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Alexandra Chrisoulidou, Gregory Kaltsas, Ioannis Ilias, and Ashley B Grossman

phaeochromocytomas and abdominal paragangliomas are benign; malignant phaeochromocytomas have been regarded as nearly 10% of all phaeochromocytomas and 15–35% of abdominal paragangliomas or even higher if related to succinate dehydrogenase B ( SDHB ) gene mutations

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Esther Korpershoek, Claudia K Stobbe, Francien H van Nederveen, Ronald R de Krijger, and Winand N M Dinjens

8.7 cm (range 0.7–15 cm) for the malignant series. Patient characteristics and clinical data are summarized in Table 1 . Table 1 Clinical data of pheochromocytoma and extra-adrenal sympathetic paraganglioma patients Patient Gender Age (years) Other

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Johanna Sandgren, Teresita Diaz de Ståhl, Robin Andersson, Uwe Menzel, Arkadiusz Piotrowski, Helena Nord, Martin Bäckdahl, Nimrod B Kiss, Michael Brauckhoff, Jan Komorowski, Henning Dralle, Ola Hessman, Catharina Larsson, Göran Åkerström, Carl Bruder, Jan P Dumanski, and Gunnar Westin

malignant pheochromocytomas have been reported to comprise up to 10% of all cases ( Bravo & Tagle 2003 , Elder et al . 2005 , Karagiannis et al . 2007 ). Malignant tumours occur, however, more frequently among abdominal paragangliomas, representing ∼20

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N B Kiss, J Geli, F Lundberg, C Avci, D Velazquez-Fernandez, J Hashemi, G Weber, A Höög, T J Ekström, M Bäckdahl, and C Larsson

included in the present study, which is focused on catecholamine-secreting tumors of the abdomen (in the following referred to as ‘pheochromocytomas’ and ‘paragangliomas’). These tumors are morphologically and functionally similar, although malignant

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P N Span, J U Rao, S B J Oude Ophuis, J W M Lenders, F C G J Sweep, P Wesseling, B Kusters, F H van Nederveen, R R de Krijger, A R M M Hermus, and H J L M Timmers

malignant sympathoadrenal paragangliomas: clinicopathologic study of 120 cases including unusual histologic features . Human Pathology 21 1168 – 1180 doi:10.1016/0046-8177(90)90155-X . Makalowska I Lin CF Makalowski W 2005 Overlapping genes in

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R Vassilopoulou-Sellin

Background: Tumors of the paraganglionic system represent a distinct, albeit uncommon, clinical entity characterized by catecholamine hypersecretion and hemodynamic instability; initial pathologic examination often cannot predict benign vs malignant behavior. An analysis of the clinical outcome of patients with known malignant tumors may serve to enhance the initial evaluation and therapeutic plan of all patients presenting with pheochromocytoma or paraganglioma.

Methods: At the University of Texas M D Anderson Cancer Center, 30 patients with malignant abdominal paraganglioma and 20 patients with malignant pheochromocytoma were diagnosed between 1971 and 1995. Their medical records were reviewed with particular attention to clinical characteristics and disease outcome.

Results: Among the 30 patients with paraganglioma, 73% were men, and 90% were younger than 50 years at the time of diagnosis. Sixteen patients have remained alive with persistent disease 0.2 to 25 years after initial diagnosis while eight patients died of their disease within 0.8 to 32 years. Regional recurrence and skeletal metastases were the most prominent events. Among the 20 patients with pheochromocytoma, 60% were men and 70% were younger than 50 years at the time of diagnosis. Ten patients have remained alive with persistent disease 0.8 to 20 years after initial diagnosis while five patients died of their disease within 1.5 to 39 years. Hypertension was a prominent presenting feature and regional recurrence was the most frequent pattern of treatment failure.

Conclusions: Important clinical differences distinguish adrenal pheochromocytomas from extra-adrenal, abdominal paragangliomas. Patients with paragangliomas are, as a group, younger men, more likely to have malignant lesions and a more aggressive clinical course. Patients with malignant pheochromocytomas usually present with hypertension, are somewhat older, and have less aggressive disease.

Acknowledgemnts

We thank the staff of the Department of Medical Informatics for database retrieval and the clinical faculty who participated in the patients' care. We thank Teo Spear for expert preparation of the manuscript. We thank Terry Smith, biostatistician, for her critical review and suggestions.

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Kimberly Perez, Heather Jacene, Jason L Hornick, Chao Ma, Nuno Vaz, Lauren K Brais, Holly Alexander, William Baddoo, Kristina Astone, Edward D. Esplin, John Garcia, Daniel M Halperin, Matthew H Kulke, and Jennifer A Chan

Malignant pheochromocytomas/paragangliomas are rare tumors for which clinical outcomes remain poorly defined and therapeutic options are limited. Approximately 27% carry pathogenic germline succinate dehydrogenase (SDHx) mutations; the presence of such mutations has been correlated with response to temozolomide. We aimed to investigate the association between SDHx and response to temozolomide. We retrospectively identified patients with malignant pheochromocytomas/paragangliomas treated with temozolomide - based chemotherapy at Dana-Farber Cancer Institute between 2003-2020. Correlation between response by RECIST 1.1 and PERCIST and presence of SDHx mutations in the germline and tumor was evaluated. 19 patients received temozolomide. 17 underwent germline assessment: nine (53%) carried a pathogenic SDHx germline mutation. 15 patients were evaluable for response by RECIST 1.1: 6 (40%) partial response, 4 (27%) stable disease, and 5 (33%) progressive disease. Overall median progression free survival was 2.2 years. Three-year overall survival was 58%. Median progression free survival was 1.3 years and 5.5 years for carriers and non-carries, respectively and overall survival was 1.5 years and not estimable for carriers and non-carriers, respectively. Response by PERCIST criteria in 9 patients correlated with the RECIST 1.1 assessment. Our series represents one of the largest analyses of patients with malignant pheochromocytomas/paragangliomas treated with temozolomide who have available germline data. The incidence of pathogenic germline SDHx mutations was similar to what has been previously published, though our analysis suggests that there may be limited association between response to temozolomide and pathogenic germline SDHx mutations.

Open access

WenQi Yuan, WeiQinq Wang, Bin Cui, TingWei Su, Yan Ge, Lei Jiang, WeiWei Zhou, and Guang Ning

malignant disease, 7 were paragangliomas of the abdomen, and the rest of the 27 patients were with benign pheochromocytomas. All of the patients were operated in between 2002 and 2006 and clinically monitored at the Shanghai Clinical Center for Endocrine and

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Birke Bausch, Ulrich Wellner, Dirk Bausch, Francesca Schiavi, Marta Barontini, Gabriela Sanso, Martin K Walz, Mariola Peczkowska, Georges Weryha, Patrizia Dall'Igna, Giovanni Cecchetto, Gianni Bisogno, Lars C Moeller, Detlef Bockenhauer, Attila Patocs, Karoly Rácz, Dmitry Zabolotnyi, Svetlana Yaremchuk, Iveta Dzivite-Krisane, Frederic Castinetti, David Taieb, Angelica Malinoc, Ernst von Dobschuetz, Jochen Roessler, Kurt W Schmid, Giuseppe Opocher, Charis Eng, and Hartmut P H Neumann

patients vs others (sporadic, VHL and SDHB ). ∥ P <0.001 refers to the high prevalence of head and neck paraganglioma (HNPs) in SDHD patients vs others (sporadic, VHL and SDHB ). ¶ P =0.005 refers to the high prevalence of malignant

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Henri J L M Timmers, Anne-Paule Gimenez-Roqueplo, Massimo Mannelli, and Karel Pacak

Succinate dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas . Journal of Clinical Endocrinology and Metabolism 92 3822 – 3828 . Astrom K Cohen JE Willett-Brozick JE Aston CE Baysal