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Rhabdomyosarcoma of the bile ducts
Rhabdomyosarcoma By Ii. S.
Nagarai,
Donald
of the Bile Ducts R. Kmetz,
and
Carol
Leitner
Rhabdomyosarcoma is a rare malignant neoplasm of the biliary tract. Including all the historical. recent probable, and established cases that are carefully classified by Hayes and Snyder,’ only 24 cases have been reported to date. Because of the extreme rarity of this tumor in this location, correct diagnosis was not made until second laparolomy or autopsy in several reported cases. The present report describes a child who recently underwent excision of such a tumor at our institution.
CASE REPORT A 4&yr-old black boy was noted to be jaundiced on 7/4/76. A tentative diagnosis of infectious hepatitis was made in the outpatient clinic, and he was treated at home with bed rest and the jaundice cleared. However, on 7/16/76 the jaundice recurred, and the patient was admitted to Norton-Children’s Hospitals on 7/19/76 with fever, abdominal pain, and jaundice. His weight was I5 kg, temperature 38.3-C. pulse rate 92 per min. and the sclera were icteric. The liver was enlarged 9 cm below the right costal margin and was tender. Spleen was not enlarged. Laboratory studies on admission included hemoglobin 9.5 g/dl, hematocrit 31”,, white blood cell (WBC) count 15,200 with shift to the left, and a normal platelet count. Prothrombin time (PT) was 22.4 (control, 11.9), partial thromboplastin time (PTT) was 82.4 set (control, 34.9 (LDH) 408 units. serum set), alkaline phosphatase measured 954 units, lactic dehydrogenase glutamic oxaloacetic transaminase (SCOT) 48 units. total protein 6.9 g/dl, albumin 2.87 g/dl. total bilirubin 12.4 mg/dl, and direct bilirubin I I.2 mg/dl. Urine was dark and urobilinogen absent. The stool was pale gray; both sickle cell preparation and the Australian antigen test were negative. The first hospital day the patient had epistaxis, which was controlled with vitamin K. His PT and PTT were brought almost to normal. During his early hospital course, the patient’s temperature spiked to 39.4-40°C. WBC count rose to 41,500. A diagnosis of obstructive jaundice with cholangitis was made. On 7/24/77, the patient was placed on ampicillin and gentamicin. All blood cultures were negative. Roentgenographic findings in the upper gastrointestinal tract, intravenous pyelogram. and bone marrow scan were within normal limits. Liver and spleen scans showed hepatomegaly with nonhomogeneous distribution of tracer in the liver. Ultrasonic examination of the liver disclosed hepatomegaly with probable dilatation of bile ducts. Two avascular areas close to porta hepatis were demonstrated on selective celiac arteriography. On 7/29/76, the patient underwent exploratory laparotomy for possible choledochal cyst with obstructive jaundice and cholangitis. The liver was found to be enlarged but free of any gross tumor. A small amount of bile was aspirated from the gallbladder. Operative cholangiogram showed a small cystic duct and dilated common duct which readily emptied into the duodenum. However. no dye could be refluxed into the proximal duct. A few nonspecific filling defects were noted in the common bile duct which was dilated up to 4 cm in diameter (Fig. I). The common duct was opened longitudinally between two stay sutures. Several soft yellow-tan polypoid masses extruded from the duct (Fig. 2). Histologic examination of frozen section suggested the diagnosis of sarcoma botryoid. The
From the Section of Pediatric Surgery. Departments of Surger?: and Pediatrics, lJniversit_v q/ Louisville School o/‘ Medicine, Health Sciences Center. and Children’s Division of NortonChildren’s Hospitals, Louisville, Kentucky. Presented before the 8th Annual Meeting of the American Pediatric Surgical Association, Acapulco, Mexico. April20-23, 1977. Address for reprint requests: Department of Surgery, the University of‘ Louisville School oj Medicrne. Health Sciences Center, Louisville, Ky., 40201. S,I977 b>, Grune & Stratton, Inc. lSSN 0022-3468. Journal of Pediatric Surgery, Vol. 12, No. 6 (December), 1977
1071
CASE REPORTS
1072
Fig. 1.
Opemtive
cholangiogrom
shows dilated common duct with
filling
defects.
extrahepatic
biliary tree was thickened and distended. The major portion of the tumor arose from the common hepatic duct. Resection of the common duct and the common hepatic duct along with the gallbladder and cystic duct was considered the best palliative treatment. The distal end of the common duct was resected close to the head of the pancreas. A sufficient cuff of the common hepatic duct was left to perform retrocolic hepaticojejunostomy and Roux-en-Y anastomosis. Several polypoid lesions at the entrance of the right and left hepatic ducts were scooped out. No gross tumor was left behind. Bile flowed freely from these ducts. Several necrotic areas were encountered in the liver parenchyma at the porta hepatis. The tinal pathology report was botryoid type rhabdomyosarcoma (Fig. 3). Ultrastructural
Polypoid .masses of Fig. 2. rhabdomyorarcoma in exci!sed part of dilated biliory tree.
CASE
REPORTS
1073
Fig. 3. examination exhibiting
Light microscopydisclosesoccasional rhabdomyoblast with crossstriation.
of the tumor myotibril
with electron
formation
microscopy
disclosed
immature
(Fig. 4). The distal part of the common
skeletal
muscle
fibers,
bile duct was free of tumor.
Liver biopsy specimen showed biliary cirrhosis and was free of tumor. During
the first 6 wk after
6000 rad through
Fig. 4. formation.
Electron
operation.
5 x 5 cm anterior
microscopy
the patient
and posterior
shows
immature
received
an estimated
ports encompassing
skeletal muscle
tumor
the porta
fiber
bed dose of hepatis.
exhibiting
Cur-
myofibrile
CASE REPORTS
1074
rent therapy consists of alternate doses of vincristine and Actinomycin D. At present. the patient is free of any complaints. Postoperative liver scan disclosed only a small area of radiation hepatitis at the porta hepatis.
DLSCUSSION Local recurrence of rhabdomyosarcoma of the bile ducts within a few months has proven to be the usual course of this disease. Akers and Needham described the longest surviving patient (who had been free of disease for more than 4 yr at that time), who had the first successful excision of the tumor and who was treated with radiaton and chemotherapy. To our knowledge, our patient is the second to have undergone such treatment. Malpas and colleagues3 reported that the treatment of children with rhabdomyosarcoma by combination of operation, radiotherapy, and chemotherapy had resulted in improved disease-free survival time. Our patient underwent radical surgical excision 3 wk after the initial presentation and has had no evidence of tumor or local recurrence for the past 9 mo.
REFERENCES 1. Hays DM, Snyder WH Jr: Boytroid sarcoma (rhabdomyosarcoma) of the bile ducts. Am J Dis Child 1l&595, 1965 2. Akers DR. Needham ME: Sarcoma botryoides (rhabdomyosarcoma) of the bile
ducts with survival. J Pediatr Surg 6:474. 197 I 3. Malpas JS, Freeman JE, Paxon A, et al: combination Radiotherapy and adjuvant chemotherapy for childhood rhabdomyosarcoma. Br Med J 1:247, 1976
Nagarai,
Donald
of the Bile Ducts R. Kmetz,
and
Carol
Leitner
Rhabdomyosarcoma is a rare malignant neoplasm of the biliary tract. Including all the historical. recent probable, and established cases that are carefully classified by Hayes and Snyder,’ only 24 cases have been reported to date. Because of the extreme rarity of this tumor in this location, correct diagnosis was not made until second laparolomy or autopsy in several reported cases. The present report describes a child who recently underwent excision of such a tumor at our institution.
CASE REPORT A 4&yr-old black boy was noted to be jaundiced on 7/4/76. A tentative diagnosis of infectious hepatitis was made in the outpatient clinic, and he was treated at home with bed rest and the jaundice cleared. However, on 7/16/76 the jaundice recurred, and the patient was admitted to Norton-Children’s Hospitals on 7/19/76 with fever, abdominal pain, and jaundice. His weight was I5 kg, temperature 38.3-C. pulse rate 92 per min. and the sclera were icteric. The liver was enlarged 9 cm below the right costal margin and was tender. Spleen was not enlarged. Laboratory studies on admission included hemoglobin 9.5 g/dl, hematocrit 31”,, white blood cell (WBC) count 15,200 with shift to the left, and a normal platelet count. Prothrombin time (PT) was 22.4 (control, 11.9), partial thromboplastin time (PTT) was 82.4 set (control, 34.9 (LDH) 408 units. serum set), alkaline phosphatase measured 954 units, lactic dehydrogenase glutamic oxaloacetic transaminase (SCOT) 48 units. total protein 6.9 g/dl, albumin 2.87 g/dl. total bilirubin 12.4 mg/dl, and direct bilirubin I I.2 mg/dl. Urine was dark and urobilinogen absent. The stool was pale gray; both sickle cell preparation and the Australian antigen test were negative. The first hospital day the patient had epistaxis, which was controlled with vitamin K. His PT and PTT were brought almost to normal. During his early hospital course, the patient’s temperature spiked to 39.4-40°C. WBC count rose to 41,500. A diagnosis of obstructive jaundice with cholangitis was made. On 7/24/77, the patient was placed on ampicillin and gentamicin. All blood cultures were negative. Roentgenographic findings in the upper gastrointestinal tract, intravenous pyelogram. and bone marrow scan were within normal limits. Liver and spleen scans showed hepatomegaly with nonhomogeneous distribution of tracer in the liver. Ultrasonic examination of the liver disclosed hepatomegaly with probable dilatation of bile ducts. Two avascular areas close to porta hepatis were demonstrated on selective celiac arteriography. On 7/29/76, the patient underwent exploratory laparotomy for possible choledochal cyst with obstructive jaundice and cholangitis. The liver was found to be enlarged but free of any gross tumor. A small amount of bile was aspirated from the gallbladder. Operative cholangiogram showed a small cystic duct and dilated common duct which readily emptied into the duodenum. However. no dye could be refluxed into the proximal duct. A few nonspecific filling defects were noted in the common bile duct which was dilated up to 4 cm in diameter (Fig. I). The common duct was opened longitudinally between two stay sutures. Several soft yellow-tan polypoid masses extruded from the duct (Fig. 2). Histologic examination of frozen section suggested the diagnosis of sarcoma botryoid. The
From the Section of Pediatric Surgery. Departments of Surger?: and Pediatrics, lJniversit_v q/ Louisville School o/‘ Medicine, Health Sciences Center. and Children’s Division of NortonChildren’s Hospitals, Louisville, Kentucky. Presented before the 8th Annual Meeting of the American Pediatric Surgical Association, Acapulco, Mexico. April20-23, 1977. Address for reprint requests: Department of Surgery, the University of‘ Louisville School oj Medicrne. Health Sciences Center, Louisville, Ky., 40201. S,I977 b>, Grune & Stratton, Inc. lSSN 0022-3468. Journal of Pediatric Surgery, Vol. 12, No. 6 (December), 1977
1071
CASE REPORTS
1072
Fig. 1.
Opemtive
cholangiogrom
shows dilated common duct with
filling
defects.
extrahepatic
biliary tree was thickened and distended. The major portion of the tumor arose from the common hepatic duct. Resection of the common duct and the common hepatic duct along with the gallbladder and cystic duct was considered the best palliative treatment. The distal end of the common duct was resected close to the head of the pancreas. A sufficient cuff of the common hepatic duct was left to perform retrocolic hepaticojejunostomy and Roux-en-Y anastomosis. Several polypoid lesions at the entrance of the right and left hepatic ducts were scooped out. No gross tumor was left behind. Bile flowed freely from these ducts. Several necrotic areas were encountered in the liver parenchyma at the porta hepatis. The tinal pathology report was botryoid type rhabdomyosarcoma (Fig. 3). Ultrastructural
Polypoid .masses of Fig. 2. rhabdomyorarcoma in exci!sed part of dilated biliory tree.
CASE
REPORTS
1073
Fig. 3. examination exhibiting
Light microscopydisclosesoccasional rhabdomyoblast with crossstriation.
of the tumor myotibril
with electron
formation
microscopy
disclosed
immature
(Fig. 4). The distal part of the common
skeletal
muscle
fibers,
bile duct was free of tumor.
Liver biopsy specimen showed biliary cirrhosis and was free of tumor. During
the first 6 wk after
6000 rad through
Fig. 4. formation.
Electron
operation.
5 x 5 cm anterior
microscopy
the patient
and posterior
shows
immature
received
an estimated
ports encompassing
skeletal muscle
tumor
the porta
fiber
bed dose of hepatis.
exhibiting
Cur-
myofibrile
CASE REPORTS
1074
rent therapy consists of alternate doses of vincristine and Actinomycin D. At present. the patient is free of any complaints. Postoperative liver scan disclosed only a small area of radiation hepatitis at the porta hepatis.
DLSCUSSION Local recurrence of rhabdomyosarcoma of the bile ducts within a few months has proven to be the usual course of this disease. Akers and Needham described the longest surviving patient (who had been free of disease for more than 4 yr at that time), who had the first successful excision of the tumor and who was treated with radiaton and chemotherapy. To our knowledge, our patient is the second to have undergone such treatment. Malpas and colleagues3 reported that the treatment of children with rhabdomyosarcoma by combination of operation, radiotherapy, and chemotherapy had resulted in improved disease-free survival time. Our patient underwent radical surgical excision 3 wk after the initial presentation and has had no evidence of tumor or local recurrence for the past 9 mo.
REFERENCES 1. Hays DM, Snyder WH Jr: Boytroid sarcoma (rhabdomyosarcoma) of the bile ducts. Am J Dis Child 1l&595, 1965 2. Akers DR. Needham ME: Sarcoma botryoides (rhabdomyosarcoma) of the bile
ducts with survival. J Pediatr Surg 6:474. 197 I 3. Malpas JS, Freeman JE, Paxon A, et al: combination Radiotherapy and adjuvant chemotherapy for childhood rhabdomyosarcoma. Br Med J 1:247, 1976