Clinical Features Analysis of 34 Patients with Retroperitoneal Paragangliomas_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

 HUHan-kui ,  ZHAOJi-chun , HUANGBin , YUANDing , YANGYi , ZENGGuo-jun , XIONGFei

Department of Vascular Surgery, West China Hospital of Sichuan University, Chengdu 610041, Sichuan Province, China;

Corresponding?author：

ZHAOJi-chun, Email: zhaojc3@126.com

Keywords：

Retroperitoneal paragangliomas; Clinical feature; Treatment

DOI：

10.7507/1007-9424.20160076

Video：

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Objective To evaluate the clinical features of the retroperitoneal paralangliomas. Method Clinical data of 34 patients with retroperitoneal paragangliomas who underwent surgery in our hospital between January 2005 and January 2015 were enrolled, all patients diagnosed by pathological examination. Results Clinical manifestation: hypertension (n=4) and headache (n=1) were the main symptoms in all 5 patients with functional tumor. Abdominal distension (n=14) or abdominal pain (n=11) were the initial symptoms in patients with nonfunctional tumor, gastrointes-tinal obstruction was observed in 1 patient, the other 3 patients were diagnosed accidently among the 29 patients with nonfunctional tumors. Blood and urine catecholamines increased notly in 5 patients with functional tumor, increased mildly in 2 patients with nonfunctional tumor; and remained at a normal level in the other patients. All the 34 patients underwent surgery, 4 patients with nonfunctional tumors who suffered from vessel wrapped needed tumor resection combining with main vessel resection or reconstruction, 1 of the 4 patients presented with vascular wall invasion. Two patients presented with lymph-vascular invasion of patients didn't received vessel resection or reconstruction. The mean value of diameter of these tumors was (9.5±5.1) cm (3.5-18.5 cm). Twenty four tumors had complete capsule, the other 10 had partial capsule; 32 tumors were well differentiated, and other 2 patients had apparent strange nucleus. Among the 34 patients, G1 tumors were confirmed in 14 patients and G2 tumors were confirmed in 20 patients. All the 34 patients were followed up with a mean value of follow-up time of 52 months (1-105 months). During the follow-up period, 6 patients suffered from recurrence and 6 patients died. The 1-year and 5-year survival rates were 86.0% and 81.5%, respectively. Conclusions Radical resection may be the only measure to cure retroperitoneal paragangliomas. Tumor combining with vascular resection or reconstruction provide a chance for patients with ‘unresectable tumors’.

Citation： HUHan-kui, ZHAOJi-chun, HUANGBin, YUANDing, YANGYi, ZENGGuo-jun, XIONGFei. Clinical Features Analysis of 34 Patients with Retroperitoneal Paragangliomas. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2016, 23(3): 274-276. doi: 10.7507/1007-9424.20160076 Copy

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6.	Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromoc-ytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab, 2011, 96(3): 717-725.
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8.	李峰, 陳小巖, 陳新, 等. Syn、CgA、melan A和inhibin α在腎上腺皮質腺瘤及嗜鉻細胞瘤中的表達及意義. 診斷病理學雜志, 2014, 21(8): 501-502.
9.	Zhang C, Tan C, Ding H, et al. Selective VEGFR inhibitors for anti-cancer therapeutics in clinical use and clinical trials. Curr Pharm Des, 2012, 18(20): 2921-2935.
10.	Park J, Song C, Park M, et al. Predictive characteristics of malignant pheochromocytoma. Korean J Urol, 2011, 52(4): 241-246.
11.	王華, 陶健, 王伯胤. CT對診斷異位嗜鉻細胞瘤的價值. 實用腫瘤雜志, 2010, 25(2): 217-219.
12.	Parenti G, Zampetti B, Rapizzi E, et al. Updated and new perspec-tives on diagnosis, prognosis, and therapy of malignant pheochro-mocytoma/paraganglioma. J Oncol, 2012, 2012: 872713.
13.	Okubo Y, Nemoto T, Wakayama M, et al. Gangliocytic paragang-lioma: a multi-institutional retrospective study in Japan. BMC Cancer, 2015, 269(15): 1-9.
14.	黃斌, 趙紀春, 馬玉奎. 原發性腹膜后腫瘤的診斷和治療. 中國普外基礎與臨床雜志, 2008, 15(8): 607-608.
15.	Norton JA, Kivlen M, Li M, et al. Morbidity and mortality of aggressive resection in patients with advanced neuroendocrine tumors. Arch Surg, 2003, 138(8): 859-866.
16.	Chang F, Vu C, Chandra A, et al. Endoscopic ultrasound-guided fine needle aspiration cytology of pancreatic neuroendocrine tumours:cytomorphological and immunocytochemical evaluation. Cytopa-thology, 2006, 17(1): 10-17.
17.	Niederle MB, Hackl M, Kaserer K, et al. Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging basedon the WHO and European Neuroendocrine Tumour Society classi-fication: an analysis based on prospectively collected parameters. Endocr Relat Cancer, 2010, 17(4): 909-918.
18.	Andersen KF, Altaf R, Krarup-Hansen A, et al. Malignant pheochro-mocytomas and paragangliomas-the importance of a multidiscip-linary approach. Cancer Treat Rev, 2011, 37(2): 111-119.
19.	郭偉, 張宏鵬. 血管外科技術在腹膜后腫瘤診療中的應用. 中華外科雜志, 2007, 45(3): 157-160.
20.	Oh DY, Kim TW, Park YS, et al. Phase 2 study of everolimus mono-therapy in patients with nonfunctioning neuroendocrine tumors or pheochromocytomas/paragangliomas. Cancer, 2012, 118(24): 6162-6170.
21.	Lowery AJ, Walsh S, Mcdermott EW, et al. Molecular and therapeuticadvances in the diagnosis and management of malignant pheochro-mocytomas and paragangliomas. Oncologist, 2013, 18(4): 391-407.
22.	Fassnacht M, Kreissl MC, Weismann D, et al. New targets and thera-peutic approaches for endocrine malignancies. Pharmacol Ther, 2009, 123(1): 117-141.
23.	Shah U, Giubellino A, Pacak K. Pheochromocytoma: implications in tumorigenesis and the actual management. Minerva Endocrinol, 2012, 37(2): 141-156.
24.	Huang H, Abraham J, Hung E, et al. Treatment of malignant pheo-chromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer, 2008, 113(8): 2020-2028.

1. 胡維維, 王輝, 陶金華, 等. 腎上腺外副神經節瘤臨床病理分析. 臨床與實驗病理學雜志, 2010, 26(1): 77-79.
2. 陳杰. 副神經節瘤. 中華病理學雜志, 2006, 35(8): 494-496.
3. Fletcher C, Bridge JA, Hogendoorn P, et al. World Health Organiza-tion classification of tumours of soft tissue and bone. Lyon: IARC Press, 2013: 236-238.
4. Kl?ppel G, Couvelard A, Perren A, et al. ENETS consensus guide-lines for the standards of care in neuroendocrine tumors: towards a standardized approach to the diagnosis of gastroenteropancreatic neuroendocrine tumors and their prognostic stratification. Neuroendocrinology, 2009, 90(2): 162-166.
5. Yao JC, Hassan M, Phan A, et al. One hundred years after ‘carcinoid’: epidemiology of and prognostic factors for neuroendocrine tumors in 35 825 cases in the United States. J Clin Oncol, 2008, 26(18): 3063-3072.
6. Ayala-Ramirez M, Feng L, Johnson MM, et al. Clinical risk factors for malignancy and overall survival in patients with pheochromoc-ytomas and sympathetic paragangliomas: primary tumor size and primary tumor location as prognostic indicators. J Clin Endocrinol Metab, 2011, 96(3): 717-725.
7. Chen H, Sippel RS, O'dorisio MS, et al. The North American Neuro-endocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas, 2010, 39(6): 775-783.
8. 李峰, 陳小巖, 陳新, 等. Syn、CgA、melan A和inhibin α在腎上腺皮質腺瘤及嗜鉻細胞瘤中的表達及意義. 診斷病理學雜志, 2014, 21(8): 501-502.
9. Zhang C, Tan C, Ding H, et al. Selective VEGFR inhibitors for anti-cancer therapeutics in clinical use and clinical trials. Curr Pharm Des, 2012, 18(20): 2921-2935.
10. Park J, Song C, Park M, et al. Predictive characteristics of malignant pheochromocytoma. Korean J Urol, 2011, 52(4): 241-246.
11. 王華, 陶健, 王伯胤. CT對診斷異位嗜鉻細胞瘤的價值. 實用腫瘤雜志, 2010, 25(2): 217-219.
12. Parenti G, Zampetti B, Rapizzi E, et al. Updated and new perspec-tives on diagnosis, prognosis, and therapy of malignant pheochro-mocytoma/paraganglioma. J Oncol, 2012, 2012: 872713.
13. Okubo Y, Nemoto T, Wakayama M, et al. Gangliocytic paragang-lioma: a multi-institutional retrospective study in Japan. BMC Cancer, 2015, 269(15): 1-9.
14. 黃斌, 趙紀春, 馬玉奎. 原發性腹膜后腫瘤的診斷和治療. 中國普外基礎與臨床雜志, 2008, 15(8): 607-608.
15. Norton JA, Kivlen M, Li M, et al. Morbidity and mortality of aggressive resection in patients with advanced neuroendocrine tumors. Arch Surg, 2003, 138(8): 859-866.
16. Chang F, Vu C, Chandra A, et al. Endoscopic ultrasound-guided fine needle aspiration cytology of pancreatic neuroendocrine tumours:cytomorphological and immunocytochemical evaluation. Cytopa-thology, 2006, 17(1): 10-17.
17. Niederle MB, Hackl M, Kaserer K, et al. Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging basedon the WHO and European Neuroendocrine Tumour Society classi-fication: an analysis based on prospectively collected parameters. Endocr Relat Cancer, 2010, 17(4): 909-918.
18. Andersen KF, Altaf R, Krarup-Hansen A, et al. Malignant pheochro-mocytomas and paragangliomas-the importance of a multidiscip-linary approach. Cancer Treat Rev, 2011, 37(2): 111-119.
19. 郭偉, 張宏鵬. 血管外科技術在腹膜后腫瘤診療中的應用. 中華外科雜志, 2007, 45(3): 157-160.
20. Oh DY, Kim TW, Park YS, et al. Phase 2 study of everolimus mono-therapy in patients with nonfunctioning neuroendocrine tumors or pheochromocytomas/paragangliomas. Cancer, 2012, 118(24): 6162-6170.
21. Lowery AJ, Walsh S, Mcdermott EW, et al. Molecular and therapeuticadvances in the diagnosis and management of malignant pheochro-mocytomas and paragangliomas. Oncologist, 2013, 18(4): 391-407.
22. Fassnacht M, Kreissl MC, Weismann D, et al. New targets and thera-peutic approaches for endocrine malignancies. Pharmacol Ther, 2009, 123(1): 117-141.
23. Shah U, Giubellino A, Pacak K. Pheochromocytoma: implications in tumorigenesis and the actual management. Minerva Endocrinol, 2012, 37(2): 141-156.
24. Huang H, Abraham J, Hung E, et al. Treatment of malignant pheo-chromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer, 2008, 113(8): 2020-2028.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Clinical Features Analysis of 34 Patients with Retroperitoneal Paragangliomas

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