Liver mass

Question 1

What to do with a patient presenting w/ fever + neutropenia?

Answer 1

Start empiric antibiotic tx ASAP for potential sepsis, continue until an infectious bacterial process has been r/o

Question 2

MC liver masses in infants <1 y?

Answer 2

Hemangioma, Hamartoma

Question 3

What liver masses require a confirmatory biopsy?

Answer 3

Hepatoblastoma, Hepatocellular carcinoma

Question 4

Potential infectious etiologies for liver mass?

Answer 4

Abscess, viral hepatitis, Entamoeba Hystolytica (if Hx international travel)

Question 5

MC cause of febrile neutropenia?

Answer 5

viral suppression of bone marrow function

Question 6

What are inflammatory pseudotumors (IPT)?

Answer 6

Rare, benign, solitary and well demarcated lesions caused by proliferation of inflammatory cells

Question 7

IPT synonyms

Answer 7

Inflammatory myofibroblastic tumor
Plasma cell granuloma
Fibrous Histiocytoma
Fibroxanthoma
Xanthogranuloma

Question 8

IPT locations

Answer 8

MC: Lungs
Extrapulmonary sites: 
Head/neck, orbit, salivary glands, lymph nodes, breast
Kidney, GU tract
Pancreas, retroperitoneum
Liver, spleen, GI tract
Soft tissue

Question 9

IPT clinical manifestations

Answer 9

Usually asymptomatic, incidental findings

In extremely rare cases they can cause mass effect

Question 10

IPT laboratory findings?

Answer 10

Elevated WBC count, elevated acute phase reactants (ESR, CRP), abnormal LFTs
Uncommonly: cytopenias (Anemia, Leukopenia/Neutropenia/Thrombocytopenia)

Question 11

IPT imaging tests

Answer 11

US, CT, MRI, PET (positron emission tomography)

Question 12

if Dx unknown, what to do?

Answer 12

Fine-needle aspiration (FNA) or open biopsy/resection

Question 13

IPT pathogenesis

Answer 13

• Cellular insult –> aberrant inflammatory response
• Activation inflammatory cells –> release of Cytokines –> activation: neutrophils, eosinophils, lymphocytes, plasma cells, Histiocytes, multinucleated giant cells, myofibroblasts and spindle-shaped cells –> cellular damage + IPT formation

Question 14

IPT etiology

Answer 14

Unknown, but 3 possibilities have been considered:
*Postinfectious, *Autoimmune, *Neoplastic (IPT > postradiation tx for malignant neoplasms, Some IPTs w/ + genetic testing for malignancy, suggesting some could be: low grade mesenchymal neoplasms + secondary inflammatory component)

Question 15

IPT (immune etiology

Answer 15

Due to IgG4-related disease: an immune mediated condition characterized by:
lymphoplasmacytic infiltration
Predominant IgG4 + plasma cells
Elevation of IgG4
Dramatic response to glucocorticoids when adm

Question 16

Viral Neutropenia

Answer 16

Transient, usually mild to moderate, rarely leads to superimposed bacterial infections, improves in 3-8 days
MC viruses (mild to moderated N): Influenza A and B, RSV, Parvovirus, Measles, Rubella, Varicella
However, Viruses causing greater suppression include:
EBV, CMV, Human herpesvirus 6 (HHV-6), Hepatitis, HIV
–> superimposed bacterial infections

Question 17

Autoimmune Neutropenia (AIN)

Answer 17

By granulocyte-specific antibodies
Typically detected incidentally btw 7-9 months of age
ANC ranging 0.5-1.0 K/mcL
Dx confirmation:
Autoantibody detection
Indirect-GIFT (Indirect granulocyte immunofluorescence test)
GAT (Granulocyte agglutination test)
Tx: Close monitoring, spontaneous remission and disappearance of autoantibodies

Question 18

Neutropenia causing recurrent or severe infections, or Pt needing a surgical procedure

Answer 18

Filgrastim-G-CSF (Granulocyte colony stimulating factor)

Question 19

Refractory cases of Neutropenia, management?

Answer 19

Intravenous immunoglobulin (IVIG)
CS
Monoclonal antibodies

Question 20

IPT Tx

Answer 20

-Usually conservative, if no clinical improvement –> surgical (full) resection
1st line: outpatient monitoring –> serial imaging and labs
>surgery, most IPT cases do not recur
-Antibiotics, NSAIDs (varying degrees of success)
-If IgG4-related disease suspected: Glucocorticoids to help tumor regression

Question 21

Differential Dx Liver mass in infants

Answer 21

• Hematologic
• Oncologic
• GI
• Infectious

Question 22

Hematologic causes of liver mass in infants

Answer 22

IgG4-related disease
Autoimmune neutropenia
Kostmann Syndrome
Congenital neutropenia

Question 23

Oncologic causes liver mass infants

Answer 23

Hepatoblastoma
Hepatocellular adenoma
Hepatocellular carcinoma
Focal nodular hyperplasia
IPT

Question 24

GI causes liver mass infants

Answer 24

Hepatic hemangioma
Hepatic hamartoma
Simple hepatic cyst
Polycystic liver disease
Hydatid cyst

Question 25

Infectious causes liver mass infants

Answer 25

Hepatic abscess
Viral hepatitis
Epstein Barr Virus
CMV
Entamoeba Hystolytica