Neuroblastoma (peds): Difference between revisions
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==Background== | ==Background== | ||
[[File:PMC4112030 40064 2013 1059 Fig3 HTML.png|thumb|Neuroblastoma: (a) Abdominal CT-scan: large heterogeneous right abdominal mass with a tumor thrombus of the inferior vena (Arrow). (b) Gross examination of the tumor: heterogeneous partially necrotizing tumor of the right adrenal gland. (c):Histology of the tumor: neuroblastoma poorly differentiated.]] | |||
*Arises from primitive ganglion cells of sympathetic nervous system | *Arises from primitive ganglion cells of sympathetic nervous system | ||
*Synthesize and secrete catecholamines | |||
*Most common neoplasm in 1st yr of life | *Most common neoplasm in 1st yr of life | ||
*May arise anywhere along sympathetic nervous system | *May arise anywhere along sympathetic nervous system | ||
| Line 6: | Line 8: | ||
**Abdomen (25%) | **Abdomen (25%) | ||
**Chest (15%) | **Chest (15%) | ||
*Mets are most common in lymph nodes, bone marrow, cortical bone, dura, orbits, liver, and skin | |||
==Clinical Features== | ==Clinical Features== | ||
*Depends on location | *Depends on location | ||
** | **Abdominal mass | ||
** | ***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> | ||
**[[Horner syndrome]] | **Abdominal pain or constipation | ||
**Spinal cord compression | **Proptosis or periorbital ecchymoses | ||
*Opsoclonus-myoclonus | **[[Horner syndrome]]: miosis, ptosis, anhidrosis | ||
** | **Paraspinal mass | ||
*Neutropenia/pancytopenia suggests bone marrow involvement | **Spinal cord compression, which can cause localized back pain or weakness | ||
**Systemic symptoms, bone pain, anemia | |||
*[[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== | ==Differential Diagnosis== | ||
*[[Wilms' tumor]] | |||
*Hepatoblastoma | |||
*Lymphoma | |||
*Other adrenal gland tumor | |||
== | ==Evaluation== | ||
*CXR | [[File:PMC4769609 gr2.png|thumb|Abdominal CT demonstrating (A) a heterogeneously enhancing mass in the pancreatic body and tail with (B) encasement of the celiac axis.]] | ||
[[File:PMC4206056 jrip-3-79-g001.png|thumb|Neuroblastoma accompanied by hyperaldosteronism.]] | |||
*Catecholamines; VMA and HVA of serum and urine | |||
*CT or MRI | |||
**Although US is often the initial radiologic study of choice for abdominal mass, primary tumor site and possible nodes on are better evaluated on CT or MRI | |||
*[[CXR]] | |||
*Biopsy for definitive diagnosis | |||
==Management== | ==Management== | ||
* | *No acute treatment | ||
==Disposition== | |||
*Admission for tumor biopsy and further workup | |||
==See Also== | ==See Also== | ||
==External Links== | |||
==References== | ==References== | ||
<references/> | |||
[[Category:Pediatrics]] | [[Category:Pediatrics]] | ||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Latest revision as of 11:17, 23 April 2022
Background
Neuroblastoma: (a) Abdominal CT-scan: large heterogeneous right abdominal mass with a tumor thrombus of the inferior vena (Arrow). (b) Gross examination of the tumor: heterogeneous partially necrotizing tumor of the right adrenal gland. (c):Histology of the tumor: neuroblastoma poorly differentiated.
- 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, primary tumor site and possible nodes on are better evaluated on CT or MRI
- CXR
- Biopsy for definitive diagnosis
Management
- No acute treatment
Disposition
- Admission for tumor biopsy and further workup