Management of a rare type of Ca appendix

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

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

3. 74 neoplasms in 7970 appendicectomy specimen • 20 are malignant Conor SJ et al. Dis Colon Rectum 1998 • 17 neoplasms in 1492 specimen Ma, KW et al. HK MJ 2010 • 41 neoplasms in 8560 specimen • adenocarcinoma (16) and carcinoid (15) Richard K.Englehardt et al. Journal of Cancer Therapy, 2010

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

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: • 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. 3rd operation with debulking done 03/2013 - 10cm 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…