Graphy Publications
Inspiring Innovations & Discoveries
International Journal of Gastroenterology Disorders & Therapy Volume 3 (2016), Article ID 3:IJGDT-122, 4 pages
Case Report
Inflammatory Pseudotumor of the Liver in Patient with Type 2 Diabetes

Malgorzata Zdunek1, Anna Ostrowska1, Piotr Wilczynski2, Slawomir Rudzki2 and Justyna Szumilo1*

1Department of Clinical Pathomorphology, Medical University of Lublin, Poland
2 Department of General Surgery, Medical University of Lublin, Poland
Prof. Justyna Szumilo MD, PhD, Department of Clinical Pathomorphology, Medical University of Lublin, 8 Jaczewskiego Str., PL-20-059, Lublin, Poland; E-mail:
06 January 2016; 09 April 2016; 11 April 2016
Zdunek M, Ostrowska A, Wilczynski P, Rudzki S, Szumilo J (2016) Inflammatory Pseudotumor of the Liver in Patient with Type 2 Diabetes. Int J Gastroenterol Disord Ther 3: 122. doi:


Inflammatory pseudotumor of the liver is a rare tumor-like lesion which has no specific clinical, laboratory or radiological findings. Despite the advances in the imaging techniques there is still possibility of misdiagnosis of this hepatic condition since it is often confused with primary or secondary neoplasms of the liver. Moreover, the gross appearance of inflammatory pseudotumor sometimes suggests malignancy. A new case of a hepatic inflammatory pseudotumor in a 65-year-old woman is reported. The patient suffered from some disorders which are thought to be induction factors for the tumor, i.e. cholelithiasis accompanied by chronic cholecystitis in the past medical history and diabetes mellitus type 2. A brief review of the literature especially regarding to histopathological features and diagnosis of hepatic inflammatory pseudotumor was also presented.

1. Introduction

Inflammatory pseudotumor (IPT) also known as inflammatory myofibroblastic tumor is a rare tumor-like lesion of unknown cause. It may arise in nearly every site of the body (e.g. breast, kidney, thyroid, salivary glands, larynx, spleen, stomach, brain, omentum, mesentery, orbit), but most commonly develops in the lung and the liver [1-8]. The first description of hepatic localization appeared in 1953 [9]. In most cases it affects adults, but in Asia, the hepatic IPT occurs often in children and young adult persons [2,6]. IPT usually presents as an asymptomatic [5], slowly growing, unilateral mass accompanied by unspecific symptoms like fever, malaise, abdominal pain and rarely vomiting, jaundice and weight loss [3,4,6,7,10]. It typically forms the solitary large (maximum dimension range from 2 to 22 cm) mass and accounts for 0.4% of all focal liver lesions [2,7]. Less often multifocal lesions have been described [2,7,11-13]. Majority cases are located in the right lobe. Radiological findings of the IPT are nonspecific but benign histopathological features constitute the hallmark of this lesion [12-14,16,17].

The aim of the description was to present a new case of hepatic inflammatory pseudotumor in patients with type 2 diabetes.

2. Case Report

A 65-year-old woman was admitted to the 1st Department of General Surgery of Medical University of Lublin (Lublin, Poland) with weakness, increased temperature and a liver mass discovered incidentally on the ultrasound scan. The right upper abdominal quadrant was tender at palpation. There were no signs of jaundice. Her previous medical history revealed classical cholecystectomy performed due to cholelithiasis about 6 years ago. Abdominal ultrasonography (USG) showed a 50x42x39 mm hypoechogenic, slightly heterogenous lesion located in the right lobe of the liver (segment 4/5). Computer tomography (CT) confirmed presence of irregular, hypoattenuating pathological mass with an irregular contrast enhancement in portal and venous phase in segments 4/5. Higher enhancement was revealed only in arterial phase. Similar subcapsular lesion with poorly visible margins (57x15 mm) was found in segment 7. Mild enlargement of mesenteric and hepatic hilar lymph nodes was observed. The primary radiological diagnosis was hepatic tumour with non-excluding malignancy or abscess but the second lesion was regarded as posttraumatic damage.

The results of laboratory tests were as follows: white blood cell count – 12.360/μL (normal range 4.000-10.500/μL), neutrophils – 76.2% (normal range 45-70%), lymphocytes – 15.7% (normal range 20-45%), monocytes – 5.3% (normal range 3.0-8.0%), red blood cell count – 3.53x106/μL (normal range 3.8-5.2x x106/μL), serum hemoglobin concentration – 10.4 g/dL (normal range 12.0-16.0 g/dL); haematocrit – 31.2 % (normal range 37.0-47.0), and serum C-reactive protein – 230.18 mg/l (normal range <5.000 mg/l). The laboratory data showed moderately elevated liver enzyme: aspartate aminotransferase (AST) – 42 U/L (normal<34 U/L) and alanine aminotransferase (ALT) – 47 U/L (normal <31 U/L).

Other results of biochemical analysis of the blood including serum platelet count and levels of serum total bilirubin, alkaline phosphatase (ALP), amylase and lipase were within normal limits. Laboratory parameters of renal function and serum level of tumor markers (α-fetoprotein) – remained within normal range. Hepatitis B virus antigen and hepatitis C virus antibody were negative. Furthermore, the patient suffered from diabetes type-2 which was controlled by diet and Metformin administration. Her fasting plasma glucose level was 99mg/dL.

An initial clinical diagnosis of hepatic tumor was made. The patient was qualified for urgent laparotomy with frozen section examination. Samples taken during surgical operation revealed prominent inflammatory process with numerous plasma cells, lymphocytes and foam cells with accompanied fibrosis. In the subsequent step, the patient underwent resection of the whole hepatic lesion. The ultimate surgical specimen sized a 6.5x5.5x3,0 cm contained an ill-defined, solid, firm, light-brown lesion with white-gray areas a 5x2,5x2 cm in dimentions (Figure 1). After fixation in 10% buffered formalin, many samples were taken for histopathological examination and then routinely processed into paraffin blocks. The slides were stained with hematoxylin and eosin (HE), mucycarmine, periodic acid Shiff (PAS), van Gieson, Prussian blue and Shikata’s orcein method for hepatitis B surface antigen (HBsAg). Furthermore, immunohistochmical reactions with smooth muscle actin (SMA), vimentin, CD34, CD117, ALK1, CD68, CD138, CD45 and Ki67 were also performed using reagents from Dako (Denmark) according to manufacturer directions.

figure 1
Figure 1: Gross appearance of surgical specimen of hepatic inflammatory pseudotumor.

Microscopically, the lesion was composed of the anastomosing fascicles of spindle cells intermingled with a variety of inflammatory cells including lymphocytes (CD45+), plasma cells (CD138+), different types of macrophages i.e. foamy histiocytes and multinucleated giant (CD68+) and few neutrophils, occasionally surrounded by collagen bundles (Figure 2A and Figure 2B). An immunohistochemical analysis revealed some SMA- (Figure 3) and vimentin-positive spindle cells, but they were negative for ALK1, CD34 and CD117. Proliferative index (Ki67) was low (5-7%). Staining for HBsAg was negative. Resection margin was free of the lesion but some scattered hepatocytes with macrovacuolar steatosis were present. There was no evidence of malignancy. A final histopathological diagnosis of IPT was established.

figure 2
Figure 2: Microscopic appearance of hepatic inflammatory pseudotumor – fascicles the spindle cells and chronic inflammatory cells in fibrous background. (H&E; objective magn. A – 10x, B – 20x).
figure 3
Figure 3: Postive immunohistochemical staining for smooth muscle actin in spindle-shaped myofibroblasts of hepatic inflammatory pseudotumor (DakoEnVisionTM+/HRP; objective magn. 20x).

Follow-up CT performed two weeks after the surgery demonstrated a small fluid-filled cystic space in the postoperative area. But apart from that the postoperative period was uneventful. The patient becomes asymptomatic one year after the surgery.

3. Discussion

IPT is an uncommon hepatic lesion considered as a benign condition usually with good prognosis, however, aggressive behavior has been occasionally reported [3,18]. They may sporadically recur usually as a consequence of incomplete resection [14,18]. Both, clinical and histopathological features of IPT did not predict the aggressive behavior [18].

The etiology and pathogenesis of this entity remain unclear. Many mechanisms and hypothesized etiologic factors like infections, trauma, autoimmunity reactions, radiotherapy, and local reaction to changes in the walls of bile ducts are suggested [1-7,10,13,18-26]. Some microorganisms that access the portal vein including Escherichia coli, Staphylococcus aureus, Klebsiella pneumoniae, Gram-positive cocci and Actinomyces were regarded as causative agents. Viral infections (Epstein-Barr virus [20], hepatitis B and C virus) [10,27] and parasitic infestations (Schistosoma mansoni) may play role in the pathogenesis [18]. Some authors suggested that disturbance of biliary drainage, e.g., due to biliary stones [1,7], recurrent pyogenic cholangitis [4,26] or sclerosing cholangitis is also important [25]. The tumor was also noted in HIV infected persons [24]. IPT is occasionally associated with systemic diseases, mostly of autoimmune background like Crohn disease [19,21], autoimmune pancreatitis [23], as well as diabetes mellitus [7,28], gout, acute myelomonocytic leukemia [29] and rectal gastrointestinal stromal tumor [30]. More recently some genetic rearrangements referring anaplastic lymphoma kinase 1 (ALK1) expression has been reported in IPT [3,7,31-33]. Chromosomal translocation involving chromosome 2p23 upon which the ALK gene is located has been identified in some cases [31-33]. Moreover a fusion of ALK gene with a partner genes such as TPM3 and TPM4 has been demonstrated [33]. Although ALK-positive and ALK-negative tumors are histologically identical, variable immunophenotype support the heterogeneity of IPTs and may indicate a different pathogenetic processes in hepatic IPT [18,31-33]. In presented case immunohistochemical reaction with ALK1 was negative.

The histological appearance of IPT can vary and thus multiple terms were given for this condition, e.g. plasma cell granuloma, inflammatory myofibroblastic tumor, histiocytoma, fibroxanthoma, xanthogranuloma or plasma cell histiocytoma [1-5,10,17,18,31,33]. According to the current WHO classification of the digestive system tumor [2], it is defined as a mesenchymal, benign, non-neoplastic and non-metastasizing mass composed of fibrous tissue and proliferated myofibroblasts with a marked inflammatory infiltration, predominantly of plasma cells. Marked cellular atypia and frequent mitoses are not found in IPTs [2,4,5,7,8]. IPT always consists of a mixture of inflammatory cells and fibrotic tissue but the relative proportion of these components can be variable. Due to microscopic heterogeneity, they were previously classified into three histologic subtypes, e.g. hyalinised sclerosing, xanthogranuloma and plasma cell granuloma [6]. However, recently only two types are distinguished: fibrohistiocytic and lymphoplasmacytic from IgG4-related disease [7,8,11]. Reported case fulfils histological criteria of fibrohistiocytic type, which is more frequent. Zen et al. [8] suggested that IPT may represent the end stage of heterogeneous inflammatory processes in the liver, including cholangitis. Recently Yang et al. [7] performed a retrospective analysis of 114 patients with hepatic IPT and proposed a clinical diagnosis guideline for establishing a correct diagnosis. It's interesting that in their study 30 patients had a history of biliary tract inflammatory disease in the past and 18 suffered from diabetes mellitus. They suggested that both above mentioned conditions are associated with development of IPT in the liver. In the present case two potentially predisposing factors were noted, i.e. cholelithiasis accompanied by chronic cholecystitis in the past medical history which could be regarded as an induction factor and successfully treated type 2 diabetes.

Regardless location IPT may be confused with true neoplasms. Precise diagnosis of lesions is difficult because they are rare conditions and clinical symptoms and laboratory findings are nonspecific. Furthermore, both clinical presentation and radiological images may be suggestive for malignancy [4,11-13,15,16,22,27,30,31]. In the liver IPT has to be distinguished from hepatocellular carcinoma [15,16,27], intrahepatic cholangiocarcinoma [11,13], metastases [12,30] and inflammatory conditions mostly abscesses [17,22]. For this reason IPT is sometimes called ‘the great mimicker” [22]. To avoid inappropriate diagnosis of neoplasm a detailed microscopic examination of the whole tumor with many samples proceeded by biopsy or frozen-section examination is necessary. Although radiographic appearance of the described tumor has not ruled out malignancy, the microscopic features were characteristic. Exclusion of neoplastic process is the primary concern, since IPT can be resolved by conservative therapy within 1-2 months (antibiotics, steroids or nonsteroidal anti-inflammatory drugs) or by surgery with excellent prognosis [11,12,34,35]. Spontaneous regression of hepatic IPT has been reported as well [36].

In conclusion, diagnosis of inflammatory pseudotumor is mostly based on microscopic examination, since its clinical and radiological presentation is frequently not characteristic. Correct diagnosis is essential for proper management.

Competing Interests

The author(s) declare that they have no competing interests.


  1. Al-Jabri T, Sanjay P, Shaikh I, Woodward A (2010) Inflammatory myofibroblastic pseudotumour of the liver in association with gall stones - a rare case report and brief review. Diagn Pathol 5: 53. View
  2. Bosman FT, Carneiro F, Hruban RH, Theise ND (2010). WHO Classification of tumours of the digestive system (4th ed.) Lyon, IARC, France, volume 3, 246 p.
  3. Coffin CM, Hornick JL, Fletcher CD (2007) Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol 31: 509-520. View
  4. Ntinas A, Kardassis D, Miliaras D, Tsinoglou K, Dimitriades A, et al. (2011) Inflammatory pseudotumor of the liver: a case report and review of the literature. J Med Case Rep 5: 196. View
  5. Schmid A, Jänig D, Bohuszlavizki A, Henne-Bruns D (1996) Inflammatory pseudotumor of the liver presenting as incidentaloma: report of a case and review of the literature. Hepatogastroenterology 43: 1009-1014. View
  6. Someren A (1978) "Inflammatory pseudotumor" of liver with occlusive phlebitis: report of a case in a child and review of the literature. Am J Clin Pathol 69: 176-181. View
  7. Yang X, Zhu J, Biskup E, Cai F, Li A (2015) Inflammatory pseudotumors of the liver: experience of 114 cases. Tumour Biol 36: 5143-5148. View
  8. Zen Y, Fujii T, Sato Y, Masuda S, Nakanuma Y (2007) Pathological classification of hepatic inflammatory pseudotumor with respect to IgG4- related disease. Mod Pathol 20: 884-894. View
  9. PACK GT, BAKER HW (1953) Total right hepatic lobectomy; report of a case. Ann Surg 138: 253-258. View
  10. Sürer E, Bozova S, Gökhan GA, Gürkan A, Elpek GO (2009) Inflammatory myofibroblastic tumor of the liver: a case report. Turk J Gastroenterol 20: 129-134. View
  11. Ahn KS, Kang KJ, Kim YH, Lim TJ, Jung HR, et al. (2012). Inflammatory pseudotumors mimicking intrahepatic cholangiocarcinoma of the liver; IgG4-positivity and its clinical significance. J Hepatobiliary Pancreat Sci 19: 405-412. View
  12. Chong A, Jeong SY, Min JJ (2009) Inflammatory pseudotumours resembling multiple hepatic metastases and their complete regression, as revealed by 18F-FDG PET/CT. Eur J Nucl Med Mol Imaging 36: 1199-1200. View
  13. Kai K, Matsuyama S, Ohtsuka T, Kitahara K, Mori D, et al. (2007) Multiple inflammatory pseudotumor of the liver, mimicking cholangiocarcinoma with tumor embolus in the hepatic vein: report of a case. Surg Today 37: 530- 533. View
  14. Chang SD, Scali EP, Abrahams Z, Tha S, Yoshida EM (2014) Inflammatory pseudotumor of the liver: a rare case of recurrence following surgical resection. J Radiol Case Rep 8: 23-30. View
  15. Iguchi H, Yamazaki H, Tsunoda H, Takahashi Y, Yokomori H (2013) A Case of Inflammatory Pseudotumor of the Liver Mimicking Hepatocellular Carcinoma on EOB-MRI and PET. Case Rep Med 2013: 594254. View
  16. Chen CB, Chou CT, Hsueh C, Lee KW, Chen YL (2013) Hepatic inflammatory pseudotumor mimicking hepatocellular carcinoma. J Chin Med Assoc 76: 299-301. View
  17. Liu XF, He BM, Ou-Yang XH, Wang ZZ, Su JG (2012) Different imaging findings of inflammatory myofibroblastic tumor of the liver. World J Gastroenterol 18: 5821-5825. View
  18. Pannain VL, Passos JV, Filho AR, Villela-Nogueira C, Caroli-Bottino A (2010). Aggressive inflammatory myofibroblastic tumor of the liver with underlying schistosomiasis: a case report. World J Gastroenterol 16: 4233- 4236. View
  19. Amankonah TD, Strom CB, Vierling JM, Petrovic LM (2001) Inflammatory pseudotumor of the liver as the first manifestation of Crohn's disease. Am J Gastroenterol 96: 2520-2522. View
  20. Arber DA, Kamel OW, van de Rijn M, Davis RE, Medeiros LJ, et al. (1995) Frequent presence of the Epstein-Barr virus in inflammatory pseudotumor. Hum Pathol 26: 1093-1098. View
  21. Papachristou GI, Wu T, Marsh W, Plevy SE (2004) Inflammatory pseudotumor of the liver associated with Crohn's disease. J Clin Gastroenterol 38: 818-822. View
  22. Patnana M, Sevrukov AB, Elsayes KM, Viswanathan C, Lubner M, et al. (2012) Inflammatory pseudotumor: the great mimicker. AJR Am J Roentgenol 198: W217-227. View
  23. Sasahira N, Kawabe T, Nakamura A, Shimura K, Shimura H, et al. (2005) Inflammatory pseudotumor of the liver and peripheral eosinophilia in autoimmune pancreatitis. World J Gastroenterol 11: 922-925. View
  24. Tai YS, Lin PW, Chen SG, Chang KC (1998) Inflammatory pseudotumor of the liver in a patient with human immunodeficiency virus infection. Hepatogastroenterology 45: 1760-1763. View
  25. Toda K, Yasuda I, Nishigaki Y, Enya M, Yamada T, et al. (2000) Inflammatory pseudotumor of the liver with primary sclerosing cholangitis. J Gastroenterol 35: 304-309. View
  26. Yoon KH, Ha HK, Lee JS, Suh JH, Kim MH, et al. (1999) Inflammatory pseudotumor of the liver in patients with recurrent pyogenic cholangitis: CT-histopathologic correlation. Radiology 211: 373-379. View
  27. Akatsu T, Wakabayashi G, Tanimoto A, Kameyama K, Kitajima M (2006) Inflammatory pseudotumor of the liver masquerading as hepatocellular carcinoma after a hepatitis B virus infection: Report of a case. Surg Today 36: 1028-1031. View
  28. Younis N, Khaleeli AA, Soran H, Monteith PG (2001) Inflammatory pseudotumour of the liver associated with diabetes mellitus. Int J Clin Pract 55: 717-719. View
  29. Isobe H, Nishi Y, Fukutomi T, Iwamoto H, Nakamuta M, et al. (1991) Inflammatory pseudotumor of the liver associated with acute myelomonocytic leukemia. Am J Gastroenterol 86: 238-240. View
  30. Matsuo Y, Sato M, Shibata T, Morimoto M, Tsuboi K, et al. (2014) Inflammatory pseudotumor of the liver diagnosed as metastatic liver tumor in a patient with a gastrointestinal stromal tumor of the rectum: report of a case. World J Surg Oncol 12: 140. View
  31. Cessna MH, Zhou H, Sanger WG, Perkins SL, Tripp S, et al. (2002) Expression of ALK1 and p80 in inflammatory myofibroblastic tumor and its mesenchymal mimics: a study of 135 cases. Mod Pathol 15: 931-938. View
  32. Chan JK, Cheuk W, Shimizu M (2001) Anaplastic lymphoma kinase expression in inflammatory pseudotumors. Am J Surg Pathol 25: 761-768. View
  33. Lawrence B, Perez-Atayde A, Hibbard MK, Rubin BP, Dal Cin P, et al. (2000) TPM3-ALK and TPM4-ALK oncogenes in inflammatory myofibroblastic tumors. Am J Pathol 157: 377-384. View
  34. Hakozaki Y, Katou M, Nakagawa K, Shirahama T, Matsumoto T (1993) Improvement of inflammatory pseudotumor of the liver after nonsteroidal anti-inflammatory agent therapy. Am J Gastroenterol 88: 1121-1122. View
  35. Jais P, Berger JF, Vissuzaine C, Paramelle O, Clays-Schouman E, et al. (1995) Regression of inflammatory pseudotumor of the liver under conservative therapy. Dig Dis Sci 40: 752-756. View
  36. Gollapudi P, Chejfec G, Zarling EJ (1992) Spontaneous regression of hepatic pseudotumor. Am J Gastroenterol 87: 214-217. View