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Management of a rare type of Ca appendix Dr. Lam Tang Yu Tuen Mun Hospital Joint Hospital Surgical Grand Round.

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Presentation on theme: "Management of a rare type of Ca appendix Dr. Lam Tang Yu Tuen Mun Hospital Joint Hospital Surgical Grand Round."— Presentation transcript:

1 Management of a rare type of Ca appendix Dr. Lam Tang Yu Tuen Mun Hospital Joint Hospital Surgical Grand Round

2 introduction  primary tumor of the appendix are rare  account for ~0.4-1 % of all gastrointestinal malignancy  ~1 % of all appendicectomy specimen

3 41 neoplasms in 8560 specimen adenocarcinoma (16) and carcinoid (15) Richard K.Englehardt et al. Journal of Cancer Therapy, 2010  17 neoplasms in 1492 specimen Ma, KW et al. HK MJ neoplasms in 7970 appendicectomy specimen 20 are malignant Conor SJ et al. Dis Colon Rectum 1998

4 primary appendiceal carcinoma classification epithelial: - mucinous (low grade to high grade, pseudomyxoma peritonei) - adenocarcinoma non-epithelial: - classical carcinoid (neuroendocrine) - goblet cell carcinoid / adenocarcinod - mesenchymal tumors: GIST, leiomyoma, sarcoma Misdraji J et al. Semin Diagn Pathol 2004

5 E.M.A. Murphy et al. British Journal of Surgery tumor recognized at time of surgery tumor < 2cm base of appendix and mesoappendix clear right hemicolectomy appendicectomy yes no

6 case presentation  55 years old gentleman, non-smoker, good past health  admitted in 08/2012 for RLQ pain, WCC 14  laparoscopy to open appendicectomy: - rupture acute inflamed appendix in retro-caecal position - ~7cm abscess around - base healthy

7 pathology : - 9cm long, diffuse dilated appendix with 1.5cm diameter at proximal end and 2cm at distal end - carcinoid tumor, mesenteric and lymphovascular invasion, margin involved

8 our patient, CT 09/2012: - heterogenous caecal mass - another mass medial to caecum, suggestive peritoneal involvement

9 laparoscopic right hemi-colectomy in 09/2012: - 5cm tumor growth at caecum with multiple enlarged mesenteric lymph node - another 5cm tumor bulk wrapped by omentum medial to caecum - loop of small bowel ~80cm from ileo-caecal valve invaded by tumor - a small pelvic nodule excised

10 pathology: - right hemi-colectomy: mixed adeno- neuroendocrine carcinoma (high grade neuroendocrine carcinoma and moderate differentiated adenocarcinoma), margin clear - pelvic nodule: high grade neuroendocrine metastatic carcinoma - no lymph node involvement (0/15)

11 goblet cell carcinoid (GCC) of appendix  variety of names: adenocarcinoid, adeno- neuroendocrine carcinoma, goblet cell carcinoid, intermediate type of carcinoid, etc  all names except GCC were omitted from WHO classification  biphasic histopathological appearance, recognized since 1960s

12  GCC accounts less than 5% of primary tumors of the appendix Gallegos NC et al. Eur J Surg Oncol 1992  3 GCC over 41 appendiceal neoplasm over 8560 specimen Richard K.Englehardt et al. Journal of Cancer Therapy, 2010

13 Payam S Pahlaven et al. world journal of surgical oncology 2005 a review from 1966 to 2004, nearly 600 cases:  mean age of presentation: 58.9 years (mean age of carcinoid: 35.9 years)  most common presentation: acute appendicitis  tend to present as diffuse thickening of whole appendix  ovaries and disseminated abdominal carcinomatosis most common distant metastasis  liver or other distant organ metastasis rare

14 Payam S Pahlaven et al. world journal of surgical oncology 2005 a review from 1966 to 2004, nearly 600 cases:  right hemicolectomy recommended if any one of following criteria are noted: - tumor size > 2cm; involvement of the base / lymph node - cellular undifferentiation; increase mitotic activity  bilateral salpingo-oophorectomy also advocated  chemotherapy 5 flurouracil and leucovorin advised  overall 5-year survival between 60% to 84%

15 Laura H. Tang, et al. Am J Surg Pathol 2008 a single center study, 63 cases:  most common growth pattern: circumferential involvement of appendiceal wall with longitudinal extension  63% patients present with stage IV disease  spectrum of histologic features and correlated with clinical behavior

16 Laura H. Tang, et la. Am J Surg Pathol 2008 a single center study, 63 cases: typical GCC (group A)minimal atypia and minimal distortion of appendiceal wall adenocarcinoma ex GCC, signet ring cell type (group B ) signficant cytologic atypia, associated destruction of the appendiceal wall adenocarcinoma ex GCC, poorly differentiated carcinoma type (group C) poorly differentiated carcinoma or a high grade neuroendocrine carcinoma for the stage IV-matched 5 year survival, group A: 100%; group B: 38% group C: 0%

17 our patient…  5 flurouracil and leucovorin, 6 cycles given  admitted in Jan 2013 for abdominal distension / sub-acute IO, resolved with conservative treatment  early FU CT arranged

18  CT 30/01/2013: - heterogenous mass at right upper abdomen in close vicinity to adjacent small bowel - another soft tissue mass in left pelvic region

19 3 rd operation with debulking done 03/ cm tumour mass arising from previous ileo- colonic anastomosis - 5cm peritoneal mass at left iliac fossa - another 7cm mass at greater omentum pathology : all are metastatic neuroendocrine carcinoma

20 H.Mahteme et al. British Journal of Surgery 2004 what else can we do… - cyto-reductive surgery and intra-peritoneal chemotherapy may help - 5-year survival: 25% - as invasive as that from colorectal adenocarcinoma with peritoneal carcinomatosis

21 follow up… - In-labeled octreotide scintigraphy - CT scan - plasma chromogranin A corresponding to tumor load - colonscopy: colorectal neoplasms found in 10% with carcinoid ; >50% with malignant epithelial tumour Conor SJ et al. Dis Colon Rectum 1998

22 bring home message…  diffuse “abnormally” dilated appendix, ?not simple appendicitis, ?goblet cell carcinoid of appendix  spectrum of clinical behavior for GCC  cyto-reductive surgery and intra-peritoneal chemotherapy may be a good option for GCC with peritoneal carcinomatosis  long term follow up for any type of Ca appendix

23 thank you any question…


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