|Year : 2015 | Volume
| Issue : 2 | Page : 220-222
Carcinoma of the cystic duct presenting as obstructive jaundice
Sunita Arvind Bamanikar1, NK Panicker1, Balaji Dhaigude2, Sangeeta Choudhary1, Archana Buch1
1 Department of Pathology, Padmashree Dr. D.Y. Patil Medical College, Hospital and Research Center, Dr. D.Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India
2 Department of Surgery, Padmashree Dr. D.Y. Patil Medical College, Hospital and Research Center, Dr. D.Y. Patil Vidyapeeth, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||13-Mar-2015|
Sunita Arvind Bamanikar
5 and 6, Siddharth Estates, North Avenue, Kalyani Nagar, Pune - 411 006, Maharashtra
Source of Support: None, Conflict of Interest: None
Cystic duct carcinoma is a rare disease. We present here a case of 33-year-old female patient who presented with obstructive jaundice. An abdominal ultrasound and computed tomography scan showed dilatation of intrahepatic biliary radicals with abrupt narrowing of the common bile duct and markedly distended gall bladder suggestive of obstructive pathology. Following an exploratory laparotomy, a markedly distended gall bladder, cystic duct specimen and infrapyloric lymph nodes were sent for histopathological examination. Histopathological examination revealed a well-differentiated adenocarcinoma of the cystic duct with no lymph node metastasis.
Keywords: Cystic duct carcinoma, hepatobiliary system, obstructive jaundice
|How to cite this article:|
Bamanikar SA, Panicker N K, Dhaigude B, Choudhary S, Buch A. Carcinoma of the cystic duct presenting as obstructive jaundice. Med J DY Patil Univ 2015;8:220-2
| Introduction|| |
The incidence of primary cystic duct carcinoma (CDC) in autopsy studies is 0.03-0.05%.  It constitutes 2.6-12.6% of the extrahepatic billiary malignancy.  The criteria for diagnosis of primary CDC were put forward by Farrar. 
A pre-operative diagnosis of CDC is difficult even with the aid of the most modern diagnostic techniques. For an early diagnosis of the disease, early exploratory laparotomy and prophylactic removal of non-functioning gallbladder (GB), followed by histopathological examination of the specimen are recommended, this being the gold standard for diagnosis of malignancy as the cause of obstruction.
Only 27 cases have been reported so far in the English literature  and 17 cases in the Japanese literature.  Most cases were characterized by a hydrops or cholecystitis. We present here a case of obstructive jaundice caused by a primary CDC in a 33-year-old female patient.
| Case Report|| |
The present case report is about a 33-year-old female patient who was admitted to the hospital with the complaints of itching all over the body and pain in abdomen since 3 months. The abdominal pain was non-radiating and not associated with vomiting and sticky stools. She was non-smoker and non-alcoholic.
The physical examination revealed tenderness in right hypochondrium and mild hepatomegaly. The laboratory findings showed: Total serum bilirubin 2.3 mg%, direct bilirubin 1.4 mg%, serum glutamic pyruvic transaminase 64 IU/L, serum alkaline phosphatase 124 IU/L, serum amylase 1317 IU/L and serum lipase 998 IU/L. An abdominal ultrasonography and computed tomography scan showed dilatation of intrahepatic biliary radicals with abrupt termination of common bile duct (CBD) suggestive of stricture and markedly distended gall bladder suggestive of obstructive pathology. On magnetic resonance cholangiopancreaticography, a diagnosis of intraluminal neoplastic lesion involving the common hepatic and CBD was offered. No definitive pathology was seen at the pancreaticobiliary junction. Patient had undergone endoscopic retrograde cholangiopancreatography with brush cytology and CBD stenting. On brush cytology, there was no evidence of malignancy. Based on these findings, an elective exploratory laporatomy was performed for massive distention of gall bladder. At surgery, cholecystectomy, resection of cystic duct and infrapyloric lymphadenectomy was performed and specimens were sent for histopathological examination.
The infrapyloric lymph nodes were enlarged and clinically malignancy was suspected. The lymph node and cystic duct stump was sent for frozen section examination. The imprints and frozen sections revealed only fat necrosis with chronic inflammation and infiltrates by foamy macrophages, which were later confirmed on paraffin sections. Gross examination showed an area of thickening and induration of the cystic duct which was adherent to the distended gall bladder in one area. Histopathological examination of the cystic duct revealed a well differentiated tubular adenocarcinoma [Figure 1]. The gall bladder was filled with gelatinous fluid, no gall stones were identified. Sections from the gall bladder showed chronic cholecystitis with marked thinning of the mucosal epithelium and presence of adenocarcinoma infiltrates in the hypertrophic muscle layer at the adherent site only.
|Figure 1: Photomicrograph of the wall of the cystic duct showing invasive adenocarcinoma (H and E, ×400)|
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| Discussion|| |
Carcinoma of the cystic duct is extremely rare. Farrar's first described diagnostic criteria for CDC which include (a) growth restricted to the cystic duct, (b) absence of neoplastic process in the gall bladder, hepatic or CBD and (c) histological evidence of carcinoma in the cystic duct.  In a study by Ozden et al. proposed a working definition of CDC as a gall bladder tumor whose site of origin is in the cystic duct. 
The rarity of reports of classical CDC must be attributed to its strict definition. The reported average length of survival for classically defined CDC was 27.2 months which was better than that of gall bladder or other extrahepatic bile duct carcinomas during the same period.  This may be due to the fact that the CDC fitting into the classic definition should be confined to the cystic duct, be small and early at onset, just sufficient to produce serious signs and symptoms of obstructive jaundice. The high resectability of this small tumor also contributes to its better prognosis. The resectability rate is <30% for extrahepatic bile duct or GB cancer, but in most cases of CDC, it is reported to be almost 100%. Another reason is that incidence of lymph node metastasis is lower in patients with CDC (0-40%) than in those with extrahepatic bile duct (about 50%) or gall bladder cancer (40-80%). ,, In our reported case the gall bladder mucosa was thinned out and the hypertrophic muscle layer was infiltrated by tumor deposits of CDC, due to direct invasion from outside.
Most reported cases were diagnosed either pre-operatively when it is suspected during surgery as in our case or post-operatively on histopathology.  The cystic duct is a short structure, which lacks a proper muscle layer and consists of a thin fibro-muscular layer and adventitia. The interstitial tissues of the hepatoduodenal ligament are composed of loose fibrous tissue, lymphatic ducts and nerve fibers. Therefore cancers in the cystic duct may progress to the adherent gall bladder wall, head of pancreas, the para-aortic fatty tissue and Glisson's capsule in the liver, the hilar duct, or any combination of these sites. 
Nakata et al. have reported that gall bladder carcinoma spreading to the cystic duct has a poor prognosis.  This should occur usually along the mucosa late in the disease with larger area of involvement of the gall bladder mucosa. Our case had a different mode of spread as indicated by the involvement of only outer muscular wall of the gall bladder, due to direct contact even when the primary was small and easily resectable. CDC may be overlooked in a patient with gall stones and a hydropic gall bladder. In the absence of gall stones in a patient with hydropic gall bladder, presenting with obstructive jaundice, possibility of CDC should be considered in the differential diagnosis.
| Conclusion|| |
This is a rare case of cystic duct adenocarcinoma occurring in a 33-year-old woman and presenting as obstructive jaundice. The prognosis of cystic duct adenocarcinoma is better than for other hepatobiliary tract cancers. Neoplasm of the cystic duct should be suspected in patients presenting with cystic duct obstruction with distended non-functioning gall bladder without evidence of stone impaction in cystic duct.
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