ONCOLOGY LETTERS 9: 2401-2403, 2015

Carcinoid tumor of the appendix: A case report

YUQING GU, NAICHAO WANG and HONGXING XU
Department of General Surgery, Taicang Hospital Affiliated to Soochow University, Taicang, Suzhou 215400, P.R. China
Received May 30, 2014; Accepted February 11, 2015
DOI: 10.3892/ol.2015.2997
Abstract. Carcinoid tumors are the most common neoplasm of
the appendix. The clinical presentation of these lesions is often
similar to that of acute appendicitis, or the tumors are asymptomatic. The carcinoids are commonly found incidentally
during histopathological examination of the resected appendix
following appendectomy or other abdominal procedures.
Appendiceal carcinoids usually behave as benign tumors and
appendicectomy alone is a sufficient treatment in the majority
of cases, while for larger lesions, right colectomy should be
performed. The prognosis of patients with local appendiceal
carcinoids is excellent. The present study reports the case of a
22yearold female patient that presented with recurrent right
lower abdominal pain of a threeyear duration. The patient
underwent successful appendectomy and recovered fourdays
later. Subsequently, an appendiceal carcinoid tumor located
at the tip of the appendix was diagnosed by histopathological
examination. Followup examination oneyear after surgery
revealed that the patient was well with no discomfort.
Introduction
Acute appendicitis is the most common abdominal emergency
encountered by the general surgeon(1). Subsequent to diagnosis by histopathological examination, primary neoplasms of
the appendix are identified in ~0.5% of all surgicallyremoved
appendices, with carcinoid tumors representing >50% of all
appendix neoplasms(2,3). Carcinoid tumors arise from the
neuroendocrine cells of the diffuse neuroendocrine system,
which are identified in numerous locations, including the lung
(25.1%), ovaries (0.5%), biliary system (0.2%) and throughout
the gastrointestinal tract (73.4%)(4). Appendiceal carcinoid
tumors are rare neuroendocrine neoplasms that usually behave
as benign tumors, while certain lesions possess the potential
for malignancy and are therefore able to metastasize(5). The
probability of metastasis of appendiceal carcinoid tumors is
low, ~4.7% of all appendiceal carcinoid tumors(6). Lymphatic

Correspondence to: Dr Hongxing Xu, Department of General

Surgery, Taicang Hospital Affiliated to Soochow University,
58South Changsheng Road, Taicang, Suzhou 215400, P.R. China
Email: [email protected]

Key words: carcinoid tumor, appendix

spread is the primary route, and hepatic metastases are

rare(2). Although not a common occurrence, primary carcinoid tumors of the appendix should be considered as a cause
of acute appendicitis during appendectomy. Surgical resection
is recommended for the treatment of appendiceal carcinoid
tumors, and the longterm prognosis of patients with appendiceal carcinoids is good(6). The present study reports the case
of a 22yearold female patient diagnosed with a carcinoid
tumor located at the tip of the appendix. Written informed
consent was obtained from the patient.
Case report
A 22yearold female patient was admitted to Taicang Hospital
Affiliated to Soochow University (Taicang, Suzhou, China)
with the complaint of recurrent right lower abdominal pain
of a threeyear duration, and an additional exacerbated attack
of pain that had lasted for the three days prior to presentation.
The patient reported a threeyear history of recurrent
dull right lower abdominal pain that had not been considered
serious enough to obtain medical assistance. Three days prior to
admittance, the aforementioned symptoms became aggravated
and the patient presented to the Taicang Hospital Affiliated
to Soochow University. Physical examination revealed a body
temperature of 36.5C, blood pressure of 111/78mmHg and
a pulse rate of 95 beats/min. During assessment, the patient
experienced light direct tenderness, but no rebound tenderness,
in the right lower abdomen. No palpable masses were observed
in the abdomen. Laboratory tests were performed, yielding
the following results: White blood cell count, 4.4x109cells/l
(normal range, 410x109cells/l); neutrophil proportion, 46.8%
(normal range, 4580%); hemoglobin level, 130g/l (normal
range, 110150g/l); platelet count, 177x109platelets/l (normal
range, 100300x10 9 platelets/l). In addition, abdominal
computed tomography revealed appendiceal inflammation,
while the tumor was too small to identify(Fig.1). The patient
possessed no previous medical history and no family medical
history of appendicitis.
The patient underwent surgery for the treatment of the
acute attack of chronic appendicitis. The appendix was identified as 7cm long and 0.8cm in diameter, demonstrating
hyperemia and edema. The resection of the appendix was
completed without complications and the patient recovered
four days later. The specimen was examined by physiological
analysis and stained using hematoxylin and eosin. At the tip
of the appendix, there was a welldifferentiated gradeIneuroendocrine tumor(Fig.2), measuring 5mm at the maximum

GU et al: CARCINOID TUMOR OF THE APPENDIX

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Figure 1. Computed tomography scan revealing the appendix, with the tumor being too small to identify, as indicated by the arrow.

Figure 2. Photomicrograph of a hematoxylin and eosinstained tissue section revealing the neuroendocrine tumor cells.

dimension, that had infiltrated few regions of the muscular

layer. The mitotic activity of the lesion was not notable.
Assessment of the Ki-67proliferative index revealed nuclear
staining in ~1% of cells. The diagnosis of appendiceal
carcinoid tumor was confirmed by positive immunostaining
for the neuroendocrine markers chromogranin A and synaptophysin. Coexisting acute purulent appendicitis was also
present. No further treatment was administered. Followup
examination oneyear after surgery revealed that the patient
was well, with no discomfort.
Discussion
Carcinoid tumors of the appendix are relatively uncommon
neoplasms. It has been previously reported that the incidence
of appendiceal carcinoids ranges between 0.3and 0.9%, as
determined using histopathological examination performed
on excised appendectomy specimens(7,8). However, appendiceal carcinoids are the most frequent tumors arising from the
appendix, comprising between 32and 57% of all appendiceal
tumors(9). Carcinoid tumors are neoplasms derived from the
subepithelial neuroendocrine cells of the appendix(10) that
rarely cause metastatic disease.
There is no specific preoperative clinical presentation for
appendiceal carcinoids. In general, appendiceal carcinoids are
either asymptomatic or present as acute appendicitis, which is
then diagnosed incidentally as appendiceal carcinoids during
surgery(11). In addition, the carcinoids can result in recurrent

episodes of abdominal pain due to partial obstruction of the

appendiceal lumen by a tumor. The presence of neuroendocrine symptoms, including flushing, diarrhea and cardiac
disease, are rarely reported(12). In the case of the present
patient, the tumor was located at the tip of the appendix, and
the patient experienced recurrent right lower abdominal pain
that had been present for three years. The surgical procedure
was performed for the treatment of appendicitis, and the carcinoid tumor was identified incidentally during the histological
examination of the excised surgical specimen.
Appendiceal carcinoids grow slowly, and the overall
prognosis is excellent(13). At present, tumor size is the most
reliable indicator for the assessment of the malignant potential of lesions. In the majority of patients with appendiceal
carcinoids, the tumor diameter is <1cm and appendectomy
alone is a sufficient treatment as carcinoids rarely metastasize. However, tumors measuring 2cm in diameter
may possess the potential for metastasis, and patients with
tumors of this size require right hemicolectomy. For tumors
measuring 12cm in diameter, the surgical options depend
on mesoappendiceal involvement and the histological
subtype of the lesion. Suitable candidates for right hemicolectomy include lesions where there is histological evidence
of mesoappendiceal extension, tumors that are located at the
base of the appendix with positive margins or involvement
of the caecum, and highgrade malignant carcinoids(14,15).
In the present patient, the tumor was 0.5cm in diameter and
therefore appendectomy alone was performed.

ONCOLOGY LETTERS 9: 2401-2403, 2015

Postoperative followup was recommended by the

National Comprehensive Cancer Network 2013Guidelines
following the excision of appendiceal carcinoids in for patients
with tumors that are >2cm in diameter. This followup procedure comprises a history and physical examination every
312months postresection and every 612months thereafter
for 10years, with consideration of followup imaging or
laboratory markers, such as 5hydroxyindoleacetic acid or
chromogranin A(16).
Overall, the longterm prognosis of appendiceal carcinoids
is extremely good. SandorandModlin evaluated 1570carcinoid tumors of the appendix that were treated at the American
National Cancer Institute between 1973 and 1991. It was
reported that the fiveyear survival rates of patients with localized, regional metastases and distant metastases in appendiceal
carcinoids were 94.0, 84.6and 33.7%, respectively, and the
overall fiveyear survival rate was 85.9%(9). Another study
with a large cohort performed by Modlinetal reported that
patients with local disease demonstrate a fiveyear survival
rate of 92%, those with regional metastases demonstrated a
fiveyear survival rate of 81% and the few with distant metastases demonstrated a fiveyear survival rate of 31%(10).
Carcinoid tumors are the most common neoplasms of the
appendix, but demonstrate no specific clinical presentation.
The presence of these tumors should be considered, particularly during surgical procedures.
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