Adrenal Tumors
Also covered as: neuroblastoma
Educational content from recorded physician discussions — not medical advice. Always talk to your child's care team about your child's situation.
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Fundamentals
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Topics in 10: Neuroblastoma
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Drs Daniel von Allmen, Todd Ponsky and Rae Hanke come together to provide you the essentials on the diagnosis and management of Neuroblastoma. Intro and outro tracks are adapted from "I dunno" by grapes, featuring J Lang, Morusque. Artist U
podcast10:25 · Dec 2020
Case-Based Learning
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Neuroblastoma
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Drs Erika Newman, Dan von Allmen and Tony Sandler join Dr. Todd Ponsky in a discussion covering the latest in neuroblastoma. Contributing editor: Rachel (Rae) Hanke, MD
Case 1: Mother who presents with 26 week fetus
with a pr
podcast56:19 · Dec 2020
In-Depth Reviews
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Thyroid Disorders
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An interactive discussion about malignant and benign pediatric thyroid disorders between Todd Ponsky, MD and Diana Diesen, MD. Dr. Diesen is assistant professor of surgery and pediatric surgery fellowship program director at the University
podcast45:51 · Dec 2020
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Every expert statement below comes from the recorded discussions, with its speaker and moment.
Topics in 10: Neuroblastoma
Neuroblastoma is the most common extracranial solid tumor in children
epidemiologicalRae Hanke0:00 ↗
The majority of neuroblastoma cases occur in children less than a year old
epidemiologicalRae Hanke0:00 ↗
Younger patients are often picked up prenatally on ultrasound or as a solid abdominal mass in two- or three-year-olds
clinicalDaniel von Allmen0:41 ↗
Patients with metastatic disease may present with bony pain or neurologic symptoms from cord compression
clinicalDaniel von Allmen0:41 ↗
Catecholamines (urine or serum) are one of the most diagnostic laboratory tests for neuroblastoma
clinicalDaniel von Allmen1:07 ↗
Cross-sectional imaging (CT or MRI) is obtained after initial ultrasound and laboratory workup
clinicalDaniel von Allmen1:39 ↗
MIBG study is helpful for confirming neuroblastoma diagnosis and demonstrating metastatic disease
clinicalDaniel von Allmen1:39 ↗
About 10% of neuroblastomas are MIBG negative
clinicalDaniel von Allmen2:20 ↗
Some centers obtain a PET scan for MIBG-negative cases to look for tumor uptake and metastatic disease
clinicalDaniel von Allmen2:20 ↗
The INRGSS staging system allows stage assignment before any invasive procedure
guidelineDaniel von Allmen2:45 ↗
Localized tumors without image-defined risk factors are categorized as L1
guidelineDaniel von Allmen2:45 ↗
Localized tumors with image-defined risk factors (encasing nerves or vessels) are L2
guidelineDaniel von Allmen2:45 ↗
Tumors with metastatic disease are categorized as M
guidelineDaniel von Allmen2:45 ↗
MS category is for children less than 18 months with metastases to bone marrow or skin
guidelineDaniel von Allmen2:45 ↗
The INRGSS was created to allow studies from different centers and countries to be compared based on pre-surgical staging
guidelineDaniel von Allmen3:36 ↗
The prior staging system required tissue diagnosis before assigning a stage
guidelineDaniel von Allmen3:36 ↗
A patient with a resectable adrenal mass and positive MIBG without metastases can be treated with primary resection via laparotomy or laparoscopy
clinicalDaniel von Allmen4:06 ↗
For large central abdominal tumors that encase major vasculature, only tissue for diagnosis is needed via open biopsy, laparoscopic biopsy, or core needle biopsy
clinicalDaniel von Allmen4:44 ↗
NMIC status is the most important biologic risk determinant from biopsy tissue
clinicalDaniel von Allmen5:14 ↗
1P and 11Q deletions should be assessed in biopsy tissue along with Shimada histology
clinicalDaniel von Allmen5:14 ↗
Neuroblastoma risk categories are very low risk, low risk, intermediate risk, or high risk
guidelineDaniel von Allmen5:45 ↗
Risk distribution is approximately 50-50 between low risk categories and high risk, with a smaller percentage intermediate risk
epidemiologicalDaniel von Allmen5:45 ↗
NMIC amplification and age greater than 18 months are the most important prognostic determinants for neuroblastoma
clinicalDaniel von Allmen6:06 ↗
High-risk patients receive aggressive chemotherapy including peripheral stem cell transplant times 2, aggressive surgery with >90% resection goal, radiation, immunotherapy, and potentially retinoic acid therapy
guidelineDaniel von Allmen6:06 ↗
Intermediate risk tumors receive varying cycles of chemotherapy based on biologic risk factors
guidelineDaniel von Allmen6:51 ↗
For intermediate risk tumors, the surgical goal is at least 50% response from initial primary tumor volume combining neoadjuvant chemotherapy and surgical resection
guidelineDaniel von Allmen6:51 ↗
Low risk patients, depending on age and diagnosis timing, could be followed with observation alone
guidelineDaniel von Allmen7:22 ↗
A Children's Oncology Group study led by Jed Nocturne showed patients less than six months with prenatally or early postnatally diagnosed localized mass can be observed with expectation that most will avoid surgery
guidelineDaniel von Allmen7:22 ↗
Patients with metastatic disease typically receive four or five cycles of neoadjuvant chemotherapy and are then reassessed
guidelineDaniel von Allmen7:55 ↗
If metastatic disease is responding to chemotherapy, the primary tumor site should be resected with attempted >90% resection
guidelineDaniel von Allmen7:55 ↗
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