Correlation Between Plasma Catecholamines, Weight, and Diabetes in Pheochromocytoma and Paraganglioma

Krumeich, Lauren N.; Cucchiara, Andrew J.; Nathanson, Katherine L.; Kelz, Rachel R.; Fishbein, Lauren; Fraker, Douglas L.; Roses, Robert E.; Cohen, Debbie L.; Wachtel, Heather

doi:10.1210/clinem/dgab401

Cited by 18 publications

(14 citation statements)

References 32 publications

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“…In our study, hypertension and diabetes were less frequent in the negative catecholamine group than the positive catecholamine group, which was expected owing to the effect of catecholamines on blood pressure and glucose metabolism [ 27 , 28 , 29 ]. Catecholamines also affect body weight during hypermetabolic and proinflammatory states [ 30 ]. As a result of a comparison between patients with negative catecholamines and patients with catecholamine-positive PPGLs, Heavner et al [ 6 ] reported that BMI was higher in patients with negative catecholamines; however, Kawashima et al [ 15 ] did not find a difference in BMI between their negative- and positive-catecholamine groups, and the results in this study were consistent with Kawashima et al’s.…”

Section: Discussionmentioning

confidence: 99%

The Clinical Characteristics of Pheochromocytomas and Paragangliomas with Negative Catecholamines

Zhao

Zhang

Meng

et al. 2022

JCM

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Pheochromocytomas and paragangliomas (PPGLs) associated with negative catecholamines are not uncommon. However, few studies have examined clinical features of patients with these tumors. In the absence of available data, it is difficult to identify characteristics of patients with potential PPGLs and normal serum and urine screens. Therefore, an analysis of patients with PPGLs was conducted retrospectively to compare the clinical features of patients with positive and negative catecholamines. This study included 214 patients, including 69 patients with negative catecholamines. Prevalence rates of diabetes (p < 0.001) and hypertension (p < 0.001) were lower and tumor diameter (p < 0.001) was smaller in the negative-catecholamine group compared with the positive-catecholamine group. Multivariable logistic regression analysis showed that extra-adrenal PPGLs were independently positively associated with negative catecholamines (p = 0.004); hypertension (p = 0.001) and tumor diameter (p = 0.016) were independently negatively associated with negative catecholamines. There was no significant difference in tumor recurrence between the two groups (mean follow-up, 20.54 ± 11.83 months) (p = 0.44). The results demonstrated that PPGL patients with negative catecholamines were more likely to have extra-adrenal tumors and less likely to have comorbidities, and these patients should also be closely monitored for tumor recurrence.

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Section: Discussionmentioning

confidence: 99%

The Clinical Characteristics of Pheochromocytomas and Paragangliomas with Negative Catecholamines

Zhao

Zhang

Meng

et al. 2022

JCM

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“…Subsequently she gained weight at a steady rate and was back to her normal weight (approximately 60 kg, BMI: 23.4 kg/m 2 ) on day 214. The initial weight stabilization and weight gain could be attributed to the alpha-blockade treatment as the excess catecholamines are known to increase metabolism in patients, resulting in weight loss ( 12 , 13 ). After approximately 2 months of alpha-blockade the patient regained the ability to swallow more solid foods which likely contributed to her further weight gain.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with catecholamine producing PPGLs have an increased risk of developing diabetes ( 13 ). This is mainly caused by high levels of circulating catecholamines leading to compromised insulin secretion from the pancreatic β-cells, decreased glucose uptake, and increased insulin resistance ( 15 ).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Giant Paraganglioma of the Skull Base With Two Somatic Mutations in SDHB and PTEN Genes

et al. 2022

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Head and neck paragangliomas (HNPGLs) are neuroendocrine tumors. They arise from the parasympathetic ganglia and can be either sporadic or due to hereditary syndromes (up to 40%). Most HNPGLs do not produce significant amounts of catecholamines. We report a case of a giant paraganglioma of the skull base with an unusually severe presentation secondary to excessive release of norepinephrine, with a good outcome considering the severity of disease. A 39-year-old Caucasian woman with no prior medical history was found unconscious and emaciated in her home. In the intensive care unit (ICU) the patient was treated for multi-organ failure with multiple complications and difficulties in stabilizing her blood pressure with values up to 246/146 mmHg. She was hospitalized in the ICU for 72 days and on the 31st day clinical assessment revealed jugular foramen syndrome and paralysis of the right n. facialis. A brain MRI confirmed a right-sided tumor of the skull base of 93.553 cm3. Blood tests showed high amounts of normetanephrine (35.1-45.4 nmol/L, ref <1.09 nmol/L) and a tumor biopsy confirmed the diagnosis of a paraganglioma. Phenoxybenzamine and Labetalol were used in high doses ((Dibenyline®, 90 mg x 3 daily) and labetalol (Trandate®, 200 + 300 + 300 mg daily) to stabilize blood pressure. The patient underwent two tumor embolization procedures before total tumor resection on day 243. Normetanephrine and blood pressure normalized after surgery (0.77 nmol/L, ref: < 1.09 nmol/L). The damage to the cranial nerve was permanent. Our patient was comprehensively examined for germline predisposition to PPGLs, however we did not identify any causal aberrations. A somatic deletion and loss of heterozygosity (LOH) of the short arm (p) of chromosome 1 (including SDHB) and p of chromosome 11 was found. Analysis showed an SDHB (c.565T>G, p.C189G) and PTEN (c.834C>G, p.F278L) missense mutation in tumor DNA. The patient made a remarkable recovery except for neurological deficits after intensive multidisciplinary treatment and rehabilitation. This case demonstrates the necessity for an early tertiary center approach with a multidisciplinary expert team and highlights the efficacy of the correct treatment with alpha-blockade.

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“…Weight loss is also a sign of pheochromocytoma and occurs because of a catecholamine-induced hypermetabolic state. In a study of 360 patients with pheochromocytomas and paragangliomas, Krumeich et al 10 showed that norepinephrine and normetanephrine levels were inversely associated with weight. In addition, Petrák et al demonstrated that chronic catecholamine overproduction leads to a hypermetabolic state characterized by increased resting energy expenditure.…”

Section: Discussionmentioning

confidence: 99%

A case of pheochromocytoma associated with liver abscess and intestinal pseudo-obstruction

Okazaki-Hada

Fukuda

Nagaoka

et al. 2022

Therapeutic Advances in Endocrinology

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Pheochromocytomas can present with various symptoms. Nonspecific manifestations of pheochromocytoma include intestinal pseudo-obstruction and weight loss. Here, we present a case of pheochromocytoma in which prolonged intestinal pseudo-obstruction due to excess catecholamines was one of the factors leading to the development of a liver abscess. An 18-year-old male patient with a history of status epilepticus and severe intellectual disability was transferred to our hospital for a thorough examination of fever and constipation that had lasted for 2 months. When admitted to our hospital, he had fever, and his body mass index was 9.5 kg/m2. Upon comprehensive examination of the patient’s fever, the blood culture was found positive for Bacteroides. Computed tomography showed findings of intestinal pseudo-obstruction and a low density region in the liver that indicated a liver abscess. Imaging studies also revealed a right adrenal mass and endocrinological test showed elevated plasma norepinephrine and urine normetanephrine levels. In addition, the right adrenal mass showed uptake on 123I-metaiodobenzylguanidine scintigraphy. These findings led to a definite diagnosis of pheochromocytoma. The patient was eventually diagnosed with a pheochromocytoma coexisting with a liver abscess. After treating the liver abscess with antibiotics and ultrasound-guided drainage, an adrenalectomy was performed. The pathological findings were consistent with pheochromocytoma. Postoperatively, the catecholamine excess normalized and intestinal pseudo-obstruction and weight loss improved. We suspected that prolonged intestinal pseudo-obstruction resulted in bacterial translocation and development of a liver abscess. To the best of our knowledge, this is the first report of a pheochromocytoma associated with a liver abscess. Moreover, the clinical presentation of this patient was unusual for pheochromocytoma, as the patient did not have typical symptoms such as hypertension or tachycardia, but rather presented with constipation, intestinal pseudo-obstruction, and weight loss. This case provides valuable insight regarding the impact of catecholamine excess on the intestinal tract and body weight.

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Correlation Between Plasma Catecholamines, Weight, and Diabetes in Pheochromocytoma and Paraganglioma

Cited by 18 publications

References 32 publications

The Clinical Characteristics of Pheochromocytomas and Paragangliomas with Negative Catecholamines

The Clinical Characteristics of Pheochromocytomas and Paragangliomas with Negative Catecholamines

Case Report: Giant Paraganglioma of the Skull Base With Two Somatic Mutations in SDHB and PTEN Genes

A case of pheochromocytoma associated with liver abscess and intestinal pseudo-obstruction

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