Adenomas can appear in any part of the billiary tract, with the gallbladder being the most common site and those in the common bile duct being very rare1. To date, only 39 cases have been described in the scientific literature2. However, despite their low prevalence, they represent a high-risk pathology, given the high percentage of progression to cholangiocarcinoma. A clear evolutionary sequence has been demonstrated from low-grade neoplasms to invasive carcinomas, due to activation of common oncogenic pathways such as KRAS mutation and over expression of p533,4. Given the rarity of their presentation, there are no clear strategies for their treatment. In global terms, there are two types of possible polyps: adenomatous polyps, which have hostile behaviour, requiring equally aggressive and early treatment; and hyperplastic polyps, which have virtually no risk of malignancy and are associated with chronic inflammation5,6. The treatment of common bile duct adenomas is a challenge for the surgeon who is faced with the difficult decision of extended resection of the bile duct vs. local resection of the adenoma, especially if, as in the case we describe, it is diagnosed in the context of acute cholecystitis.
We present the case of a 75-year-old woman with a history of arterial hypertension and previous cholelithiasis, who came to the emergency department with pain in the right hypochondrium, jaundice and fever. Laboratory tests showed hyperbilirubinaemia of 10.2 mg/dL, with mild transaminasemia and elevated alkaline phosphatase up to 289 IU. An abdominal CT scan was performed which identified findings compatible with acute cholecystitis associated with Mirizzi syndrome. Urgent laparoscopic cholecystectomy and exploration of the biliary tract with extraction of multiple lithiasis and purulent material was indicated. Subsequently, a structure was identified in the mid-distal common bile duct compatible with an adenoma of approximately 1 MD, making it impossible to explore the distal bile duct with a choledochoscope. Given a very dilated common bile duct with cholangitis, the adenoma was not resected so as not to complicate the postoperative evolution, leaving a Kehr tube in the choledochotomy and planning more specific postoperative studies.
The postoperative period progressed favourably and the study was completed with cholangio-magnetic resonance imaging (MRI) (Fig. 1a), which confirmed the presence of the adenoma in the middle third of the common bile duct and endoscopic retrograde cholangiography (ERCP) assisted with Spyglass with serial biopsies. The diagnosis of the biopsies was compatible with adenoma without signs of dysplasia, and local resection was performed laparoscopically one month after the initial operation (Fig. 1b). A longitudinal choledochotomy was performed, identifying and resecting the polyp, with primary closure of the bile duct. The perioperative anatomo-pathological study excluded the presence of dysplasia, which was confirmed in the definitive study (Fig. 1c).
(a). Cholangio-NMR showing nodular signal void in the middle common bile duct (blue arrow), compatible with adenoma. (b) Image of the polyp in the middle third of the common bile duct during laparoscopic resection. (c) Pathology study showing biliary type glands surrounded by fibrous stroma and with mild associated inflammation.
According to the few cases described in the literature (Table 1), common bile duct adenoma predominantly affects males in their sixth decade of life, generally associated with long-standing abdominal pain, fever and progressive obstructive jaundice, with or without cholangitis, with only three asymptomatic cases described2. The clinical picture is that the adenoma leads to bile duct obstruction, and secondarily to acute cholangitis or choledocholithiasis, but more specific studies, such as NMR-cholangiography and ERCP, are able to define its true nature. Similar cases could be treated initially with ERCP, resolving the acute picture and postponing surgical intervention to a later stage. Our usual attitude is to try to resolve the pathology in a single stage, so we indicate surgery directly. Furthermore, in this case the initial diagnosis was Mirizzi syndrome, a situation in which, in our opinion, surgery offers better results than ERCP.
Cases of bile duct adenomas described in the literature, including sex, age, tumour location, therapeutic approach taken and anatomo-pathological result obtained.
| Author | Sex | Age | Site | Treatment | Pathology |
|---|---|---|---|---|---|
| Ariche et al. (2002)1 | F | 77 | C | LE, Hepatojejunostomy | AV |
| Burhans y Myers (1971)2 | F | 64 | CHI | LE | AP |
| F | 76 | CP | CUR | A | |
| Hultén et al. (1970)3 | M | 61 | DD | LE, hepatoduodenostomy | AP |
| M | 80 | DD | CUR, choledochoduodenostomy | AP | |
| Shemesh (1985)4 | M | 58 | DD | LE | TA |
| Sturgis et al. (1992)5 | F | 81 | DD | EE | ATV |
| Futami et al. (1997)6 | F | 40 | DD | LE | A |
| Jao et al. (2003)7 | M | 60 | DD | EE | ATV |
| Ibrarullah et al. (2003)8 | F | 33 | DD | Hepatojejunostomy | A |
| Katsinelos et al. (2006)9 | M | 58 | DD | DPC | A |
| Kim et al. (2008)10 | M | 55 | DD | DPC | ATV |
| Aparajita et al. (2009)11 | F | 75 | CP | DPC | AP |
| Akaydin et al. (2010)12 | M | 60 | CP | LE, Hepatojejunostomy | ATV |
| Munshi y Hassan. (2010)13 | F | 69 | DD | EE | AP |
| Prachayakul et al. (2012)14 | M | 53 | DD | EE | TA |
| Sirimontaporn et al. (2013)15 | M | 73 | MD | EE | A |
| Styne et al. (1986)16 | F | 59 | LHD | LE | P |
| Cardoza et al. (1988)17 | F | 53 | CHC | LE | P |
| Jennings et al. (1990)18 | M | 58 | CHC | LE | AV |
| Colarian y Wescott. (2001)19 | F | 78 | CHC | Hepatojejunostomy | AV |
| Sotona et al. (2010)20 | M | 58 | LHD | LE Hepatojejunostomy | PA |
| Ho y Lee. (2006)21 | M | 15 | CD | LE | PA |
| Loh et al. (1994)22 | F | 72 | CD | LE + cholecystectomy. | PA |
| Liu et al. (2014)23 | F | 61 | CD | EE | ATV |
| O’Shea et al. (2002)24 | M | 75 | CHC y LHD | LE | AV |
| Morris-Stiff et al. (2010)25 | F | 73 | CHC y LHD | LE, Hepatojejunostomy | PA |
| Hanafy et al. (1993)26 | M | 76 | C y CD | LE | AV |
| Xu y Chen. (2008)27 | F | 27 | CP y LHD | DPC | AV |
| Saxe et al. (1988)28 | M | 64 | DD | DPC | AV |
| Blot et al. (1996)29 | M | 84 | DD | LE | AV |
| Inagaki et al. (1999)30 | M | 73 | DD | DPC | PA |
| Chang et al. (2001)31 | M | 51 | DD | Rejection of surgery. | PA |
| Aggarwal et al. (2003)32 | M | 55 | MD | DPC | A |
| Lou et al. (2003)33 | M | 47 | DD | LE | TA |
| Fletcher et al. (2004)34 | M | 74 | DD | DPC | PA |
| Loh et al. (2015)35 | F | 85 | DD | Rejection of surgery | PA |
| F | 78 | DD | Choledochojejunostomy | A | |
| F | 61 | CHC | LE, Hepatojejunostomy | AV | |
| Current case | F | 75 | MD | Local laparoscopic resection | PA |
Pathology. A: Unspecified adenoma; ATA: Tubular Adenoma; ATV: Adenoma Tubulovillous; AV: Adenoma Villous; P: Papilloma; PA: Papillary Adenoma; Sex: F: female. M: male.
Site: C: common bile duct; CD: cystic duct; CDP: cephalic duodenopancreatectomy; CHC: common hepatic duct; CHI: left hepatic duct; DD: distal bile duct; MD: middle bile duct; PD: proximal bile duct; Treatment: CUR: curettage; EE: endoscopic excision; LE: local excision (surgical).
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Surgical resection strategies depend on the presence of severe dysplasia or malignancy. Most adenomas show signs of malignancy at diagnosis so, if possible, resection should involve the extrahepatic bile duct and be associated with a pancreaticoduodenectomy if the distal third is involved or the margins are affected. On the other hand, if the adenoma shows no signs of atypia/dysplasia, local resection and follow-up is the best strategy. However, definitive diagnosis can only be assured in resected specimens. ERCP biopsies can be suggestive, but have diagnostic limitations because they are difficult to access despite the use of Spyglass, and samples are limited to a part of the adenoma.
It is important to point out that more than 60% of resected adenomas described in the literature show anatomopathological signs of mild atypia7–9, so that, a priori, the best strategy may be local resection of the adenoma and enlargement only in cases of proven atypia, avoiding aggressive interventions with high postoperative morbidity and mortality.
In this case, we describe the laparoscopic approach for the first time in the literature as a treatment for choledochal adenoma, demonstrating the efficacy of a less aggressive option, with better recovery, prior to a second aggressive intervention if necessary.
Knowledge of the pathology and its different therapeutic approaches is important for better management, since if it progresses to cholangiocarcinoma the prognosis is poor despite treatment, and we must be aware of all the strategies available to avoid this detrimental outcome in our patients.
