A pediatric case of pheochromocytoma without apparent hypertension associated with von Hippel-Lindau disease

Igaki, Junko; Nishi, Akira; Satô, Takeshi; Hasegawa, Tomonobu

doi:10.1297/cpe.27.87

Cited by 5 publications

(3 citation statements)

References 18 publications

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“…In individual case reports, patient weight loss was significant (>10 kg), mostly in elderly women. [7,[13][14][15][16] Among 79 patients with PCC, four cases (5.1%) demonstrated a preoperative weight loss of > 10%. [17] Other studies reported weight loss rates of 10.5% to 6% (including children) but did not provide specific descriptions of the extent of weight loss.…”

Section: Abnormal Energy Metabolism In Patients With Ppglmentioning

confidence: 99%

Main mechanisms and clinical implications of alterations in energy expenditure state among patients with pheochromocytoma and paraganglioma: A review

Yang,

Zhou,

Zhao

et al. 2024

Medicine

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Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors with diverse clinical presentations. Alterations in energy expenditure state are commonly observed in patients with PPGL. However, the reported prevalence of hypermetabolism varies significantly and the underlying mechanisms and implications of this presentation have not been well elucidated. This review discusses and analyzes the factors that contribute to energy consumption. Elevated catecholamine levels in patients can significantly affect substance and energy metabolism. Additionally, changes in the activation of brown adipose tissue (BAT), inflammation, and the inherent energy demands of the tumor can contribute to increased resting energy expenditure (REE) and other energy metabolism indicators. The PPGL biomarker, chromogranin A (CgA), and its fragments also influence energy metabolism. Chronic hypermetabolic states may be detrimental to these patients, with surgical tumor removal remaining the primary therapeutic intervention. The high energy expenditure of PPGL has not received the attention it deserves, and an accurate assessment of energy metabolism is the cornerstone for an adequate understanding and treatment of the disease.

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Section: Abnormal Energy Metabolism In Patients With Ppglmentioning

confidence: 99%

Main mechanisms and clinical implications of alterations in energy expenditure state among patients with pheochromocytoma and paraganglioma: A review

Yang,

Zhou,

Zhao

et al. 2024

Medicine

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show abstract

“…As in adults, some children with PPGLs may be normotensive and asymptomatic ( 24 ), particularly when tumors are found as part of surveillance programs involving family members with a known mutation of a tumor susceptibility gene ( 25 ). Children with incidentally discovered adrenal or extra-adrenal masses based on imaging studies for reasons other than a suspected PPGL can also be normotensive and asymptomatic and have a catecholamine-producing tumor ( 26 , 27 ).…”

Section: Modes Of Clinical Suspicionmentioning

confidence: 99%

Biochemical Diagnosis of Catecholamine-Producing Tumors of Childhood: Neuroblastoma, Pheochromocytoma and Paraganglioma

et al. 2022

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Catecholamine-producing tumors of childhood include most notably neuroblastoma, but also pheochromocytoma and paraganglioma (PPGL). Diagnosis of the former depends largely on biopsy-dependent histopathology, but this is contraindicated in PPGL where diagnosis depends crucially on biochemical tests of catecholamine excess. Such tests retain some importance in neuroblastoma though continue to largely rely on measurements of homovanillic acid (HVA) and vanillylmandelic acid (VMA), which are no longer recommended for PPGL. For PPGL, urinary or plasma metanephrines are the recommended most accurate tests. Addition of methoxytyramine to the plasma panel is particularly useful to identify dopamine-producing tumors and combined with normetanephrine also shows superior diagnostic performance over HVA and VMA for neuroblastoma. While use of metanephrines and methoxytyramine for diagnosis of PPGL in adults is established, there are numerous pitfalls for use of these tests in children. The establishment of pediatric reference intervals is particularly difficult and complicated by dynamic changes in metabolites during childhood, especially in infants for both plasma and urinary measurements, and extending to adolescence for urinary measurements. Interpretation of test results is further complicated in children by difficulties in following recommended preanalytical precautions. Due to this, the slow growing nature of PPGL and neglected consideration of the tumors in childhood the true pediatric prevalence of PPGL is likely underappreciated. Earlier identification of disease, as facilitated by surveillance programs, may uncover the true prevalence and improve therapeutic outcomes of childhood PPGL. For neuroblastoma there remain considerable obstacles in moving from entrenched to more accurate tests of catecholamine excess.

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“…However, pheochromocytomas may be associated with Von Hippel-Lindau (VHL) disease, familial paraganglioma, and multiple endocrine neoplasia type 2A. [4] Hereditary, bilateral, extra-adrenal, and multifocal pheochromocytomas are more common in children than adults. [5] This condition is rare and may be overlooked.…”

Section: Introductionmentioning

confidence: 99%

Feokromasitomanın İlk Bulgusu Olarak Paroksismal Baş Ağrısı, Olgu Sunumu

Zorlu

Seyhan

Dur

et al. 2021

Journal of Contemporary Medicine

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Pheochromocytoma is a rare tumor that develops from the chromaffin cells of the sympathetic nervous system. Although hypertension is the most common manifestation of pheochromocytoma, it is less common in children than in adults. This condition may be overlooked like in our patient who had an undiagnosed headache for several years. Our patient was admitted to our emergency department due to the increase in headache, which lasted for about 6 years, and a neurological evaluation was performed. Due to the high blood pressure in her follow-up, she was examined further. Abdominal USG revealed a 36x26x27 mm solid lesion in the right adrenal gland with a mild hyperechogenic appearance with a cystic component. With supporting findings in magnetic resonance imaging and high catecholamine levels in blood, she was diagnosed with pheochromocytoma. Our aim in sharing this case is to emphasize the importance of measuring blood pressure in patients accurately and at each examination, evaluating them with percentile charts, and monitoring them closely.

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A pediatric case of pheochromocytoma without apparent hypertension associated with von Hippel-Lindau disease

Cited by 5 publications

References 18 publications

Main mechanisms and clinical implications of alterations in energy expenditure state among patients with pheochromocytoma and paraganglioma: A review

Main mechanisms and clinical implications of alterations in energy expenditure state among patients with pheochromocytoma and paraganglioma: A review

Biochemical Diagnosis of Catecholamine-Producing Tumors of Childhood: Neuroblastoma, Pheochromocytoma and Paraganglioma

Feokromasitomanın İlk Bulgusu Olarak Paroksismal Baş Ağrısı, Olgu Sunumu

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