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Arab Journal of Gastroenterology 13 (2012) 150–152
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Case Report
Gallbladder adenomyomatosis with tubercular portal lymphadenopathy masquerading as gallbladder carcinoma Ramdip Ray a, Rajesh Dey a, Souvik Chatterjee a, Pradipta Guha b,⇑ a b
Department of General Surgery, Medical College, Kolkata, 88 College Street, Kolkata 700073, West Bengal, India Department of General Medicine, Calcutta National Medical College, Kolkata, 24 Gorachand Road, Kolkata 700014, West Bengal, India
a r t i c l e
i n f o
Article history: Received 14 March 2011 Accepted 12 August 2012
Keywords: Gallbladder adenomyomatosis Fundal variant Tuberculosis Portal lymphadenopathy Carcinoma mimicker
a b s t r a c t Gallbladder carcinoma is more likely to occur in elderly females and the presence of periportal adenopathy often signifies advanced disease. Such patients are generally not taken up for surgery and are treated palliatively. Isolated periportal tuberculosis without the evidence of disease elsewhere is in itself a rarity. Here we present a case study of gallbladder mass suspected of being gallbladder carcinoma with portal lymphatic metastasis actually turning out to be that of gallbladder adenomyoma with periportal tuberculosis. This case illustrates how mass lesions of the gallbladder are commonly and falsely interpreted to be malignant. Crown Copyright Ó 2012 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved.
Introduction Gallbladder carcinoma is a common entity in this part of the world, particularly the Indian Gangetic river basin. Adenomyomatosis of the gallbladder being a degenerative condition, is characterised by the proliferation of the mucosa of the gallbladder wall, which forms protrusions and sinus tracts penetrating a hypertrophied muscular layer: the so-called Rokitansky–Aschoff sinuses (RASs). With adenomyomatosis being identified in at least 5% of cholecystectomy specimens, it is usually an incidental finding at the histologic examination of gallbladders or at radiological investigations such as ultrasonography performed for the detection of calculi. Visualisation of RASs on ultrasonography, computed tomography (CT) scan or magnetic resonance imaging (MRI) only occasionally detects adenomyomatosis in an acalculous gallbladder. Most of the patients with adenomyomatosis of the gallbladder are asymptomatic. However, right upper-quadrant pain can occur similar to the pain produced by cholelithiasis. Treatment of symptomatic adenomyomatosis consists of cholecystectomy by a laparoscopic or laparotomic approach whenever possible. Apart from this, isolated abdominal tuberculous lymphadenopathy is an extremely rare clinical finding, which makes the diagnosis even more difficult. These two rare clinical entities altogether make the ⇑ Corresponding author. Address: Department of General Medicine, Calcutta National Medical College, Kolkata, 335, Nandan Nagar, Belgharia, Kolkata 700083, West Bengal, India. Tel.: +91 33 25412415, mobile: +91 9433126469; fax: +91 33 22198727. E-mail address:
[email protected] (P. Guha).
clinical scenario very difficult for the physician to differentiate from gallbladder carcinoma.
Case report A woman aged 75 years was admitted to our institution with a history of mild to moderate pain over right upper abdomen for the last 6 months. The pain was colicky in nature and especially occurred after fatty food intake. In the past 3 months, the attacks became more frequent, intense and distressing and not relieved by analgesic medications. She also noticed a gradually enlarging swelling over the same site for the same duration. She did not have any history of loss of weight, anorexia and jaundice. Her past medical records were insignificant and she did not have any comorbidities. On examination, she was of average build and nutritional status. Her abdomen was soft with a firm, well-defined lump over the upper abdomen, which was fixed to the underlying structures but was free from overlying skin. Keeping in mind the clinical aspect of this female patient, a provisional diagnosis of gallbladder mass probably due to a malignant process was made. In blood biochemical markers, no significant haematological abnormalities were detected. Her liver function tests and pancreatic enzymes were normal, too. The patient was human immunodeficiency virus (HIV) seronegative. An ultrasound abdomen revealed wall thickening in the fundal region of the gallbladder without any other significant finding. Contrast-enhanced CT scan of the abdomen revealed a structure with increased attenuation in the fundus of the gallbladder, which did not change in position
1687-1979/$ - see front matter Crown Copyright Ó 2012 Arab Journal of Gastroenterology. Published by Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.ajg.2012.08.006
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R. Ray et al. / Arab Journal of Gastroenterology 13 (2012) 150–152
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Fig. 4. Microscopical view of periportal node showing granuloma and central caseation with amorphous eosinophilic material along with typical Langhans’ giant cells.
Fig. 1. Axial contrast-enhanced CT scan showing focal gallbladder wall thickening at gallbladder fundus containing multiple small cystic spaces.
Fig. 2. Macroscopically the specimen of gallbladder showing a thickened wall mainly over fundal region but without any calculi.
on altered decubitus (Fig. 1). The rest of the biliary tree was normal. The patient was put up for operation keeping in mind the diagnosis of gallbladder carcinoma and prepared accordingly. A right subcostal incision was made which was later extended vertically in the midline. The gallbladder mass was palpated. No obvious signs of hepatic or peritoneal metastasis were present. Macroscopically, the specimen of the gallbladder was 8 4 cm, with a thickened wall mainly over the fundal region, but without any calculi (Fig. 2). On kocherisation, posterior pancreatico-duodenal lymph nodes were seen enlarged and there were enlarged pericholedochal and periportal lymph nodes as well. The portal triad was skeletonised and the periportal lymph nodes were removed to the right of the coeliac axis. A wedge of liver with a 2-cm margin was removed as well and sent to the pathology laboratory. The patient developed respiratory acidosis on the 4th postoperative day and had to be put on mechanical ventilation. She was weaned successfully and made a gradual recovery. The histopathology report quite astonishingly revealed a hyperplastic mucosa with diffusely placed focal erosions and a thickened musculosa layer. RASs into and through the musculosa were also noted. The adjacent fat tissue layer was almost completely replaced by a fibrous layer. The RAS, inflammatory and fibrotic changes were found spread mainly over the gallbladder fundal wall and were characteristic of the histologic appearance of the fundal variety of adenomyomatosis of the gallbladder (Fig. 3). Hence, our diagnosis changed to adenomyoma and adenomatous (tubular) polyp of the gallbladder. Dissected periportal lymph nodes showed features of granulomatous inflammation consistent with tuberculous lymphadenopathy (Fig. 4). Then, we reviewed our clinical examination to detect any other tubercular focus. However, we could not detect any other pathological site except for the periportal lymph node group. The patient was referred to the general physician to treat tuberculosis, where she received antitubercular therapy and is doing well at present.
Discussion
Fig. 3. Microscopical appearance of gallbladder adenomyomatosis showing cystic protrusions in the thickened gallbladder wall lined with glandular epithelium and surrounded by muscle fibres.
Gallbladder carcinoma is quite a common disease entity in people residing in the Gangetic basin in the eastern part of India. It generally presents in the elderly age group with pain in the abdomen and jaundice and most patients seek medical attention at a later stage, when only a minority of patients remain operable at presentation.
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R. Ray et al. / Arab Journal of Gastroenterology 13 (2012) 150–152
Adenomyomatosis is a benign condition of the gallbladder characterised by muscular wall hypertrophy, an overgrowth from the mucosal layer producing an intramural sinus called RASs [1]. The condition can be classified morphologically into three types: diffuse (generalised), segmental (annular) and the localised. In the diffuse variety, we can find thickening of the muscular layer, mucosal surface irregularity and small cystic structures within the wall [1]. The segmental type causes focal strictures. The most common site of localised variety is fundus and in that case, it is called adenomyoma [2]. Patients of gallbladder adenomyomatosis might present with abdominal pain as in the present case but the majority of the fundal type is asymptomatic [3]. The distinctive multimodality imaging features of adenomyomatosis are due to a thickened gallbladder containing bile with cholesterol crystals, sludge or calculi [4]. The aetiology of gallbladder adenomyomatosis is not yet completely understood. Its incidence varies from 2% to 33% [5]. In the fundal type of adenomyomatosis, a macroscopically intraluminal rounded mass is seen in the fundus of the gallbladder. Histological diagnosis is easy, as it has distinctive microscopic features from other types. The smooth muscle cell is positive for alpha-smooth muscle actin [6]. Our case was depicted as ‘fundal variant of adenomyomatosis’ because of both macroscopic and microscopic features. The differential diagnosis of fundal type adenomyomatosis consists of few pathologic lesions which also cause wall thickening leading to protrusion into the lumen. Among them, the most common with fatal consequences is adenocarcinoma of the gallbladder. Other common but protruding lesions of the gallbladder are adenomatous polyp, hyperplastic polyp and cholesterol polyps. Apart from these, metastatic diseases such as malignant melanoma should be kept in the differential diagnosis [7]. There are different tools available to diagnose this pathological condition. The oral cholecystogram can be used to diagnose adenomyomatosis by observing the filling of RASs with contrast material [3]. Ultrasound and MRI being the preferred radiological examinations, characteristically show intramural cysts in the gallbladder. Focal or segmental adenomyomatosis may simulate gallbladder carcinoma radiologically and may create a difficulty in differentiating the carcinoma [2]. On ultrasound scan, echogenic foci, the absence of cystic space and the presence of vascularity within the lesion need further evaluation to differentiate it from carcinoma [2]. The characteristic finding in CT scan is gallbladder wall thickening along with a rosary sign, which is formed by the mucosal outgrowth and intramural sinus tracts surrounded by the nonenhancing hypertrophied muscular layer of gallbladder wall [3]. Isolated periportal tuberculous lymphadenopathy is a rare clinical phenomenon. Abdominal tuberculosis is a common entity in India and has diverse presentation ranging from mild abdominal pain to ascites and intestinal obstruction. There are multiple reports of periportal adenopathy causing compression of the biliary tree leading to obstructive jaundice. However, isolated tubercular periportal adenopathy without any evidence of disease elsewhere is a rarity and there are only few reported cases of the same. It is quite evident that the number of patients of tuberculosis is slowly increasing worldwide, mainly because of the burden of HIV infection [8]. Extrapulmonary tuberculosis has become more common following the increase in co-morbid HIV disease prevalence [9], and abdominal tuberculosis is one of the most common forms [10]. However, the uniqueness of our case study was that the patient was HIV seronegative; still, she developed this isolated
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form of abdominal tuberculosis. Preoperative diagnosis is often difficult because of the diverse clinical manifestation, and in many cases the diagnosis is confirmed during exploratory laparotomy or after histopathological examination report [10]. Isolated abdominal tuberculous lymphadenopathy is extremely rare, which makes the clinical scenario even more challenging. It is a common finding that most cases of abdominal tuberculous lymphadenopathy are associated with the infection of other organs [11]. Isolated periportal lymphadenitis is uncommon, and is very difficult to differentiate from the neoplastic disease process. Although the exact cause of adenomyomatosis is still unknown, persistent irritation by any chronic disease process should be evaluated thoroughly in future. We wish to highlight this case study because of the presence of dual pathology in a single patient: Gallbladder adenomyomatosis presenting as a mass lesion in CT scan of the abdomen along with isolated periportal tuberculous adenopathy, as mass lesion in the gallbladder on CT scan is usually taken to be gallbladder carcinoma, which misdirects further patient management. Hence, it is prudent to keep in mind that all gallbladder masses are not neoplastic lesions. Hence, we should remain vigilant regarding this fact during evaluation of a patient with a gallbladder mass lesion. Proper evaluation and early initiation of therapy may alter the life expectancy of these patients. The presence of such rare features simultaneously in a patient makes this case study unique as there are only very few reported articles till date. Author contributions Ramdip Ray and Rajesh Dey contributed equally to this work; Pradipta Guha and Souvik Chatterjee designed and performed the research; Pradipta Guha wrote the article. Conflicts of interest The authors declared that there was no conflict of interest. References [1] Hagga JR, Dogra VS, Forsting M, et al. (5th ed.) CT and MRI of the whole body. Nova Science Publishers Inc., USA, 2009. [2] Rumack C, Wilson S, Charboneau JW, et al. Diagnostic ultrasound. 3rd ed. Mosby; 2004. [3] Dogra VS. Adenomyomatosis [homepage on the Internet]; 2009. [4] Levy AD, Murakata LA, Abbott RM, et al. From the archives of the AFIP. Benign tumors and tumorlike lesions of the gallbladder and extrahepatic bile ducts: radiologic–pathologic correlation. Armed Forces Institute of Pathology. Radiographics 2002;22(2):387–413. [5] Williams I, Slavin G, Cox A, et al. Diverticular disease (adenomyomatosis) of the gallbladder: a radiological–pathological survey. Br J Radiol 1986;59(697): 29–34. [6] Handra Luca A, Terris B, Couvelard A, et al. Adenomyoma and adenomyomatous hyperplasia of the vaterian system: clinical, pathological and new immunohistochemical features of 13 cases. Mod Pathol 2003;16(6): 530–6. [7] Kasahara Y, Sonobe N, Tomiyoshi H, et al. Adenomyomatosis of the gallbladder: a clinical survey of 30 surgically treated patients. Nippon Geka Hokan 1992;61(2):190–8. [8] Frieden TR, Sterling TR, Munsiff SS, et al. Tuberculosis. Lancet 2003;362(9387): 887–99. [9] Shafer RW, Edlin BR. Tuberculosis in patients infected with human immunodeficiency virus: perspective on the past decade. Clin Infect Dis 1996;22(4):683–704. [10] Uygur Bayramicli O, Dabak G, Dabak R. A clinical dilemma: abdominal tuberculosis. World J Gastroenterol 2003;9(5):1098–101. [11] Malik A, Saxena NC. Ultrasound in abdominal tuberculosis. Abdom Imaging 2003;28(4):574–9.
