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. 2024 Nov 2;14(1):26456.
doi: 10.1038/s41598-024-75354-9.

Long-term outcomes and prognostic factors of metastatic or recurrent pheochromocytoma and paraganglioma: a 20-year review in a single institution

Fumio Ishizaki  1 , Takahiro Taguchi  2 , Masaki Murata  3 , Sayaka Hoshino  3 , Tomotaka Toba  3 , Keisuke Takeda  3 , Masayuki Tasaki  3 , Kazutoshi Yamana  3 , Takashi Kasahara  3   4 , Tatsuhiko Hoshii  3 , Kenji Obara  3 , Kazuhide Saito  3 , Yoshihiko Tomita  3
Affiliations

Long-term outcomes and prognostic factors of metastatic or recurrent pheochromocytoma and paraganglioma: a 20-year review in a single institution

Fumio Ishizaki et al. Sci Rep. .

Abstract

Pheochromocytoma and paraganglioma (PPGL) represent a group of rare neuroendocrine tumors known for their potential to metastasize. This study provides a comprehensive retrospective evaluation of 15 patients diagnosed with metastatic or recurrent PPGL at our institution over a two-decade span (2000-2020). Our primary objectives were to delineate the long-term clinical outcomes and pinpoint key prognostic determinants. Median duration from initial PPGL diagnosis to the onset of metastasis or recurrence stood at 5.8 years. Predominant sites for metastasis included the bone, lung, lymph nodes, and peritoneum. A salient finding was that surgical interventions targeting metastatic lesions significantly improved prognosis. Further analysis revealed that a Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) exceeding 7 closely associated with unfavorable outcomes. These insights not only underscore the clinical variability of PPGL's progression but also highlight the pivotal role of surgical management for metastatic or recurrent cases. The value of the PASS score as an informative prognostic tool was evident, suggesting its utility in shaping future therapeutic approaches. Given the intricacies of PPGL, collaborative studies involving larger patient cohorts will be crucial to optimize management strategies and prognostication.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Overall survival in patients with metastatic or recurrent PPGL.
Fig. 2
Fig. 2
Overall survival in patients diagnosed with metastatic or recurrent PPGL with or without surgical intervention.
See this image and copyright information in PMC

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