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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 50  |  Issue : 2  |  Page : 81-84

Management of primary neoplasms of the appendix


Department of Surgery, Division of General Surgery, Renai Branch, Taipei City Hospital, Taiwan

Date of Submission21-Jul-2016
Date of Decision09-Sep-2016
Date of Acceptance21-Oct-2016
Date of Web Publication18-Apr-2017

Correspondence Address:
Ming-Jui Yang
Department of Surgery, Division of General Surgery, Renai Branch, Taipei City Hospital, No. 10, Section 4, Renai Road, Taipei
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/fjs.fjs_25_17

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  Abstract 

Primary appendiceal adenocarcinoma is a rare disease, constituting <0.5% of all neoplasms of the gastrointestinal origin. Primary appendiceal adenocarcinoma presents classically as an inflammatory condition such as acute appendicitis, and its preoperative diagnosis is difficult. In this case series, we report the cases of five patients with appendiceal neoplasms who underwent laparoscopic resection of appendiceal tumors and have thus far been healthy without any evidence of recurrence. Opinions regarding the most appropriate management of appendiceal tumors are variable. Recently, laparoscopic resection of appendiceal tumors has been added as a surgical option. If adequate preoperative assessment and definite diagnosis can be performed, laparoscopic resection of appendiceal tumors might become a feasible surgical option instead of open surgery in selected patients.

Keywords: Acute appendicitis, laparoscopic surgery, primary neoplasm of the appendix, right hemicolectomy


How to cite this article:
Wang SL, Yang MJ, Ting CT, Chen PC. Management of primary neoplasms of the appendix. Formos J Surg 2017;50:81-4

How to cite this URL:
Wang SL, Yang MJ, Ting CT, Chen PC. Management of primary neoplasms of the appendix. Formos J Surg [serial online] 2017 [cited 2019 Oct 19];50:81-4. Available from: http://www.e-fjs.org/text.asp?2017/50/2/81/204664


  Introduction Top


Primary appendiceal adenocarcinoma is a rare disease,[1] constituting <0.5% of all neoplasms of the gastrointestinal origin. This disease was first reported by Berger [2] in 1882. Appendiceal neoplasms present clinically as an inflammatory condition, such as acute appendicitis.[3] The preoperative diagnosis of this disease is often difficult. Therefore, it is usually diagnosed by the pathological examination of surgical specimens. Generally, either appendectomy or right hemicolectomy is performed.

Laparoscopic techniques have also been used in recent years. We included five patients with appendiceal neoplasms in our series [from 2006 to 2014; [Table 1]. Two patients presented with acute appendicitis, one presented with colon intussusception, and two presented with cecal tumors. Here, we report the surgical experiences of five patients with appendiceal neoplasms and review optimal surgical procedures, particularly in the laparoscopic era.
Table 1: Patients' summary

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  Case Reports Top


Case 1

A 56-year-old female presented with pain in the right lower quadrant of the abdomen. Blood examination revealed leukocytosis. Abdominal computed tomography (CT) revealed an engorged appendix with fat stranding [Figure 1] and [Figure 2]. Therefore, a diagnosis of acute appendicitis was made, and appendectomy was performed. Pathological findings showed mucinous adenocarcinoma of the appendix with perforation. Therefore, the right hemicolectomy was performed with complete pathological resection [Figure 3]. The postoperative course was uneventful. She has remained healthy for 5 years after the surgery with no evidence of recurrence.
Figure 1: Abdominal computed tomography (coronal section) revealed an engorged appendix (arrowhead) with fat stranding

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Figure 2: Abdominal computed tomography (sagittal section) revealed an engorged appendix (arrowhead) with fat stranding

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Figure 3: Resected specimen of right hemicolectomy

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Case 2

A 78-year-old male presented with a 2-day history of pain in the right lower quadrant of the abdomen. On the basis of the findings of abdominal CT, a diagnosis of acute appendicitis was established. Thus, he underwent appendectomy. However, because pathological findings revealed mucinous adenocarcinoma of the appendix, the right hemicolectomy was performed. He has remained healthy for 5 years after the surgery without any evidence of recurrence.

Case 3

A 45-year-old female presented with ileal and colonic obstruction. Abdominal CT revealed ileocolic intussusception. Thus, the right hemicolectomy was performed along with the resection of the intussusception lesion. An appendiceal tumor was observed as the leading point [Figure 4]. Final pathological reports revealed mucinous adenocarcinoma of the appendix. She has remained healthy for 6 years after the surgery.
Figure 4: One appendiceal tumor (arrowhead) measuring 4.0 cm × 1.8 cm × 1.5 cm in size was identified as the leading point

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Case 4

A 74-year-old female underwent colonoscopy for colon cancer screening, which revealed a carcinoma in the cecal area. Abdominal CT revealed a 5 cm × 2 cm × 2 cm tumor in the cecal area. Therefore, we performed elective right hemicolectomy. Furthermore, pathological reports revealed an appendiceal mucinous adenocarcinoma, pT3N0M0. No obvious cancer recurrence was noted during the 9-year follow-up period.

Case 5

A 57-year-old female presented with a several months history of intermittent abdominal pain in the right lower quadrant. Fiber colonoscopy and abdominal CT revealed a broad-based polypoid cecal mass in the cecum. She underwent laparoscopic right hemicolectomy. Furthermore, pathological findings revealed an appendiceal mucinous adenocarcinoma arising in a tubulovillous adenoma. She has remained healthy for 2 years after the surgery.


  Discussion Top


Although colon cancer is a very common malignancy, primary carcinomas of the appendix are rare.[1] The incidence of primary appendiceal adenocarcinoma ranges from 0.01% to 0.11% for all appendectomies.[3],[4] An appendiceal neoplasm often presents clinically as acute appendicitis. The preoperative diagnosis of appendiceal neoplasms is often difficult. Therefore, they are usually identified during the pathological examination of surgical specimens. Depending on the pathological findings, clinicians sometimes consider performing additional right hemicolectomy as a secondary procedure to obtain an improved oncological outcome. In fact, two of our patients underwent appendectomy for appendicitis, followed by right hemicolectomy for long-term survival.

Appendiceal malignancies are classified as carcinoid type mucinous adenocarcinomas, colonic type adenocarcinomas, signet ring cell type carcinomas, and malignant carcinoid carcinomas. Carcinoid tumor is the most frequently encountered carcinoma type. A metastatic disease may be encountered in advanced stages if diagnosed or treated late. Turaga et al.[5] reported that the histological subtype is a crucial predictor of disease-free and overall survival in patients with appendiceal neoplasms. Patients with noncarcinoid adenocarcinomas of the appendix, with neoplasms >2 cm in size and neoplasms involving the base of the appendix or the mesoappendix, should be considered for immediate right hemicolectomy to obtain an optimal outcome.[4],[6],[7],[8],[9]

An accurate preoperative diagnosis to distinguish between tumors of the appendix and acute appendicitis is necessary for selecting an appropriate surgical procedure. Abdominal CT, ultrasonography, and endoscopy may facilitate preoperative diagnosis and assessment.[10],[11],[12]

In recent years, laparoscopic appendectomy has been widely used. However, indications for the laparoscopic resection of appendiceal tumors are beginning to be established. However, in their 10-year review, Bucher et al.[13] indicated that laparoscopic appendectomy may involve the risk of inadequate resection. In case of doubt regarding laparoscopic resection, the procedure may be modified to laparotomy for improved exploration and right hemicolectomy. In 1993, Schmidt et al.[14],[15] reported that the conversion to open appendectomy may be the most prudent surgical judgment when appendiceal neoplasm is suspected.

All our patients had mucinous adenocarcinomas, which are usually slow-progressing, well-differentiated cancers. However, perforation may result in metastasis or pseudomyxoma peritonei.[16] Even after long-term follow-up after surgery, our patients are alive without recurrence thus far.

Of our five cases, two underwent emergency appendectomy followed by the right hemicolectomy. If a definite pathological report including frozen section pathology and preoperative image study evaluation is available, laparoscopic surgery can be selected instead of open laparotomy.


  Conclusion Top


Right hemicolectomy is recommended for most appendiceal adenocarcinomas. In our opinion, if definite diagnosis and preoperative assessment results are available, laparoscopic resection of an appendiceal tumor might be a more feasible surgical option than open surgery in selected cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

The study was supported by Renai Branch, Taipei City Hospital.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Benedix F, Reimer A, Gastinger I, Mroczkowski P, Lippert H, Kube R; Study Group Colon/Rectum Carcinoma Primary Tumor. Primary appendiceal carcinoma – Epidemiology, surgery and survival: Results of a German multi-center study. Eur J Surg Oncol 2010;36:763-71.  Back to cited text no. 1
    
2.
Deng K, Zhang CQ, Wang GL. Primary appendiceal mucinous adenocarcinoma mimicking bladder carcinoma: A case report and review of the literature. Wei Li Oncol Lett 2014;7:1270-2.  Back to cited text no. 2
    
3.
Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: Retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum 1998;41:75-80.  Back to cited text no. 3
    
4.
Rutledge RH, Alexander JW. Primary appendiceal malignancies: Rare but important. Surgery 1992;111:244-50.  Back to cited text no. 4
    
5.
Turaga KK, Pappas SG, Gamblin T. Importance of histologic subtype in the staging of appendiceal tumors. Ann Surg Oncol 2012;19:1379-85.  Back to cited text no. 5
    
6.
Rossi G, Valli R, Bertolini F, Sighinolfi P, Losi L, Cavazza A, et al. Does mesoappendix infiltration predict a worse prognosis in incidental neuroendocrine tumors of the appendix? A clinicopathologic and immunohistochemical study of 15 cases. Am J Clin Pathol 2003;120:706-11.  Back to cited text no. 6
    
7.
Kulke MH, Mayer RJ. Carcinoid tumors. N Engl J Med 1999;340:858-68.  Back to cited text no. 7
    
8.
Deans GT, Spence RA. Neoplastic lesions of the appendix. Br J Surg 1995;82:299-306.  Back to cited text no. 8
    
9.
O'Donnell ME, Badger SA, Beattie GC, Carson J, Garstin WI. Malignant neoplasms of the appendix. Int J Colorectal Dis 2007;22:1239-48.  Back to cited text no. 9
    
10.
Pickhardt PJ, Levy AD, Rohrmann CA Jr., Kende AI. Primary neoplasms of the appendix: Radiologic spectrum of disease with pathologic correlation. Radiographics 2003;23:645-62.  Back to cited text no. 10
    
11.
Takehara Y, Takahashi M, Isoda H, Kaneko M, Mochizuki K, Yuasa H, et al. Adult intussusception with an appendiceal mucocele diagnosed by CT and ultrasonography. Radiat Med 1989;7:139-42.  Back to cited text no. 11
    
12.
Sandor A, Modlin IM. A retrospective analysis of 1570 appendiceal carcinoids. Am J Gastroenterol 1998;93:422-8.  Back to cited text no. 12
    
13.
Bucher P, Mathe Z, Demirag A, Morel P. Appendix tumors in the era of laparoscopic appendectomy. Surg Endosc 2004;18:1063-6.  Back to cited text no. 13
    
14.
Schmidt JS, Maier D, Raj P, Remine SG. Laparoscopic management of appendiceal intussusception associated with villous adenocarcinoma. J Laparoendosc Surg 1994;4:369-73.  Back to cited text no. 14
    
15.
González Moreno S, Shmookler BM, Sugarbaker PH. Appendiceal mucocele. Contraindication to laparoscopic appendectomy. Surg Endosc 1998;12:1177-9.  Back to cited text no. 15
    
16.
Paolucci V, Schaeff B, Schneider M, Gutt C. Tumor seeding following laparoscopy: International survey. World J Surg 1999;23:989-95.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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