Neuroblastoma (peds): Difference between revisions
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==Background== | ==Background== | ||
*Arises from primitive ganglion cells of sympathetic nervous system | *Arises from primitive ganglion cells of sympathetic nervous system | ||
*Synthesize and secrete catecholamines | *Synthesize and secrete catecholamines | ||
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*Depends on location | *Depends on location | ||
**Abdominal mass | **Abdominal mass | ||
***2/3 of primary neuroblastoma arise in the abdomen | ***2/3 of primary neuroblastoma arise in the abdomen<ref>https://www.uptodate.com/contents/clinical-presentation-diagnosis-and-staging-evaluation-of-neuroblastoma?search=neuroblastoma%20children&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1</ref> | ||
**Abdominal pain or constipation | **Abdominal pain or constipation | ||
**Proptosis or periorbital ecchymoses | **Proptosis or periorbital ecchymoses | ||
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==Evaluation== | ==Evaluation== | ||
*Catecholamines; VMA and HVA of serum and urine | *Catecholamines; VMA and HVA of serum and urine | ||
*CT or MRI | *CT or MRI | ||
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[[Category:Pediatrics]] | [[Category:Pediatrics]] | ||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Revision as of 11:14, 23 April 2022
Background
- Arises from primitive ganglion cells of sympathetic nervous system
- Synthesize and secrete catecholamines
- Most common neoplasm in 1st yr of life
- May arise anywhere along sympathetic nervous system
- Adrenal gland (40%)
- Abdomen (25%)
- Chest (15%)
- Mets are most common in lymph nodes, bone marrow, cortical bone, dura, orbits, liver, and skin
Clinical Features
- Depends on location
- Abdominal mass
- 2/3 of primary neuroblastoma arise in the abdomen[1]
- Abdominal pain or constipation
- Proptosis or periorbital ecchymoses
- Horner syndrome: miosis, ptosis, anhidrosis
- Paraspinal mass
- Spinal cord compression, which can cause localized back pain or weakness
- Systemic symptoms, bone pain, anemia
- Abdominal mass
- Opsoclonus-myoclonus: rapid, dancing eye movements, rhythmic jerking of trunk or limbs, and/or ataxia
- paraneoplastic syndrome highly associated with neuroblastoma
- Neutropenia/pancytopenia suggests bone marrow involvement
Differential Diagnosis
- Wilms' tumor
- Hepatoblastoma
- Lymphoma
- Other adrenal gland tumor
Evaluation
- Catecholamines; VMA and HVA of serum and urine
- CT or MRI
- Although US is often the initial radiologic study of choice for abdominal mass, you need to look at the primary tumor site and possible nodes on CT or MRI
- CXR
- Biopsy for definitive diagnosis
Management
- Admission for tumor biopsy and further workup