Yun Mi Choi1, Tae-Yon Sung2, Won Gu Kim1, Jong Jin Lee3, Jin-Sook Ryu3, Tae Yong Kim1, Won Bae Kim1, Suck Joon Hong2, Dong Eun Song4, Young Kee Shong1. 1. Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. 2. Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. 3. Department of Nuclear Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. 4. Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Abstract
OBJECTIVES: Malignant pheochromocytoma (PCC)/paraganglioma (PG) are rare neuroendocrine malignancies, and their clinical courses and prognoses are not well understood. This study aimed to evaluate prognostic factors associated with the survival of malignant PCC/PG. METHODS: This retrospective study reviewed 299 patients with PCC and 46 with PG treated between 1997 and 2013 at our single tertiary hospital. Malignant PCC/PG was defined as the presence of distant metastasis or recurrence at sites where neuroendocrine tissue is normally not present. RESULTS: Twenty-seven patients (9%) were confirmed with malignant PCC and six patients (13%) with malignant PG. Twenty-seven patients (82%) had distant metastases, nine patients (27%) presented with a metastasis at the initial diagnosis, whilst 24 patients (73%) were diagnosed with malignant PCC/PG during follow-up (median, 4.3 year). The median survival was 7.2 years, and the 5 year survival rate was 75.4%. Older age (>45 years), larger tumor size (>6 cm), synchronous metastasis, and absence of surgical excision were associated with poor survival by univariate analysis. By multivariate analysis, older age (HR = 4.3, P = 0.02) and synchronous metastasis (HR = 4.3, P = 0.01) were significantly associated with a poor prognosis. CONCLUSIONS: Patients with malignant PCC/PG have diverse clinical courses. Poor survival was independently associated with older age and synchronous metastasis.
OBJECTIVES:Malignant pheochromocytoma (PCC)/paraganglioma (PG) are rare neuroendocrine malignancies, and their clinical courses and prognoses are not well understood. This study aimed to evaluate prognostic factors associated with the survival of malignant PCC/PG. METHODS: This retrospective study reviewed 299 patients with PCC and 46 with PG treated between 1997 and 2013 at our single tertiary hospital. Malignant PCC/PG was defined as the presence of distant metastasis or recurrence at sites where neuroendocrine tissue is normally not present. RESULTS: Twenty-seven patients (9%) were confirmed with malignant PCC and six patients (13%) with malignant PG. Twenty-seven patients (82%) had distant metastases, nine patients (27%) presented with a metastasis at the initial diagnosis, whilst 24 patients (73%) were diagnosed with malignant PCC/PG during follow-up (median, 4.3 year). The median survival was 7.2 years, and the 5 year survival rate was 75.4%. Older age (>45 years), larger tumor size (>6 cm), synchronous metastasis, and absence of surgical excision were associated with poor survival by univariate analysis. By multivariate analysis, older age (HR = 4.3, P = 0.02) and synchronous metastasis (HR = 4.3, P = 0.01) were significantly associated with a poor prognosis. CONCLUSIONS:Patients with malignant PCC/PG have diverse clinical courses. Poor survival was independently associated with older age and synchronous metastasis.
Authors: Helena Leijon; Satu Remes; Jaana Hagström; Johanna Louhimo; Hanna Mäenpää; Camilla Schalin-Jäntti; Markku Miettinen; Caj Haglund; Johanna Arola Journal: Hum Pathol Date: 2018-12-08 Impact factor: 3.466
Authors: Helena Leijon; Tuomas Kaprio; Annamari Heiskanen; Tero Satomaa; Jukka O Hiltunen; Markku M Miettinen; Johanna Arola; Caj Haglund Journal: J Clin Endocrinol Metab Date: 2017-11-01 Impact factor: 5.958
Authors: Javier López-Gómez; Ma Alejandra Salazar-Álvarez; Rodrigo Y Adame; Alejandro Alfaro-Goldaracena; Erwin R Flores-Vazquez; Sergio H Gonzalez-Infante; Alejandro E Padilla-Rosciano; Horacio N López-Basave Journal: Int J Surg Case Rep Date: 2016-10-22