台灣癌症醫誌 (J. Cancer Res. Pract.) 1(1), 82-87, 2014 journal homepage:www.cos.org.tw/web/index.asp
Case Report Goblet Cell Carcinoid of Appendix Kung-Chen Ho1*, Chien-Liang Liu1, Jie-Jen Lee1, Tsan-Pai Liu1, Wen-Chin Ko1, Jiunn-Chang Lin1,2 1
Department of Surgery, Mackay Memorial Hospital, Taipei, Taiwan Mackay Medicine, Nursing and Management College, Taipei, Taiwan
2
Abstract. Carcinoid tumors are rare and slow-growing malignancies which occur most frequently in the appendix as its primary malignancy. Goblet cell carcinoid belongs to a subgroup of carcinoids and occurs in exclusively the appendix. Goblet cell carcinoid of the appendix is usually discovered incidentally after appendectomy. We report a 44-year-old woman, who underwent laparoscopic appendectomy for acute appendicitis. The final pathological diagnosis was goblet cell carcinoid. After a series of gastrointestinal examination, no evidence of local or distant lesions was found. The patient decided to be closely followed -up. There have been no signs of progression of the disease more than 1 year. Keywords : goblet cell carcinoid, adenocarcinoma, appendix
病例報告 闌尾的杯狀細胞類癌 何恭誠 1* 劉建良 1 1 2
李居仁 1 劉滄柏 1
柯文清 1
林俊昌 1,2
財團法人馬偕紀念醫院 一般外科 馬偕醫護管理專科學校
中文摘要 類癌是一種少見而生長緩慢的惡性腫瘤,是闌尾常見的原發性惡性腫瘤。杯狀細胞 類癌屬於類癌的次分類常發生在闌尾。闌尾的杯狀細胞類癌通常在術前難以診斷,術後 才意外發現。本篇文章將提出一 44 歲女性因急性闌尾炎接受腹腔鏡闌尾切除。病理報告 確診為杯狀細胞類癌。在一系列的腸胃道檢查後並沒有發現其他局部侵犯或轉移的證 據,於是決定病人接受持續的追蹤,不再作進一步手術。截至今時已超過一年,病人沒 有出現轉移的狀況。
關鍵字: 杯狀細胞類癌、腺癌、闌尾
INTRODUCTION
0.9% of specimens from appendectomy [2-4]. Goblet
Appendectomy for acute appendicitis is the most
cell carcinoid, a subgroup comprising approximately
common urgent operation, corresponding to 15 pro-
6% of carcinoids, and is almost always found only in
cedures per 100,000 population [1]. Carcinoids of the
the appendix [5,6]. Goblet cell carcinoid is a separate
appendix are rare occurring in approximately 0.3 to
entity from adenocarcinoma and such carcinoids were
Open access under CC BY-NC-ND license.
K. C. Ho et al./JCRP 1(2014) 82-87
83
B
A
C
Figure 1. (A and C) CT with contrast: swelling of appendix with increased wall suggesting appendicitis (white arrow); Enlargement and heterogeneity of uterus suggestive of endometrosis (white circle); (B) hypodense nodule at posterior S8 segment of liver with irregular border suggesting benign cyst (black triangle) first recognized in 1974 [7].
A 44-year-old woman came to our emergency
Controversy exists over the management following
room because of right lower quadrant pain and vom-
appendectomy, especially in patients with low level
ited several times. She had undergone laparoscopic
malignancies [4,6]. In this report a 44-year-old woman
surgery for endometriosis 10 years before. Physical
underwent laparoscopic appendectomy for acute ap-
examination revealed muscle guarding and rebound
pendicitis. Unexpectedly, the specimen showed goblet
tenderness in the right lower quadrant, and no obvious
cell carcinoid on the tip of the appendix.
tumor was palpated. No signs of carcinoid syndrome were observed. Preoperative blood tests demonstrated
CASE REPORT
systemic inflammation with an elevated white blood cell count (13300/μL) with left shifting (Neut. 93%). According to her clinical features, acute appendicitis
*Corresponding author: Kung-Chen Ho M.D.
was highly suspected on account of 8 points of the
*通訊作者:何恭誠醫師
Alvardo scoring system.
Tel: +886-2-25433535
The abdominal computed tomography scan demon-
Fax: +886-2-25433642
strated a swollen appendix and a low-density area in
E-mail:
[email protected]
the appendiceal lumen (Figure 1). According to the
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K. C. Ho et al./JCRP 1(2014) 82-87
was swollen and hypertrophied without tumor-
A
suggesting appearance. The patient was discharged home on the fifth postoperative day. Pathologic examination of the surgical specimen revealed increased inflammatory cells and fibrinopurulent exudates coating the serosa (Figure 3). Diagnosis of acute suppurative appendicitis was established. However, tumor cells resembling signet-rings that contain a rich and lucent cytosol were observed at the tip of the appendix, and these cells formed multiple lesions or cell nests in the submucosal layer (Figure 4). There was no venous or lymphatic invasion. Immunohistochemical stains focusing on neuroendo-
B
crine markers, involving CD-56, Chromogranin-A and Appendix
Synaptophysin were performed. The results were positive for CD-56 and Synaptophysin, and weakly positive for Chromogranin-A (Figure 5). According to these findings, the pathological diagnosis was goblet cell carcinoid with acute suppurative appendicitis. Post-operatively, the gastrointestinal and neuroendocrine profile was obtained, including liver soCecum
nography, panendoscopy, colonoscopy and urinary
Omentum
levels of 5-hydroxyindoleacetic acid (5-HIAA). She
Figure 2. (A) Engorged appendix with fibrin coating,
hepatic hemangioma was considered benign on ac-
adhered to mesentery and some omentum,
count of sonographic findings. There were no doubtful
grossly suggestive of acute appendicitis. (B)
lesions in the stomach, duodenum or colon under
Schematic picture in right bases on picture
panendoscopy and colonoscopy. The level of serum
in A
5-hydroxytrptyphan (5-HT) was within normal limits.
refused the detection of plasma Chromogranin A. The
Despite the small area of cancer cells confined within the appendiceal tip and no evidence of any dispatient’s statement, the nodule of the liver had not
tant metastasis, the patient was thought to carry a high
grown for 10 years (Figure 1B). Collectively, these
risk of malignancy. She preferred however conserva-
findings suggested acute appendicitis, and the patient
tive treatment to a right hemicolectomy, and is still
underwent laparoscopic appendectomy.
under close observation.
Intraoperatively, some amount of purulent ascites was found in the right lower quadrant of the abdomen.
DISCUSSION
The swollen and inflamed appendix was severely ad-
Goblet cell carcinoid of the appendix was first
hered to the peritoneum and the mesentery of the ter-
recognized as a distinct entity in 1974 and remains a
minal ileum. Inflammation was noted at the appendix
rare variant of carcinoids of the appendix. The peak
(Figure 2). Macroscopically, the entire appendix wall
age is between 53 and 58 years with a female pre-
K. C. Ho et al./JCRP 1(2014) 82-87
85
Figure 3. (A) cross section, H&E stain (40X), acute suppurative appendicitis. (A) Inflammatory cells seen between muscle layer along with herniation of mucosa (black triangle); (B) H&E stain (40X) It is coated with fibrinopurulent exudate (black arrow)
Figure 4. (A) coronary section of tip of appendix, H&E stain (40X), Goblet cell carcinioid some showing gland-like structures distributed in submucosa (oval annotation), resembling invasive adenocarcinoma in pattern; (B) H&E stain (200X), High-power field of submucosa showing, aggregate signet-ring cells (arrow)
dominance of 4 to 1 [1]. It is usually discovered after
Similar to typical carcinoids, goblet cell carcinoid of
appendectomy for acute appendicitis incidentally.
appendix is usually asymptomatic and found inci-
86
K. C. Ho et al./JCRP 1(2014) 82-87
A
B
C
Figure 5. (A) CD-56 stain(100X), definitely positive; (B) Chromogranin-A stain (100X), weakly positive; (C) Synaptophysin stain (200X), definitely positive
dentally; it rarely presents with metastasis [8]. They
rather than to those of typical carcinoid [10].
can cause appendicitis as a result of luminal obstruc-
Most authors agree that the goblet cell carcinoids
tion, but this is not common as they are most often
of the appendix are clinically more aggressive than
located at the tip of the organ [4].
typical carcinoids. However, there is still debate about
The term carcinoid syndrome refers to the system-
their management, especially whether appendectomy
ic signs and symptoms resulting from the release of
alone is an adequate treatment or whether a right
neuroendocrine mediators by some carcinoid tumors.
hemicolectomy is indicated [5]. Byrn et al. reviewed
Cutaneous flushing, diarrhea, and cardiac valvular
16 cases of gastrointestinal goblet cell carcinoid and
lesions are the most common manifestations of the
did not support right hemicolectomy for patient with
carcinoid syndrome. However, only 8 to 10% of all
non-metastatic goblet cell carcinoid of the appendix
carcinoid tumors are associated with the carcinoid
[11].
syndrome, while ileal carcinoids are usually associated with hepatic metastasis [9].
Laura et al. divided 63 cases of goblet call carcinoid into 3 groups (Group A: typical; Group B: sig-
According to the retrospective image review by
net ring cell type; Group C: poorly differentiated ade-
Courtney et al. carcinoids of the appendix were all less
nocarcinoma type) on the basis of their histologic fea-
than 1.5 cm in size. Possibly as a result of their small
tures [12].
size, none of the carcinoid tumors in the current study
In their study, both the 3-year and the 5-year dis-
has been identified on preoperative examination [2,3].
ease-specific survival of group A were 100%; they
S van Eeden et al. collected specimens included 16
were 85% and 36% in group B and 17% and 0% in
goblet cell carcinoid, 14 typical carcinoids, 19 colonic
group C, respectively. They investigated 6 cases in
adencarcinoma and 10 appendiceal mucinous cystad-
Group A confirmed to be in less than stage II. Those
enocarcinomas and pointed out immunohistochemical
patients in this group had only received simple ap-
comparability among those specimens. The results
pendectomy without other complicated procedures.
suggested that histopathological features of goblet cell
In our case, a tumor of less than 1 cm was found
carcinoid were similar to those of adenocarcinoma
on the tip of the appendix, and there was not evidence
K. C. Ho et al./JCRP 1(2014) 82-87
of metastasis in the peritoneum. Conservative treatment was considered appropriate as the disease was thought to be in stage I.
87
features. Eur J Radiol 82(1): 85-89, 2013. 4. Goede AC, Caplin ME, Winslet MC. Carcinoid tumor of the appendix. Br J Surg 90: 1317-1322, 2003.
CONCLUSIONS
5. Toumpanakis C, Standish RA, Baishnab E, et al.
Goblet cell carcinoids, found only in the appendix,
Goblet cell carcinoid tumors (adenocarcinoid) of
are uncommon tumors with histological features of
the appendix. Dis Colon Rectum 50(3): 315-322,
both adenocarcinoma and carcinoid tumor. In our
2007.
present case, the patient presented a typical pattern of
6. Suzuki O, Ono K, Sekishita Y, et al. Laparoscopic
acute appendicitis. The diagnosis of goblet cell car-
two-stage surgery for goblet cell carcinoid of the
cinoid was not made preoperatively or during surgery.
appendix: report of a case and review of the Jap-
However for our patient with low clinical staging,
anese literature. Surg Laparosc Endosc Percu-
simple appendectomy seemed to be have procedured
tan Tech 16: 106-108, 2006.
results similar to those of the Laura series [12]. Byrn
7. Mitra B, Pal M, Paul B, et al. Goblet cell car-
et al. did not suggest right hemicolectomy or oopho-
cinoid of appendix: A rare case with literature re-
rectomy for patient without metastasis [11]. Our pa-
view. Int J Surg Case Rep 4: 334-337, 2013.
tient decided to be kept closely followed. Long-term
8. Läuffer JM, Zhang T, Modlin IM. Review article:
follow-up with In-labelled octreotide scintigraphy and
current status of gastrointestinal carcinoids. Ali-
colonoscopy, however, is required to monitor possible
ment Pharmacol Ther 13: 271-287, 1999.
metastasis in the future [5].
9. Kenneth RH. Chapter 240 Carcinoid syndrome. Goldman's Cecil Medicine, 24th ed, 1509-1511.
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