Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2022  |  Volume : 27  |  Issue : 3  |  Page : 348--350

Case of botryoid rhabdomyosarcoma mimicking biliary hydatid disease

Suha Mohammad Akbar1, Harish Chandra Tudu2, Subrat Kumar Mohanty3, Amaresh Mishra1, Sruti Mohanty2,  
1 Department of Surgery, KIMS, Bhubaneswar, Odisha, India
2 Department of Pediatric Surgery, KIMS, Bhubaneswar, Odisha, India
3 Department of Surgery, Pediatric Surgery Unit, KIMS, Bhubaneswar, Odisha, India

Correspondence Address:
Dr. Subrat Kumar Mohanty
Department of Surgery, Pediatric Surgery Unit, KIMS, Bhubaneswar - 751 024, Odisha


Hepatobiliary rhabdomyosarcoma (RMS) is a soft tissue malignant musculoskeletal tumor of the biliary tract. It is rare tumor, mostly seen in children and mimics common benign biliary condition. Here, we present a case of 1 year 9 months child of biliary RMS who presented with obstructive jaundice and diagnosed after biopsy. This case highlights the unusual childhood malignancy of bile duct RMS in obstructive jaundice with atypical imaging findings.

How to cite this article:
Akbar SM, Tudu HC, Mohanty SK, Mishra A, Mohanty S. Case of botryoid rhabdomyosarcoma mimicking biliary hydatid disease.J Indian Assoc Pediatr Surg 2022;27:348-350

How to cite this URL:
Akbar SM, Tudu HC, Mohanty SK, Mishra A, Mohanty S. Case of botryoid rhabdomyosarcoma mimicking biliary hydatid disease. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Jun 25 ];27:348-350
Available from:

Full Text


Rhabdomyosarcoma (RMS) is a malignant tumor and biliary tract involvements are rare. It is usually identified in children and reported incidence is 1% of cancer among children aged between 0 and 14 years.[1] Tumor progression is usually rapid and most of them present in early childhood. Preoperative diagnosis is difficult as it mimics other benign biliary conditions of obstructive jaundice. Management includes tissue diagnosis by biopsy, staging, metastatic workup, and appropriate treatment in the forms of surgical excision followed by chemotherapy and radiotherapy.

 Case Report

A female child of 1 year 9 months age presented with intermittent jaundice, fever, vomiting, and anorexia for 1 month. Physical examination found to have gross pallor with icterus and poor general condition. Investigation revealed hemoglobin of 6 g% and deranged liver function test with total bilirubin - 10.38 mg/dl, direct bilirubin - 8.88 mg/dl, and Gamma glutamyl transferase (GGT) - 616 U/L. Ultrasonography and magnetic resonance cholangiopancreatography (MRCP) showed intraductal cystic lesions in the common bile duct (CBD) with distended gall bladder with bilobar intrahepatic biliary tree dilatation, suggestive of hydatid cyst of extrahepatic biliary duct [Figure 1].{Figure 1}

Contrast-enhanced computed tomography (CECT) of the abdomen and pelvis showed dilated biliary channels with multiple cystic hypodense lesions with no features of distant metastasis. The child was treated for cholangitis and prepared for surgery. On exploratory laparotomy, CBD was dilated all along from porta to lower end with multiple cystic lesions in the CBD obstructing it with mucocele of gall bladder [Figure 2]. No periductal lymphadenopathy or involvement of the liver parenchyma was observed. Cholecystectomy, excision of entire CBD from porta to retro pancreatic part with Roux en Y portojejunostomy of patent left and the right hepatic duct was done. Postoperative recovery was uneventful with normalization of liver function test. Histopathology showed gall bladder and bile duct mucosa and sub-epithelium with tumor of spindle cell and rhabdomyoblasts forming a condensed cambium layer underneath [Figure 3]. Immunohistochemical analysis showed tumor positive for desmin and myogenin while negative for myo-D1. Bone scan showed no signs of metastasis. Postoperatively, there was some residual disease at porta hepatis revealed as ill-defined heterogeneously enhancing lesion on CT scan. She was categorized as stage III intergroup RMS and received chemotherapy with six cycles of vincristine, actinomycin D, and cyclophosphamide along with radiotherapy. The patient received external beam radiotherapy of 36 Gy over 20 fractions in 4 weeks. Follow-up was done at 3rd month, 6 month, and at 1 year with a review of clinical parameters, liver function test, and ultrasound abdomen. Ultrasound scan showed no dilatation of intrahepatic biliary radicals and the child was thriving well without any signs of jaundice or recurrence.{Figure 2}{Figure 3}


RMS is a very common soft tissue tumor in the pediatric population, but biliary affection is rare. Common sites in children are head and neck, genitourinary tract, and retroperitoneum.[2] Typical presentation is of obstructive jaundice and includes intermittent jaundice, fever, anorexia, and acholic stools, decreased appetite with or without abdominal distension. Preoperative diagnosis mimic more common benign conditions mostly choledochal cyst and others such as choledocholithiasis, stricture, and rarely neoplasm of the biliary tree. In this case, initial suspicion was hydatid cyst due to multiple intraductal cystic lesions. Ultrasound is the first imaging modality, but often inconclusive. Here, in this patient ultrasound revealed solid cystic lesion filling proximal and mid-CBD with minimal central IHBR dilatation which was supported by MRCP giving a false impression of hydatid disease of CBD. Diagnosis is suspected on CECT, magnetic resonance imaging and percutaneous transhepatic cholangiography and is frequently made during surgical intervention or liver biopsy. Typical CT complements sonographic evaluation and determines operability. Imaging also plays a vital role for preoperative staging.[3] In the present case suspicion of mass with malignancy was made on surgical exploration and later confirmed by biopsy.

Unlike other sites, biliary tract RMS contains tumor of embryonal or botryoid histology. Immunohistochemical analysis is very useful for diagnosis of embryonal variety with 95% tumor positive for desmin and myogenin.[4] Tumor cells, in this case, were botryoid histology and immunopositive for desmin (cytoplasmic) and myogenin (nuclear). Favorable prognostic factors for cure include radical surgery with complete removal of localized tumor with negative margins, botryoid histology, tumor size <5 cm, and age of the patient <10 years without any detectable distant metastasis at diagnosis.[5] The recommended radiation doses ranged from 30.6 Gy to 44.8 Gy depending on the extent of surgery and response to chemotherapy.[6] Late detection of tumor, its critical location, and frequent involvement of liver accounts for low survival, even though the tumor has favorable histology. Here in this child, there was no evidence of local or distant spread even at 1 year of follow-up. Although rare this case illustrates the importance of considering malignancy of biliary tree as differential diagnosis of childhood obstructive jaundice in atypical imaging findings.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


1Ali S, Russo MA, Margraf L. Biliary rhabdomyoscarcoma mimicking choledochal cyst. J Gastrointestin Liver Dis 2009;18:95-7.
2Baker KS, Anderson JR, Link MP, Grier HE, Qualman SJ, Maurer HM, et al. Benefit of intensified therapy for patients with local or regional embryonal rhabdomyosarcoma: Results from the Intergroup Rhabdomyosarcoma Study IV. J Clin Oncol 2000;18:2427-34.
3Abhishek M, Ashish V, Amrita K, Ritu O, Ram S, Arvind S, et al. Botryoid rhabdomyosarcoma of common bile duct: Diagnostic imaging features of an unusual pathology: Case report and review. Asian J Oncol 2016;2:49.
4Morotti RA, Nicol KK, Parham DM, Teot LA, Moore J, Hayes J. An immunohistochemical algorithm to facilitate diagnosis and subtyping of rhabdomyosarcoma: The children's oncology group experience. Am J Surg Pathol 2006;30:962-8.
5Zampieri N, Camoglio F, Corroppolo M, Cecchetto M, Ornis S, Ottolenghi A. Botryoid rhabdomyosarcoma of the biliary tract in children: A unique case report. Eur J Cancer Care (Engl) 2006;15:463-6.
6Urla C, Warmann SW, Sparber-Sauer M, Schuck A, Leuschner I, Klingebiel T, et al. Treatment and outcome of the patients with rhabdomyosarcoma of the biliary tree: Experience of the Cooperative Weichteilsarkom Studiengruppe (CWS). BMC Cancer 2019;19:945.