Math Framework, Appendix B - Curriculum Frameworks (CA Dept of ...
Management of a rare type of Ca appendix
description
Transcript of Management of a rare type of Ca appendix
![Page 1: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/1.jpg)
Management of a rare type of
Ca appendix
Dr. Lam Tang Yu
Tuen Mun Hospital
Joint Hospital Surgical Grand Round
![Page 2: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/2.jpg)
introduction
primary tumor of the appendix are rare
account for ~0.4-1 % of all gastrointestinal malignancy
~1 % of all appendicectomy specimen
![Page 3: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/3.jpg)
• 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
![Page 4: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/4.jpg)
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
![Page 5: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/5.jpg)
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
![Page 6: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/6.jpg)
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
![Page 7: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/7.jpg)
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
![Page 8: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/8.jpg)
our patient, CT 09/2012:- heterogenous caecal mass- another mass medial to caecum, suggestive peritoneal involvement
![Page 9: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/9.jpg)
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
![Page 10: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/10.jpg)
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)
![Page 11: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/11.jpg)
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
![Page 12: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/12.jpg)
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
![Page 13: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/13.jpg)
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
![Page 14: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/14.jpg)
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%
![Page 15: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/15.jpg)
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
![Page 16: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/16.jpg)
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%
![Page 17: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/17.jpg)
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
![Page 18: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/18.jpg)
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
![Page 19: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/19.jpg)
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
![Page 20: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/20.jpg)
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
![Page 21: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/21.jpg)
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
![Page 22: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/22.jpg)
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
![Page 23: Management of a rare type of Ca appendix](https://reader036.fdocuments.in/reader036/viewer/2022062800/568142dc550346895daf3597/html5/thumbnails/23.jpg)
thank you
any question…