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Jack in the Duct

A Case of Common Biliary Duct Schwannoma

Kolhe, Kailash M MD1; Amarapurkar, Anjali MD2; Ingle, Meghraj MD, DNB1; Pandey, Vikas MD, DM1; Amonkar, Mamata MD1; Khairnar, Harshad MD, DM1; Chauhan, Shamshersingh G MD1; Walke, Swapnil MD1; Shukla, Akash MD, DM1

doi: 10.14309/crj.0000000000000223
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1Department of Gastroenterology, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India

2Department of Pathology, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India

Correspondence: Kailash Kolhe, MD, Department of Gastroenterology, Lokmanya Tilak Municipal Medical College and General Hospital, RB2 Central Railway Quarters, Sion W, Sion, Mumbai, Maharashtra 400022, India (kkthedoc27@gmail.com).

Received July 06, 2019

Accepted August 16, 2019

Online date: October 17, 2019

This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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CASE REPORT

A 46-year-old woman was admitted with a history of painless, intermittent, self-resolving, cholestatic jaundice, without preceding prodrome for the past 1 year. She had 3 similar episodes without any significant past medical history. There was no history of significant weight loss, anorexia, hepatic decompensation, or any surgery in the past. Laboratory investigations showed a normal hemogram, conjugated hyperbilirubinemia with total bilirubin of 12 mg/dL and direct bilirubin of 9.6 mg/dL, normal aminotransferases, of 38 IU/L, elevated alkaline phosphatase (1,123 IU/L), and an albumin level of 3.4 g/dL with a gamma-glutamyl transferase (GGT) of 63 U/L. Hepatitis A, B, and C virus serology results were negative.

Ultrasonography of the abdomen showed an 8 mm dilatation of the mid- and proximal common biliary duct (CBD) with intrahepatic biliary radicle dilatation (IHBRD). A contrast-enhanced computed tomography scan of the abdomen revealed an ill-defined lesion in the lower CBD, with IHBRD. Magnetic resonance cholangiopancreatography demonstrated IHBRD and T2 hyperintense focal cystic bulbous dilatation of the left hepatic duct (Figure 1).

Figure 1

Figure 1

Endoscopic ultrasound was suggestive of an eccentric globular mass with dilated CBD and IHBRD (Figure 2). Endoscopic retrograde cholangiopancreatography revealed an ulcerated, pinkish fleshy mass hanging out of papilla intermittently. Cholangiogram demonstrated dilated mid- and proximal CBD with IHBRD with rounded filling defect in the left hepatic duct. Spyglass cholangioscopy (Boston Scientific, Marlborough, MA) showed a polypoidal lesion with a smooth surface and ulcerated stalk attached at the hilum (Figure 3). Biopsies were taken during cholangioscopy using SpyBite forceps (Boston Scientific, Marlborough, MA). CBD stenting was performed.

Figure 2

Figure 2

Figure 3

Figure 3

Biopsy showed fascicles of benign spindle cells mixed with sparse inflammatory cells. Immunohistochemistry showed S100 positivity suggestive of Schwannoma of the biliary tract (Figure 4). Our patient did not consent for definitive surgery and is currently asymptomatic. CBD Schwannoma arises from sympathetic and parasympathetic nerve fibers around the wall of the CBD. Treatment is resection of CBD and hepaticojejunostomy.1 There are only 18 reported cases until now, which showed female predominance and an average age of 43 years. The most common symptom at presentation was jaundice.2

Figure 4

Figure 4

Almost all the reported cases showed involvement of the extrahepatic bile ducts. In a similar case, reported by Madhusudan et al, it was shown to be involving both intrahepatic and extrahepatic bile ducts.3 Our case is a unique one as it is arising from the isolated intrahepatic bile duct, pedunculated, intermittently prolapsing and presenting as intermittent obstructive jaundice. Our case has no other features suggestive of neurofibromatosis. Keeping the possibility of such tumor and timely referral after endoscopic retrograde cholangiopancreatography is the key to diagnosis and preventing surgery. Also use of cholangioscopy-guided biopsy was fundamental in preoperative diagnosis of the lesion, unlike previous cases where the diagnosis was based on postsurgical histopathology.

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DISCLOSURES

Author contributions: All authors contributed equally to this manuscript. KM Kolhe is the article guarantor.

Financial disclosure: None to report.

Previous presentation: This case report was presented as a poster at the 2018 Asia Pacific Association of Study of Liver Meeting; March 14-18, 2018; New Delhi, India.

Informed consent was obtained for this case report.

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REFERENCES

1. Jung JH, Joo KR, Chae MJ, et al. Extrahepatic biliary schwannomas: A case report. J Korean Med Sci. 2007;22:549–52.
2. Marin Campos C, Garcia Sanz I, Mun[Combining Tilde]oz de Nova JL, Valde[Combining Acute Accent]s de Anca A, Marti[Combining Acute Accent]n Pe[Combining Acute Accent]rez ME. Schwannoma of the biliary tract resembling cholangiocarcinoma: A case report and review. Ann R Coll Surg Engl. 2016;98:e143–46.
3. Madhusudhan KS, Srivastava DN, Dash NR, Gupta C, Gupta SD. Case report. Schwannoma of both intrahepatic and extrahepatic bile ducts: A rare case. Br J Radiol. 2009;82:e212–5.
© 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.