RESUMEN
Background/Objective: Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors. Here, we report an unusual case of synchronous PPGL in an asymptomatic patient with tuberous sclerosis complex (TSC). Case Report: A 49-year-old woman with a history of TSC and end-stage renal disease was referred for evaluation of bilateral adrenal and retroperitoneal masses. She denied chest pain, palpitations, headaches, or previous hypertensive crisis. The laboratory test results showed a plasma normetanephrine level of 20.20 nmol/L (normal range, 0.00-0.89 nmol/L) and plasma chromogranin A level Chromogranin A (CgA) levels of 1518 ng/mL (normal range, 0-103 ng/mL). The plasma metanephrine level was normal. After α-blockade, the patient underwent bilateral adrenalectomy and retroperitoneal mass excision. Pathology confirmed these lesions to be pheochromocytoma and composite paraganglioma/ganglioneuroma, respectively. Her plasma normetanephrine level normalized postoperatively, and the chromogranin A levels improved to 431 ng/mL. Discussion: Routine imaging has increased the incidental diagnosis of PPGL. Diagnostic workup includes measurement of the urinary and/or plasma metanephrine and catecholamine levels followed by tumor localization. Patients with young age, syndromic lesions, bilateral PPGL, or unilateral disease with a positive family history should have genetic testing. Definitive treatment is surgical after α-blockade. Conclusion: This case highlights a rare presentation of bilateral PPGL in a patient with TSC.
RESUMEN
INTRODUCTION: Previous studies have shown that autonomic dysfunction is associated with shorter survival in patients with advanced cancer. We examined the association between heart rate variability, a measure of autonomic function, and survival in a large cohort of patients with cancer. METHODS: We retrospectively examined the records of 651 patients with cancer who had undergone ambulatory electrocardiogram monitoring for 20 to 24 hours. Time domain heart rate variability (SD of normal-to-normal beat interval [SDNN]) was calculated using power spectral analysis. Survival data were compared between patients with SDNN ≥ 70 milliseconds (Group 1, n = 520) and SDNN < 70 milliseconds (Group 2, n = 131). RESULTS: Two groups were similar in most variables, except that patients in group 2 had a significantly higher percentage of male patients (P = 0.03), hematological malignancies (P = 0.04), and use of non-selective serotonin reuptake inhibitor antidepressants (P = 0.04). Patients in group 2 had a significantly shorter survival rate (25% of patients in group 2 died by 18.7 weeks vs. 78.9 weeks in group 1 patients; P < 0.0001). Multivariate analysis showed that SDNN < 70 milliseconds remained significant for survival (hazard ratio 1.9 [95% confidence interval: 1.4-2.5]) independent of age, cancer stage, and performance status. CONCLUSION: The presence of cancer in combination with decreased heart rate variability (SDNN < 70 milliseconds) is associated with shorter survival time.