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Teratoma arising from anomalous common bile ducts: a case report
Teratoma Arising From Anomalous Common Bile Ducts: A Case Report By Mehmet Demircan, Sema Uguralp, Murat Mutus, Ramazan Kutlu, and Bulent Mızrak Malatya, Turkey
Teratoma arising from extrahepatic common ducts is very rare entity. The authors found 2 teratoma cases originating from common bile duct in the literature. As a third case, the authors report on a 4-month-old girl with benign cystic teratoma arising from distal common hepatic bile duct and with
anomalous common bile ducts. Surgical management of the patient also is discussed. J Pediatr Surg 39:E1-E2. © 2004 Elsevier Inc. All rights reserved. INDEX WORDS: Bile duct tumor, teratoma of bile duct.
T
were found: hemoglobin, 9.5 g/dL (normal range, 10.6 to 14.5 g/dL); alkaline phosphatase, 1390 U/L (normal range, 64 to 300 U/L); alanine aminotransferase (ALT) 964 U/L (normal range, 5 to 30 U/L); gamma glutamyl transpeptidase, 346 U/L (normal range, 7 to 49 U/L); indirect bilirubin, 1.0 mg/dL (normal range, 0.1 to 0.8 mg/dL); direct bilirubin, 3.2 mg/d (normal range, 0.1 to 0.5 mg/dL). Alpha-fetoprotein level was normal with respect to the patient’s age (77.8 ng/mL), and carcinoembryonic antigen level was also normal (1.72 ng/mL). Although CA 125 level was elevated (27 U/mL), CA 19.9 and CA 15.3 levels were within normal ranges. A plain film of the abdomen showed a large upper abdominal cystic mass compressing and displacing the stomach and intestine to the left. Sonography showed a cystic mass including a little solid component on the right side that had compressed left lobe of the liver and extended to pelvis. The right kidney was displaced to the lateral by the mass, but no hydronephrosis was observed. Contrast-enhanced axial computerized tomography scan showed that cystic lesions with smooth edges and different densities occupied the whole right abdomen and passed the midline to the left abdomen (Fig 1). At laparotomy, the huge cystic mass (15 cm in diameter) with solid components passed the midline and extended to the pelvis. The cystic mass originated from a point just distal to the common hepatic bile duct where it was occluded by tumor. Gallbladder and choledocal duct were under pressure by the tumor. Abnormally, choledocal duct was leaving from the apex of the gallbladder and was thin and elongated (2 mm in diameter and 15 cm in length), and reach duodenum passing over the mass. Operative cholangiogram showed dilated intrahepatic biliary tract, and no passage of the contrast media into the duodenum was detected. The mass was excised totally together with distal part of common hepatic duct. Choledocal duct was ligated at just the point to enter the duodenum and excised totally. A decision was made for an end-to-side anastomosis between the proximal part of the common hepatic duct and fundus of the gallbladder. The apex of the gallbladder was tubularized for 2 cm in length and then anastomozed to the antimesenteric side of the jejunum 10 cm from the ligament of Treitz (Fig 2).
HE MOST common causes of obstructive jaundice in early childhood are choledocal cyst and rhabdomyosarcoma.1,2 The teratoma of the common bile ducts is a very rare phenomenon. To our knowledge, there have been 2 cases of teratoma of the common bile duct in the English-language literature.1,2 In this case report, a child with teratoma arising from common hepatic bile duct and with anomalous common bile ducts is presented, and its surgical management is discussed. CASE REPORT A 4-month-old girl was admitted for progressive jaundice and asymptomatic progressive abdominal distension lasting for a month. His past medical history was unremarkable. The child was observed to be icteric on physical examination. Abdominal examination found a prominent distention and a huge, well-defined mass that was nonfixed with a smooth surface at the right upper and lower quadrants and was passing over to the other side of the midline. The child otherwise appeared well. On laboratory investigation, the following abnormalities
Fig 1.
Appearance of the tumor on computerized tomography.
Journal of Pediatric Surgery, Vol 39, No 4 (April), 2004: E1-E2
From the Departments of Pediatric Surgery, Radiology, and Pathology, Inonu University Faculty of Medicine, Malatya, Turkey. ¨ zal Tıp Address reprint requests to Dr Mehmet Demircan, T. O Merkezi, C¸ocuk Cerrahisi Anabilim Dalı, 44069 Malatya, Tu¨rkiye (Turkey). © 2004 Elsevier Inc. All rights reserved. 1531-5037/04/3904-0035$30.00/0 doi:10.1016/j.jpedsurg.2003.12.036 e1
e2
DEMIRCAN ET AL
Fig 2. Schematic diagram of the teratoma and bile ducts (A) and final appearance of the bile ducts after surgical procedure (B).
Macroscopic and histopathologic examination of the specimen found multilocular cystic mass (size of 11 ⫻ 10 ⫻ 6 cm) and solid component consisting of squamous epithelium, skin adnexae, mature glial tissue, and fat. Immature neuronal tissue or malignant tissue was not detected. At 4 years follow-up, the course was uneventful, and the patient did not experience any episodes of cholangitis.
DISCUSSION
Teratomas are neoplasms containing elements derived from the 3 primary germ layers, and the most common sites for teratoma are sacrococcygeal, mediastinal, and retroperitoneal regions and gonads. Teratomas rarely occur in abdominal organs such as the liver.3-6 Teratoma of the common bile ducts is an extremely rare entity. We found only 2 case reports of teratoma arising from the common bile duct in the English-language literature.1,2 There was no morphologic and radiologic description in first case report.1 In the second case report, investigators have emphasized the radiologic description and differential diagnosis of the teratoma of common bile duct.2 Interestingly, our patient also had anatomic abnormalities of the extrahepatic biliary ducts. These abnormalities were described as follows: common hepatic duct entering directly into the gallbladder, absence of a cystic duct, and a very thin and elongated choledocal duct exiting from the apex of the gallbladder. These abnormalities may have occurred if the teratoma began growth
during the embryogenesis of the biliary tract, or the teratoma may have developed from abnormal structures of the common biliary tracts. The majority of teratomas in children are benign. Excision of the tumor is the treatment of choice for benign teratomas and is associated with a good prognosis. Usually, surgeons prefer to use Roux-en-Y hepaticoportojejunostomy to correct surgical disorders of the porta hepatis area. But the most common complication of this procedure is cholangitis. In our patient, we preferred to use the gallbladder as a bile port. We first performed an end-to-side anastomosis between the hepatic common duct and gallbladder (ductocystostomy). Then the apex of the gallbladder was tubularized for 2 cm in length, and an end-to-side anastomosis between tubularized gallbladder and jejunum (cystojejunostomy) was performed. Thus, 2 anastomoses were performed on the gallbladder. Follow-up over 4 years showed that the patient did not have any episodes of cholangitis. The radiologic and biochemical test of the patient’s liver functions are within normal ranges. As reported in the literature, a Whipple operation had been performed in one of the cases because of the clinical impression that the tumor was a malignancy of the common bile duct, such as rhabdomyosarcoma.1 We think that this approach is quick radical for benign pathology such as teratoma.
REFERENCES 1. Frexes M, Neblett WW 3rd, Holcomb GW Jr: Spectrum of biliary disease in childhood. South Med J 79:1342-1349, 1986 2. Kim WS, Choi BI, Lee YS, et al: Endodermal sinus tumour associated with benign teratoma of the common bile duct. Pediatr Radiol 23:59-60, 1993 3. Witte DP, Kissane JM, Askin FB: Hepatic teratomas in children. Pediatr Pathol 1:81-92, 1983
4. Alam K, Maheshwari V, Aziz M: Teratoma of the liver-a case report. Indian J Pathol Microbiol 41:457-459, 1998 5. Rao PL, Venkatesh A, Murthy VS: Cystic teratoma in the bare area of liver. Indian J Pediatr 54:275-278, 1987 6. Abe H, Ikeda K, Watanabe I: Teratoma of the ligamentum teres of the liver—A case report. Fukushima J Med Sci 23:1-10, 1976
Teratoma arising from extrahepatic common ducts is very rare entity. The authors found 2 teratoma cases originating from common bile duct in the literature. As a third case, the authors report on a 4-month-old girl with benign cystic teratoma arising from distal common hepatic bile duct and with
anomalous common bile ducts. Surgical management of the patient also is discussed. J Pediatr Surg 39:E1-E2. © 2004 Elsevier Inc. All rights reserved. INDEX WORDS: Bile duct tumor, teratoma of bile duct.
T
were found: hemoglobin, 9.5 g/dL (normal range, 10.6 to 14.5 g/dL); alkaline phosphatase, 1390 U/L (normal range, 64 to 300 U/L); alanine aminotransferase (ALT) 964 U/L (normal range, 5 to 30 U/L); gamma glutamyl transpeptidase, 346 U/L (normal range, 7 to 49 U/L); indirect bilirubin, 1.0 mg/dL (normal range, 0.1 to 0.8 mg/dL); direct bilirubin, 3.2 mg/d (normal range, 0.1 to 0.5 mg/dL). Alpha-fetoprotein level was normal with respect to the patient’s age (77.8 ng/mL), and carcinoembryonic antigen level was also normal (1.72 ng/mL). Although CA 125 level was elevated (27 U/mL), CA 19.9 and CA 15.3 levels were within normal ranges. A plain film of the abdomen showed a large upper abdominal cystic mass compressing and displacing the stomach and intestine to the left. Sonography showed a cystic mass including a little solid component on the right side that had compressed left lobe of the liver and extended to pelvis. The right kidney was displaced to the lateral by the mass, but no hydronephrosis was observed. Contrast-enhanced axial computerized tomography scan showed that cystic lesions with smooth edges and different densities occupied the whole right abdomen and passed the midline to the left abdomen (Fig 1). At laparotomy, the huge cystic mass (15 cm in diameter) with solid components passed the midline and extended to the pelvis. The cystic mass originated from a point just distal to the common hepatic bile duct where it was occluded by tumor. Gallbladder and choledocal duct were under pressure by the tumor. Abnormally, choledocal duct was leaving from the apex of the gallbladder and was thin and elongated (2 mm in diameter and 15 cm in length), and reach duodenum passing over the mass. Operative cholangiogram showed dilated intrahepatic biliary tract, and no passage of the contrast media into the duodenum was detected. The mass was excised totally together with distal part of common hepatic duct. Choledocal duct was ligated at just the point to enter the duodenum and excised totally. A decision was made for an end-to-side anastomosis between the proximal part of the common hepatic duct and fundus of the gallbladder. The apex of the gallbladder was tubularized for 2 cm in length and then anastomozed to the antimesenteric side of the jejunum 10 cm from the ligament of Treitz (Fig 2).
HE MOST common causes of obstructive jaundice in early childhood are choledocal cyst and rhabdomyosarcoma.1,2 The teratoma of the common bile ducts is a very rare phenomenon. To our knowledge, there have been 2 cases of teratoma of the common bile duct in the English-language literature.1,2 In this case report, a child with teratoma arising from common hepatic bile duct and with anomalous common bile ducts is presented, and its surgical management is discussed. CASE REPORT A 4-month-old girl was admitted for progressive jaundice and asymptomatic progressive abdominal distension lasting for a month. His past medical history was unremarkable. The child was observed to be icteric on physical examination. Abdominal examination found a prominent distention and a huge, well-defined mass that was nonfixed with a smooth surface at the right upper and lower quadrants and was passing over to the other side of the midline. The child otherwise appeared well. On laboratory investigation, the following abnormalities
Fig 1.
Appearance of the tumor on computerized tomography.
Journal of Pediatric Surgery, Vol 39, No 4 (April), 2004: E1-E2
From the Departments of Pediatric Surgery, Radiology, and Pathology, Inonu University Faculty of Medicine, Malatya, Turkey. ¨ zal Tıp Address reprint requests to Dr Mehmet Demircan, T. O Merkezi, C¸ocuk Cerrahisi Anabilim Dalı, 44069 Malatya, Tu¨rkiye (Turkey). © 2004 Elsevier Inc. All rights reserved. 1531-5037/04/3904-0035$30.00/0 doi:10.1016/j.jpedsurg.2003.12.036 e1
e2
DEMIRCAN ET AL
Fig 2. Schematic diagram of the teratoma and bile ducts (A) and final appearance of the bile ducts after surgical procedure (B).
Macroscopic and histopathologic examination of the specimen found multilocular cystic mass (size of 11 ⫻ 10 ⫻ 6 cm) and solid component consisting of squamous epithelium, skin adnexae, mature glial tissue, and fat. Immature neuronal tissue or malignant tissue was not detected. At 4 years follow-up, the course was uneventful, and the patient did not experience any episodes of cholangitis.
DISCUSSION
Teratomas are neoplasms containing elements derived from the 3 primary germ layers, and the most common sites for teratoma are sacrococcygeal, mediastinal, and retroperitoneal regions and gonads. Teratomas rarely occur in abdominal organs such as the liver.3-6 Teratoma of the common bile ducts is an extremely rare entity. We found only 2 case reports of teratoma arising from the common bile duct in the English-language literature.1,2 There was no morphologic and radiologic description in first case report.1 In the second case report, investigators have emphasized the radiologic description and differential diagnosis of the teratoma of common bile duct.2 Interestingly, our patient also had anatomic abnormalities of the extrahepatic biliary ducts. These abnormalities were described as follows: common hepatic duct entering directly into the gallbladder, absence of a cystic duct, and a very thin and elongated choledocal duct exiting from the apex of the gallbladder. These abnormalities may have occurred if the teratoma began growth
during the embryogenesis of the biliary tract, or the teratoma may have developed from abnormal structures of the common biliary tracts. The majority of teratomas in children are benign. Excision of the tumor is the treatment of choice for benign teratomas and is associated with a good prognosis. Usually, surgeons prefer to use Roux-en-Y hepaticoportojejunostomy to correct surgical disorders of the porta hepatis area. But the most common complication of this procedure is cholangitis. In our patient, we preferred to use the gallbladder as a bile port. We first performed an end-to-side anastomosis between the hepatic common duct and gallbladder (ductocystostomy). Then the apex of the gallbladder was tubularized for 2 cm in length, and an end-to-side anastomosis between tubularized gallbladder and jejunum (cystojejunostomy) was performed. Thus, 2 anastomoses were performed on the gallbladder. Follow-up over 4 years showed that the patient did not have any episodes of cholangitis. The radiologic and biochemical test of the patient’s liver functions are within normal ranges. As reported in the literature, a Whipple operation had been performed in one of the cases because of the clinical impression that the tumor was a malignancy of the common bile duct, such as rhabdomyosarcoma.1 We think that this approach is quick radical for benign pathology such as teratoma.
REFERENCES 1. Frexes M, Neblett WW 3rd, Holcomb GW Jr: Spectrum of biliary disease in childhood. South Med J 79:1342-1349, 1986 2. Kim WS, Choi BI, Lee YS, et al: Endodermal sinus tumour associated with benign teratoma of the common bile duct. Pediatr Radiol 23:59-60, 1993 3. Witte DP, Kissane JM, Askin FB: Hepatic teratomas in children. Pediatr Pathol 1:81-92, 1983
4. Alam K, Maheshwari V, Aziz M: Teratoma of the liver-a case report. Indian J Pathol Microbiol 41:457-459, 1998 5. Rao PL, Venkatesh A, Murthy VS: Cystic teratoma in the bare area of liver. Indian J Pediatr 54:275-278, 1987 6. Abe H, Ikeda K, Watanabe I: Teratoma of the ligamentum teres of the liver—A case report. Fukushima J Med Sci 23:1-10, 1976