One-Year Progression-Free Survival of Therapy-Naive Patients With Malignant Pheochromocytoma and Paraganglioma

Hescot, Ségolène; Leboulleux, Sophie; Amar, Laurence; Vezzosi, Delphine; Borget, Isabelle; Bournaud-Salinas, Claire; Fouchardière, Christelle De La; Libé, Rossella; Cao, Christine Do; Niccoli, Patricia; Tabarin, Antoine; Raingeard, I.; Chougnet, C.; Giraud, Sophie; Gimenez-Roqueplo, Anne-Paule; Young, Jacques; Borson‐Chazot, Françoise; Bertherat, Jérôme; Wémeau, Jean-Louis; Bertagna, Xavier; Plouin, Pierre‐François; Schlumberger, Martin; Baudin, Éric; networks, COrtico-MEdullo Tumeurs Endocrines

doi:10.1210/jc.2013-1907

Cited by 102 publications

(74 citation statements)

References 29 publications

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“…18 F-fluorodeoxyglucose positron emission tomography (FDG-PET) is the most sensitive scintigraphic method for assessing MPPs (11). In addition, scintigraphy with 123 I-metaiodobenzylguanidine (MIBG) can be used to determine whether patients are candidates for targeted radiotherapy with 131 I-MIBG (12,13).…”

Section: Characterization Before Therapymentioning

confidence: 99%

“…In the absence of validated prognostic parameters, in asymptomatic patients with MPPs with no threatening masses, radiographic studies to assess disease progression should be conducted every 3 months for the first year. The results of these assessments will refine prognosis, the need and type of treatment (discussed in the following sections), and the follow-up modality of these patients (18).…”

Section: Characterization Before Therapymentioning

confidence: 99%

“…Recent breakthroughs in genetics and imaging, as well as a median overall survival duration of more than 5 years in most studies, have made the prognostic characterization of these patients less straightforward (2,8,18).…”

Section: Prognosismentioning

confidence: 99%

“…Finally, death from other cancers, although rare, should be considered, especially in the context of genetic syndromes (19,20). The reported 5-year overall survival rates of MPP patients range from 40 to 77% (2,8,15,18). One study found the presence of a SDHB mutation, after adjustment for tumor burden, to be the single most powerful prognostic factor, though this observation is yet to be confirmed (8).…”

Section: Prognosismentioning

confidence: 99%

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THERAPY OF ENDOCRINE DISEASE: Treatment of malignant pheochromocytoma and paraganglioma

Baudin

Habra

Deschamps

et al. 2014

European Journal of Endocrinology

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100

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Metastatic pheochromocytomas and paragangliomas (MPPs) present clinicians with three major challenges: scarcity, complexity of characterization, and heterogeneous behavior and prognosis. As with the treatment for all neuroendocrine tumors, the control of hormonal symptoms and tumor growth is the main therapeutic objective in MPP patients. A significant number of MPP patients still die from uncontrolled hormone secretion. In addition, the management of MPPs remains palliative. Steps forward include proper characterization of MPP patients at large cancer referral centers with multidisciplinary teams; improved strategies to stratify patients prognostically; and implementation of trials within national and international networks. Progress in the molecular characterization and staging of MPPs constitutes the basis for significant treatment breakthroughs.

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Section: Characterization Before Therapymentioning

confidence: 99%

Section: Characterization Before Therapymentioning

confidence: 99%

Section: Prognosismentioning

confidence: 99%

Section: Prognosismentioning

confidence: 99%

See 2 more Smart Citations

THERAPY OF ENDOCRINE DISEASE: Treatment of malignant pheochromocytoma and paraganglioma

Baudin

Habra

Deschamps

et al. 2014

European Journal of Endocrinology

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100

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“…However, considering the frequently encountered longterm stable disease, or very slow progression, and the absence of curative options for metastasized PGL patients, the benefits and side effects of therapeutic interventions should be carefully weighed. The timing to systemic therapy in these patients has become the subject of debate because, a recent retrospective study in therapy-naïve patients with malignant PGL/PCC has shown that nearly half of patients achieved stable disease at 1 year and that therefore, in symptom-free patients, a wait-and-scan surveillance policy until imaging proof of progression, preferably by Response Evaluation Criteria in Solid Tumors (RECIST), seems appropriate as first-line treatment (82). A recent systematic review and meta-analysis in MIBG-positive cases have shown that treatment with therapeutic doses of 131 I-MIBG resulted in an objective tumor response in 30% of patients and stabilization of disease in 57% of patients (81,83), and an objective hormonal response in 50% of patients.…”

Section: Malignant Pglmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Clinical management of paragangliomas

Corssmit

Romijn

2014

European Journal of Endocrinology

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Paragangliomas (PGLs) are rare vascular, neuroendocrine tumors of paraganglia, which are associated with either sympathetic tissue in adrenal (pheochromocytomas (PCCs)) and extraadrenal (sympathetic paraganglioma (sPGLs)) locations or parasympathetic tissue of the head and neck paragangliomas (HNPGLs). As HNPGLs are usually benign and most tumors grow slowly, a wait-and-scan policy is often advised. However, their location in the close proximity to cranial nerves and vasculature may result in considerable morbidity due to compression or infiltration of the adjacent structures, necessitating balanced decisions between a wait-and-see policy and active treatment. The main treatment options for HNPGL are surgery and radiotherapy. In contrast to HNPGLs, the majority of sPGL/PCCs produces catecholamines, in advanced cases resulting in typical symptoms and signs such as palpitations, headache, diaphoresis, and hypertension. The state-of-the-art diagnosis and localization of sPGL/PCCs are based on measurement of plasma and/or 24-h urinary excretion of (fractionated) metanephrines and methoxytyramine (MT). sPGL/PCCs can subsequently be localized by anatomical (computed tomography and/or magnetic resonance imaging) and functional imaging studies ( 123 I-metaiodobenzylguanidine-scintigraphy, 111 In-pentetreotide scintigraphy, or positron emission tomography with radiolabeled dopamine or dihydroxyphenylalanine). Although most PGL/PCCs are benign, factors such as genetic background, tumor size, tumor location, and high MT levels are associated with higher rates of metastatic disease. Surgery is the only curative treatment. Treatment options for patients with metastatic disease are limited. PGL/PCCs have a strong genetic background, with at least one-third of all cases linked with germline mutations in 11 susceptibility genes. As genetic testing becomes more widely available, the diagnosis of PGL/PCCs will be made earlier due to routine screening of at-risk patients. Early detection of a familial PGL allows early detection of potentially malignant PGLs and early surgical treatment, reducing the complication rates of this operation.

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Malignant Tumors of the Adrenal Gland

Lee¹,

Habra²,

Campbell³

2022

Holland‐Frei Cancer Medicine

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Overview Optimal evaluation and treatment of malignant or potentially malignant adrenal tumors including pheochromocytoma/paraganglioma and adrenal cortical carcinoma can be complex. It requires appropriate diagnostic imaging and endocrine hormone evaluation, and benefits from expert multidisciplinary coordination, by medical oncologists, endocrinologists, and surgeons, as well as additional specialists, for example genetic counselors. Recent advances in diagnostic imaging, including functional imaging such as PET, have facilitated early detection and diagnosis, enhanced the accuracy of staging, and improved treatment planning. Good‐risk patients with resectable localized, recurrent, or limited metastatic disease are usually recommended to undergo either minimally invasive or open surgery. Advances in minimally invasive and open surgical techniques have improved surgical outcomes. There is no defined role for adjuvant therapy following resection of pheochromocytoma/paraganglioma. Adjuvant therapy for patients with adrenal cortical carcinoma, most commonly single‐agent mitotane, is considered selectively. Systemic treatment options for patients with locally recurrent, unresectable, or metastatic malignant adrenal carcinoma include targeted treatments for patients with metastatic pheochromocytoma/paraganglioma, and combination chemotherapy with or without mitotane for patients with adrenal cortical carcinoma. Radiation therapy for patients with malignant adrenal tumors of either histology is also employed selectively. Emerging genetic and molecular data, as well as clinical trials, increasingly inform treatment strategies and choice of systemic therapy.

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One-Year Progression-Free Survival of Therapy-Naive Patients With Malignant Pheochromocytoma and Paraganglioma

Abstract: Half of the therapy-naive patients with MPP achieved stable disease at 1 year. In symptom-free patients with MPP, a wait-and-see antitumor policy seems appropriate as first line. Modality for a prospective follow-up is proposed.

Cited by 102 publications

References 29 publications

THERAPY OF ENDOCRINE DISEASE: Treatment of malignant pheochromocytoma and paraganglioma

THERAPY OF ENDOCRINE DISEASE: Treatment of malignant pheochromocytoma and paraganglioma

MANAGEMENT OF ENDOCRINE DISEASE: Clinical management of paragangliomas

Malignant Tumors of the Adrenal Gland

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