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Graeme Eisenhofer, Stefan R Bornstein, Frederieke M Brouwers, Nai-Kong V Cheung, Patricia L Dahia, Ronald R de Krijger, Thomas J Giordano, Lloyd A Greene, David S Goldstein, Hendrik Lehnert, William M Manger, John M Maris, Hartmut P H Neumann, Karel Pacak, Barry L Shulkin, David I Smith, Arthur S Tischler, and William F Young Jr

malignant pheochromocytoma. There are also no reliable histopathological methods for distinguishing benign from malignant tumors. Instead, malignancy requires evidence of metastases at non-chromaffin sites distant from that of the primary tumor. Although

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Jens Waldmann, Volker Fendrich, Julia Holler, Malte Buchholz, Ernst Heinmöller, Peter Langer, Annette Ramaswamy, Birgit Samans, Martin K Walz, Matthias Rothmund, Detlef K Bartsch, and Emily P Slater

Malignant PG 37 20 PGS (SDHD) 15 y HM DM, distant metastases; PC, pheochromocytoma; PG, paraganglioma; n, no; y, yes; PGS, paraganglioma syndrome; LM, liver metastases; LN, lymph node metastases; LG, lung metastases; BN, bone metastases; P, peritoneum; HM

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Goswin Y Meyer-Rochow, Nicole E Jackson, John V Conaglen, Denis E Whittle, Muthusamy Kunnimalaiyaan, Herbert Chen, Gunnar Westin, Johanna Sandgren, Peter Stålberg, Elham Khanafshar, Daniel Shibru, Quan-Yang Duh, Orlo H Clark, Electron Kebebew, Anthony J Gill, Rory Clifton-Bligh, Bruce G Robinson, Diana E Benn, and Stan B Sidhu

unknown ( Neumann et al . 2002 , Adler et al . 2008 ). Approximately 10% of pheochromocytoma cases are malignant ( DeLellis et al . 2004 ); however, this malignant potential cannot be determined histologically, and there is currently no diagnostic test

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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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Erwan Thouënnon, Alice Pierre, Yannick Tanguy, Johann Guillemot, Destiny-Love Manecka, Marlène Guérin, L'houcine Ouafik, Mihaela Muresan, Marc Klein, Jérôme Bertherat, Hervé Lefebvre, Pierre-François Plouin, Laurent Yon, and Youssef Anouar

, there is currently no means to identify, predict, or cure malignant pheochromocytomas. Despite distinction attempts based on biochemical measurements of dihydroxyphenylalanine (DOPA) and dopamine production, histological criteria such as tumor cell

Open access

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

similar to those detected by CGH analysis. The most obvious was the gain of erythroblastic leukemia viral oncogene homolog 2 ( ERBB-2 ) oncogene located on chromosome 17q that was more common in malignant pheochromocytomas. Moreover, we analyzed the ERBB

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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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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.