Cholangiocarcinoma

BY

DR. ABIODUN POPOOLA

CASE SUMMARY

NAME: A.S

GENDER: MALE

AGE: 57 YRS

OCCUPATON: Politician

RELIGION: CHRISTIAN

MARITAL STATUS: MARRIED

Presenting Complaint

Recurrent yellowness of the eyes x 6months

Progressive loss of weight x 5 months

Bilateral leg swelling x 2wks

History

Associated with dark coloured urine, pale colouredstool as well as pruritus.

No history of fever or abdominal pain or swelling

Developed intermittent bilateral leg swelling 2 weeks prior to presentation, no associated history of cough ,dyspnea or chest pain

No history of easy bruising or altered sleep pattern, no haematemesis, or passage of melena stool

No history of nausea , vomiting, abdominal pain or swelling, no change in bowel habits .

History

He is not a known hypertensive or diabetic nor peptic ulcer disease patient

No history of blood transfusion

No history of previous surgery

Does not smoke but drinks alcohol 2 bottles per day

Physical examination

Middle age man, not in any distress, not pale, icteric+, acyanosed, afebrile, nil pitting pedal edema.

Abdomen: No areas of tenderness. Liver is not enlarged, kidneys are not ballotable bilaterally and the spleen is not enlarged

DRE: good anal hygiene,normal sphincteric tone examining finger stained with pale stool

Vitals: PR: 66b/m BP: 113/71mmhg

Investigations

Biochemical: 20/6/17

LFT Result Ref. ranges Result Ref.

ranges

Total

bilirubin

18.14mg/dl 0-1.0mg/dl Total protein 7.25g/dl 6.3-8.6g/dl

Direct

bilirubin

9.41mg/dl 0-0.6mg/dl Albumin 3.74 g/dl 3.7-5.6g/dl

Indirect

bilirubin

8.73mg/dl 0.1-1.0mg/dl globulin 3.57g/dl 1.8-3.6g/dl

SGOT 196.8 iu/l 5-40iu/l A/G ratio 1.07 1.1-2.2g/dl

SGPT 168 iu/l 7-45iu/l

ALP 550iu/l 70-260iu/l

GGT 797.3 iu/l 8-78iu/l

Renal

function test

Result Units Reference range

Na+ 132.9 mmol/l 135-145

K+ 4.16 mmol/l 3.5-5.5

Cl- 98.9 mmol/l 96-109

Hco3- 20.01 mmol/l 17-31

Urea 16.94 mg/dl 22-50

Cr 0.23 mg/dl 0.9-1.4

fbs 106 mg/dl 70-110

Tumour markers:

Markers Result Reference ranges

Alpha feto protein 1362 iu/ml 0-7iu/ml

Ca 19:9 297 u/ml 0-37u/ml

CEA 3.3 ng/ml Smoker: 8.5ng/mlNon-smoker: 5.0ng/ml

Hematologic:

Parameter Result Unit Reference ranges

Full Blood Count

PCV/Haematocrit 43.3 % 40.5-52.5

WBC 7230 mm3 3500-11000

Neutrophils 61.7 % 40-75

Lymphocytes 29.1 % 19.0-46.0

Monocytes 5.5 % 2.5-10.0

Platelet count 359,000 /mm3 100,000-400,000

Radiological:

CXR –Normal.

Abdominopelvic USS- 20/6/17:

Liver is normal sized, spans 153mm.

The parenchymal echotexture is normal.

The common bile duct is dilated to 12mm and contains an irregularly shaped isoechoic mass measuring 38x36mm

USS

Radiological:

CT abdominopelvic scan done 21/6/2017 revealed minimally enhancing soft tissue density lesion in almost the entire common bile duct with abrupt narrowing at the proximal CBD with dilatation of the hepatic ducts and IHBR

Gall bladder is overdistended with hypo to isointense content and calcification along lateral wall.

There is loss of fat plane with liver with area of hypodensity in adjacent liver parenchyma measuring 25 x14mm(Liver infiltration)

MRCP not done

Biopsy not done

SUMMARY57 year old man who presented with 6months recurrent yellowness of the eyes,

progressive weight loss of 5 months and bilateral leg swelling of 2wks.

Abnormal liver enzymes.

High Alkaline Phophatase value -550iu/l

High GGT value 797.3 iu/l

High Alpha feto protein value 1362 iu/mlHigh C A 19. 9 value 297 u/mlUSS -Dilated CBD due to bile duct massCT-Soft tissue density lesion in almost the entire common bile duct with abrupt narrowing at the proximal CBD with dilatation of the hepatic ducts and IHBR

Overdistended gall bladder Stage IIIB -

Based on the above findings he was then managed as a case of cholangiocarcinoma

He received 6 courses of chemotherapy (gemcitabine and eloxatin) with pre-chemotherapy workup which he completed in February in 2018 and there was significant regression of symptoms .

Follow up

An abdomino-pelvic CT scan done 23/01/18 compared to the pre chemotherapy CT scan shows a stable residual disease.

He however presented with progressive right abdominal swelling associated with right hypochondialpain in august 2018.

A comparative abdominopelvic CT scan done showed interval progression of the disease, the gall bladder mass with liver infiltration is larger. There is also interval infiltration of the hepatic flexure.

Follow

Ca 19.9 : 25.94IU/ml (0-37)

CEA : 2.65 IU/ml

He was worked up for a second line chemotherapy but he defaulted.

He re-presented on 19/11/18 at the surgical emergency with complains of inability to pass stool, distended abdomen, hiccups.

Abdominopelvic CT scan done same day revealed extensive progression of disease process with involvement of colon, duodenum, liver metastasis ,increased biliary dilatation . with bowel perforation as a new finding.

LFT 21/11/18LFT

Total bilirubin 13.35Mg/dl Total protein 6.94g/dl

Direct bilirubin 9.49Mg/dl Albumin 2.53 g/dl

Indirect bilirubin 4,71Mg/dl globulin --

SGOT 146.8 iu/l

SGPT 60 iu/l

ALP 427 iu/l

GGT 197.3 iu/l

CXR; nodular opacities in both lung fields

Ass: Metastatic deposit.

His condition further deteriorated despite necessary supportive treatment and passed on 22/11/18.

CHOLANGIOCARCINOMADR POPOOLA A.O

CASE 2

Name: Mr. S. O.

Age: 36 years

Address: Ago Palace Way Isolo.

Religion: Christian

Occupation: Freight forwarder

He was referred from GASTRO clinic on the 2nd of April, 2015 with complaints of

• Weight loss x 6 months

• Yellowness of the eyes x 4 months

History of presenting complaints.• Weight loss was progressive despite good appetite at onset of

illness, though he developed anorexia with early satiety later on, associated with generalized weakness.

• Yellowness of the eyes was associated passage of coke coloured urine, pale stools and generalized pruritus. No hx of contact with indiv with jaundice,

• No hx of Sharing of sharps, scarification marks, tattoos, blood transfusions.

• Px does not take alcohol, herbal concoctions or smoke cigarettes

Medical/ Surgical history

• Px is not a known hypertensive, diabetic or PUD px. Genotype is AA and blood group O+.

• Patient had a background history of recurrent diarrhea of 16years duration for which 4yrs prior to presentation, he visited Gwagwalada Specialist Hospital Abuja where he did a colonoscopy and was diagnosed as having Ulcerative Colitis

• He was placed on Tabs Mesalazine 800mg bid, Tabs Prednisolone 30mg dly, Tabs Metronidazole 400mg tds, Tabs Ciprofloxacin 500mg bid

Family

• Married .

• Positive family history of similar symptoms in elder sister who died a year earlier from cholangiocarcinoma, no known family hx of hypertension, diabetes, sickle cell disease, or malignancy.

SUMMARY

36 year old man who presented with 6months history of progressively weight loss and symptoms suggestive of obstructive jaundice.

He has a background history of diarrheal disease of 16years for which he had colonoscopy and histologic diagnosis of ulcerative colitis.

He has a positive family history of cholangiocarcinoma in a deceased sister.

Examination findings

• A young man, chronically ill looking ,pale, icteric, afebrile, acyanosed, not dehydrated, grade 3 digital clubbing, nil peripheral lymphadenopathy, nil pedal oedema.

ABDOMINAL EXAM: Abdomen was full, moved with respiration, no areas of tenderness. The liver was 4cm palpable below the right costal region , liver span was 16cm, soft non tender, smooth surface.

DRE: No rectal masses, gloved finger was stained with brownish stool.

CVS: PR- 92bpm, mod. Volume, regular; BP- 100/60mmHg.

CNS: No signs of Overt encephalopathy

Other system examinations were essentially normal.

Work up plan

• LFT, FBC+ ESR, Stool for occult blood and m/c/s,

• Serology; HIV, HBsAg, Anti HCV,

• Tumour markers; CEA, CA19-9, AFP.

• Abdominal USS, ABD CT Scan, Repeat Colonoscopy.

Results

• Abd USS finding- Normal sized liver with homogenous parenchyma and vascular architecture. The hepatic vessels appear normal. However, the portal channels and biliary tree are dilated. No intrahepatic mass seen. But there is a well defined tissue mass at the portal hepatis measuring 1.9 by 1.8cm

• The gall bladder is normal in size but the wall is thickened with a halo of fluid. No gall stones sighted.

• Pancreas, spleen, kidneys and GIT are normal.

• IMPRESSION- (1). Cholecystitis (2). Intrahepatic biliary obstruction (3). portal hepatis lymphadenopathy

• ABD CT Scan- shows hepatomegaly with intra hepatic ductal dilatation, no intrahepatic mass with dilated large bowel loops

Investigation Results

• LFT showed Increased transaminases; ALT (100mg/dl) 21/2 x ULN

• ALP (837 IU/L) 3X ULN, Conjugated hyper bilirubinaemia, hypo albuminaemia ( 3.2mg/dl), Normal total protein

• Clotting profile- PT= 21.0s (control 16.0s) INR= 1.8

• FBC +ESR; PCV-38% HB- 12.4g/dl, WBC- 8.4x 109/l, differentials- N- 51% L- 35% M-10% E- 4%, Platelet- 92x 109/l. ESR- 100mm/ hr.

• Stool m/c/s, occult blood- FOBT= Negative, starch granules present.

• HIV- Negative

• HBsAg- Negative

• Anti HCV- Negative

Results contd

• Tumour markers- CEA- 0.61ng/ml (<5.0ng/ml) ,CA19-9 688ng/ml (<40ng/ml), AFP – Normal

• Assessment now was that of Inflammatory Bowel Disease complicated by Cholangiocarcinoma.

• He was asked to do a repeat colonoscopy and a consult was written to see the general surgeons.

• However, px opted to travel in June 2015 to South Africa for further treatment since he has family members there.

• After series of investigations in SA which included ERCP, Abd CT Scans, MRI, Colonoscopies, biopsy with histology, he was diagnosed as having

• 1. Cholangiocarcinoma type 3A-Klatskin tumour and hilar mass with invasion of the right and left portal veins and

With extensive lymphadenopathy- hepatoduodenal and supra pancreatic lymph nodes.

• 2. Colonic high grade dysplasia

• He subsequently had a Right lobe hepatectomy with portal vein reconstruction and also a hepaticojejunostomy.

• He had an eventful post op period with episodes of wound breakdown and drainage of pus from wound site, also developed post op Ascites.

• Abd CT Scan done a month after the surgical procedure showed left common iliac vein thrombosis extending into the internal and external branches.

Post op treatment

• After spending a year in SA, Px had to return to Nigeria due to expiration of his Visa.

• He was referred to continue follow up visits .

• Also he was to be on yearly repeat surveillance colonoscopy since he had a high risk for colon cancer and may need total colectomy

LASUTH Gastro/Oncology clinic review• A month after returning to the country, he attended follow up

clinic . His clinical condition was found to have deteriorated and he was offered admission which he declined.

• Repeat FBC showed Anaemia with leukocytosis. Also LFTs done were deranged and also had elevated urea and creatinine values.

• A week later he was rushed to the surgical emergency with features of multiple organ failure (liver, kidney, respiratory ) complicated by sepsis and hepatic encephalopathy.

• He died within 30 minutes of presentation.

???• The role of -CHEMOTHERAPY

-RADIOTHERAPY

-PALLIATIVE CARE

in advanced Cholangiocarcinoma.

•THANK YOU