Master Arman, a 10-year old male, presented with abdominal pain, vomiting, jaundice and itching for 3 months. Imaging showed a dilated common bile duct containing soft tissue. Histopathology of the cyst contents suggested a small round blue cell tumor. The patient underwent choledochotomy with cyst removal and T-tube insertion. Histopathology then confirmed malignant small round blue cell tumor. Radiotherapy and chemotherapy were recommended post-surgery to prevent recurrence, as small round blue cell tumors are malignant.

1. CASE PRESENTATION
Dr. Sarwar Ahmed Sobhan and Dr Md Ab Quiyum
Resident, Phase-B
Hepatobiliary, pancreatic and liver transplant surgery
BSMMU

2. Particulars of the patient
• Name: Master Arman
• Age/sex: 10 years/male
• Reg no: 22/6
• Bed: cabin-422A
• Occupation: student
• Address: Kalkini, Madaripur

3. Chief complaints
• Abdominal pain and vomiting for 3 months
• Yellowish discoloration of skin for 3 months
• Pruritus for 3 months
• Fever for 3 months

4. History of presenting illness
According to the patient he was reasonably well 3 months back. Then he
developed pain in the epigastric region which was gradual in onset, spasmodic,
intermittent, no radiation was present, no aggravating or relieving factors were
noticed. Pain was associated with vomiting of same duration. The vomitus
contained only food particles, was not bile stained, aggravated by food intake.
He also noticed yellowish discoloration of his eyes and skin. It was associated
with generalized itching. The yellowish discoloration of sclera and skin gradually
increased and decreased but it was never absent. He also complained of low
grade fever which was on and off initially but became high grade later on. Fever
subsided only after taking antipyretics. Fever was associated with occasional
chills and rigor. His bowel and bladder habits were normal except yellowish
discoloration of urine and occasional constipation. He had no history weight
loss.

5. Past history
• He suffered from acute hepatitis due to HAV 5 years back.
Personal history
• No significant history
Family history
• Consanguineous marriage between his parents (cousins)

6. Examination
General condition: ill-looking, average built, average nutrition,
decubitus on choice
Height:
Weight:
BMI:

7. Vital parameters:
• Heart rate: 87bpm
• Respiratory rate: 17 breaths/min
• Temperature: 100 deg F
• Blood pressure: 100/70 mm of Hg

8. • Anemia: Absent
• Icterus: Present
• Lymphadenopathy: Absent
• Cyanosis: Absent
• Clubbing: Absent
• Oedema: Absent
• Dehydration: Absent

9. Systemic examination
Abdomen examination:
Inspection:
• Shape: scaphoid
• Flanks not full, umbilicus inverted and placed centrally
• No scar marks, venous prominence or visible cough impulse
• Hernial orifices intact

10. Palpation: no local rise of temperature, soft, non-tender, no
palpable mass or organomegaly
Percussion: tympanic note heard all over the abdomen
Auscultaion: bowel sounds present, 3-4 per minute

11. Other systemic examination revealed normal findings

12. Salient features
• Master Arman, 10 years old male patient hailing from Kalkini,
Madaripur presented with the features of intermittent
obstructive jaundice with cholangitis for 3 months with
vomiting of same duration. He is icteric but other examinations
revealed normal findings.

13. Provisional diagnosis
• choledocholithiasis

14. Differential diagnosis
• Choledochal cyst

15. Investigations:
Complete blood counts:
• Haemoglobin: 9.7 g/dl
• Total count: 3,600/cumm
• Platelets: 5,60,00/cumm
Liver function test:
• Serum bilirubin: 7.8 mg/dl
• Alkaline phosphatase: 292 U/L
• PT: 30.3 seconds
• INR: 2.64
• SGPT: 63 U/L
• Serum albumin: 2.6 g/dl

16. Tumor markers
• CA 19-9: 547.5 U/ml
• CEA : 3.05 ng/ml

17. USG of Whole abdomen
• Liver: mildly enlarged with homogenous echotexture
• Biliary channels: Fusiform dilatation of CBD (3.8cm X 5.2 cm X
5.8cm) with soft tissue structure seem which extends distal part
of CBD compressing the head of pancreas.
• Gallbladder: is contracted. Wall is thickened.
• Pancreas: normal
• Impression: suggestive of choledochal cyst (infected or
hemorrhagic- type IVb). Mild hepatomegaly

18. MRCP

19. MRCP findings

20. CE-CT of abdomen

21. Pancreatic enzymes:
• Serum amylase: 38 U/L
• Serum lipase: 9.0 U/L

22. Investigations for anesthesia fitness
• Renal function tests:
– Serum creatinine: 0.5 mg/dl
– Sodium (Na): 137.0 mmol/L
– Potassium (K): 4.3 mmol/L
• Blood group: B positive
• Chest X-ray PA view: normal
• ECG: sinus rhythm
• Echocardiography: normal findings. LVEF: 62%

23. Investigations cont.…
• HBsAg: negative
• Anti-HCV: negative
• Random blood sugar: 6.2 mmol/L

24. • The patient was optimized and surgery was planned.
• Planned procedure: excision of choledochal cyst with Roux-en-
Y hepaticojejunostomy.

25. Operation : choledochotomy with removal of cyst
content with t-tube insertion
• Procedure
• Incision: bilateral subcostal
• Choledochotomy done and the soft tissue, necrotic tissue and
tumerous tissues cleared form the bile ducts.
• Distal and proximal duct patency ensured by passing a dilator.
• A 7 Fr t-tube placed in the CBD.
• A right subhepatic drain placed.
• Cyst content and cyst wall sent for histopathological study

26. Peroperative findings
• Gallbladder was enlarged and distended.
• CBD was widely dilated, wall thin and
edematous.
• Cystic dilatation of CBD contain large amount
of soft tissue, necrotic tissues and tumerous
tissues upto intrahepatic bile ducts.
• White bile within the bile ducts.

27. Choledochal
cyst

28. Content
removed
from
cyst

29. Cyst cavity
after
removal of
content

30. Postoperative period
• Postoperative recovery was uneventful.

31. Postoperative investigations
21/02/2020 29/02/2020 08/02/2020
Serum bilirubin (mg/dl) 7.3 5.3 3.8
ALP (U/L) 282.0 326.0
SGPT (U/L) 87.0 52.0
PT (seconds) 11.2 14.0
Serum albumin (g/dl) 34.0 38.0

32. Histopathology reports:
• Suggestive of malignant small round blue cell
tumor.

33. T-tube
cholangiogram

34. Small round blue cell tumor
•
• A group of childhood tumors that is
characterized by a similar
appearance under the microscope.
The appearance is that of small,
round, primitive cells that stain blue
with conventional staining techniques
for biopsy analysis.

35. Small round blue cell tumors
• round cells with increased nuclear-cytoplasmic
ratio.
• includes entities such as
• 1. peripheral neuroectodermal tumor,
• 2.rhabdomyosarcoma,
• 3.synovial sarcoma,
• 4. non-Hodgkin's lymphoma, 5.neuroblastoma,
• 6.hepatoblastoma,
• 7.Wilms' tumor, and
• 8.desmoplastic small round cell tumor.
• malignant tumors
• Need exact typing {imm
• As treatments are differe
Large round cell – Squamous cell carcinoma,
adenocarcinoma, melanoma, RMS, lymphoid
tumors, paraganglioma
For curiosity

36. Recommendation :
Radiologically RMS is often misdiagnosed as choledochal cyst. RMS should be considered in the differential
diagnosis of choledochal cyst even if imaging is not suggestive for RMS.
RMS need surgical excision and roux en y hepaticojejunostomy followed by chemotherapy. Redo surgery is
indicated if residual disease remains which is followed by radiotherapy and chemotherapy as per need.
2 cases: Excision of CBD with cholecystectomy with a roux en Y portoenterostomy was performed

37. Procedure done :
laparotomy with open exploration of CBD which revealed a
10cm x 4 cm myxomatous polypoidal tumor filling the lumen
and adhering to the CBD wall. A cholecystectomy with
complete resection of the common bile duct and a Rouxen-Y
hepatico-jejunostomy was performed.
outcome : After three chemotherapy cycles, showed no recurrence or residual tumor considered complete response. At week 17 after
the start of chemotherapy, the child received an Intensity Modulated Radiotherapy (IMRT) while continuing chemotherapy cycles. Imaging
scans performed quarterly for one year and a half after the end of the therapy did not reveal any evidence of recurrence or metastatic
disease.

38. Take home message
%
• Blue cell tumor affecting only 1% CBD tumor group.
D/D
• Considerrhabdomyosarcomaas D/D ofCholedochalcyst
Rx
• Surgery followed by CR therapy or Biopsy ,chemotherapy followed by surgery is still a matter of
debate.
• Though recurrence or residual disease is key concern but data showed complete response is possible

39. • .
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