Clinicopathological Conference November 11, 2005

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Clinicopathological ConferenceNovember 11,
2005

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History

• An 84-year-old woman was brought to the ER of the
  WFH on Dec 9, 2004 because of disturbance in
  consciousness for one day.

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History

• 84 y/o woman, was noted to have disturbed
  consciousness by her family on the night of Dec 8
• Brought to NTUH
• Urosepsis was the initial impression
• No bed available
• Transfer to WFH on Dec 9

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History

• On arrival, physical examination
• GCS E4V4M1, BP 121/80 mmHg, TPR 36/93/16
• Bilateral basal rales, irregular heart beat,
  murmur (-)
• Abdomen distended and tenderness over RUQ, with
  positive peritoneal sign
• No pitting edema of the lower extremities

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History

• Laboratory examinations
• WBC 8760/ul
• Neut Lymph Mono Eos Baso
• 65.3 21.2 13 0.3 0.2
• Hb 9.6gm/dl Platelet 104K/ul
• PT 23.6 sec INR 2.11 APTT 45.2/35.2
• AST 70U/L, ALT 97 U/L, Bil T/D 1.96/1.04
• Alb 3.3g/dl, amylase 62U/L, lipase 226 U/L
• U/AWBC gt 100/HPF

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History

• Brain CT old territorial infarction at the right
  PCA and left MCA
• Abd CT gallstones, 2 hepatic tumors (peripheral
  part of S5 and left lobe) with hemoperitoneum,
  pancreatic tail tumor, bilateral renal cysts,
  contracted left kidney and suspected left renal
  left adnexal tumor

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History

• Ruptured hepatoma with hemoperitoneum was
  impressed
• Emergent angiography was done hypervascular
  tumor in right lobe of liver, no active bleeder
  nor extravasation found
• TAE was performed to obliterate tumor vessels
• Transferred to SICU for intensive care

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Past medical history

• Diabetes mellitus, hypertension for 50 years
  under medical control
• CAD s/p PTCA 10 years ago
• Atrial fibrillation
• Cerebral infarction in the territory of left MCA
  in 2003, with sequelae of right hemiplegia,
  dysphagia and aphasia
• Chronic hepatitis B

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Clinical course

• In SICU, unstable hemodyanamics despite fluid
  challenge and blood transfusion
• Repeat abd CT ongoing internal bleeding was
  suspected
• Family refused surgical exploration and any
  invasive treatment

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Clinical course

• Respiratory distress developed since Dec 13
• ABG respiratory acidosis
• CXR opacification of left lung
• Endotracheal intubation was not done due to
  family had signed DNR
• Condition deteriorated
• Patient died at 730 pm on Dec 14, 2004

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Laboratory Data- hemograms
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Laboratory Data- Coagulation profile
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Laboratory Data- Biochemistries
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Laboratory Data- Biochemistries
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Laboratory Data- Urinalysis
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Laboratory Data- Arterial blood gas
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Image Studies

• - to be presented

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Summary

• This 84 y/o woman, with past history of DM,
  hypertension, CAD, old cerebral infarction,
  atrial fibrillation, chronic hepatitis B was
  admitted because of consciousness disturbance
• Abd CT revealed ruptured hepatic tumor with
  hemoperitoneum pancreatic tail cystic tumor
• Emergent TAE was performed
• Unstable hemodynamics persisted despite fluid
  challenge and blood transfusion
• Respiratory distress developed 4 days after TAE
• ? Death

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Discussion

• Etiologies and management of ruptured hepatic
  tumor with hemoperitoneum
• Differential diagnosis of pancreatic cystic
  tumor
• Benign or malignant?
• Primary or metastasis?
• Multiple primary malignant neoplasm (MPMN)
• Causes of death
• Hypovolemic shock
• Respiratory failure

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Ruptured hepatic tumor with hemoperitoneum
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Ruptured hepatic tumor with hemoperitoneum-
Etiologies

• Malignant
• Hepatocellular carcinoma
• Secondary liver cancer

• Benign
• Cirrhosis
• Hepatic angioma
• Hepatic adenoma

• For this patient
• History of chronic hepatitis B with liver
  cirrhosis
• 2) AFP 1066.49 ng/ml
• ? Favor ruptured hepatoma with hemoperitoneum

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Ruptured hepatic tumor with hemoperitoneum-
Etiologies

• Malignant
• Hepatocellular carcinoma
• Secondary liver cancer

• Benign
• Cirrhosis
• Hepatic angioma
• Hepatic adenoma

• For this patient
• History of chronic hepatitis B with liver
  cirrhosis
• 2) AFP 1066.49 ng/ml
• ? Favor ruptured hepatoma with hemoperitoneum

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Ruptured hepatoma with hemoperitoneum

• Life threatening condition
• Incidence 5-26
• Mortality high 33-67
• Clinical manifestations
• Sudden onset of abdominal pain, hypovolemic
  shock, abdominal distension etc
• ( may be misdiagnosed if symptoms are
  atypical)

Am J of Emergency 2005 23 730-736
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Ruptured hepatoma with hemoperitoneum

• Mechanisms
• Central necrosis in a rapidly growing hepatoma
• ? predispose the numerous pathological
  dilated vessels within the tumor ? hemorrhage and
  venous congestion inside the tumor
• Coagulopathy due to the underlying cirrhosis
• Minor trauma cause a sudden increase in pressure
  within the tumor

Eur J Surg Oncology 2000 26 770-772
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Ruptured hepatoma with hemoperitoneum

• Characteristics of ruptured hepatoma
• Large tumors
• Tumors with hump sign
• Minimal thickness of peritumor liver parenchyma
• Tumors in left lobe

J Clin Gastroenterology 1995 21(3) 238-242
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Ruptured hepatoma with hemoperitoneum- Diagnosis

• History of hepatoma
• Typical clinical symptoms- sudden onset of
  abdominal pain, peritoneal sign, shock.
• Abdominal paracentesis revealed
• non-coagulable blood
• Abdominal CT scan

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Ruptured hepatoma with hemoperitoneum- Management

• Conservative treatment
• Transcatheter arterial embolization (TAE)
• Surgical treatment

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Management of ruptured hepatoma with
hemoperitoneum

• Conservative treatment
• Is effective if there was no evidence of
  hemodyanamic instability
• For patients with poor general condition or poor
  liver reserve (surgical intervention or TAE may
  resulted in hepatic failure)
• With/without elective surgery

Dig Surg 2002 19 109-113
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Management of ruptured hepatoma with
hemoperitoneum

• Transcatheter arterial embolization (TAE)
• Blood supply of hepatoma is mainly by hepatic
  artery
• Less invasive than surgical intervention
• Chemoembolization or embolization
• Demonstrated - hypervascular tumor
• - extravasation
  0-23.5 (angiography detects bleeding rate 1
  ml/min)

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Management of ruptured hepatoma with
hemoperitoneum

• Transcatheter arterial embolization (TAE)
• Control bleedding 70-100
• Preclude in patients with portal vein thrombosis
• One month survival rate 62 (vs 50 29 in
  patients underwent emergency operation
  conservative treatment) Am J Emer Med 2005 23
  730-736
• Major causes of death after emergent TAE
• Uncontrolled bleeding
• Rerapture (35 ) Radiat Med 19886
  150-156.
• Hepatic failure

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Management of ruptured hepatoma with
hemoperitoneum

• Surgical hemostasis
• Ligation of the hepatic artery, suturing of the
  bleeding tumor, perihepatic packing, resection of
  the ruptured tumor
• Mortality 4-75
• 3- year disease free survival rates 14.5 (vs
  39.9 non-ruptured case)
• No difference in the rates of peritoneal
  dissemination, distant metastasis or intrahepatic
  recurrence between ruptured and non ruptured
  group
• 
  
  Hepatogastroenterol 1995 42
  166-168
• Not suitable in patients with poor liver reserve

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Am J Emer Med 2005 23 730-736
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Ruptured hepatic tumor with hemoperitoneum-
Prognostic factors

• Early mortality of ruptured hepatoma was
• determined by
• Prerupture disease state
• Liver function at the time of rupture
• Severity of hemorrhage
• Was not dependent on the modality of immediate
• treatment

J Clin Oncol 2001 19 3725-3732
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Management of ruptured hepatoma with
hemoperitoneum

• Individualized !

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Ruptured hepatoma with hemoperitoneum

• Ongoing internal bleeding was found via
• repeated abdominal CT in this patient
• Causes failed embolization ? Rerapture?
• Management- repeat TAE ? surgical
• intervention ?

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• Pancreatic cystic tumor

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Pancreatic cystic tumor

• Benign / Borderline neoplasms / Malignant
• Primary cystic tumors / Result from the cystic
  degeneration of solid tumors

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Pancreatic cystic tumor

• Neoplastic epithelial tumors
• Intraductal papillary mucinous neoplasm ( IPMN )
• Mucinous cystic neoplasm ( MCN, MCT )
• Serous cystic neoplasm ( SCN )
• Solid-pseudopapillary neoplasm ( SPN )
• Acinar cell cystadenoma/carcinoma
• Neuroendoscrine tumor, cystic
• Ductal carcinoma , cystic change
• Metastatic tumor, cystic change

• Neoplastic nonepithelial tumors
• Lymphangioma
• Nonneoplastic epithelial tumors
• Retention cyst
• Nonneoplastic epithelial tumors
• Pseudocyst
• Parasite cyst

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Characteristics of pancreatic cystic neoplasms

• NEJM 2004 3511218-1226

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Characteristics of pancreatic cystic neoplasms
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Characteristics of pancreatic cystic neoplasms
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Microcystic v.s. macrocystic neoplasm
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Characteristics of pancreatic cystic neoplasms
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(A)
(B)
IPMN branch duct type (A), main duct type (B),
combined type (C). a adenocarcinoma, b
adenoma, c hyperplasia, m mucus.
(C)
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Characteristics of pancreatic cystic neoplasms
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Characteristics of pancreatic cystic neoplasms
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Cystic pancreatic tumor
Pseudocyst VHL disease Adult PCKD Parasitic cyst
Pseudocyst Microadenoma Macroadenoma Ductoectatic
adenocarcinoma
multiple
solitary
Fibrous wall (pseudocyst)
Cyst size ? gt or lt 2 cm
1.Renal cysts ? 2.Hemangioblastoma ? Solid
pancreatic tumor ? Family history 3. Infectious
S/S history ?
Unilocular or multilocucar ?
Calcification Sunburst or mural?
Vascularity hypo or hyper
Age, Gender Serum tumor markers EUS, FNAB CT,
ERCP, MRCP
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Nature of the pancreatic cystic tumor in this
patient ?

• Primary pancreatic tumors
• IPMN ?
• Ductal adenocarcinoma with cystic change?
• Nonpancreatic primary tumors with metastasis to
  the pancreas
• Hepatoma with pancreatic metastasis ?

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Nature of the pancreatic cystic tumor in this
patient ?

• Nonpancreatic primary tumors with metastasis to
  the pancreas Uncommon. Colon, lung, breast,
  kidney, stomach, ovaries, melanoma etc
• Hepatoma with pancreatic metastasis ?
• Extrahepatic metastases of HCC 64
• - lungs, regional lymph node, kidney, bone,
• adrenal glands
• Rarely metastasis to pancreas (2.7-5.6)
• mainly direct invasion
• Less likely in this patient.

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Nature of the pancreatic cystic tumor in this
patient ?

• Primary pancreatic tumors
• IPMN ?
• Ductal adenocarcinoma with cystic change?

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Abdominal CT

• Bilateral renal cysts , contracted kidneys, left
  renal lesion
• Left adnexal lesion

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Multiple Primary Malignant Neoplasm (MPMN)

• First described by Billroth in 1889
• Prevalence 0.734-11.7
• MPMN may occur at any age, but the patients with
  MPMN tend to be older than those with single
  primary malignant neoplasm
• Most malignancies with MPMN involve those of
  respiratory, gastrointestinal and genitourinary
  systems
• Preponderance of men with MPMN is caused
  primarily by the high frequency of prostatic
  cancer

Am J Clinc Oncol 200326(1) 79-83
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Am J Clinc Oncol 200326(1) 79-83
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MPMN - Synchronous vs Metachronous

• Synchronous (34)
• Predominance of men
• Smokers and drinkers
• Frequency of aerodigestive tumors are higher
• Lower frequency of mesenchymal tumors
• Older

• Metachronous (66 )
• Younger
• Percentage of breast tumors was higher in the
  metachronous primary tumor group
• Frequency of lung cancer was higher among
  synchronous and metachronous secondary tumors

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Am J Clin Oncol 2000 23(4) 364-370
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Multiple Primary Malignant Neoplasm (MPMN)

• Genetic factors / Environmental factors
• Embryologically similar tissues might develop
  multiple primary tumors when exposed
  simultaneously to certain hormones or to
  carcinogens
• Field cancerization epithelial lining exposed to
  tobacco and alcohol? premalignant / malignant
  cytologic changes? increased risk for multiple
  independent tumor development

Am J Clin Oncol 2000 23(4) 364-370
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Causes of death

• Uncontrolled internal bleeding ? Hypovolemic
  shock
• Respiratory distress (occurred 4 days after TAE)?
  Respiratory failure
• ABG respiratory acidosis
• no apparent hypoxemia
• CXR opacification of left lung- collapsed
  left
• lung
• ( Pulmonary embolism is not likely in this
  case)

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Final clinical diagnosis

• Ruptured hepatoma with hemoperitoneum
• and hypovolemic shock
• Respiratory failure
• Pancreatic cystic tumor IPMN ? Ductal
  adenocarcinoma with cystic change ?
• Left renal and adnexal tumor- nature?
• Urinary tract infection
• Chronic hepatitis B
• DM, HTN, old cerebral infarction, CAD

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Thank you for your attention!