Transcript Carcinoid tumour of the appendix: Our experience

Carcinoid tumour of the appendix:
Experience at a district general hospital
A Powell-Chandler, S Gurjar, P Strauss, J Adamek,
B Biswas, MC Parker, A McIrvine, P Hanek, R Bhardwaj
Department of Surgery, Darent Valley Hospital
Aim
• To review our management of carcinoid
tumour of the appendix
• To set guidelines for future management
Introduction
• Carcinoid- from German ‘karzinoid’
• Carcinoma-like in appearance but benign behaviour
• First described by Otto Lurbasch in 1888, termed karzinoid by
Siegfried Oberndorfer in 1907
• Appendiceal carcinoid tumours are rare
– 0.3-0.9% appendicectomies¹
• Often found incidentally following appendicectomy
• Management guidelines often unfamiliar & may be unclear
• WHO classification of appendiceal tumours
Well differentiatedbenign
behaviour
Well differentiateduncertain
behaviour
Well differentiatedlow grade
malignant
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Non functioning
Confined to
appendiceal wall
<2cm
No vascular
invasion
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
Enteroglucagon
producing
Confined to
subserosa
>2cm or
vascular
invasion

Poorly
differentiatedhigh grade
malignant
Well to

moderately
differentiated
Invasion to
mesoappendix or
beyond or
metastases
Small cell
carcinoma
• Right hemicolectomy should be performed & 5 year follow-up needed if
– Tumour size greater than 2cm
– Invasion of serosal surface
– Goblet cell or adenocarcinoid features
• Tumours less than 1cm that have been completely resected need no followup
Ramage et al, Gut 2005; 54: iv1-iv16
• Right hemicolectomy should be performed & referral to specialist centre if
– Tumour larger than 2cm
– There is involvement of the base of the appendix or mesoappendix
– There are features of goblet cell carcinoid or adenocarcinoma
• Further investigation may be needed if appendix perforated
Murphy et al, British Journal of Surgery 2006; 93: 783-792
Method
• Retrospective analysis of all appendix
histopathology specimens from Jan 2005-Dec
2010
• Review of records of all patients diagnosed
with carcinoid tumours
Results
• 1300 appendicectomies
• 16 cases carcinoid (1.23%)
• 10 female
– median age 27 (range 9-54) years
• 6 male
– median age 52 (range 26-75) years
Presenting Features
• 13 presented as appendicitis
– 1 perforated, 2 gangrenous, 5 inflamed, 5 ‘normal’
• 1 chronic abdominal pain
• 1 abdominal pain & weight loss
• 1 bowel obstruction
Initial Management
• 15 appendicectomies
– 8 laparoscopic
– 7 open
• 1 right hemicolectomy
Histopathology
• Mean tumour size 13.5mm (range 2-55mm, SD 17mm)
10
9
8
7
Number of 6
5
patients 4
3
2
1
0
0-9mm
10-19mm
Size of tumour
>20mm
• Location of tumour
4 throughout appendix
1 at base
3 mid-appendix
8 at tip
• Resection margin negative in 10 patients
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11 well-differentiated classical carcinoid
3 goblet-cell carcinoid
1 adenocarcinoid
1 large cell neuroendocrine
Classical carcinoid tumour
Goblet cell carcinoid tumour
Outcome
Presenting features
Acute appendicitis
(13)
Abdominal pain
& weight loss(1)
Chronic abdominal
Pain (1)
Bowel obstruction
(1)
7 laparoscopic
6 open
appendicectomies
Lap to open
appendicectomy
Laparoscopic
appendicectomy
Right
hemicolectomy
3 tumours
>2cm
Extensive goblet
cell tumour
10mm tumour
completely excised
Infiltrating lymph
nodes
Discharged
Referred for
chemotherapy
10 tumours
<2cm
9 completely
excised
1 extending to
mesoappendix
Right
hemicolectomy
Right
hemicolectomy
Discharged
Referred to
Neuroendocrine
MDT
Tumour completely
excised
Tumour completely
excised
CT
Octreotide scan
Chromogranin
Yearly follow-up
Yearly follow-up
All normal
Yearly follow-up
• Median follow-up 29 months
(range 16-61 months)
• No cases of recurrence
Literature Review
• 5 similar series since 2000
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–
Tchana-Sato et al, World Journal of Gastroenterology 2006; 7: 6699-6701
In’t Hof et al, Journal of Gastrointestinal Surgery 2008; 12: 1436-8
Machado et al, Tropical Gastroenterology 2004; 25(1): 36-9
Guraya et al, Saudi Medical Journal 2005; 26(3): 434-7
Koskun et al, Turkish Journal of Trauma & Emergency Surgery 2006; 12(2): 150-4
• Retrospective reviews of 1237-6777 appendicectomies
• DVH highest number of carcinoid cases
– 16 v 5-11
• DVH highest incidence
– 1.23% v 0.16-0.6%
• DVH highest number of hemicolectomies
– 6 v 0-1
Conclusion
• Incidence of appendiceal carcinoid tumours found to be higher
than reported in the literature
• All were diagnosed incidentally
• Can be managed by appendicectomy alone if small & welldifferentiated
• Require hemicolectomy if >2cm
• Recommend discussion at neuroendocrine MDM if malignant
features present
• Overall good prognosis
Management of carcinoid tumours of the appendix diagnosed
after appendicectomy
Tumour <2cm
Yes
No
Evidence of perforation, spread
beyond appendix, goblet cell or
adenocarcinoid features
Discuss at neuroendocrine
MDM
Yes
No
Staging CT
Right hemicolectomy
Discuss at neuroendocrine
MDM
May require further surgery
Can be discharged
Yearly follow-up
Acknowledgements
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Dr Tripathi
Dr Holder
Mr Stewart
Mr Gurjar
Mr Strauss
Mr Adamek
Mr Biswas
Mr Parker
Mr McIrvine
Mr Hanek
Mr Bhardwaj
Thank you
References
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¹ Murphy EMA, Farquharson SM, Moran BJ. Management of an unexpected appendiceal neoplasm. British
Journal of Surgery 2006; 93: 783-792.
² Ramage JK, Davies AHG, Ardill J, et al. Guidelines for the management of gastroenteropancreatic
neuroendocrine (including carcinoid) tumours. Gut 2005; 54: iv1-iv16.
³ Murphy EMA, Farquharson SM, Moran BJ. Management of an unexpected appendiceal neoplasm. British
Journal of Surgery 2006; 93: 783-792.
Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer 1997; 79: 813-29.
Tchana-Sato V, Detry O, Polus M, Thiry A, Detroz B, Maweja S, Hamoir E, Defechereux T, Coimbra C, De
Roover A, Meurisse M, Honore P. Carcinoid tumor of the appendix: A consecutive series from 1237
appendectomies. World Journal of Gastroenterology 2006; 7: 6699-6701.
[1] In’t Hof KH, van der Wal HC, Kazemier G, Lange JF. Carcinoid tumour of the appendix: An analysis of
1485 consecutive emergency appendicectomies. Journal of Gastrointestinal Surgery 2008; 12: 1436-8.
[1] Machado NO, Chopra P, Pande G. Appendiceal tumour- retrospective clinicopathological analysis.
Tropical Gastroenterology 2004; 25(1): 36-9.
[1] Guraya SY, Khairy GA, Ghallab A, Al-Saigh A. Carcinoid tumors of the appendix. Saudi Medical
Journal 2005; 26(3): 434-7.
[1] Coskun H, Bostanci O, Dilege ME, Mihmanli M, Yilmaz B, Akgun I, Yildirim S. Carcinoid tumors of
appendix: treatment and outcome. Turkish Journal of Trauma & Emergency Surgery 2006; 12(2): 150-4.
[1] Modlin IM, Lye KD, Kidd M. A 5 decade analysis of 13715 carcinoid tumors. Cancer 2003; 97(4): 93459.