Neuroblastoma Flashcards
What is the most common malignancy in infants?
Neuroblastoma
What is the most common extracranial solid tumor of childhood?
Neuroblastoma
Neuroblastoma is most common in what race?
Caucasians
Pathophysiology of Neuroblastoma
- SNS
- Solid, soft mass with areas of hemorrhage, calcification, and necrosis
- Small, round blue cell (psuedorosettes)
From a molecular standpoint, what signifies the most aggressive tumor?
N-myc
Clinical presentation of neuroblastoma
Abdominal mass –> abdominal distension, pain, constipation, blueberry muffin sign
- Paraspinal mass –> spinal cord compression with change in gait or sensation, avoiding use of limb
- Bone metastasis (bone pain, limp, if orbital involvement proptosis)
- Bone marrow involvement (low grade fever, maliase)
Between Wilms and neuroblastoma, which is “looks like shit?”
Neuroblastoma
What is commonly involved when it comes to bone metastasis?
Orbital/skull invovleemnt
Further clinical presentation of neuroblastoma
- Horner syndrome (ptosis, miosis, anhidrosis)
- Catecholamine secretion –> HTN
- Intractable diarrhea due to VIP
Diagnostic workup of neuroblastoma
- H and P
- CT or MRI
- Bone films
- MIBG scan**
- Urinary catecholamines (VMA, HVA)
- Biopsy tumor tissue for n-myc, ALK mutation, other studies)
Common sites of metastasis
- Bone marrow
- Bone (orbits/skull, extremities)
- Lymph nodes
- Soft tissue
- Liver
- Lung
- Late stage = leptomeningeal involvement
What stage is when a patient is diagnosed under one year of age?
Stage 4S
- Distant metastasis present
- Huge liver
Stage MS (under 18 months, NO BONE METS)
What patients do the best?
-Stage 4S
What patients are classified as high risk?
- N-myc amplification
- Ploidy
Treatment is based on what?
- Age
- Stage
- Risk category
