Table of Contents    
Case Report
 
Clinical review of the carcinoid tumor of the cystic duct: A rare tumor at a rare site
Rasees Fawwaz Alotaibi1, Yara Ali Alnashwan2, Maryam Mohammed Alomar3, Shaukat Ahmad Bojal4, Mohammed Saad Alqahtani5, Iftikhar Khan6
1MBBS, Radiology Resident, Medical Imaging Department, King Fahad Specialist Hospital, Dammam, Saudi Arabia.
2MBBS, Medical Intern, University of Dammam, Dammam, Saudi Arabia.
3MBBS, Medical Intern, University of Dammam, Dammam, Saudi Arabia.
4MD, Hepatobiliary Surgeon, Section of Hepatobiliary Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia.
5MD, Transplant and Hepatobiliary Surgeon, Department of Transplant Surgery and Section of Hepatobiliary Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia.
6MD, Transplant Surgeon, Department of Transplant Surgery, King Fahad Specialist Hospital, Dammam, Saudi Arabia.

Article ID: 100053IJHPDRA2016
doi:10.5348/ijhpd-2016-53-CR-9

Address correspondence to:
Dr. Iftikhar Khan
MOTC, KFSH, PO Box: 15215
MBC: 067, Dammam 31444
Saudi Arabia

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How to cite this article:
Alotaibi RF, Alnashwan YA, Alomar MM, Bojal SA, Alqahtani MS, Khan I. Clinical review of the carcinoid tumor of the cystic duct: A rare tumor at a rare site. Int J Hepatobiliary Pancreat Dis 2016;6:43–47.


Abstract
Introduction: Carcinoid tumor arising in the cystic duct is extremely rare. This is the 23rd case reported in English literature.
Case Report: A case of carcinoid tumor in the cystic duct of a 68-year old lady is described that was found incidentally after laparoscopic cholecystectomy.
Conclusion: Presentation, diagnosis, treatment and prognosis are discussed based on the review of the reported cases. It usually presents with biliary symptoms and the diagnosis is incidental. Radical resection and follow up are recommended because of its malignant potential.

Keywords: Carcinoid tumor, Cystic duct, Neuroendocrine tumor


Introduction

Carcinoid tumor arising in the cystic duct is exceedingly rare. Over a period of 32 years, since 1984, when Goodman et al. [1] described the first case, only 22 cases of the cystic ductal carcinoid tumors have been reported. We present another similar case followed by review of literature. This is the first case reported from the Middle East.


Case Report

A 68-year-old female had undergone laparoscopic cholecystectomy for symptomatic gallstones in another hospital, was referred for incidentally discovered carcinoid tumor in the cystic duct. She was a known case of diabetes mellitus, hypertension and osteoporosis. Her symptom was post-prandial right upper quadrant pain for one year. At the time of her presentation to our hospital, she was asymptomatic with unremarkable examination. Her complete blood count, liver function tests and serum chromogranin A, serotonin and octreotide levels were within normal range. Imaging of her abdomen and chest by computed tomography (CT), magnetic resonance (MRI) and positron emission tomography (PET) did not reveal any metastatic deposit.

Macroscopic review of the gallbladder revealed a nodular tumor in the cystic duct which was 0.7 cm in diameter. Microscopic examination showed nests, cords and trabeculae of small cells with granular chromatin which were positive for synaptophysin, chromogranin, and CD56 on immunohistochemistry (Figure 1). The tumor invaded the perineural and muscle tissue. This was consistent with well-differentiated carcinoid tumor.

The patient underwent resection of the cystic duct stump, liver segments 4B and 5 and clearance of hepatoduodenal lymph nodes. Appendectomy was also done because of a nodule in the mesoappendix which turned out to be benign fibrosis. No residual tumor was found on frozen and permanent sections. Her postoperative recovery was unremarkable. At 16th month follow-up, she is doing well with no evidence of recurrent disease on MRI and PET scans.


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Figure 1: (A) Photomicrograph of the carcinoid tumor in the cystic duct, showing nests, cords and trabeculae of small cells (H&E stain, x200), (B) Positivity for synaptophysin, (C) Chromogranin, and (D) CD56.



Discussion

Carcinoid tumors, arising from the enterochromaffin cells, are extremely rare and account for 0.49% of all malignancies [2]. Only 0.2–2.0% of carcinoid tumors are found in the extra-hepatic bile ducts [3]. Cystic duct carcinoid tumors represent 11–20% of all extra-hepatic bile duct carcinoid tumors [3], highlighting the rarity of this particular tumor at this particular site. Ours is the 23rd case reported in English literature (Table 1) [1] [2] [4][5][6][7][8] [9][10][11][12][13] [14][15][16][17][18] [19][20][21].

Review of the reported cases demarcates the clinical presentation into two groups. Isolated cystic duct involvement usually presents with clinical picture similar to symptomatic gallstones. Gallstones were present in two-thirds of the reported cases. When main hepatic/bile duct gets involved then obstructive jaundice is the usual presentation. Endocrine symptoms are rare and only one patient, who had multiple carcinoid tumors at various locations as part of the multiple endocrine neoplasia type-I (MEN-I) syndrome, presented with Zollinger-Ellison syndrome [17]. Majority of the patients were in their fifth and sixth decades and were females. All cases with isolated cystic duct carcinoid tumor were diagnosed incidentally after cholecystectomy on histological examination. The yield of endoscopic retrograde cholangiopancreatography (ERCP) and biopsy in cases that presented with obstructive jaundice was low and only one patient was diagnosed correctly preoperatively [13].

One-third of the patients had evidence of metastasis. Synchronous spread to the regional lymph nodes was seen in four patients [1][2] [11][21]. Synchronous liver deposits were present in two patients [13] [17]. Metachronous liver metastasis occurred in two patients [10] [16]. It is important to note that the locations of liver metastasis were away from the gallbladder bed which requires re-assessment of the value of adding the resection of liver segments 4 and 5 in the primary clearance.

Complete surgical resection is the best treatment for the fit patient. Isolated cystic duct carcinoid tumor requires clearance of the cystic duct stump and the hepatoduodenal lymph nodes. This can be achieved with open or laparoscopic approaches [20]. Involvement of the main hepatic/bile ducts requires excision of the extra-hepatic bile ducts, clearance of the lymph nodes and reconstruction with hepaticojejunostomy. Liver metastasis can be treated by locoregional modalities such as radio frequency ablation, ethanol injection and chemoembolization. Hormone therapy with somatostatin analogues may be considered in frail patients and those with endocrine symptoms. Radiotherapy and chemotherapy should be reserved for surgically unfit, recurrent or metastatic disease.

During the reported follow-up that ranged between 6 and 49 months, one patient died at sixth month due to liver metastasis [10]. Due to their malignant potential, the patients ought to be followed-up by imaging.


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Table 1: Cystic duct carcinoid cases




Conclusion

In conclusion, cystic duct carcinoid tumor is a rare tumor which presents either with biliary symptoms or obstructive jaundice. The preoperative diagnosis is difficult. Radical surgical resection is the best treatment. Follow-up is recommended because of relatively low but definite malignant potential.


Acknowledgements

We would like to acknowledge Dr. Miral Mohammed Mashhour, Department of Pathology, King Fahad Specialist Hospital, Dammam, Saudi Arabia for kindly providing the photomicrographs of the histological slides.


References
  1. Goodman ZD, Albores-Saavedra J, Lundblad DM. Somatostatinoma of the cystic duct. Cancer 1984 Feb 1;53(3):498–502.   [CrossRef]   [Pubmed]    Back to citation no. 1
  2. Liew EW, Usatoff V. Cystic duct carcinoid tumor with ensuing lymph node metastasis. Surgical Practice 2011;15:145–7.    Back to citation no. 2
  3. Michalopoulos N, Papavramidis TS, Karayannopoulou G, Pliakos I, Papavramidis ST, Kanellos I. Neuroendocrine tumors of extrahepatic biliary tract. Pathol Oncol Res 2014 Oct;20(4):765–75.   [CrossRef]   [Pubmed]    Back to citation no. 3
  4. Nicolescu PG, Popescu A. Carcinoid tumor of the cystic duct. Morphol Embryol (Bucur) 1986 Oct-Dec;32(4):275–7.   [Pubmed]    Back to citation no. 4
  5. Chittal SM, Ra PM. Carcinoid of the cystic duct. Histopathology 1989 Dec;15(6):643–6.   [CrossRef]   [Pubmed]    Back to citation no. 5
  6. Rugge M, Sonego F, Militello C, Guido M, Ninfo V. Primary carcinoid tumor of the cystic and common bile ducts. Am J Surg Pathol 1992 Aug;16(8):802–7.   [CrossRef]   [Pubmed]    Back to citation no. 6
  7. Ishibashi T, Nagai H, Yasuda Y, Furuta K, Saito K, Kanazawa K. Multiple carcinoid tumors of the gallbladder and cystic duct. J Hep Bil Pancr Surg 1995;2(1):77–84.   [CrossRef]    Back to citation no. 7
  8. Meyer CN, Geerdsen J, Christensen N. Limited surgical excision of a localized carcinoid tumor of the cystic duct. Dig Surg 1997;14:423–5.    Back to citation no. 8
  9. Shah IA, Schlageter MO, Wong SX, Gani OS. Carcinoid tumor of the cystic duct. Dig Surg 1998;15(4):372–3.   [CrossRef]   [Pubmed]    Back to citation no. 9
  10. Oikawa I, Hirata K, Katsuramaki T, Mukaiya M, Sasaki K, Satoh M. Neuroendocrine carcinoma of the extrahepatic biliary tract with positive immunostaining for gastrin-releasing peptide: report of a case. Surg Today 1998;28(11):1192–5.   [CrossRef]   [Pubmed]    Back to citation no. 10
  11. Hermina M, Starling J, Warner TF. Carcinoid tumor of the cystic duct. Pathol Res Pract 1999;195(10):707–9; discussion 710.   [CrossRef]   [Pubmed]    Back to citation no. 11
  12. Aronsky D, Z'graggen K, Stauffer E, Lange J, Klaiber C. Primary neuroendocrine tumors of the cystic duct. Digestion 1999 Sep-Oct;60(5):493–6.   [CrossRef]   [Pubmed]    Back to citation no. 12
  13. Hubert C, Sempoux C, Berquin A, Deprez P, Jamar F, Gigot JF. Bile duct carcinoid tumors: an uncommon disease but with a good prognosis? Hepatogastroenterology 2005 Jul-Aug;52(64):1042–7.   [Pubmed]    Back to citation no. 13
  14. Stavridi F, Chong H, Chan S, Goldsmith C, Reddy M, Glees J, Benepal T. Neuroendocrine tumour of the cystic duct: a case report and literature review. J Gastrointest Cancer 2007;38(1):32–3.   [CrossRef]   [Pubmed]    Back to citation no. 14
  15. Sethi H, Madanur M, Srinivasan P, Portmann B, Heaton N, Rela M. Non-functioning well-differentiated neuroendocrine tumor of the extrahepatic bile duct: an unusual suspect? Hepatobiliary Pancreat Dis Int 2007 Oct;6(5):549–52.   [Pubmed]    Back to citation no. 15
  16. Felekouras E, Petrou A, Bramis K, et al. Malignant carcinoid tumor of the cystic duct: a rare cause of bile duct obstruction. Hepatobiliary Pancreat Dis Int 2009 Dec;8(6):640–6.   [Pubmed]    Back to citation no. 16
  17. Price TN, Thompson GB, Lewis JT, Lloyd RV, Young WF. Zollinger-Ellison syndrome due to primary gastrinoma of the extrahepatic biliary tree: three case reports and review of literature. Endocr Pract 2009 Nov-Dec;15(7):737–49.   [CrossRef]   [Pubmed]    Back to citation no. 17
  18. Ioannidis O, Cheva A, Paraskevas G, et al. Neuroendocrine tumor of the cystic duct. Acta Gastroenterol Belg 2012 Sep;75(3):357–60.   [Pubmed]    Back to citation no. 18
  19. Lim HU, Chan CC, Knotts FB. An incidental finding of carcinoid tumor of the cystic duct. J Surg Case Rep 2013 Apr 4;2013(4). pii: rjt021.   [CrossRef]   [Pubmed]    Back to citation no. 19
  20. Garland J, O'Leary E, Haggerty J, Zorc TG. Carcinoid tumor of the cystic duct: case report, literature review and comparison of surgical approaches. Case Rep Oncol 2014 Sep 3;7(3):621–4.   [CrossRef]   [Pubmed]    Back to citation no. 20
  21. Hong N, Kim HJ, Byun JH, Neuroendocrine neoplasms of the extrahepatic bile duct: radiologic and clinical characteristics. Abdom Imaging 2015 Jan;40(1):181–91.   [CrossRef]   [Pubmed]    Back to citation no. 21

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Author Contributions
Rasees Fawwaz Alotaibi – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Yara Ali Alnashwan – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Maryam Mohammed Alomar – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Shaukat Ahmad Bojal – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published.
Mohammed Saad Alqahtani – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Iftikhar Khan – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
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Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2016 Rasees Fawwaz Alotaibi et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.