Year : 2021 Month : December Volume : 10 Issue : 45 Page : 4062-4064,

Botryoid Rhabdomyosarcoma of Biliary Tree - An Unusual Presentation of Obstructive Jaundice in a Child

Jayalatha Nethagani1, Dhinesh Ram2, Priyanka Govula3, Sridhar Devu4, Sai Sree Harsha Varada5

1, 2, 4 Department of Radiology, MNJ Institute of Oncology and Regional Cancer Center, Hyderabad, Telangana, India.
3,5 Department of Radiology, Osmania Medical College, Hyderabad, Telangana, India.

CORRESPONDING AUTHOR

Dr. Jayalatha Nethagani, MNJ Institute of Oncology and Regional Cancer Center, Red Hills Road, Lakdikapul, Hyderabad-500004, Telangana, India.
Email : jayanethagani@gmail.com

PRESENTATION OF CASE

A 5-year-old male child presented with complaints of abdominal pain, swelling, yellowish discoloration of eyes and altered stools for 4 months. No h/o fever/similar complaints in family/recent vaccinations/recent travel/blood transfusion. Normal vaccination for age.

 

Ultrasound


Subsequently contrast enhanced computed tomography (CECT) of the patient was done and it showed – ill-defined heterogeneously enhancing mass lesion measuring 68 mm x 50 mm x 70 mm at the level of confluence of right and left hepatic duct with upstream dilatation of intra-hepatic biliary radicles. Lesion is seen encasing portal vein, hepatic artery with no evidence of tumour thrombosis. Moderate ascites, few heterogeneously enhancing nodules in peri-portal region and few enlarged lymph nodes noted at porta hepatis was observed.

 

 

 


PROVISIONAL DIAGNOSIS

  • Rhabdomyosarcoma of biliary duct
  • Choledochal cyst with sludge
  • Abdominal tuberculosis

PATHOLOGICAL DISCUSSION

  • Microscopy showed multiple irregular coarse cellular tissue arranged in loose sheets with cells having indistinct cell walls and round to ovoid to spindle dark nucleus.
  • Many of them showed perinuclear vacuolation with moderate nuclear atypia.
  • Intervening stoma shows focal hyalinization and fibrocollagenous tissue with fibromuscular and fibro-adipose tissue.

 

Immunohistochemistry (IHC)

Positive for vimentin and desmin.

 

Findings Consistent With Botryoid Rhabdomyosarcoma

CASE DISCUSSION

  • Rhabdomyosarcoma ranks fourth among common paediatric tumours and it’s the most common malignant soft tissue tumour in paediatric age group (5 - 10 %). Tumour has slight predilection towards male children and median age being 3 years of age. Biliary rhabdomyosarcoma clinically presents with symptoms of obstructive jaundice (60 – 80 %), abdominal distension, hepatomegaly and altered stools with elevated liver enzymes and elevated billirubin levels biochemically.1 Imaging plays an important role in differentiating this rare cause of obstructive jaundice from other common causes such as choledochal cyst, choledocholithiasis, stricture.2 Tumour arises as an intra-biliary mass or from choledochal cyst and gives an appearance of cluster of grapes similar to sarcoid botryoides of bladder and vaginahistologically.3
  • Based on site of tumour, regional and distant metastases, staging system is developed by The intergroup rhabdomyosarcoma study group.2 Another prognostically significant classification provided by international classification of rhabdomyosarcoma based on histological subtypes as being of poor prognosis with alveolar RMS and superior prognosis with botryoid and spindle cell RMS and intermediate prognosis with typical embryonal RMS.1

 

Imaging Features

  • Ultrasound - being the primary imaging modality for evaluation of jaundice with abdominal pain, it reveals dilatation of intra and extra hepatic bile duct with intra-ductal mass with colour flow on Doppler.2 Imaging differentials for mass at porta hepatis includes mass arising from liver (hepatoblastoma/hcc/undifferentiated embryonal sarcoma), pancreatic neoplasms (pancreaticoblastoma), metastatic lesions to liver, choledochal cyst with sludge.3 Commonly lesion is mistaken for choledochal cyst with sludge and can be differentiated by absence of colour flow in the latter.
  • Computed tomography - Primarily for extension of tumour and distant metastases. Presents as heterogeneous intra biliary mass with varying pattern of enhancement.4,5
  • MRCP - Provides information about anatomy of biliary tree, tumour extent, local and distant metastases.6
  • MRI - T1-Isointense to skeletal muscle, T2-hyperintense with heterogeneous enhancement post contrast. Common differentials include choledochal cyst with sludge when the tumour has central necrosis.2
  • Endoscopic retrograde cholangio-pancreatography (ERCP) - To obtain biopsy from the lesion and to visualize biliary tree and relieving obstruction by stent.
  • Chemotherapy is recommended for extensive disease using vincristine, dactinomycin and cyclophosphamide. Radiotherapy is recommended for post-operative residual tumour or with failure ofchemotherapy.6,7

FINAL DIAGNOSIS

Botryoid rhabdomyosarcoma of biliary tract

REFERENCES

1
  1. Mathew D, de Lima H, Mahomed N. Embryonal rhabdomyosarcoma of the biliary tree in a paediatric patient - a rare cause of obstructive jaundice. S Afr J Radiol 2019;23(1):1662.
  2. Kinariwala DJ, Wang AY, Melmer PD, et al. Embryonal rhabdomyosarcoma of the biliary tree: a rare cause of obstructive jaundice in children which can mimic choledochal cysts. Indian J Radiol Imaging 2017;27(3):306-9.
  3. Nemade B, Talapatra K, Shet T, et al. Embryonal rhabdomyosarcoma of the biliary tree mimicking a choledochal cyst. J Can Res Ther 2007;3(1):40-2.
  4. Mane A, Verma A, Kar AG, et al. Botryoid rhabdomyosarcoma of common bile duct: diagnostic imaging features of an unusual pathology: Case report and review. Asian J Oncol 2016;2(1):49-52.
  5. Thampy R, Elsayes KM, Menias CO, et al. Imaging features of rare mesenychmal liver tumours: beyond haemangiomas. Br J Radiol 2017;90(1079):20170373.
  6. Kırlı EA, Parlak E, Oğuz B, et al. Rhabdomyosarcoma of the common bile duct: an unusual cause of obstructive jaundice in a child. Turk J Pediatr 2012;54(6):654-7.
  7. Chowdhary S, Kumar D, Sharma SP. Rhabdomyosarcoma of biliary tract misdiagnosed as choledocal cyst-two cases and review of literature. World Journal of Surgical Research 2016;5:17-21.

DISCLOSURE AND FUNDING

Disclosure forms provided by the authors are available with the full text of this article at jemds.com

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Financial or other competing interests: None.

Disclosure forms provided by the authors are available with the full text of this article at jemds.com.

How to cite this article

Nethagani J, Ram D, Govula P, et al. Botryoid rhabdomyosarcoma of biliary tree - an unusual presentation of obstructive jaundice in a child.J Evolution Med Dent Sci 2021;10(45):4062-4064, DOI: 10.14260/jemds/2021/822

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