Joint Hospital Surgical Grand Round Management of a

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

introduction � � � primary tumor of the appendix are rare account for ~0. 4 -1 % of all gastrointestinal malignancy ~1 % of all appendicectomy specimen

• 74 neoplasms in 7970 appendicectomy specimen • 20 are malignant Conor SJ et al. Dis Colon Rectum 1998 • 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 2010

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

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

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 - ~7 cm abscess around - base healthy �

pathology : - 9 cm long, diffuse dilated appendix with 1. 5 cm diameter at proximal end and 2 cm at distal end - carcinoid tumor, mesenteric and lymphovascular invasion, margin involved

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

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

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

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

� 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

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 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 > 2 cm; 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% Payam S Pahlaven et al. world journal of surgical oncology 2005

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 Laura H. Tang, et al. Am J Surg Pathol 2008

a single center study, 63 cases: typical GCC (group A) adenocarcinoma ex GCC, signet ring cell type (group B ) adenocarcinoma ex GCC, poorly differentiated carcinoma type (group C) minimal atypia and minimal distortion of appendiceal wall signficant cytologic atypia, associated destruction of the appendiceal wall 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% Laura H. Tang, et la. Am J Surg Pathol 2008

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

� 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

3 rd operation with debulking done 03/2013 - 10 cm tumour mass arising from previous ileocolonic anastomosis - 5 cm peritoneal mass at left iliac fossa - another 7 cm mass at greater omentum pathology: all are metastatic neuroendocrine carcinoma

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 H. Mahteme et al. British Journal of Surgery 2004

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

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

thank you any question…