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Inflammatory pseudotumor of the liver
CLINICAL IMAGING 1994;18:283-285
283
INFLAMMATORY PSEUDOTUMOR OF THE LIVER IVAN NOI, MD, NORMAN LOBERANT, AND ISAAC COHEN, MD
We present a case of inflammatory pseudotumor of the liver (IPL), a rare benign hepatic lesion. A 66-year-old woman with fever, and right abdominal pain and mass underwent abdominal sonography and computed tomography. Both showed multiple hepatic masses. Ultmsound guided needle liver biopsy showed a dense chronic inflammatory lesion with innumerable foamy histiocytes and spindle fibroblasts consistent with IPL. She improved with conservative treatment. Clinical, pathologic, and imaging findings at IPL are reviewed. KEY WORDS:
Benign liver mass; Benign hepatic mass INTRODUCI’ION Inflammatory pseudotumor of the liver (IPL) is a rare condition, first described by Pack and Baker in 1953 (1). Neither the etiology nor the pathophysiology of the condition have been fully elucidated. The clinical presentation includes epigastric pain, anemia, fever, weight loss, and palpable liver mass. Useful imaging modalities include sonography, computed tomography, nuclear medicine scan, and angiography. The initial presumptive diagnosis with all these modalities is invariably liver tumor. The diagnosis is generally established either by open or percutaneous liver biopsy.
MD,
nal pain. Physical examination revealed a palpable mass, presumed to be hepatic in origin. There was no jaundice. There was edema of both legs. Laboratory examinations showed an erythrocyte sedimentation rate of 120 mm/hr (markedly elevated), and alkaline phosphatase level of 446 IU (normal < 180 IU; 208 of hepatic origin, 238 of osseous origin). Results of the remaining liver function studies were normal. There was mild anemia; the white blood cell count was 17,200/mm3 with 84% neutrophils. Blood and urine cultures grew no organisms. A chest radiograph appeared normal. Abdominal sonography showed an enlarged liver with multiple round hypoechoic foci in both lobes (Figure 1). There was no dilatation of the biliary tree, and no abnormality in the area of the porta hepatis. Doppler sonography was not available. Barium enema and gastroscopy demonstrated normal findings. Computed tomography of the abdomen revealed multiple low-density foci scattered in the liver (Figure 2), with no other abnormality. Needle biopsy of the liver was performed. Results
CASE REPORT A 66-year-old woman, previously in good health, was admitted with fever of 39% and right upper abdomi-
From the Departments of Radiology (I.N., N.L.) and Pathology (I.C.), Western Galilee Regional Hospital, Nahariya, Israel. Address reprint requests to: Normal Loberant, MD, Department of Radiology, Western Galilee Regional Hospital, Nahariya, Israel. Received July 27, 1993; revised November 1, 1993. 0 1994 ElsevierScienceInc. 655 Avenue of the Americas, 0899-7CVl/94/$7.00
New York, NY 10010
FIGURE 1. Oblique sonogmmshows adjacent, round, inhomogeneous lesions in the right lobe of the liver.
284
NOI ET AL.
CLINICAL IMAGING ML.
18, NO. 4
FIGURE 2. Axiel contrast-enhanced computed tomography shows three hypodense lesions in the liver. The posterior lesions correspond to those in Figure 1.
of studies for microorganisms, including bacteria, ameba, and parasites, were negative. Histology revealed a xanthogranulomatous lesion exhibiting a dense chronic inflammatory infiltrate with plasma cells and lymphocytes, in addition to innumerable foamy histiocytes and spindle fibroblasts (Figure 3). Normal hepatocytes surrounded this exuberant cellular process. These features are consistent with the cellular type of IPL. The patient was treated conservatively with a combination of cephazolin, aztreonam (Azactam), chloroquine, and metronidazole, the former two providing broad antibacterial coverage and the latter two antiamebic coverage. She responded well clinically, and was afebrile after 2 weeks. Repeat abdominal sonography revealed significant regression of the hepatic lesions.
B FIGURE 3. Liver needle biopsy specimens. (A)Low-power micrograph shows the dense cellular process with surrounding normel hepatocytes. (B) High-power magnification shows that the majority of the inflammatory cells are histiocytes with foamy cytoplasm.
DISCUSSION
Approximately 40 cases of IPL have been described in the medical literature (2-13). Patients have ranged in age from 1 to83 years. There is a male preponderance. Presenting clinical signs and symptoms include fever, weight loss, anemia, abdominal pain, and a palpable abdominal mass. Laboratory evidence generally supports infectious disease (1). Some of the patients had visited tropical or subtropical countries (including India and Southeast Asia) prior to the onset of illness (g), suggesting an infectious etiology; however, serological and bacteriological studies have not revealed the etiology to date. Imaging studies reveal either single or multiple lesions. The differential diagnosis is extensive, includ-
ing primary or metastatic carcinoma, and various benign focal lesions including complex cysts, abscess, hematoma, adenoma, degenerated hemangioma, focal nodular hyperplasia, and lipoma (14). The majority of patients with IPL have undergone surgical resection of lesions. The prognosis is generally good, although illness-related deaths have been recorded (9). Biliary obstruction has occasionally been associated with IPL (2,11). Spontaneous regression of lesions has been observed (10). Sonography of the liver generally reveals a mass lesion. Less frequent findings include multiple nodules, thickening of portal vein walls, and vessels coursing through the lesion. Computed tomography character-
OCTOBER-DECEMBER
1994
istically shows low-density lesions that do not enhance with intravenous contrast material. Liver scintigrams using technetium consistently show photopenic hepatic lesions. In their review of nine patients with IPL, Horiuchi et al. (9) divided IPL into two predominant histological groups: (1) xanthogranuloma (cellular type), and (2) hyalinized sclerosing. Two other cases were characterized as primarily plasma cell granuloma and as inflamed granulation tissue. Foamy histiocytes and evidence of fibrosis were the histological hallmarks, being present in all patients; plasma cells were present in all but one, and obliterating phlebitis was seen in five of the nine. The benign nature of the process is suggested by the following clinical, imaging, and histological features: (1) thepersistence of hepatic lesions in a patient whose general condition remains satisfactory; (2) hepatic findings in a patient who does not have cirrhosis; (3) normal alpha-fetoprotein levels in the presence of a hepatic mass; (4) obliterating phlebitis of major portal veins with thickening of their walls; and (5) portal vein with thickened walls coursing through the lesion, a finding inconsistent with hepatocellular carcinoma though occasionally seen in lymphoma. Computed tomography and abdominal sonography are used routinely in the evaluation of patients with abdominal complaints, and focal hepatic mass lesions are discovered with regularity. Primary and metastatic lesions are common, but the diagnosis of IPL, though rare, should be included in the differential diagnosis in appropriate clinical circumstances. Given the benign nature of the condition, every effort must be made for diagnosis before a patient is subjected to surgery.
INFLAMMATORY
PSEUDOTUMOR
OF THE LIVER
285
REFERENCES 1. Pack GT, Baker HW. Total right hepatic lobectomy: report of a case. Ann Surg 1953;138:253-258. 2. Haith EE, Kepes JJ, Holder TM. The inflammatory pseudotumor involving the common bile duct of a six-year old boy: successful pancreatico-duodenectomy. Surgery 1964;56:436-441. 3. Hertzer NR, Hawk WA, Hermann RE. Inflammatory lesions of the liver which simulate tumor: report of two cases in children, Surgery 1971;69:839-846. 4. Someren A. “Inflammatory pseudotumor” of liver with occlusive phlebitis. Report of a case in a child and review of the literature. Am J Clin Path01 1978:69:176-181. 5. Chen KTK. Inflammatory pseudotumor of the liver. Hum Path01 1984;15:694-696. 6. Heneghan MA, Kaplan CG, Priebe CJ, Partin J, Partin JS. Inflammatory pseudotumor of the liver: a rare case of obstructive jaundice and portal hypertension in a child. Pediatr Radio1 1984;14:433-435. z
Anthony PP, Telesinghe PU. Inflammatory pseudotumor liver. J Clin Path01 1986;39:761-768.
of the
8. Kessler E, Turani H, Kayser S, Bar-Ziv J, Chaimoff CH. Inflammatory pseudotumor of the liver. Liver 1988;8:17-23. 9. Horiuchi R, Uchida T, Kojima T, Shikata T. Inflammatory pseudotumor of the liver: clinicopathologic study and review of the literature. Cancer 1990;65:1583-1590. 10. Gollapudi P, Chejfec G, Zarling EJ. Spontaneous regression hepatic pseudotumor. Am J Gastroenterol 1992;87:214-217.
of
11. Pokorny CS, Painter DM, Waugh RC, McCaughan GW, Gallagher ND, Tattersall MI-I. Inflammatory pseudotumor of the liver causing biliary obstruction: treatment by biliary stenting with 5-year follow-up. J Clin Gastroenterol 1991;13:338-341. 12
Andreola S, Cattoretti G, Lombardi L, Audisio RA, Regalia E, Bosch JP, Mazzaferro V, Doci R. Inflammatory pseudotumor of the liver: report of two cases. Tumori 1990;76:517-523.
13. Lopez JI, Eizaguirre B, Nevada M, Ruiz-Jaureguizuria JC. Inflammatory pseudotumor of the liver: report of a case and literature review. APMIS 1990;98:1022-1026. 14. Vassiliades VG. Focal and diffuse benign hepatic disease: correlative imaging. Semin Ultrasound CT MR 1992;13:313-335.
283
INFLAMMATORY PSEUDOTUMOR OF THE LIVER IVAN NOI, MD, NORMAN LOBERANT, AND ISAAC COHEN, MD
We present a case of inflammatory pseudotumor of the liver (IPL), a rare benign hepatic lesion. A 66-year-old woman with fever, and right abdominal pain and mass underwent abdominal sonography and computed tomography. Both showed multiple hepatic masses. Ultmsound guided needle liver biopsy showed a dense chronic inflammatory lesion with innumerable foamy histiocytes and spindle fibroblasts consistent with IPL. She improved with conservative treatment. Clinical, pathologic, and imaging findings at IPL are reviewed. KEY WORDS:
Benign liver mass; Benign hepatic mass INTRODUCI’ION Inflammatory pseudotumor of the liver (IPL) is a rare condition, first described by Pack and Baker in 1953 (1). Neither the etiology nor the pathophysiology of the condition have been fully elucidated. The clinical presentation includes epigastric pain, anemia, fever, weight loss, and palpable liver mass. Useful imaging modalities include sonography, computed tomography, nuclear medicine scan, and angiography. The initial presumptive diagnosis with all these modalities is invariably liver tumor. The diagnosis is generally established either by open or percutaneous liver biopsy.
MD,
nal pain. Physical examination revealed a palpable mass, presumed to be hepatic in origin. There was no jaundice. There was edema of both legs. Laboratory examinations showed an erythrocyte sedimentation rate of 120 mm/hr (markedly elevated), and alkaline phosphatase level of 446 IU (normal < 180 IU; 208 of hepatic origin, 238 of osseous origin). Results of the remaining liver function studies were normal. There was mild anemia; the white blood cell count was 17,200/mm3 with 84% neutrophils. Blood and urine cultures grew no organisms. A chest radiograph appeared normal. Abdominal sonography showed an enlarged liver with multiple round hypoechoic foci in both lobes (Figure 1). There was no dilatation of the biliary tree, and no abnormality in the area of the porta hepatis. Doppler sonography was not available. Barium enema and gastroscopy demonstrated normal findings. Computed tomography of the abdomen revealed multiple low-density foci scattered in the liver (Figure 2), with no other abnormality. Needle biopsy of the liver was performed. Results
CASE REPORT A 66-year-old woman, previously in good health, was admitted with fever of 39% and right upper abdomi-
From the Departments of Radiology (I.N., N.L.) and Pathology (I.C.), Western Galilee Regional Hospital, Nahariya, Israel. Address reprint requests to: Normal Loberant, MD, Department of Radiology, Western Galilee Regional Hospital, Nahariya, Israel. Received July 27, 1993; revised November 1, 1993. 0 1994 ElsevierScienceInc. 655 Avenue of the Americas, 0899-7CVl/94/$7.00
New York, NY 10010
FIGURE 1. Oblique sonogmmshows adjacent, round, inhomogeneous lesions in the right lobe of the liver.
284
NOI ET AL.
CLINICAL IMAGING ML.
18, NO. 4
FIGURE 2. Axiel contrast-enhanced computed tomography shows three hypodense lesions in the liver. The posterior lesions correspond to those in Figure 1.
of studies for microorganisms, including bacteria, ameba, and parasites, were negative. Histology revealed a xanthogranulomatous lesion exhibiting a dense chronic inflammatory infiltrate with plasma cells and lymphocytes, in addition to innumerable foamy histiocytes and spindle fibroblasts (Figure 3). Normal hepatocytes surrounded this exuberant cellular process. These features are consistent with the cellular type of IPL. The patient was treated conservatively with a combination of cephazolin, aztreonam (Azactam), chloroquine, and metronidazole, the former two providing broad antibacterial coverage and the latter two antiamebic coverage. She responded well clinically, and was afebrile after 2 weeks. Repeat abdominal sonography revealed significant regression of the hepatic lesions.
B FIGURE 3. Liver needle biopsy specimens. (A)Low-power micrograph shows the dense cellular process with surrounding normel hepatocytes. (B) High-power magnification shows that the majority of the inflammatory cells are histiocytes with foamy cytoplasm.
DISCUSSION
Approximately 40 cases of IPL have been described in the medical literature (2-13). Patients have ranged in age from 1 to83 years. There is a male preponderance. Presenting clinical signs and symptoms include fever, weight loss, anemia, abdominal pain, and a palpable abdominal mass. Laboratory evidence generally supports infectious disease (1). Some of the patients had visited tropical or subtropical countries (including India and Southeast Asia) prior to the onset of illness (g), suggesting an infectious etiology; however, serological and bacteriological studies have not revealed the etiology to date. Imaging studies reveal either single or multiple lesions. The differential diagnosis is extensive, includ-
ing primary or metastatic carcinoma, and various benign focal lesions including complex cysts, abscess, hematoma, adenoma, degenerated hemangioma, focal nodular hyperplasia, and lipoma (14). The majority of patients with IPL have undergone surgical resection of lesions. The prognosis is generally good, although illness-related deaths have been recorded (9). Biliary obstruction has occasionally been associated with IPL (2,11). Spontaneous regression of lesions has been observed (10). Sonography of the liver generally reveals a mass lesion. Less frequent findings include multiple nodules, thickening of portal vein walls, and vessels coursing through the lesion. Computed tomography character-
OCTOBER-DECEMBER
1994
istically shows low-density lesions that do not enhance with intravenous contrast material. Liver scintigrams using technetium consistently show photopenic hepatic lesions. In their review of nine patients with IPL, Horiuchi et al. (9) divided IPL into two predominant histological groups: (1) xanthogranuloma (cellular type), and (2) hyalinized sclerosing. Two other cases were characterized as primarily plasma cell granuloma and as inflamed granulation tissue. Foamy histiocytes and evidence of fibrosis were the histological hallmarks, being present in all patients; plasma cells were present in all but one, and obliterating phlebitis was seen in five of the nine. The benign nature of the process is suggested by the following clinical, imaging, and histological features: (1) thepersistence of hepatic lesions in a patient whose general condition remains satisfactory; (2) hepatic findings in a patient who does not have cirrhosis; (3) normal alpha-fetoprotein levels in the presence of a hepatic mass; (4) obliterating phlebitis of major portal veins with thickening of their walls; and (5) portal vein with thickened walls coursing through the lesion, a finding inconsistent with hepatocellular carcinoma though occasionally seen in lymphoma. Computed tomography and abdominal sonography are used routinely in the evaluation of patients with abdominal complaints, and focal hepatic mass lesions are discovered with regularity. Primary and metastatic lesions are common, but the diagnosis of IPL, though rare, should be included in the differential diagnosis in appropriate clinical circumstances. Given the benign nature of the condition, every effort must be made for diagnosis before a patient is subjected to surgery.
INFLAMMATORY
PSEUDOTUMOR
OF THE LIVER
285
REFERENCES 1. Pack GT, Baker HW. Total right hepatic lobectomy: report of a case. Ann Surg 1953;138:253-258. 2. Haith EE, Kepes JJ, Holder TM. The inflammatory pseudotumor involving the common bile duct of a six-year old boy: successful pancreatico-duodenectomy. Surgery 1964;56:436-441. 3. Hertzer NR, Hawk WA, Hermann RE. Inflammatory lesions of the liver which simulate tumor: report of two cases in children, Surgery 1971;69:839-846. 4. Someren A. “Inflammatory pseudotumor” of liver with occlusive phlebitis. Report of a case in a child and review of the literature. Am J Clin Path01 1978:69:176-181. 5. Chen KTK. Inflammatory pseudotumor of the liver. Hum Path01 1984;15:694-696. 6. Heneghan MA, Kaplan CG, Priebe CJ, Partin J, Partin JS. Inflammatory pseudotumor of the liver: a rare case of obstructive jaundice and portal hypertension in a child. Pediatr Radio1 1984;14:433-435. z
Anthony PP, Telesinghe PU. Inflammatory pseudotumor liver. J Clin Path01 1986;39:761-768.
of the
8. Kessler E, Turani H, Kayser S, Bar-Ziv J, Chaimoff CH. Inflammatory pseudotumor of the liver. Liver 1988;8:17-23. 9. Horiuchi R, Uchida T, Kojima T, Shikata T. Inflammatory pseudotumor of the liver: clinicopathologic study and review of the literature. Cancer 1990;65:1583-1590. 10. Gollapudi P, Chejfec G, Zarling EJ. Spontaneous regression hepatic pseudotumor. Am J Gastroenterol 1992;87:214-217.
of
11. Pokorny CS, Painter DM, Waugh RC, McCaughan GW, Gallagher ND, Tattersall MI-I. Inflammatory pseudotumor of the liver causing biliary obstruction: treatment by biliary stenting with 5-year follow-up. J Clin Gastroenterol 1991;13:338-341. 12
Andreola S, Cattoretti G, Lombardi L, Audisio RA, Regalia E, Bosch JP, Mazzaferro V, Doci R. Inflammatory pseudotumor of the liver: report of two cases. Tumori 1990;76:517-523.
13. Lopez JI, Eizaguirre B, Nevada M, Ruiz-Jaureguizuria JC. Inflammatory pseudotumor of the liver: report of a case and literature review. APMIS 1990;98:1022-1026. 14. Vassiliades VG. Focal and diffuse benign hepatic disease: correlative imaging. Semin Ultrasound CT MR 1992;13:313-335.