Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician

Sharma, Sona; Fishbein, Lauren

doi:10.1016/j.eprac.2023.07.027

Cited by 3 publications

(7 citation statements)

References 67 publications

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“…The management of metastatic PPGLs is challenging as there are only practiced standards of systemic therapy, that have been extensively adopted outside of controlled clinical trials [ 10 , 11 , 21 , 51 , 52 , 53 , 54 ]. To date, the only FDA-approved treatment option in metastatic disease is HSA 131 MIBG in the US.…”

Section: Therapeutic Optionsmentioning

confidence: 99%

“…The choice of the first line treatment and of subsequent therapy approaches depends on the patients’ characteristics, clinical symptoms, tumor type, radioisotope tumor avidity, disease burden (limited/extensive) as well as its aggressivity (limited stable disease, extensive disease with slow/rapid progression) and available resources [ 11 , 14 , 21 , 25 , 54 ].…”

Section: Therapeutic Optionsmentioning

confidence: 99%

“…Chemotherapy should be considered as the first line treatment in patients who have no significant uptake of radiotracers or with clear rapid disease progression, associated with high tumor burden, or with severe uncontrolled symptoms due to CMNs secretion or mass effect, in order to obtain disease control and/or symptom palliation [ 10 , 11 , 30 , 54 , 55 ].…”

Section: Therapeutic Optionsmentioning

confidence: 99%

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The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

Bracigliano,

Marretta,

Guerrera

et al. 2024

Pharmaceuticals

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Pheochromocytomas (PCCs) and Paragangliomas (PGLs), commonly known as PPGLs to include both entities, are rare neuroendocrine tumors that may arise in the context of hereditary syndromes or be sporadic. However, even among sporadic PPGLs, identifiable somatic alterations in at least one of the known susceptibility genes can be detected. Therefore, about 3/4 of all PPGL patients can be assigned to one of the three molecular clusters that have been identified in the last years with difference in the underlying pathogenetic mechanisms, biochemical phenotype, metastatic potential, and prognosis. While surgery represents the mainstay of treatment for localized PPGLs, several therapeutic options are available in advanced and/or metastatic setting. However, only few of them hinge upon prospective data and a cluster-oriented approach has not yet been established. In order to render management even more personalized and improve the prognosis of this molecularly complex disease, it is undoubtable that genetic testing for germline mutations as well as genome profiling for somatic mutations, where available, must be improved and become standard practice. This review summarizes the current evidence regarding diagnosis and treatment of PPGLs, supporting the need of a more cluster-specific approach in clinical practice.

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Section: Therapeutic Optionsmentioning

confidence: 99%

Section: Therapeutic Optionsmentioning

confidence: 99%

Section: Therapeutic Optionsmentioning

confidence: 99%

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The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

Bracigliano,

Marretta,

Guerrera

et al. 2024

Pharmaceuticals

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“…Both sympathetic and parasympathetic pheochromocytomas may exhibit paroxysmal or persistent hypertension, headache, palpitation, syncope, anxiety, hyperglycemia, and sweating as the most common symptoms. 4 Additionally, symptoms such as orthostatic hypotension, pallor, nausea, vomiting, weight loss, constipation, abdominal pain, flushing, weakness, fever, tremors, acute anxiety, and panic attacks may occur. 5 Biochemical screening involves measuring plasma free catecholamines or urine-fractionated catecholamines.…”

Section: Introductionmentioning

confidence: 99%

A comparative analysis of surgically excised hereditary and sporadic pheochromocytomas: Insights from a single‐center experience

Nasiroglu Imga,

Deniz,

Tural Balsak

et al. 2024

The Kaohsiung J of Med Scie

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Pheochromocytoma is a tumor that usually originating from adrenal medullary chromaffin cells and producing one or more catecholamines, can manifest as hereditary or sporadic. While the majority pheochromocytomas are sporadic, hereditary forms are often associated with genetic syndromes such as von Hippel–Lindau, multiple endocrine neoplasia type 2, and neurofibromatosis type 1. This study aims to analyze data from our series of surgically excited pheochromocytoma patients and compare the characteristics between hereditary and sporadic cases. We retrospectively evaluated 33 diagnosed pheochromocytoma patients, documenting clinical features, surgical complications, and tumor characteristics in both hereditary and sporadic cases. Among the patients, 21% (7 individuals) had hereditary pheochromocytoma, while 79% (26 individuals) had sporadic cases. During diagnosis, hereditary pheochromocytoma patients exhibited a significantly lower mean age compared to the sporadic group (26.4 ± 9.9 years vs. 50.4 ± 14.0 years; p < 0.001). The maximum tumor size was also lower in hereditary cases compared to sporadic cases (p = 0.004). Adrenal tumor localization analysis showed that 63.6% were right‐sided, 24.2% were left‐sided, and 12.1% were bilateral. Laboratory analysis revealed significantly higher urinary norepinephrine levels in hereditary pheochromocytoma patients (p = 0.021). Our findings suggest that hereditary pheochromocytoma cases are characterized by a younger age at diagnosis, smaller tumor size, and a higher prevalence of multiple bilateral adrenal adenomas. We recommend genetic testing for all pheochromocytoma patients, particularly those with early‐onset disease and bilateral adrenal tumors.

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“…Pheochromocytoma (PHEO) is a rare neuroendocrine tumour. It differs from paraganglioma (PGL) by the site of origin; in the case of PHEO, the chromaffin cells of the adrenal gland are located in the adrenal medulla [ 1 ]. In the latest classification of the World Health Organisation (WHO) published in 2022, it is referred to as intra-adrenal PGL, as both are histologically the same.…”

Section: Introductionmentioning

confidence: 99%

Differences between Patients with Sporadic and Familial Pheochromocytoma—Is It Possible to Avoid Genetic Testing in Certain Patients?

Muñoz,

Febrero,

Abellán

et al. 2024

Biomedicines

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Background: Pheochromocytoma (PHEO) is a rare neuroendocrine tumour with a strong genetic link, which therefore may modify its clinical behaviour and prognosis. The aim of the study is to evaluate the epidemiological and clinical differences between patients with sporadic and familial PHEO, as well as the specific differences in the index cases. Methods: A retrospective analysis of 136 patients in a tertiary hospital (1984–2021). Epidemiological, clinical, and histological variables were analysed. Statistics: SPSS 28.0 software was used. Univariate and multivariate logistic regression analyses were performed. p < 0.05 was considered statistically significant. Results: 64.71% of the cases (n = 88) presented a genetic mutation (familial cases). Additionally, 32.39% (n = 23) corresponded to index cases and the rest to screening cases. The main differences between patients with familial and sporadic PHEO were age (OR = 0.93 (0.89–0.97)), blood pressure-related symptoms (OR = 0.22 (0.06–0.89)), bilaterality (OR = 15.49 (3.76–63.84)), and size (OR = 0.70 (0.54–0.92)). Among patients with sporadic PHEO and index cases, only bilaterality was significant (OR = 13.53 (1.24–144.34)). Conclusions: Patients with familial PHEO diagnosed by screening differ from sporadic cases in terms of age, clinical features, and size. However, patients with sporadic PHEO only differ from index cases by a lower presence of bilaterality, which reaffirms the importance of genetic screening of patients with PHEO and their relatives.

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Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician

Cited by 3 publications

References 67 publications

The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

A comparative analysis of surgically excised hereditary and sporadic pheochromocytomas: Insights from a single‐center experience

Differences between Patients with Sporadic and Familial Pheochromocytoma—Is It Possible to Avoid Genetic Testing in Certain Patients?

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