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Inflammatory pseudotumor of the liver in children: Report of cases and review of the literature
Inflammatory Pseudotumor of the Liver in Children: Report of Cases and Review of the Literature By Yoshinobu Hata, Fumiaki Sasaki, Shinichi Matuoka, Hiromi Hamada, Kazunori Taguchi, Tohru Hasumi, Kunihiro Manabe, Junichi Uchino, Takayuki Nojima, Yasunori Hujioka, Kazuaki Inoue, and Yutaka Morita Sapporo, Japan @Two pediatric cases of inflammatory pseudotumor of liver were reported and compared with seven previously repotted cases. Clinical presentation was variable but often consisted of fever and vague abdominal symptoms. These two cases were considered to be clinically malignant because angiographically one showed hypervascularity and the other showed the portal vein draining into the lesion to be occluded. There are four previous reports of similar cases complicated with occlusive phlebitis within and around the lesion. All but two of the reported cases underwent major hepatectomy or liver transplantation. However, because of the benign nature of the lesion, the treatment might be more conservative after adequate diagnostic procedures, including needle biopsy, are applied. Copyright o 1992 by W.B. Saunders Company INDEX WORDS:
Liver, inflammatory
pseudotumor,
pediatric.
I
pseudotumor has been reNFLAMMATORY ported to occur in many organs, including the lung and orbit. However, only 31 cases have been reported in the liver since the first report of Pack and Baker in 1953.’ Among them, there were only seven cases in children. These benign lesions have been frequently misdiagnosed to be primary liver cancer. Because it is so rare, a detailed analysis of clinicopathological characteristics of these lesions has not been elicited. In this paper two pediatric cases of inflammatory pseudotumor of the liver are reported and compared with seven previously reported cases. Then, we attempted to clarify the clinicopathological characteristics of this lesion in children.
ing and interdigitating with hepatic parenchyma. was not conspicuous (Fig 2). He was completely years after surgery.
Case
Mitotic symptom
activity free I6
2
A h-year-old girl was admitted to this hospital with a Z-month history of slight fever and 2-week history of abdominal pain and vomiting. On admission her liver was enlarged and nontender. Laboratory data showed elevation of SGOT (101 KU), SGPT (72 KU), and alakalin phosphatase (122 KAU). Ultrasonography of the liver showed a complex, ill-defined, and hypoechogenic mass in the left lobe of the liver and dilated right intrahepatic bile ducts. Computed tomography scan showed a poorly marginated lowdensity mass in the left lobe of the liver that enhanced homogenously to the same degree as the surrounding parenchyma. Selective hepatic arteriograms showed stretching and irregularity of the branch arteries of the left hepatic artery in the arterial phase, faint parencymal opacification of the left lobe in the capillary phase and no visualisation of the left portal branch in the portal phase (Fig 3). At operation, there was a solid mass in the left lobe of the liver. and the lesion extended to the hepatic hilum. A left hepatic lobectomy. with resection of the extrahepatic bile duct including the gallbladder, and hepaticojejunostomy were carried out. A hard and solitary tumor-like lesion, 9.0 x 6.0 x 6.0 cm in size, was removed. The cut surfaces of the lesion were yellowish white and firm with an irregular margin. Microscopic examination showed a proliferation of densely hyalinized collagenous tissue infiltrated with a variety of cells. Throughout the fibrous stroma, there was an infiltration of cells among which mature plasma cells predominated. There was evidence of inflammation involving the vessel wall of the large- and medium-sized veins, with a gradual filling of the lumen with connective tissue rich in capillaries and inflammatory cells. Many of the veins showed complete obliteration of the lumen with total sclerosis (Fig 4). She remained symptom free 6 years after surgery.
CASE REPORTS DISCUSSION Case I A 7-year-old boy was admitted to this hospital with a l-week history of fever, abdominal pain, vomiting, and diarrhea. On physical examination the liver was enlarged, firm, and tender. The spleen was not palpable and lymphadenopathy was absent. Laboratory data showed a slight elevation of bilirubin (total bilirubin 2.3 mg/dL, direct bilirubin 1.1 mg/dL) without any elevation of hepatic enzymes. Selective hepatic arteriograms showed an hepatic mass located in the inferior lateral aspect of the liver with hypervascularity (Fig 1). At laparotomy, a solitary mass in the right lobe of the liver was noticed. A right hepatic lobectomy with cholecystectomy was performed. A hard and solitary tumor-like lesion, 4.0 x 4.0 x 3.5 cm in size, was removed. The cut surfaces of the lesion were yellowish white and firm. It appeared to be fairly well circumscribed, but not encapsulated. Microscopical examination of the resected specimen showed a proliferation of fibrous tissue replac-
Journal of Pediatric Surgery, Vol27, No 12 (December),
1992: pp 1549-l 552
Lesions classified as “inflammatory pseudotumor,” “postinflammatory” or “plasma cell granuloma” have been found in a variety of locations: the lung, orbit, parotid, pleura, stomach, ovary, and liver. These localized benign proliferations, composed predomi-
From the First Department of Surgery and the Depatiment of Pathology, Hokkaido University Hospital. and College of Medical Technology Hokkaido University, Sapporo, Japan. Address reprint requests to Yoshinobu Hata, MD, the First Department of Surgery, Hokkaido University School of Medicine, N-15 W-7. SapporQ, Japan. Copyright o 1992 by W.B. Saunders Company 0022-3468192/2712-0026$03.00/0
HATA ET AL
Fig 1. Selective hepatic arteriograms show a hepatic mass located in the inferior lateral aspect of the liver with hypervascularity ([A] arterial phase and [B] capillary phase).
Fig 2. (A) The cut surfaces of the lesion were yellowish white and firm. It appeared to be fairly well circumscribed, but not encapsulated. (B) Microscopic examination showed a proliferation of fibrous tissue replacing and interdigitating with hepetic parenchyma (H&E, original magnification, x200).
nantly of plasma cells and other elements supported by fibrous stroma, are of unknown etiology. At all sites, inflammatory pseudotumors clinically masquerade as malignant growths.2 Thirty-one cases of inflammatory pseudotumor of the liver have been reported in the literature, of which only 8 were female.3 Nine pediatric cases of inflammatory pseudotumor of the liver, including the present cases, have been collected.4-8 Six of the seven Table 1. Inflammatory Investigators Hertzer
et al4
Age
SC!X
lyr
F
female patients were pediatric cases, the youngest being 10 months old, which indicates a predominance of these cases for females (Table 1). Abdominal pain was the chief complaint in most cases. Four cases, including the present, had fever as the chief complaint. The lesion was solitary in all
Pseudotumor of the Liver-Summary
Presentation
Fever, jaundice, hepatospleno-
Site
Size
ML
?
of Pediatric Cases Phlebitis
?
Treatment
Biopsy
Outcome
Recovery
megaly Somere+
4yr
M
Fever, abdominal pain, vomiting
AP
8x6x6cm
+
Right lobectomy
Recovery
Daimaru et al5
9mo
F
Anemia, abdominal mass
L
lOx9x7cm
+
Left lobectomy
Recovery
?
Recovery
8yr
F
Jaundice, abdominal pain
APM
+
Transplantation
Kaneko et al7
10mo
F
Anemia, vomiting, abdominal mass
L
15xlZx12cm
?
Lateral segmentectomy
Recovery
Anthony et aIs
1Oyr
F
Abdominal pain, vomiting, diarrhea
AP
9cm
Right lobectomy
Recovery
Anthony et al*
12yr
M
Abdominal pain, night sweats
A
5cm
+ _
Partial resection
Recovery
Present case 1
7 yr
M
Fever, abdominal pain, vomiting,
A
4x4x3.5cm
_
Right lobectomy
Recovery
ML
9x6x6cm
+
Left lobectomy
Recovery
Heneghan et aI5
diarrhea Present case 2
6yr
F
Fever, abdominal pain, vomiting
Abbreviations: M, medial segment; L, lateral segment; A, anterior segment; P, posterior segment.
INFLAMMATORY
PSEUDOTUMOR
OF THE LIVER
cases. Its size varied from 3.5 to 15 cm. The right lobe of the liver was affected in five cases. Of clinical importance is the differential diagnosis of this lesion from hepatic malignancies. Most hepatoblastoma or hepatocellular carcinomas show hypervascularity by angiography and a few show a hypovascular or avascular nature. However, in these cases, one showed hypervascularity and the other showed hypovascularity. These cases were considered to be malignant, because one showed hypervascularity and the other showed the occlusion of the left branch of the portal vein angiographically. Thus, no specific angiographical findings exist. Histological appearances covered a wide range, but a plasma cell component was invariably present and may predominate in these cases. Five cases, including ours, demonstrated the findings of occlusive phlebitis within and around the lesion. These unusual changes were present in the medium and large veins8 In one of our cases, these inflammatory changes involved the
Fig 4. (A) The cut surfaces of the lesion were yellowish white and firm with an irregular margin. (B) Microscopic examination showed a proliferation of densely hyalinized collagenous tissue infiltrated with a variety of cells. There was evidence of inflammation involving the vessel wall of the large and medium-sized veins, with a gradual filling of the lumen with connective tissue rich in capillaries and inflammatory cells (Masson-trichrome, original magnification x 100).
hepatic hilum including the bifircation of the hepatic ducts. Of the total number of reported cases of pseudotumor, six patients underwent major hepatic resections and one was treated by liver transplantation. Two underwent biopsy or partial resection. All recovered after surgery. Thus, the treatment might be more conservative because of this benign nature, after adequate diagnostic procedures, including needle biopsy, are applied. REFERENCES
Fig 3. Selective hepatic arteriograms showed stretching and irregularity of the branch arteries of the left hepatic artery in (A) the arterial phase (arrow) and no visualization of the left portal branch in the (B) portal phase (arrow).
1. Pack GT, Baker HW: Total right hepatic lobectomy. Report of a case. Ann Surg 13853-58, 1953 2. Someren A: “Inflammatory pseudotumor” of liver with occlusive phlebitis. Report of a case in a child and review of the literature. Am J Clin Pathol69:176-181, 1978 3. Horiuchi R, Uchida T. Kajima T. et al: Inflammatory pseudotumor of the liver. Clinicopathologic study and review of the literature. Cancer 65:1583-1590. 1990
1552
4. Hertzer NR, Hawk WA, Hermann RE: Inflammatory lesions of the liver which simulate tumor. Report of two cases in children. Surgery 69:839-846,197l 5. Daimaru U, Tuneyoshi M, Enjoji M, et al: Inflammatory pseudotumor. Pediatr Oncol19:241-242,1983 6. Heneghan MA, Kaplan CG, Priebe Cl, et al: Inflammatory
HATA ET AL
pseudotumor of the liver. A rare cause of obstructive jaundice and portal hypertention in a child. Pediatr Radio1 14:433-435, 1984 7. Kaneko T, Mitarai Y, Wakasugi K, et al: A case of inflammatory pseudotumor of liver. J Jpn Sot Pediatr Surg 20:875-879,1984 8. Anthony PP, Telesinghe PU: Inflammatory pseudotumor of the liver. J Clin Pathol39:761-768, 1986
Liver, inflammatory
pseudotumor,
pediatric.
I
pseudotumor has been reNFLAMMATORY ported to occur in many organs, including the lung and orbit. However, only 31 cases have been reported in the liver since the first report of Pack and Baker in 1953.’ Among them, there were only seven cases in children. These benign lesions have been frequently misdiagnosed to be primary liver cancer. Because it is so rare, a detailed analysis of clinicopathological characteristics of these lesions has not been elicited. In this paper two pediatric cases of inflammatory pseudotumor of the liver are reported and compared with seven previously reported cases. Then, we attempted to clarify the clinicopathological characteristics of this lesion in children.
ing and interdigitating with hepatic parenchyma. was not conspicuous (Fig 2). He was completely years after surgery.
Case
Mitotic symptom
activity free I6
2
A h-year-old girl was admitted to this hospital with a Z-month history of slight fever and 2-week history of abdominal pain and vomiting. On admission her liver was enlarged and nontender. Laboratory data showed elevation of SGOT (101 KU), SGPT (72 KU), and alakalin phosphatase (122 KAU). Ultrasonography of the liver showed a complex, ill-defined, and hypoechogenic mass in the left lobe of the liver and dilated right intrahepatic bile ducts. Computed tomography scan showed a poorly marginated lowdensity mass in the left lobe of the liver that enhanced homogenously to the same degree as the surrounding parenchyma. Selective hepatic arteriograms showed stretching and irregularity of the branch arteries of the left hepatic artery in the arterial phase, faint parencymal opacification of the left lobe in the capillary phase and no visualisation of the left portal branch in the portal phase (Fig 3). At operation, there was a solid mass in the left lobe of the liver. and the lesion extended to the hepatic hilum. A left hepatic lobectomy. with resection of the extrahepatic bile duct including the gallbladder, and hepaticojejunostomy were carried out. A hard and solitary tumor-like lesion, 9.0 x 6.0 x 6.0 cm in size, was removed. The cut surfaces of the lesion were yellowish white and firm with an irregular margin. Microscopic examination showed a proliferation of densely hyalinized collagenous tissue infiltrated with a variety of cells. Throughout the fibrous stroma, there was an infiltration of cells among which mature plasma cells predominated. There was evidence of inflammation involving the vessel wall of the large- and medium-sized veins, with a gradual filling of the lumen with connective tissue rich in capillaries and inflammatory cells. Many of the veins showed complete obliteration of the lumen with total sclerosis (Fig 4). She remained symptom free 6 years after surgery.
CASE REPORTS DISCUSSION Case I A 7-year-old boy was admitted to this hospital with a l-week history of fever, abdominal pain, vomiting, and diarrhea. On physical examination the liver was enlarged, firm, and tender. The spleen was not palpable and lymphadenopathy was absent. Laboratory data showed a slight elevation of bilirubin (total bilirubin 2.3 mg/dL, direct bilirubin 1.1 mg/dL) without any elevation of hepatic enzymes. Selective hepatic arteriograms showed an hepatic mass located in the inferior lateral aspect of the liver with hypervascularity (Fig 1). At laparotomy, a solitary mass in the right lobe of the liver was noticed. A right hepatic lobectomy with cholecystectomy was performed. A hard and solitary tumor-like lesion, 4.0 x 4.0 x 3.5 cm in size, was removed. The cut surfaces of the lesion were yellowish white and firm. It appeared to be fairly well circumscribed, but not encapsulated. Microscopical examination of the resected specimen showed a proliferation of fibrous tissue replac-
Journal of Pediatric Surgery, Vol27, No 12 (December),
1992: pp 1549-l 552
Lesions classified as “inflammatory pseudotumor,” “postinflammatory” or “plasma cell granuloma” have been found in a variety of locations: the lung, orbit, parotid, pleura, stomach, ovary, and liver. These localized benign proliferations, composed predomi-
From the First Department of Surgery and the Depatiment of Pathology, Hokkaido University Hospital. and College of Medical Technology Hokkaido University, Sapporo, Japan. Address reprint requests to Yoshinobu Hata, MD, the First Department of Surgery, Hokkaido University School of Medicine, N-15 W-7. SapporQ, Japan. Copyright o 1992 by W.B. Saunders Company 0022-3468192/2712-0026$03.00/0
HATA ET AL
Fig 1. Selective hepatic arteriograms show a hepatic mass located in the inferior lateral aspect of the liver with hypervascularity ([A] arterial phase and [B] capillary phase).
Fig 2. (A) The cut surfaces of the lesion were yellowish white and firm. It appeared to be fairly well circumscribed, but not encapsulated. (B) Microscopic examination showed a proliferation of fibrous tissue replacing and interdigitating with hepetic parenchyma (H&E, original magnification, x200).
nantly of plasma cells and other elements supported by fibrous stroma, are of unknown etiology. At all sites, inflammatory pseudotumors clinically masquerade as malignant growths.2 Thirty-one cases of inflammatory pseudotumor of the liver have been reported in the literature, of which only 8 were female.3 Nine pediatric cases of inflammatory pseudotumor of the liver, including the present cases, have been collected.4-8 Six of the seven Table 1. Inflammatory Investigators Hertzer
et al4
Age
SC!X
lyr
F
female patients were pediatric cases, the youngest being 10 months old, which indicates a predominance of these cases for females (Table 1). Abdominal pain was the chief complaint in most cases. Four cases, including the present, had fever as the chief complaint. The lesion was solitary in all
Pseudotumor of the Liver-Summary
Presentation
Fever, jaundice, hepatospleno-
Site
Size
ML
?
of Pediatric Cases Phlebitis
?
Treatment
Biopsy
Outcome
Recovery
megaly Somere+
4yr
M
Fever, abdominal pain, vomiting
AP
8x6x6cm
+
Right lobectomy
Recovery
Daimaru et al5
9mo
F
Anemia, abdominal mass
L
lOx9x7cm
+
Left lobectomy
Recovery
?
Recovery
8yr
F
Jaundice, abdominal pain
APM
+
Transplantation
Kaneko et al7
10mo
F
Anemia, vomiting, abdominal mass
L
15xlZx12cm
?
Lateral segmentectomy
Recovery
Anthony et aIs
1Oyr
F
Abdominal pain, vomiting, diarrhea
AP
9cm
Right lobectomy
Recovery
Anthony et al*
12yr
M
Abdominal pain, night sweats
A
5cm
+ _
Partial resection
Recovery
Present case 1
7 yr
M
Fever, abdominal pain, vomiting,
A
4x4x3.5cm
_
Right lobectomy
Recovery
ML
9x6x6cm
+
Left lobectomy
Recovery
Heneghan et aI5
diarrhea Present case 2
6yr
F
Fever, abdominal pain, vomiting
Abbreviations: M, medial segment; L, lateral segment; A, anterior segment; P, posterior segment.
INFLAMMATORY
PSEUDOTUMOR
OF THE LIVER
cases. Its size varied from 3.5 to 15 cm. The right lobe of the liver was affected in five cases. Of clinical importance is the differential diagnosis of this lesion from hepatic malignancies. Most hepatoblastoma or hepatocellular carcinomas show hypervascularity by angiography and a few show a hypovascular or avascular nature. However, in these cases, one showed hypervascularity and the other showed hypovascularity. These cases were considered to be malignant, because one showed hypervascularity and the other showed the occlusion of the left branch of the portal vein angiographically. Thus, no specific angiographical findings exist. Histological appearances covered a wide range, but a plasma cell component was invariably present and may predominate in these cases. Five cases, including ours, demonstrated the findings of occlusive phlebitis within and around the lesion. These unusual changes were present in the medium and large veins8 In one of our cases, these inflammatory changes involved the
Fig 4. (A) The cut surfaces of the lesion were yellowish white and firm with an irregular margin. (B) Microscopic examination showed a proliferation of densely hyalinized collagenous tissue infiltrated with a variety of cells. There was evidence of inflammation involving the vessel wall of the large and medium-sized veins, with a gradual filling of the lumen with connective tissue rich in capillaries and inflammatory cells (Masson-trichrome, original magnification x 100).
hepatic hilum including the bifircation of the hepatic ducts. Of the total number of reported cases of pseudotumor, six patients underwent major hepatic resections and one was treated by liver transplantation. Two underwent biopsy or partial resection. All recovered after surgery. Thus, the treatment might be more conservative because of this benign nature, after adequate diagnostic procedures, including needle biopsy, are applied. REFERENCES
Fig 3. Selective hepatic arteriograms showed stretching and irregularity of the branch arteries of the left hepatic artery in (A) the arterial phase (arrow) and no visualization of the left portal branch in the (B) portal phase (arrow).
1. Pack GT, Baker HW: Total right hepatic lobectomy. Report of a case. Ann Surg 13853-58, 1953 2. Someren A: “Inflammatory pseudotumor” of liver with occlusive phlebitis. Report of a case in a child and review of the literature. Am J Clin Pathol69:176-181, 1978 3. Horiuchi R, Uchida T. Kajima T. et al: Inflammatory pseudotumor of the liver. Clinicopathologic study and review of the literature. Cancer 65:1583-1590. 1990
1552
4. Hertzer NR, Hawk WA, Hermann RE: Inflammatory lesions of the liver which simulate tumor. Report of two cases in children. Surgery 69:839-846,197l 5. Daimaru U, Tuneyoshi M, Enjoji M, et al: Inflammatory pseudotumor. Pediatr Oncol19:241-242,1983 6. Heneghan MA, Kaplan CG, Priebe Cl, et al: Inflammatory
HATA ET AL
pseudotumor of the liver. A rare cause of obstructive jaundice and portal hypertention in a child. Pediatr Radio1 14:433-435, 1984 7. Kaneko T, Mitarai Y, Wakasugi K, et al: A case of inflammatory pseudotumor of liver. J Jpn Sot Pediatr Surg 20:875-879,1984 8. Anthony PP, Telesinghe PU: Inflammatory pseudotumor of the liver. J Clin Pathol39:761-768, 1986