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Treatment of Malignant Pheochromocytoma and Paraganglioma

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Treatment of Malignant Pheochromocytoma and Paraganglioma E Baudin and others Therapy for malignant 171:3 R111–R122 Review chromaffin tumors THERAPY OF ENDOCRINE DISEASE Treatment of malignant pheochromocytoma and paraganglioma Eric Baudin, Mouhammed Amir Habra1, Frederic Deschamps, Gilbert Cote1, Frederic Dumont, Maria Cabanillas1, J Arfi-Roufe, A Berdelou, Bryan Moon1, Abir Al Ghuzlan, Shreyaskumar Patel1, Sophie Leboulleux and Camilo Jimenez1,† De´ partement de Me´ decine Nucle´ aire et de Cance´ rologie Endocrinienne, Radiologie Interventionnelle, Chirurgie, Imagerie, Institut Gustave Roussy, Universite´ Paris Sud, 114 Rue Edouard Vaillant 94805 villejuif Cedex, Paris, France Correspondence and 1Department of Endocrine Neoplasia and Hormone Disorders, Unit 1461, The University of Texas MD Anderson should be addressed to Cancer Center, 1400 Pressler Street, Houston, Texas 77030, USA E Baudin or C Jimenez †(C Jimenez is now at The University of Texas MD Anderson Cancer Center, 1400 Pressler Street Unit 1461, Houston, Email Texas 77004, USA) [email protected] or [email protected] Abstract 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. European Journal of European Journal of Endocrinology Endocrinology (2014) 171, R111–R122 Introduction Malignant pheochromocytomas and paragangliomas MPPs to be differentiated from the metastases of gastro- (MPPs) constitute a subgroup of neuroendocrine tumors enteropancreatic NETs (1). The diagnosis of MPPs is made (NETs) defined by the presence of chromaffin tissues in extra- either at the time of first evaluation by thorough radio- adrenal or paraganglion sites. Because they are NETs, MPPs graphic evaluation and/or surgical exploration or over long- are defined by their endocrine tumor morphology and term follow-up lasting months, years, or even decades. The positive immunohistochemical staining forchromogranin A presence of clinical predictors of metastases and overall and synaptophysin; negative staining for keratins allows survival, such as the size, location (adrenal or extra-adrenal), Invited Author’s profile Eric Baudin is assistant professor at GustaveRoussy (University of Paris-Sud), where he chairs the Endocrine Tumor Board. He is also co-chair of the French network for adrenal cancer (COMETE) and scientific coordinator or the French network of neuroendocrine tumors (GTE-RENATEN). He served as member of the ENSAT, PRESSOR and, ENETS, associations. His main field of interest concerns endocrine tumors. He is involved in many subjects, including prognostic stratification, treatment with radioactive isotopes, chemotherapy and molecular targeted therapy, but also biomarker development. He has been an investigator for most prospective clinical trials on endocrine tumors. He has published more than 200 original articles in the field of endocrine tumors and has received awards from the French Academy of Medicine and of Sciences. www.eje-online.org Ñ 2014 European Society of Endocrinology Published by Bioscientifica Ltd. DOI: 10.1530/EJE-14-0113 Printed in Great Britain Downloaded from Bioscientifica.com at 09/26/2021 12:02:39AM via free access Review E Baudin and others Therapy for malignant 171:3 R112 chromaffin tumors and genotype of the primary tumor, can help clinicians many years. The minimum information that should be determine the required radiographic studies to asses disease included in all MPP patients’ records is given in Table 1. extension at the time of the discovery of the primary tumor Advances in genetics and imaging studies have and during the long-term follow-up (2).Thus,patientswith revealed that, compared with other diseases, MPPs lack pheochromocytomas larger than 5 cm, paragangliomas of validated prognostic indicators and therapies. The most any size, and/or carriers of SDHB mutations need radio- robust studies of the characterization of MPP patients at graphic studies that can detect metastases in the thoracic, the time of the diagnosis have suggested that metastatic abdominal, and pelvic cavities and the skeletal tissue or adrenal and extra-adrenal tumors occur at equal rates and recurrences in the primary tumor bed (2). that the survival rates of metastatic adrenal and extra- The incidence of MPPs is !1 per 1 million people/year. adrenal tumors overlap (2); w60% of MPP patients have Approximately 10–20% of pheochromocytomas and para- tumor burden- and hormone-related manifestations gangliomas are malignant. Malignancy is much more (e.g., pain, hypertension, and constipation) (3); most common in sympathetic paragangliomas than in pheochro- MPPs produce noradrenaline and normetanephrine mocytomas, the incidence of which is higher (2). Owing to and/or dopamine and methoxytyramine, which partly the low incidence of MPPs, few prospective studies have reflects the higher risk of malignancy associated with investigated potential therapies for the disease. Thus, SDHB mutations and extra-adrenal locations (4, 5, 6, 7, 8); advances in the understanding and management of MPPs most MPP patients have apparently sporadic tumors or largely depend on retrospective studies’ findings, expert tumors associated with germline or somatic mutations consensus, and clinicians’ experience with other NETs. The of the succinate dehydrogenase subunit B (SHDB) gene implementation of networks for researching MPPs, such as (2,9,10). The high prevalence of SDHB mutations the European Network for the Study of Adrenal Tumors and (30–50% of patients) supports the screening of all patients the Pheochromocytoma and Paraganglioma Research Sup- with MPPs for such mutations. port Organization, is a major step forward in the treatment Progress in imaging is remarkable. 18F-fluorodeoxyglu- of the disease. This review focuses on the therapeutic cose positron emission tomography (FDG-PET) is the most management of adult patients with MPPs. sensitive scintigraphic method for assessing MPPs (11).In addition, scintigraphy with 123I-metaiodobenzylguanidine (MIBG) can be used to determine whether patients are Characterization before therapy candidates for targeted radiotherapy with 131I-MIBG (12, MPPs must be precisely characterized according to 13). All foci of uptake should be correlated with guided European Journal of Endocrinology standardized criteria before therapy because this standar- conventional review or imaging. Computed tomography dized characterization not only affects the therapeutic with optimal i.v. administration of contrast material has a management of the disease, including the decision to high sensitivity (90–95%) for detecting primary tumors and treat, but also constitutes a modality for improving the metastatic and extra-adrenal lesions larger than 1 cm. The understanding of a very rare cancer, the research of which usual sites of metastatic involvement include the lymph is expected to primarily involve retrospective studies for nodes (80% of patients), bones (71%), liver (50%), and Table 1 Minimum information to record at the presentation of patients with malignant pheochromocytomas and paragangliomas. Age and gender Performance status and comorbidities Primary tumor location (If multiple tumors are present, the tumor that is O5 cm and/or associated with local spread or regional positive lymph nodes is considered the primary tumor.) Hormone- and tumor-related symptoms, including hypertension, constipation, and pain Serum chromogranin A levels and urinary and/or plasma metanephrine, normetanephrine, and methoxytyramine levels indexed to urinary creatinine levels Genetic syndrome Best scintigraphic imaging among 18F-fluorodeoxyglucose positron emission tomography, metaiodobenzylguanidine scintigraphy, and somatostatin receptor scintigraphy Bone, lung, liver, and lymph node tumor burden determined by head and neck, thoracic, abdominal, and pelvic computed tomography and/or magnetic resonance imaging in early arterial phase and guided computed tomography and/or magnetic resonance imaging Morphological tumor progression at 3 months, without therapy if feasible Acquisition of frozen or paraffin-embedded tumor tissue for research purposes www.eje-online.org Downloaded from Bioscientifica.com at 09/26/2021 12:02:39AM via free access Review E Baudin and others Therapy for malignant 171:3 R113 chromaffin tumors A metastases found are mandatory (15). 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). In the characterization of MPPs, pathological differ- entiation and proliferative index have no prognostic value, which is in stark contrast
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