Management of a rare type of

Ca appendix

Dr. Lam Tang Yu

Tuen Mun Hospital

Joint Hospital Surgical Grand Round

introduction

primary tumor of the appendix are rare

account for ~0.4-1 % of all gastrointestinal malignancy

~1 % of all appendicectomy specimen

• 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

• 74 neoplasms in 7970 appendicectomy specimen • 20 are malignant

Conor SJ et al. Dis Colon Rectum 1998

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

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

right hemicolectomy

appendicectomy

yes

yes

yes

no

no

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

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

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

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

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)

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

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

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 appendicitistend to present as diffuse thickening of whole appendix

ovaries and disseminated abdominal carcinomatosis most common distant metastasisliver 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 > 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%

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

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 walladenocarcinoma 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%

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

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

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

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 tumourConor 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…