Clinical features, treatment and prognostic factors of primary appendiceal tumor_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

LUO Huan ¹ , LI Jianxin ¹ , GOU Haoxian ² ,  YANG Qingqiang ¹

1. Department of Gastrointestinal Surgery, Affiliated Hospital of Southwest Medical University, Luzhou, Sihcuan 646000, P. R. China;
2. Sichuan Key Laboratory of Nuclear Medicine and Molecular Imaging, Affiliated Hospital of Southwest Medical University, Luzhou, Sihcuan 646000, P. R. China;

Corresponding?author：

YANG Qingqiang, Email: yangqingqiang121@163.com

Keywords：

primary appendiceal tumor; clinical characteristic; treatment method; prognosis

DOI：

10.7507/1007-9424.202301007

Video：

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Objective To explore the clinical features, treatment methods and prognostic factors of primary appendiceal tumors. Methods The clinicopathologic data of 82 patients with primary appendiceal tumor who met the inclusion and exclusion criteria and were treated in the Affiliated Hospital of Southwest Medical University from January 2013 to November 2022 were retrospectively collected, and the clinicopathologic characteristics of the patients were summarized. Log-rank and Cox proportional hazard regression models were further used for univariate and multivariate survival analysis for patients without other malignant tumors. Results Among the 82 patients with primary appendiceal tumor, 9 cases (11.0%) were complicated with other malignant tumors. Low-grade mucinous tumors were the most common (51.2%, 42/82), 25 cases were complicated with appendicitis and 12 cases were complicated with intestinal obstruction. Common clinical manifestations included abdominal pain, changes in bowel habits, abdominal distension, vomiting, nausea, etc. Among the patients without other malignancies, 69 patients were followed up for a median of 32 months (4–106 months), 18 of whom died. The 1-year, 2-year, 3-year and 5-year cumulative survival rates were 91.1%, 87.5%, 80.5% and 60.7%, respectively. Multivariate Cox proportional hazard regression analysis showed that platelet-to-lymphocyte ratio (P=0.004), anemia (P=0.015), tumor metastasis (P=0.007) and diabetes mellitus (P=0.011) were prognostic factors. Among them, patients with platelet-to-lymphocyte ratio >190.1, anemia, tumor metastasis and diabetes mellitus had poor prognosis. Conclusions The main pathological type of primary appendiceal tumors is low grade mucinous tumors. It lacks characteristic clinical manifestations, with abdominal pain and changes in bowel habits as the main manifestations, and can be accompanied by appendicitis and intestinal obstruction. There is no unified standard for treatment. For patients characterized by prognostic risk factors including platelet-to-lymphocyte ratio >190.1, anemia, tumor metastasis and diabetes, further monitoring and intervention are required.

Citation： LUO Huan, LI Jianxin, GOU Haoxian, YANG Qingqiang. Clinical features, treatment and prognostic factors of primary appendiceal tumor. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2023, 30(6): 721-727. doi: 10.7507/1007-9424.202301007 Copy

1.	Murphy EM, Farquharson SM, Moran BJ. Management of an unexpected appendiceal neoplasm. Br J Surg, 2006, 93(7): 783-792.
2.	Garland-Kledzik M, Maithel S, Jafari MD, et al. Misdiagnosis of appendiceal neoplasms as ovarian tumors: impact of prior gynecologic surgery on definitive cytoreduction and HIPEC. Eur J Surg Oncol, 2022, 48(2): 449-454.
3.	Behera PK, Rath PK, Panda R, et al. Primary appendiceal mucinous adenocarcinoma. Indian J Surg, 2011, 73(2): 146-148.
4.	Gortchev G, Tomov S, Dimitrov D, et al. Appendiceal mucocele presenting as a right adnexal mass: a case report. Obstet Gynecol Int, 2010, 2010: 281053. doi: 10.1155/2010/281053.
5.	程研, 譚震, 王長順. 原發性闌尾腺癌的臨床特點及預后影響因素. 中華臨床醫師雜志 (電子版), 2013, 7(8): 3568-3570.
6.	Misdraji J. Appendiceal mucinous neoplasms: controversial issues. Arch Pathol Lab Med, 2010, 134(6): 864-870.
7.	Glasgow SC, Gaertner W, Stewart D, et al. The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms. Dis Colon Rectum, 2019, 62(12): 1425-1438.
8.	呂靖芳, 湯慶超. 中國闌尾腫瘤多學科綜合治療專家共識 (2021版). 中華結直腸疾病電子雜志, 2021, 10(3): 225-231.
9.	Nitecki SS, Wolff BG, Schlinkert R, et al. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg, 1994, 219(1): 51-57.
10.	Shaib WL, Assi R, Shamseddine A, et al. Appendiceal mucinous neoplasms: diagnosis and management. Oncologist, 2017, 22(9): 1107-1116.
11.	武穎超, 文龍, 竇偉冬, 等. 原發性闌尾腫瘤的臨床病理分析及手術決策. 中華胃腸外科雜志, 2021, 24(12): 1065-1072.
12.	Cortina R, McCormick J, Kolm P, et al. Management and prognosis of adenocarcinoma of the appendix. Dis Colon Rectum, 1995, 38(8): 848-852.
13.	Tsang ES, McConnell YJ, Schaeffer DF, et al. Outcomes of surgical and chemotherapeutic treatments of goblet cell carcinoid tumors of the appendix. Ann Surg Oncol, 2018, 25(8): 2391-2399.
14.	Pai RK, Beck AH, Norton JA, et al. Appendiceal mucinous neoplasms: clinicopathologic study of 116 cases with analysis of factors predicting recurrence. Am J Surg Pathol, 2009, 33(10): 1425-1439.
15.	Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum, 1998, 41(1): 75-80.
16.	Esmer-Sanchez DD, Martinez-Ordaz JL, Roman-Zepeda P, et al. Appendiceal tumors. Clinicopathologic review of 5,307 appendectomies. Cir Cir, 2004, 72(5): 375-378.
17.	Tang LH, Shia J, Soslow RA, et al. Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix. Am J Surg Pathol, 2008, 32(10): 1429-1443.
18.	Bucher P, Gervaz P, Ris F, et al. Surgical treatment of appendiceal adenocarcinoid (goblet cell carcinoid). World J Surg, 2005, 29(11): 1436-1439.
19.	Varisco B, McAlvin B, Dias J, et al. Adenocarcinoid of the appendix: is right hemicolectomy necessary? A meta-analysis of retrospective chart reviews. Am Surg, 2004, 70(7): 593-599.
20.	P?rvanov P. Primary malignant appendiceal tumors. Khirurgiia (Sofiia), 1997, 50(4): 46-49.
21.	Shenoy S. Goblet cell carcinoids of the appendix: tumor biology, mutations and management strategies. World J Gastrointest Surg, 2016, 8(10): 660-669.
22.	Shaib W, Krishna K, Kim S, et al. Appendiceal neuroendocrine, goblet and signet-ring cell tumors: a spectrum of diseases with different patterns of presentation and outcome. Cancer Res Treat, 2016, 48(2): 596-604.
23.	Wang G, Li Q, Chen W. Chemotherapy in the treatment of different histological types of appendiceal cancers: a SEER based study. BMC Cancer, 2021, 21(1): 778. doi: 10.1186/s12885-021-08502-3.
24.	Almasri SS, Paniccia A, Hammad AY, et al. The role of adjuvant chemotherapy following right hemicolectomy for non-metastatic mucinous and nonmucinous appendiceal adenocarcinoma. J Gastrointest Surg, 2022, 26(1): 171-180.
25.	Lieu CH, Lambert LA, Wolff RA, et al. Systemic chemotherapy and surgical cytoreduction for poorly differentiated and signet ring cell adenocarcinomas of the appendix. Ann Oncol, 2012, 23(3): 652-658.
26.	Zakka K, Williamson S, Jiang R, et al. Is adjuvant chemotherapy beneficial for stage Ⅱ–Ⅲ goblet cell carcinoid/goblet cell adenocarcinoma of the appendix? Surg Oncol, 2021, 36: 120-129.
27.	Asare EA, Compton CC, Hanna NN, et al. The impact of stage, grade, and mucinous histology on the efficacy of systemic chemotherapy in adenocarcinomas of the appendix: analysis of the National Cancer Data Base. Cancer, 2016, 122(2): 213-221.
28.	Alexander-Sefre F, Chandrakumaran K, Banerjee S, et al. Elevated tumour markers prior to complete tumour removal in patients with pseudomyxoma peritonei predict early recurrence. Colorectal Dis, 2005, 7(4): 382-386.
29.	Carmignani CP, Hampton R, Sugarbaker CE, et al. Utility of CEA and CA19-9 tumor markers in diagnosis and prognostic assessment of mucinous epithelial cancers of the appendix. J Surg Oncol, 2004, 87(4): 162-166.
30.	Fournier K, Rafeeq S, Taggart M, et al. Low-grade appendiceal mucinous neoplasm of uncertain malignant potential (LAMN-UMP): prognostic factors and implications for treatment and follow-up. Ann Surg Oncol, 2017, 24(1): 187-193.
31.	Taflampas P, Dayal S, Chandrakumaran K, et al. Pre-operative tumour marker status predicts recurrence and survival after complete cytoreduction and hyperthermic intraperitoneal chemotherapy for appendiceal Pseudomyxoma Peritonei: analysis of 519 patients. Eur J Surg Oncol, 2014, 40(5): 515-520.
32.	Baratti D, Kusamura S, Martinetti A, et al. Prognostic value of circulating tumor markers in patients with pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol, 2007, 14(8): 2300-2308.
33.	van Ruth S, Hart AA, Bonfrer JM, et al. Prognostic value of baseline and serial carcinoembryonic antigen and carbohydrate antigen 199 measurements in patients with pseudomyxoma peritonei treated with cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol, 2002, 9(10): 961-967.
34.	Chua TC, Chong CH, Liauw W, et al. Inflammatory markers in blood and serum tumor markers predict survival in patients with epithelial appendiceal neoplasms undergoing surgical cytoreduction and intraperitoneal chemotherapy. Ann Surg, 2012, 256(2): 342-349.
35.	張體碩. 預后營養指數 (PNI) 及炎癥標記物對卵巢癌預后的影響. 吉林: 吉林大學, 2021.
36.	Sugarbaker PH. Epithelial appendiceal neoplasms. Cancer J, 2009, 15(3): 225-235.

1. Murphy EM, Farquharson SM, Moran BJ. Management of an unexpected appendiceal neoplasm. Br J Surg, 2006, 93(7): 783-792.
2. Garland-Kledzik M, Maithel S, Jafari MD, et al. Misdiagnosis of appendiceal neoplasms as ovarian tumors: impact of prior gynecologic surgery on definitive cytoreduction and HIPEC. Eur J Surg Oncol, 2022, 48(2): 449-454.
3. Behera PK, Rath PK, Panda R, et al. Primary appendiceal mucinous adenocarcinoma. Indian J Surg, 2011, 73(2): 146-148.
4. Gortchev G, Tomov S, Dimitrov D, et al. Appendiceal mucocele presenting as a right adnexal mass: a case report. Obstet Gynecol Int, 2010, 2010: 281053. doi: 10.1155/2010/281053.
5. 程研, 譚震, 王長順. 原發性闌尾腺癌的臨床特點及預后影響因素. 中華臨床醫師雜志 (電子版), 2013, 7(8): 3568-3570.
6. Misdraji J. Appendiceal mucinous neoplasms: controversial issues. Arch Pathol Lab Med, 2010, 134(6): 864-870.
7. Glasgow SC, Gaertner W, Stewart D, et al. The American Society of Colon and Rectal Surgeons, Clinical Practice Guidelines for the Management of Appendiceal Neoplasms. Dis Colon Rectum, 2019, 62(12): 1425-1438.
8. 呂靖芳, 湯慶超. 中國闌尾腫瘤多學科綜合治療專家共識 (2021版). 中華結直腸疾病電子雜志, 2021, 10(3): 225-231.
9. Nitecki SS, Wolff BG, Schlinkert R, et al. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg, 1994, 219(1): 51-57.
10. Shaib WL, Assi R, Shamseddine A, et al. Appendiceal mucinous neoplasms: diagnosis and management. Oncologist, 2017, 22(9): 1107-1116.
11. 武穎超, 文龍, 竇偉冬, 等. 原發性闌尾腫瘤的臨床病理分析及手術決策. 中華胃腸外科雜志, 2021, 24(12): 1065-1072.
12. Cortina R, McCormick J, Kolm P, et al. Management and prognosis of adenocarcinoma of the appendix. Dis Colon Rectum, 1995, 38(8): 848-852.
13. Tsang ES, McConnell YJ, Schaeffer DF, et al. Outcomes of surgical and chemotherapeutic treatments of goblet cell carcinoid tumors of the appendix. Ann Surg Oncol, 2018, 25(8): 2391-2399.
14. Pai RK, Beck AH, Norton JA, et al. Appendiceal mucinous neoplasms: clinicopathologic study of 116 cases with analysis of factors predicting recurrence. Am J Surg Pathol, 2009, 33(10): 1425-1439.
15. Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum, 1998, 41(1): 75-80.
16. Esmer-Sanchez DD, Martinez-Ordaz JL, Roman-Zepeda P, et al. Appendiceal tumors. Clinicopathologic review of 5,307 appendectomies. Cir Cir, 2004, 72(5): 375-378.
17. Tang LH, Shia J, Soslow RA, et al. Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix. Am J Surg Pathol, 2008, 32(10): 1429-1443.
18. Bucher P, Gervaz P, Ris F, et al. Surgical treatment of appendiceal adenocarcinoid (goblet cell carcinoid). World J Surg, 2005, 29(11): 1436-1439.
19. Varisco B, McAlvin B, Dias J, et al. Adenocarcinoid of the appendix: is right hemicolectomy necessary? A meta-analysis of retrospective chart reviews. Am Surg, 2004, 70(7): 593-599.
20. P?rvanov P. Primary malignant appendiceal tumors. Khirurgiia (Sofiia), 1997, 50(4): 46-49.
21. Shenoy S. Goblet cell carcinoids of the appendix: tumor biology, mutations and management strategies. World J Gastrointest Surg, 2016, 8(10): 660-669.
22. Shaib W, Krishna K, Kim S, et al. Appendiceal neuroendocrine, goblet and signet-ring cell tumors: a spectrum of diseases with different patterns of presentation and outcome. Cancer Res Treat, 2016, 48(2): 596-604.
23. Wang G, Li Q, Chen W. Chemotherapy in the treatment of different histological types of appendiceal cancers: a SEER based study. BMC Cancer, 2021, 21(1): 778. doi: 10.1186/s12885-021-08502-3.
24. Almasri SS, Paniccia A, Hammad AY, et al. The role of adjuvant chemotherapy following right hemicolectomy for non-metastatic mucinous and nonmucinous appendiceal adenocarcinoma. J Gastrointest Surg, 2022, 26(1): 171-180.
25. Lieu CH, Lambert LA, Wolff RA, et al. Systemic chemotherapy and surgical cytoreduction for poorly differentiated and signet ring cell adenocarcinomas of the appendix. Ann Oncol, 2012, 23(3): 652-658.
26. Zakka K, Williamson S, Jiang R, et al. Is adjuvant chemotherapy beneficial for stage Ⅱ–Ⅲ goblet cell carcinoid/goblet cell adenocarcinoma of the appendix? Surg Oncol, 2021, 36: 120-129.
27. Asare EA, Compton CC, Hanna NN, et al. The impact of stage, grade, and mucinous histology on the efficacy of systemic chemotherapy in adenocarcinomas of the appendix: analysis of the National Cancer Data Base. Cancer, 2016, 122(2): 213-221.
28. Alexander-Sefre F, Chandrakumaran K, Banerjee S, et al. Elevated tumour markers prior to complete tumour removal in patients with pseudomyxoma peritonei predict early recurrence. Colorectal Dis, 2005, 7(4): 382-386.
29. Carmignani CP, Hampton R, Sugarbaker CE, et al. Utility of CEA and CA19-9 tumor markers in diagnosis and prognostic assessment of mucinous epithelial cancers of the appendix. J Surg Oncol, 2004, 87(4): 162-166.
30. Fournier K, Rafeeq S, Taggart M, et al. Low-grade appendiceal mucinous neoplasm of uncertain malignant potential (LAMN-UMP): prognostic factors and implications for treatment and follow-up. Ann Surg Oncol, 2017, 24(1): 187-193.
31. Taflampas P, Dayal S, Chandrakumaran K, et al. Pre-operative tumour marker status predicts recurrence and survival after complete cytoreduction and hyperthermic intraperitoneal chemotherapy for appendiceal Pseudomyxoma Peritonei: analysis of 519 patients. Eur J Surg Oncol, 2014, 40(5): 515-520.
32. Baratti D, Kusamura S, Martinetti A, et al. Prognostic value of circulating tumor markers in patients with pseudomyxoma peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol, 2007, 14(8): 2300-2308.
33. van Ruth S, Hart AA, Bonfrer JM, et al. Prognostic value of baseline and serial carcinoembryonic antigen and carbohydrate antigen 199 measurements in patients with pseudomyxoma peritonei treated with cytoreduction and hyperthermic intraperitoneal chemotherapy. Ann Surg Oncol, 2002, 9(10): 961-967.
34. Chua TC, Chong CH, Liauw W, et al. Inflammatory markers in blood and serum tumor markers predict survival in patients with epithelial appendiceal neoplasms undergoing surgical cytoreduction and intraperitoneal chemotherapy. Ann Surg, 2012, 256(2): 342-349.
35. 張體碩. 預后營養指數 (PNI) 及炎癥標記物對卵巢癌預后的影響. 吉林: 吉林大學, 2021.
36. Sugarbaker PH. Epithelial appendiceal neoplasms. Cancer J, 2009, 15(3): 225-235.

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CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Clinical features, treatment and prognostic factors of primary appendiceal tumor

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