg/dl, provoking a seizure within 30 minutes. During her hospitalization, recurrent hypoglycemia was found during the postprandial period rather than intermittent fasting periods requiring dextrose infusion and octreotide. A Hypoglycemia panel sent during the initial episode was consistent with endogenous hyperinsulinism (Serum glucose=35, Insulin level=24, Proinsulin=166, C-peptide=0.9, BHB=undetectable, Sulfonurea=negative). A subsequent CT of the abdomen/pelvis revealed a mass associated with the pancreatic tail, measuring 4.1 x 4.4 x 5.2 cm concerning for pancreatic malignancy. A fine needle aspiration followed by a distal pancreatectomy and splenectomy with histopathological and immuno-histochemical evaluation confirming a well differentiated (grade 1), 5.0 x 4.5 x 3.0 cm, neuroendocrine tumor (insulinoma). Her recovery, post-operatively, was complicated by an intraabdominal abscess, left pleural effusion, necessitating insulin therapy for hyperglycemia believed to be secondary to morbid obesity, weight gain, and insulin resistance. Hypoglycemic symptoms resolved, blood glucose normalized, and insulin therapy was weaned over the following 3 months and she remains on metformin to date with no evidence of recurrence. Conclusion: Although rare, an insulinoma should be considered in the differential diagnosis of any individual with recurrent episodes of frequent symptomatic hypoglycemia. The classical clinical manifestation of an insulinoma is a fasting hypoglycemia, with distinct episodes of autonomic symptoms. However, postprandial symptoms have been reported with increasing frequency. Here we present a case of surgically confirmed insulinoma with predominantly post-prandial hypoglycemia.
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