CARCINOID OF MECKEL’S DIVERTICULUM; A CASE REPORT AND SYSTEMATIC
LITERATURE REVIEW
Emily Ryon, MSIII. University of Miami Miller School of Medicine
Introduction: Meckel’s diverticulum (MD) is the most common congenital anomaly of the
small intestine, occurring in 0.5-2.5% of the general population (1). Tumors within MD are a
rare complication, but recent research indicates tumors of MD occur at higher frequency than the
rest of the ileum (2). Carcinoid is the most common malignancy reported, accounting for 76.5%
of all cancers in MD (2). Carcinoid tumors present a diagnostic challenge in that they are often
advanced before they become symptomatic, and produce non-specific symptoms (3). There are
several large-scale reviews reporting occurrence of carcinoid in MD (4,5,6), however details
concerning diagnostic and operative decision-making are lacking.
Objectives: In this case report, we describe a 44 year-old man with abdominal pain who was
found to have mesenteric lymph node metastasis of a primary carcinoid tumor in MD, and who
underwent segmental ileal resection. In addition, we present a systematic literature review of
case reports over the past 25 years in order to better characterize diagnostic and operative
modalities regarding this rare entity.
Methods: A PubMed search was performed using the terms “Meckel’s Diverticulum” and
“Carcinoid” and was limited to case reports and case series over the past 25 years. Papers were
excluded if they were not in English or not accessible in electronic format. Tumors were staged
by the author according to AJCC criteria (7).
Results: The search yielded 20 articles and 28 case reports total that met inclusion criteria. There
were only 5 case reports published in the United States over the past 25 years, and none in the
past 5 years. The majority of cases (61.8%) were discovered incidentally on imaging or during
operations for unrelated conditions. CT identified lymph node and liver metastasis, but never the
primary tumor. Of the cases where MD was discovered intra-operatively, only 25% reported a
palpable mass in the diverticulum. In the other 75%, MD was removed prophylactically and the
tumor was discovered on pathology. Either transverse or wedge diverticulectomy were
performed when MD was discovered intra-operatively, and in 2 cases a subsequent segmental
resection was required due to discovery of regional disease. In symptomatic cases, the most
common presentation was Meckel’s diverticulitis secondary to tumor necrosis (36.4%), followed
by lower GI bleeding and carcinoid syndrome (diarrhea, flushing). A variety of diagnostic
modalities were used to locate the primary tumor, including arteriography, CT enterography,
RBC nuclear scan, octreoscan, and MIBG scan. Of the incidentally discovered tumors, 37.5%
demonstrated regional or distant metastasis (Stage III or IV), whereas in symptomatic cases, the
percentage was greater at 55.6%. Only 16 cases reported details of follow-up, with an average
follow-up time of 1.73 years. Of those, only one reported a recurrence, a case that was
diagnosed at advanced stage.
Conclusion: Carcinoid tumors of Meckel’s Diverticulum are often metastatic at the time of
diagnosis, and the majority are discovered incidentally. This review demonstrates the
heterogeneity of diagnostic tools and the need for better reporting of follow-up.
1. Mudatsakis N, Paraskakis S, Lasithiotakis K, Andreadakis E, Karatsis P. Acute appendicitis
and carcinoid tumor in Meckel’s diverticulum. Three pathologies in one: a case report. Tech
Coloproctol (2011) 15 (Suppl 1):S83–S85.
2. Thirunavukarasu P, Sathaiah M, Sukumar S, et al. Meckel's Diverticulum—A High-Risk
Region for Malignancy in the Ileum: Insights From a Population-Based Epidemiological Study
and Implications in Surgical Management. Ann Surg. 2011 February ; 253(2): 223–230.
3. Shebani KO, Souba WW, Finkelstein DM, et al. Prognosis and survival in patients with
gastrointestinal tract carcinoid tumors. Ann Surg. 1999; 229:815–821.
4. Stone PA, Hofeldt MJ, Campbell JE, et al. Meckel Diverticulum: Ten-year Experience in
Adults Southern Medical Journal 2004; 97: 1038-1041
5. Modlin IM, Shapiro MD, Kidd M. An Analysis of Rare Carcinoid Tumors: Clarifying These
Clinical Conundrums. World J. Surg. 2005; 29: 92–101.
6. Park JJ, Wolff BG, Tollefson MK, et al. Meckel diverticulum: the Mayo Clinic experience
with 1476 patients (1950–2002). Ann Surg. 2005; 241:529–533.
7. Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New
York, NY: Springer, 2010, pp 181-9.
Table 1
Sex
Male
Female
Table 2
Mean Age
51.4
58.3
Presentation
Incidental Finding
Appendectomy
Colectomy
Aortoiliac disease
Cholecystectomy
Sigmoid diverticulitis
Hematuria
Hernia repair
Hysterectomy
Intussusception
LoA
n (%)
21 (75)
7 (25)
17 (61.8)
3
3
2
2
2
1
1
1
1
1
Symptomatic
11 (39.2)
Diverticulitis1
4
Bleeding2
3
Carcinoid Syndrome
3
Obstruction
1
_________________________________________________________
1
On pathology, the tumor was identified as the source of
necrosis and micro-perforation in 3 of 4 cases.
2 On pathology, the tumor was identified as the source of
ulceration, not ectopic glandular tissue
Table 4. Tumor size, procedure and followup by stage
I
IIA
IIB
IIIA
IIIB
IV
NS
Size
0.3
0.9
1.5
Divert.
3
2
4
SR
1
4
0
Ave. f/u (y)
3.5
3
3
1.6
2.2
1.0
12
0
13
5
5
1
2.7
2.8
Divert. – transverse or wedge diverticulectomy, SR –
Segmental Ileal Resection, NS – not staged
1 In one case, subsequent segmental resection was
recommended, but refused by patient
2 Wedge diverticulectomy with removal of solitary
mesenteric lymph node
3 One report did not specify a method of resection. None of
the un-staged cases reported follow-up time
Symptomatic Cases
Total
11 (100)
Pre-op diagnosis of carcinoid
4 (36.4)
Tumor palpable during operation
2 (18.2)
Stage at diagnosis1
I
IIA
IIB
IIIA
IIIB
IV
Not staged
Average tumor size (cm)
_______
1 (9.1)
3 (27.3)
2 (18.2)
3 (27.3)
2 (18.2)
1.22
1
Staged by author using pathology report and American Joint
Committee on Cancer (AJCC) Criteria
Table 3.
Incidental Cases _______________
Total
17 (100)
Diagnosis
MD discovered at laparotomy
LN discovered at laparotomy
Liver met found on imaging
MD causing intussusception
12 (70.6)
2 (11.8)
2 (11.8)
1 (5.8)
Tumor palpable during operation
4 (23.5)
Stage at diagnosis
I
IIA
IIB
IIIA
IIIB
IV
Not staged
Average tumor size (cm)
1
3 (17.6)
3 (17.6)
4 (23.5)
4 (23.5)
2 (11.8)
1 (5.9)
1.25
Staged by author using pathology report and American Joint
Committee on Cancer (AJCC) Criteria