Randomized trial comparing phenoxybenzamine and doxazosine for preoperative treatment of patients with a pheochromocytoma (PRESCRIPT)

Buitenwerf, Edward; Osinga, TE; Timmers, Hjlm; Lenders, Jwm; Feelders, RA; Eekhoff, Emw; Haak, H.; Corssmit, Epm; Valk, GD; GrooteVeldman, R; Dullaart, Rpf; Links, TP; Voogd, MF; Wietasch, Jkg; Kerstens, MN

doi:10.1530/endoabs.56.oc7.5

EJEA

2018

DOI: 10.1530/endoabs.56.oc7.5

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Randomized trial comparing phenoxybenzamine and doxazosine for preoperative treatment of patients with a pheochromocytoma (PRESCRIPT)

Edward Buitenwerf¹,

TE Osinga²,

Hjlm Timmers³

et al.

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Tumor metabolism in pheochromocytomas: clinical and therapeutic implications

Jeeyavudeen,

Mathiyalagan,

Fernandez James

et al. 2024

Exploration of Targeted Anti-Tumor Therapy

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Pheochromocytomas and paragangliomas (PPGLs) have emerged as one of the most common endocrine tumors. It epitomizes fascinating crossroads of genetic, metabolic, and endocrine oncology, providing a canvas to explore the molecular intricacies of tumor biology. Predominantly rooted in the aberration of metabolic pathways, particularly the Krebs cycle and related enzymatic functionalities, PPGLs manifest an intriguing metabolic profile, highlighting elevated levels of oncometabolites like succinate and fumarate, and furthering cellular malignancy and genomic instability. This comprehensive review aims to delineate the multifaceted aspects of tumor metabolism in PPGLs, encapsulating genetic factors, oncometabolites, and potential therapeutic avenues, thereby providing a cohesive understanding of metabolic disturbances and their ramifications in tumorigenesis and disease progression. Initial investigations into PPGLs metabolomics unveiled a stark correlation between specific genetic mutations, notably in the succinate dehydrogenase complex (SDHx) genes, and the accumulation of oncometabolites, establishing a pivotal role in epigenetic alterations and hypoxia-inducible pathways. By scrutinizing voluminous metabolic studies and exploiting technologies, novel insights into the metabolic and genetic aspects of PPGLs are perpetually being gathered elucidating complex interactions and molecular machinations. Additionally, the exploration of therapeutic strategies targeting metabolic abnormalities has burgeoned harboring potential for innovative and efficacious treatment modalities. This review encapsulates the profound metabolic complexities of PPGLs, aiming to foster an enriched understanding and pave the way for future investigations and therapeutic innovations in managing these metabolically unique tumors.

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Tumor metabolism in pheochromocytomas: clinical and therapeutic implications

Jeeyavudeen,

Mathiyalagan,

Fernandez James

et al. 2024

Exploration of Targeted Anti-Tumor Therapy

View full text Add to dashboard Cite

show abstract

Chromaffin-cell Tumors in Pregnancy: A Case Series and Systematic Review

Rappaport

Skierczynski

Dungy-Poythress

et al. 2018

World Journal of Endocrine Surgery

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Aim:We describe three chromaffin-cell tumors managed during pregnancy as well as systematically review case reports published from 2014 to 2018. Chromaffin-cell tumors are rare catecholamine-producing tumors that can arise from the adrenal medulla, where they are referred to as pheochromocytomas, or from extra-adrenal chromaffin tissue, referred to as paragangliomas. The incidence of chromaffin-cell tumors identified during pregnancy is extremely rare, with an incidence of 0.32 cases per 100,000 pregnancy years. Cases:We describe diagnosis and management during pregnancy of a 25-year-old with a 7.3 cm right pheochromocytoma, a 23-year-old with metastatic paraganglioma and SDHB mutation, and a 28-year-old with MEN2A and a left pheochromocytoma. We performed a systematic review of cases utilizing MEDLINE, EMBASE and Google Scholar with the terms (pheochromocytoma or paraganglioma) and (pregnancy or pregnant) within the timeframe 2014 through 2018 (searched on April 9th, 2018). We found that emergency cesarean section delivery (p < 0.05), maternal heart failure or pulmonary edema (p<0.05) and fetal or neonatal death (p < 0.05) were more common in women with a late or postpartum diagnosis of a chromaffin-cell tumor compared to women with diagnosis during or before pregnancy. Conclusion:Chromaffin-cell tumors are rare during pregnancy. However, morbidity is severe and requires an early diagnosis for the best possible outcomes. Hypertension during pregnancy is the most common presenting symptom of these catecholamineproducing tumors. Severe hypertension, labile hypertension or hypertension before 20 weeks, without proteinuria or lower extremity edema, should raise suspicion for a chromaffin-cell tumor. Management should consist of an experienced multi-disciplinary team at a tertiary referral hospital to ensure the best outcomes.A 25-year-old G3P0202 at 27 weeks gestation was admitted to an outside hospital due to elevated home blood pressures and headaches. On admission, her blood pressure was 152/96 mm Hg with a heart rate of 68 to 133 bpm. Further evaluation revealed elevated liver enzymes with an alanine aminotransferase of 151 U/L (6 to 65

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Randomized trial comparing phenoxybenzamine and doxazosine for preoperative treatment of patients with a pheochromocytoma (PRESCRIPT)

Cited by 2 publications

References 0 publications

Tumor metabolism in pheochromocytomas: clinical and therapeutic implications

Tumor metabolism in pheochromocytomas: clinical and therapeutic implications

Chromaffin-cell Tumors in Pregnancy: A Case Series and Systematic Review

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