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…