Clinical Pathological Conference

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Clinical Pathological Conference

• Shrujal Baxi, M.D.
• Chief Resident
• Department of Medicine
• November 9, 2007

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Chief Complaint

• An 83 year-old man presents with three days of
  intermittent chest pain

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History of Present Illness

• Six months prior to admission when he noted
  decreased exercise tolerance and was found to
  have a normocytic anemia thought to be
  Myelodysplastic syndrome, but no work up done at
  that time
• About five months prior to admission, pt noted a
  nonproductive, chronic cough that was worse in
  evenings and relieved with prn albuterol therapy
• One month prior to admission, the patient again
  started experiencing increasing shortness of
  breath.
• 5-10lb weight loss over last few months, night
  sweats, subjective fevers

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History of Present Illness

• On day of admission, pt presented with three
  days of intermittent chest pain that was
  substernal and radiated to his left arm and
  shoulder. It was sharp and stabbing in nature
  and worse with inspiration. The episodes would
  last hours and were variably relieved with
  sublingual nitroglycerin.

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• Past Medical History
• Hypertension 20 years
• Diabetes 10 years
• Hypercholesterolemia 10 years
• Past Surgical History
• Appendectomy
• Medications (outpatient)
• Glyburide
• Ramipril
• Atenolol
• Erythropoietin and iron
• albuterol prn

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• Allergies none
• Family History
• Brother died at 55 of MI. No family history of
  malignancy, inflammatory conditions
• Social History
• Born in the United States, patient fought in East
  Asia during World War II. He has no recent
  travel.
• 50 pack year tobacco history, quit 35 years ago.
  No alcohol use. No illicit drug use. Pt lives
  with wife in upstate New York. Pt worked in
  construction prior to retiring at the age of 69.
• ROS otherwise noncontributory

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Physical Exam

• General Well developed male with evidence of
  respiratory distress who appears younger than
  stated age
• Vital Signs BP 105/68 HR 120, regular, RR 20,
  Temp 98.2, SpO2 92 room air
• HEENT Oropharynx clear and dry
• Lymph Nodes No cervical, axillary or inguinal
  lymphadenopathy
• Neck Supple, jugular venous distention difficult
  to assess

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Physical Exam

• Pulmonary Decreased breath sounds at bases, 1/3
  up bilaterally. Dull to percussion
• Heart Decreased heart sounds, tachycardic,
  regular rhythm, pulsus paradoxus of 22
• Abdominal Soft, nontender, nondistended, normal
  bowel sounds, with liver span of 14cm and
  dullness in Traubes space
• Extremities No peripheral edema, 2 peripheral
  pulses
• Skin No rashes, no purpura, no petechia

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Admission Labs
Laboratory On Admission Reference Range
Hemoglobin (g/dl) 10.1 13-18
Hematocrit () 29.5 40-52
White Cell Count (per mm3) 7,200 4,500-11,000
Differential Count ()
Neutrophils 53 42-75
Lymphocytes 22 20-50
Monocytes 7 2-12
Eosinophils 18 0-7
Mean Corpuscular Volume 83.2 80-95
Platelet Count (per mm3) 195,000 150-450,000
MVP 7.3 7.5-10.5
Partial-thromboplastin time, activated (sec) 33.6 23.3-35.6
Prothrombin time (sec) 18.2 10.0-13.8
INR 1.5 .9-1.2
Lactate Dehydrogenase/LDH 348 110-225
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Admission Labs
Laboratory On Admission Reference Range
Sodium (mmol/liter) 141 135-145
Potassium (mmol/liter) 4.1 3.5-5.0
Chloride (mmol/liter) 104 100-110
Carbon dioxide (mmol/liter) 28 24-32
Urea nitrogen (mg/dl) 21 6-22
Creatinine (mg/dl) .7 .4-1.2
Glucose 95 65-115
Calcium (mg/dl) 8.5 8.5-10.5
Magnesium (mmol/liter) 0.8 0.7-1.0
Phosphorus (mmol/liter) 2.9 2.6-4.5
Aspartate aminotransferase (U/liter) 25 10-42
Alanine aminotransferase (U/liter) 18 10-42
Total Bilirubin (g/dl) 2.6 0.1-1.2
Alk Phos 109 42-121
Total Protein (g/dl) 6.1 6.4-8.2
Albumin (g/dl) 4.2 3.8-5.1
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EKG
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Upon Admission

• A prompt cardiac evaluation revealed a moderate
  to large pericardial effusion with right atrial
  collapse with a question of a right atrial mass.
  Pt was admitted to CCU for further evaluation. A
  diagnostic procedure was performed

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T1
T2
STIR
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PATHOLOGY Dr. Hui Tsou Clinical Assistant
Professor Department of Pathology  
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Final Diagnosis

• Diffuse Large B-Cell Lymphoma (DLBCL) with
  primary cardiac involvement
• - CD45, CD20
• - CD3-, CD15-, CD30-, CD10-

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Primary Cardiac Tumors

• Prevalence-.002-.025 at autopsy
• 75 benign in nature
• Systemic embolization is presenting symptom in
  25-50 of cases
• Metastatic tumors 10-40X more likely than primary
  tumor

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Primary Cardiac Tumors

• Benign (75 of all cases)
• Myxoma
• Rhabdomyoma
• Fibroma
• Teratoma
• Malignant (25 of all cases)
• Sarcoma (majority)
• Angiosarcoma
• Rhabdomyosarcoma
• Lymphoma
• Histiocytoma

• Malignant (25 of all cases) Sarcoma
• Angiosarcoma
• Rhabdomyosarcoma
• Fibrosarcoma
• Leiomyosarcoma
• Other
• Lymphoma
• Histiocytoma

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Primary Cardiac Lymphoma (PCL)

• Defined as presence of Non-Hodgkins Lymphoma
  confined to the heart or pericardium
• PCL represents lt2.0 of 1 cardiac tumors and
  0.5 of extranodal lymphomas
• More common in immunocompromised
• Increased incidence due to AIDS and improved
  imaging techniques

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Lymphoma

• Now the 5th most common cancer diagnosed in both
  men and women
• Represent 4 of all cancers
• Approximately 63,000 cases diagnosed annually
• Age at diagnosis is 60 with more than 50 over
  the age of 65
• 5 year survival is 63 and 10 year survival is 49

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Pathophysiology
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Pathophysiology
Assignment of Human B-Cell Lymphomas to Their
Normal B-Cell Counterparts
Kuppers R et al. N Engl J Med 19993411520-1529
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Pathophysiology
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PCL

• Common presentations of this uncommon diagnosis
  are based on location of tumor
• Right-sided heart failure
• Precordial chest pain
• Pericardial effusion
• Superior vena cava syndrome
• Arrhythmia
• CHF
• Constitutional Symptoms

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Pathogenesis of Disease
Tumor Mass from replicating atypical lymphoma
cells
Environmental Factors
Release of Cytokines (TNF, IL-6)
Mutation to Oncogene of Lymphoid Cell
Tissue invasion of right atrium and septal wall
Weight Loss
Night Sweats
Pericardial Effusion
Atrial Fibrillation
Anemia of Chronic Disease
Pleural effusions
cough
dyspnea
chest pain
fatigue
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Diagnostic Studies

• Labs ? LDH, ? IL-2, ? ESR
• ECG AV block, RBBB, Inverted T waves, Low
  voltage
• CXR Pleural Effusion and/or Cardiomegaly
• Echocardiography
• Hypoechoic masses in the R atrium with
  pericardial effusion
• TTE difficulty visualizing pulmonary vessels,
  SVC, R atrium

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Diagnostic Studies

• CT
• Appears hypodense or isodense relative to
  adjacent myocardium
• Contrast heterogenous enhancement
• MRI
• T1 images Hypointense and Dark
• T2 images Hyperintense and Bright
• Gadolinium Heterogenous enhancement
• Useful in making diagnosis and assessing response
  to RX
• Nuclear medicine techniques
• Gallium 67
• Technetium-99m hexakis-2-methoxyisobutyl
  isonitrile
• Thallium-201

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Diagnostic Studies

• Tissue is the Issue
• Pericardial fluid
• Diagnostic in 67 of cases
• Tissue biopsy
• Mediastinoscopy
• Thoracoscopic biopsy
• TEE guided biopsy
• Endomyocardial transvenous biopsy
• Exploratory thoracotomy

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Treatment

• Treatment for DLBCL is the chemotherapy regimen
  of R-CHOP
• RRituximab
• CCyclophosphamide
• HAdriamycin
• OVincristine
• PPrednisone
• Alternative regimens include
• COP
• CHOP
• Bone Marrow Transplant

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Follow-Up

• Upon admission, pt had pleural and pericardial
  drains placed
• While work-up continuing, patient developed rapid
  afib controlled with low-dose b-blocker
• Due to concern of significant atrial wall
  involvement of disease, first 2 cycles of R-CHOP
  given in CCU setting with continuous cardiac
  monitoring
• Patient is currently disease free after receiving
  a complete course of R-CHOP

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Thank you

• Dr. Srichai-Parsia
• Dr. Kahn
• Dr. Hui Tsou
• Dr. Blaser
• Dr. Grieco
• Dr. Ballard
• Dr. Mark Fisch