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CASE REPORT Table of Contents   
Year : 2008  |  Volume : 51  |  Issue : 3  |  Page : 382-385
Hepatic tuberculosis mimicking Klatskin tumor: A diagnostic dilemma

1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Surgical Disciplines, All India Institute of Medical Sciences, New Delhi, India

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Tubercular involvement of liver is rare and usually occurs in association with pulmonary or miliary tuberculosis, as diffuse involvement without recognizable pulmonary tuberculosis or rarely in a localized form, which presents as a tuberculoma or tubercular abscess. We report the case of a 22-year-old boy presenting with features of obstructive jaundice and a clinico-radiological picture highly suggestive of a perihilar cholangiocarcinoma (Klatskin tumor), but found to have tubercular involvement of porta hepatis. We review the literature on this unusual presentation, highlight the considerable diagnostic challenge such cases can pose, and also emphasize the need to consider tuberculosis in differential diagnosis of lesions involving the porta hepatis, particularly in areas endemic for the disease.

Keywords: Cholangiocarcinoma, hepatic tuberculosis, Klatskin tumor

How to cite this article:
Arora R, Sharma A, Bhowate P, Bansal VK, Guleria S, Dinda AK. Hepatic tuberculosis mimicking Klatskin tumor: A diagnostic dilemma. Indian J Pathol Microbiol 2008;51:382-5

How to cite this URL:
Arora R, Sharma A, Bhowate P, Bansal VK, Guleria S, Dinda AK. Hepatic tuberculosis mimicking Klatskin tumor: A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2008 [cited 2022 Aug 17];51:382-5. Available from: https://www.ijpmonline.org/text.asp?2008/51/3/382/42517

   Introduction Top

Hepatic involvement is seen in less than 1% of all cases of tuberculosis. [1] This may occur in association with pulmonary or miliary tuberculosis, as diffuse involvement without recognizable pulmonary tuberculosis (granulomatous hepatitis) or the rare localized form, which presents as a tuberculoma or tubercular abscess. [2] Clinical and radiological manifestations of tuberculosis are protean and often mimic those associated with malignant lesions, thereby leading to erroneous clinical diagnosis and subsequent unnecessary surgical interventions.

Klatskin tumor is a term applied to cholangiocarcinoma occurring in the perihilar region of liver. We report the case of a 22-year-old male presenting with clinical and radiological features suggestive of a Klatskin tumor and found to have tubercular involvement of porta hepatis region on histopathological examination. This report highlights the difficulty in arriving at a correct preoperative diagnosis in such cases, and emphasizes the need of considering tuberculosis in differential diagnoses of lesions involving the porta hepatis and liver.

   Case History Top

A 23-year-old male presented with complaints of loss of appetite, yellowish discoloration of sclera, and high-colored urine for 9 months, and pruritus with passage of clay-colored stools for 8 months. There was significant weight loss during the last 6 months. There was no past history of tuberculosis or diabetes mellitus.

Physical examination revealed icterus. There was no palpable lymphadenopathy. Perabdomen examination showed non-tender hepatomegaly 5 cm below right costal margin. The gall bladder was not palpable. The rest of systemic examination was within normal limits.

Hematological profile was normal. Liver function tests showed total bilirubin of 12.1 mg% (conjugated 9.0 mg% and unconjugated 3.1 mg%). Alkaline phosphatase (2122 IU/l) and transaminase levels (SGOT - 116 IU/l and SGPT - 95 IU/l) were also elevated. Serology for human immunodeficiency virus (HIV) and hepatitis B virus was negative.

Chest X-ray was within normal limits. There was no evidence to suggest active or past tubercular lesion. Ultrasound abdomen showed enlarged liver with maintained echotexture. Intrahepatic biliary radicals were dilated. Contrast-enhanced computerized tomograph (CECT) of abdomen revealed dilatation of intrahepatic biliary radicals and evidence of a thrombus in right branch of portal vein [Figure 1]. Distal common bile duct was not well visualized. The gall bladder was contracted. The hepatic artery was normal. There was no evidence of free fluid or intraabdominal lymphadenopathy. An endoscopic retrograde cholangiopancreatography (ERCP) was attempted, but had to be abandoned as the endoscopic cannula could not be negotiated through the ampulla of Vater. Instead, a magnetic resonance cholangiopancreatography (MRCP) was performed, which revealed intrahepatic biliary radical dilatation involving both the lobes with a block at porta hepatis. Distal common bile duct was collapsed. Upper gastrointestinal endoscopy was normal. A clinico-radiologic diagnosis of cholangiocarcinoma (Klatskin tumor) was made and patient taken up for diagnostic laparoscopy and exploratory laparotomy.

Intraoperatively, a tumor was seen at confluence of right and left hepatic ducts. The right portal vein was thrombosed. An extended right hepatic lobectomy and was performed.

Pathological findings

A specimen of right lobe of liver with proximal part of common bile duct and attached gall bladder measuring 19 × 14 × 6 cm was received. On sectioning, a grey-white firm lesion with irregular margins measuring about 2 × 2 cm was seen at the porta hepatis [Figure 2]. The gall bladder was grossly unremarkable.

Sections from the lesion revealed multiple epithelioid cell granulomas, some showing a central area of caseation necrosis with Langhans giant cells and areas of fibrosis [Figure 3] and [Figure 4]. Adjacent liver parenchyma showed portal and periportal fibrosis and inflammation with marked bile ductular proliferation and evidence of intrahepatic cholestasis [Figure 5]. Hepatic duct and proximal part of common bile duct included in the specimen were uninvolved. No evidence of tumor was seen in any of the sections. Stains for acid-fast bacilli and fungus were negative. However, polymerase chain reaction (PCR), performed with devRf4/devR r3 primers, was positive in the tissue from the lesion. A final diagnosis of tubercular lesion at porta hepatis was thus made.

   Discussion Top

Tuberculosis in its varied forms is widely prevalent in tropics. It continues to be a leading cause of morbidity and mortality in the developing regions of the world, and has witnessed resurgence elsewhere as a result of the AIDS epidemic. While diagnosis of pulmonary tuberculosis is easy to establish, patients of abdominal and hepatic tuberculosis pose a considerable diagnostic problem. Hepatic tuberculosis usually presents as a diffuse involvement of liver with or without a recognizable focus of pulmonary tuberculosis or as localized involvement in form of an abscess or tuberculoma. [2]

Isolated hepatic tuberculosis is extremely rare with less than 30 cases being reported in the literature. [3] Pathophysiology of localized hepatic tuberculosis is similar to other forms of extrapulmonary tuberculosis, which result from hematogenous spread and implantation of tubercle bacilli from a primary pulmonary focus and subsequent regression of the primary focus. [4]

Clinical features of hepatic tuberculosis include pyrexia, variable degree of hepatosplenomegaly along with constitutional symptoms such as loss of weight, appetite, and anemia. Jaundice is extremely rare and occurs as a result of lymphadenopathy at porta hepatis, obstruction in the common bile duct or by portal inflammatory stricture. In addition, only about 25% of patients with hepatic tuberculosis have elevated liver enzymes. [2],[5] In the present case, obstructive jaundice was seen, apparently due to compression of hepatic ducts by the lesion at porta hepatis associated with elevated serum levels of liver enzymes.

Hepatic tuberculosis may show overlap of clinical features with several conditions including viral hepatitis, collagen vascular disorders and hepatic involvement in systemic infections including enteric fever and malignancies. [5] Indeed, cases of hepatic tuberculosis mimicking hepatocellular carcinoma [6] and gall bladder carcinoma [3] have been described in the literature. However, presentation of hepatic tuberculosis mimicking Klatskin tumor, involving porta hepatis as in the present case, has not been described previously. As is evident, definitive diagnosis of hepatic tuberculosis only on clinical, radiological, or biochemical findings is difficult because of the close similarity in clinical and laboratory profile of various disorders mentioned above. In a recent series of 682 cases of liver resections for suspected hepatocellular carcinoma, eight cases of hepatic tubercular pseudotumors were found, further emphasizing the diagnostic difficulty in such cases. [7]

Radiological diagnosis of hepatic tuberculosis also presents a diagnostic challenge, particularly in the localized form. On computerized tomography, appearance varies from a hypodense mass with or without post-contrast rim enhancement to a heterogeneous density of the necrotic center. Ultrasonography may reveal hypoechoic or rarely hyperechoic nodular lesions. [8] The present case, however, due to its unusual location presented with radiological findings strongly suggestive of a Klatskin tumor, namely, diffuse dilatation of intrahepatic biliary radicals, abrupt cutoff just beyond hepatic duct confluence with a hypodense mass lesion at the porta hepatic. [9] In addition, a thrombus was also seen in right branch of portal vein further emphasizing the possibility of a malignant lesion; indeed cases of hepatic tuberculosis presenting with portal vein thrombosis mimicking a malignancy are on record. [10],[11]

Microscopically hepatic tuberculosis encompasses a wide spectrum of morphological changes varying from caseating or non-caseating epithelioid cell granulomas to cases showing only caseation necrosis without a granulomatous component. Hersch [4] noted that tubercular granulomas in the miliary or diffuse form were located in the hepatic lobules in contrast to the localized form, where the granulomas were predominantly portal in location. Other non-specific histological findings include Kupffer cell hyperplasia, focal hepatocytes necrosis with round-cell infiltration, and portal inflammation. Smith et al. [12] have divided the histopathological features into reactive, hyporeactive, and non-reactive, based on degree of caseation necrosis and the number of AFB seen in the sections, reflecting individual cellular immunity. However, the absence of AFB should not detract from the diagnosis, particularly in areas endemic for tuberculosis. Differential diagnosis of granulomatous lesions elsewhere also holds true in hepatic lesions and includes sarcoidosis, fungal infections, and collagen vascular diseases. Utility of PCR has recently been evaluated for diagnostic evidence in difficult cases such as the localized form and those with negative AFB staining. Diaz et al. [13] found 57% of hepatic granulomas to be positive for mycobacterial antigen by PCR analysis. PCR has a potentially important role in improving the diagnostic accuracy of extrapulmonary tuberculosis. Maximum sensitivity and specificity can be achieved by the combined use of clinical diagnosis, histopathology, and PCR. [14] In the present case also, although AFB stain was negative in the lesion, PCR provided the diagnostic evidence.

Treatment of hepatic tuberculosis includes the standard four-drug regimen with Rifampicin, Isoniazid, Ethambutol, and Pyrazinamide as for any other form of extrapulmonary tuberculosis. [15] Even in cases where a surgical procedure has been carried out before the diagnosis of tuberculosis is evident, [16] there is a rationale for post-surgical antitubercular chemotherapy as this would help in eliminating any residual disease, and also treat any undetected primary focus.

   Conclusion Top

The present case highlights the diagnostic difficulties encountered with atypical presentation of tuberculosis. This represents the first reported occurrence of localized hepatic tuberculosis clinically and radiologically mimicking a Klatskin tumor. A high index of suspicion and a close differential diagnosis of tuberculosis is therefore must when dealing with such cases.

   References Top

1.Mert A, Ozaras R, Tabak F, Ozturk R, Bilir M. Localized hepatic tuberculosis. Eur J Intern Med 2003;14:511.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Purl AS, Nayyar AK, Vij JC. Hepatic Tuberculosis. Indian J Tub 1994;41:131-3.  Back to cited text no. 2    
3.Bikhchandani J, Malik VK, Kumar V, Sharma S. Hepatic tuberculosis mimicking carcinoma gall bladder. Indian J Gastroenterol 2005;24:25.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Hersch C. Tuberculosis of the liver. A study of 200 cases. S Afr Med J 1964;38:906.  Back to cited text no. 4    
5.Singh DS, Das AK, Aurora AL, Chandrasekhar S, Bisht DB, Tandon BN. Indian J Tub 1988:35:193-6.  Back to cited text no. 5    
6.Brookes MJ, Field M, Dawkins DM, Gearty J, Wilson P. Massive primary hepatic tuberculoma mimicking hepatocellular carcinoma in an immunocompetent host. Medscape Gen Med 2006;8:11.  Back to cited text no. 6    
7.Xing X, Li Hong, Liu WG. Hepatic segmentectomy for treatment of hepatic tuberculous pseudotumor. Hepatobiliary Pancreat Dis Int 2005;4:565-8.  Back to cited text no. 7    
8.Buxi TB, Vohra RB, Sujatha Y, Chawla D, Byotra SP, Gupta PS, et al. CT appearances in macronodular hepatosplenic tuberculosis: A review with five additional new cases. Comput Med Imaging Graph 1992;16:381-7.  Back to cited text no. 8  [PUBMED]  
9.Shizaki Y, Wakayama T, Okada Y, Kobayashi T. Magnetic resonance cholangiography for evaluation of obstructive jaundice. Am J Gastroenterol 1993;88:2072-7.  Back to cited text no. 9  [PUBMED]  
10.Venkatesh SK, Tan LK, Siew EP, Putti TC. Macronodular: Hepatic tuberculosis associated with portal vein thrombosis and portal hypertension. Australas Radiol 2005;49:322-4.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.Ka MM, Mbengue M, Dangau JM, Pouye A, Toure-Fall AO, Leye A, et al. Portal and splenic veins thrombosis reveling a miliary tuberculosis of the liver. Dakar Med 2004;49:150-2.  Back to cited text no. 11    
12.Smith MB, Boyars MC, Veasey S, Woods GL. Generalized tuberculosis in the acquired immune deficiency syndrome. Arch Pathol Lab Med 2000;124:1267-74.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Diaz ML, Herrera T, Lopez-Vidal Y, Calva JJ, Hernandez R, Palacios GR, et al. Polymerase chain reaction for the detection of Mycobacterium tuberculosis DNA in tissue and assessment of its utility in the diagnosis of hepatic granulomas. J Lab Clin Med 1996;127:359-63.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]
14.Kesarwani RC, Pandey A, Misra A, Singh A. Polymerase chain reaction (PCR): Its comparison with conventional techniques for diagnosis of extra-pulmonary tubercular diseases. Indian J Surg 2004;66:84-8.  Back to cited text no. 14    
15.Plumber ST, Pipalia DH, Vora IM, Bhambhure N, Naik SR. Hepatic granulomas: Profile and follow up of 10 cases responding to antituberculous therapy. J Assoc Physician India 1987;35:207.  Back to cited text no. 15    
16.Saluja SS, Ray S, Pal S, Kukeraja M, Srivastava DN, Sahni P, et al. Hepatobiliary and pancreatic tuberculosis: A two decade experience. BMC Surg 2007;7:10.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]

Correspondence Address:
Alok Sharma
Department of Pathology, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.42517

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]

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