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Year : 2001  |  Volume : 11  |  Issue : 2  |  Page : 101-102
Case report : Sarcoma botryoides of the common bile duct

Department of Radiology, Lady Hardinge Medical College & Associated Smt. Sucheta Kripalani Hospital & Kalawati Saran Childrens' Hospital, New Delhi 1, India

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Keywords: rhabdomyosarcoma, bile duct, sarcoma botryoides

How to cite this article:
Mathew M, Narula M K, Chadha R. Case report : Sarcoma botryoides of the common bile duct. Indian J Radiol Imaging 2001;11:101-2

How to cite this URL:
Mathew M, Narula M K, Chadha R. Case report : Sarcoma botryoides of the common bile duct. Indian J Radiol Imaging [serial online] 2001 [cited 2021 Feb 26];11:101-2. Available from:
Embryonal rhabdomyosarcoma of the bile duct is a very rare malignancy. Our recent experience with a case of sarcoma botryoides of the common bile duct has prompted us to report this unusual tumor.

   Case Report Top

A five-years-old girl presented with a one-month history of jaundice, fever and progressive abdominal distension. On examination, the child had a palpable lump in the right hypochondrium. Laboratory investigations revealed obstructive jaundice.

On sonographic evaluation, the liver was found to be enlarged, with dilatation of the intrahepatic radicles in the right and left lobes. A large well-defined mass measuring 6x6 cm was seen at the porta hepatis, anterior to the portal vein [Figure - 1]. It was seen to extend from the confluence of the right and left hepatic ducts in the superior position, to the level of the head of pancreas in the inferior position. The common bile duct was not visualized separate from the mass. A small multicystic subhepatic collection was seen adjacent to the gall bladder. Computed tomography, confirmed the findings of a bile duct mass with inferior extension to the level of pancreatic head. The lesion showed mixed attenuation values (40-90 HU) with enhancing polypoidal masses within the common bile duct [Figure - 2]. Ultrasound guided fine needle aspiration cytology revealed few clusters of cells with anisonucleosis, scanty cytoplasm and dispersed chromatin with fibrillary basement membrane material suggestive of a round cell tumor.

The patient subsequently underwent laparotomy. The liver was found to be grossly enlarged. A greenish yellow, myxomatous polypoidal tumor was seen arising from the common bile duct, with dense adhesions to the gall bladder, stomach and duodenum. The gall bladder was excised and dissection was performed around the tumor as the mass could not be excised in toto. Histopathological examination of the tissue suggested a diagnosis of botryoid type of embryonal rhabdomyosarcoma of the common bile duct. The patient was started on chemotherapy with a multidrug regime comprising of vincristine, actinomycin D and cyclophosphamide. The mass initially underwent a diminution in size. Radiation therapy was started on the tumor bed after six weeks of chemotherapy. The mass again began to increase in size at the end of one year of therapy, and the patient subsequently expired.

   Discussion Top

Embryonal rhabdomyosarcomas constitute about 10 to 15% of malignant solid tumors occurring in early childhood. These are usually localised in the genitourinary tract and in the soft tissues of the head and neck [1]. Involvement of the biliary tract is extremely rare. The first case was reported by Wilks and Moxon in 1875 [2].

In comparison with embryonal rhabdomyosarcomas at other sites, the tumors of the biliary tract are commoner in women, characterised by an earlier onset and shorter duration of survival. Intermittent jaundice with or without abdominal distension, fever and anorexia is the typical presentation [3].

Sarcoma botryoides of the extrahepatic bile duct is rarely diagnosed pre-operatively. Radiological evaluation is a vital pre-requisite to the appropriate management of these tumors. In a child presenting with hepatomegaly or biliary retention, ultrasonography should be the first examination [4]. It demonstrates the tumor mass at the hepatic hilum, surrounded by fluid areas; this pattern would be suggestive of an intraluminal tumor. It may reveal a mass containing multiple septations and mural masses delineating cystic areas. Computed tomography complements sonographic evaluation and determines operability. On computed tomography, the attenuation of the mass is lower than that of the normal liver, the tumor may seem almost cystic and tumor calcification is unusual [4]. In our patient, ultrasonography revealed the typical pattern of a tumor mass at the hepatic hilum with extension down to the lower end of the common bile duct. The mass was hypodense and enhanced on contrast administration.

Based on sonographic and CT findings, a mass lesion of the common bile duct was diagnosed and the possibility of rhabdomyosarcoma was considered. Needle aspiration cytology revealed it to be a round cell tumor, which was confirmed to be embryonal rhabdomyosarcoma on histopathology. The possibility of other tumors of the hepatobiliary system seemed fairly remote due to the common duct origin of the mass.

Most embryonal rhabdomyosarcomas of the biliary tree are of the botryoid type. They tend to form polypoidal masses of soft gelatinous, pinkish grey tissue that tend to obstruct bile flow [4] resulting in jaundice as in our case. Spread initially occurs by local invasion or to the regional lymph nodes rather than by distant metastases [2]. They spread within the liver and then to the retroperitoneum and lungs.

Rhabdomyosarcomas have been divided into four clinical stages by the Intergroup Rhabdomyosarcoma Study (NIH) based on the extent of the disease and the type of surgery performed [2]. These are Stage I - localized disease completely resected, Stage II - microscopic residual following surgical excision, Stage III - gross residual disease following surgery, and Stage IV - metastatic disease [5]. Accordingly our patient fell into clinical group III (gross residual disease following surgery). Survival progressively decreases for patients from clinical Group I (localised disease completely resected) to Group IV (metastatic disease) [5]. Patients with botryoid or embryonal lesions have the best survival. There are three currently recognised modalities of treating children with sarcomas - surgical removal (if feasible), radiation therapy and systemic chemotherapy. Only with multidisciplinary management has any patient shown prolonged survival. The longest reported follow-up is fourteen years, the patient having received a combination of radiotherapy and chemotherapy following surgery [3].

   References Top

1.Friedburg H, Kauffmann GW, Bohm N, Fiedler L, Jobke A. Sonographic and computed tomographic features of embryonal rhabdomyosarcoma of the biliary tract. Pediatr Radiol 1984; 14: 436.   Back to cited text no. 1  [PUBMED]  
2.Mihara S, Matsumoto H, Tokunaga F, Yano H, Ota M, Yamashita S. Botryoid rhabdomyosarcoma of the gall-bladder in a child. Cancer 1982; 49: 812.   Back to cited text no. 2  [PUBMED]  
3.Ruymann FB, Raney RB, Crist WM, Lawrence W, Lindberg RD, Soule EH. Rhabdomyosarcoma of the biliary tree in childhood. Cancer 1985; 56: 575.   Back to cited text no. 3    
4.Geofrray A, Couanet D, Montagne JP, Leclere J, Flamant F. Ultrasonography and computed tomography for diagnosis and follow-up of biliary duct rhabdomyosarcomas in children. Pediatr Radiol 1987; 17: 127.   Back to cited text no. 4    
5.Crist WM, Garnsey L, Beltangady MS, et al. Prognosis in children with rhabdomyosarcoma; a report of the intergroup rhabdomyosarcoma studies I and II. J Clin Oncol 1990; 8: 443.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]

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M Mathew
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