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 appendixTo review our management of carcinoid tumour of the appendix To set guidelines for future managementTo 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 rareAppendiceal carcinoid tumours are rare – % appendicectomies¹ Often found incidentally following appendicectomyOften found incidentally following appendicectomy

Management guidelines often unfamiliar & may be unclearManagement guidelines often unfamiliar & may be unclear WHO classification of appendiceal tumoursWHO classification of appendiceal tumours Well differentiated- benign behaviour Well differentiated- uncertain behaviour Well differentiated- low grade malignant Poorly differentiated- high grade malignant  Non functioning  Confined to appendiceal wall  <2cm  No vascular invasion  Enteroglucagon producing  Confined to subserosa  >2cm or vascular invasion  Well to moderately differentiated  Invasion to mesoappendix or beyond or metastases  Small cell carcinoma

Right hemicolectomy should be performed & 5 year follow-up needed ifRight 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 follow- upTumours less than 1cm that have been completely resected need no follow- up Ramage et al, Gut 2005; 54: iv1-iv16 Ramage et al, Gut 2005; 54: iv1-iv16 Right hemicolectomy should be performed & referral to specialist centre ifRight 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 perforatedFurther investigation may be needed if appendix perforated Murphy et al, British Journal of Surgery 2006; 93: Murphy et al, British Journal of Surgery 2006; 93:

Method Retrospective analysis of all appendix histopathology specimens from Jan 2005-Dec 2010Retrospective analysis of all appendix histopathology specimens from Jan 2005-Dec 2010 Review of records of all patients diagnosed with carcinoid tumoursReview of records of all patients diagnosed with carcinoid tumours

Results 1300 appendicectomies1300 appendicectomies 16 cases carcinoid (1.23%)16 cases carcinoid (1.23%)

10 female10 female – median age 27 (range 9-54) years 6 male6 male –median age 52 (range 26-75) years

Presenting Features 13 presented as appendicitis13 presented as appendicitis –1 perforated, 2 gangrenous, 5 inflamed, 5 ‘normal’ 1 chronic abdominal pain 1 chronic abdominal pain 1 abdominal pain & weight loss 1 abdominal pain & weight loss 1 bowel obstruction 1 bowel obstruction

Initial Management 15 appendicectomies15 appendicectomies –8 laparoscopic –7 open 1 right hemicolectomy1 right hemicolectomy

Histopathology Mean tumour size 13.5mm (range 2-55mm, SD 17mm)Mean tumour size 13.5mm (range 2-55mm, SD 17mm)

Location of tumour 4 throughout appendix 1 at base 3 mid-appendix 8 at tip Resection margin negative in 10 patients

11 well-differentiated classical carcinoid11 well-differentiated classical carcinoid 3 goblet-cell carcinoid3 goblet-cell carcinoid 1 adenocarcinoid1 adenocarcinoid 1 large cell neuroendocrine1 large cell neuroendocrine Classical carcinoid tumour Goblet cell carcinoid tumour

Outcome Presenting features Acute appendicitis (13) 7 laparoscopic 6 open appendicectomies 10 tumours <2cm 9 completely excised Discharged 1 extending to mesoappendix Referred to Neuroendocrine MDT CT Octreotide scan Chromogranin All normal Yearly follow-up 3 tumours >2cm Right hemicolectomy Tumour completely excised Yearly follow-up Abdominal pain & weight loss(1) Lap to open appendicectomy Extensive goblet cell tumour Right hemicolectomy Tumour completely excised Yearly follow-up Chronic abdominal Pain (1) Laparoscopic appendicectomy 10mm tumour completely excised Discharged Bowel obstruction (1) Right hemicolectomy Infiltrating lymph nodes Referred for chemotherapy

Median follow-up 29 monthsMedian follow-up 29 months (range months) (range months) No cases of recurrenceNo cases of recurrence

Literature Review 5 similar series since similar series since 2000 –Tchana-Sato et al, World Journal of Gastroenterology 2006; 7: –In’t Hof –In’t Hof et al, Journal of Gastrointestinal Surgery 2008; 12: –Machado et al, Tropical Gastroenterology 2004; 25(1): 36-9 –Guraya et al, Saudi Medical Journal 2005; 26(3): –Koskun et al, Turkish Journal of Trauma & Emergency Surgery 2006; 12(2): Retrospective reviews of appendicectomiesRetrospective reviews of appendicectomies DVH highest number of carcinoid casesDVH highest number of carcinoid cases –16 v 5-11 DVH highest incidenceDVH highest incidence –1.23% v % DVH highest number of hemicolectomiesDVH highest number of hemicolectomies –6 v 0-1

Conclusion Incidence of appendiceal carcinoid tumours found to be higher than reported in the literatureIncidence of appendiceal carcinoid tumours found to be higher than reported in the literature All were diagnosed incidentallyAll were diagnosed incidentally Can be managed by appendicectomy alone if small & well- differentiatedCan be managed by appendicectomy alone if small & well- differentiated Require hemicolectomy if >2cmRequire hemicolectomy if >2cm Recommend discussion at neuroendocrine MDM if malignant features presentRecommend discussion at neuroendocrine MDM if malignant features present Overall good prognosisOverall good prognosis

Management of carcinoid tumours of the appendix diagnosed after appendicectomy Tumour <2cm Yes Evidence of perforation, spread beyond appendix, goblet cell or adenocarcinoid features Yes Discuss at neuroendocrine MDM May require further surgery No Can be discharged No Discuss at neuroendocrine MDM Staging CT Right hemicolectomy Yearly follow-up

Acknowledgements Dr Tripathi Dr Holder Mr Stewart Mr GurjarMr Gurjar Mr StraussMr Strauss Mr AdamekMr Adamek Mr BiswasMr Biswas Mr ParkerMr Parker Mr McIrvineMr McIrvine Mr HanekMr Hanek Mr BhardwajMr Bhardwaj

Thank you

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