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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Adrenal tumors in children present unique diagnostic and surgical challenges. A 13-year-old girl with FAP syndrome illustrates key principles: a 14 cm calcified left retroperitoneal mass was discovered incidentally during colectomy, displacing the left kidney inferiorly [e4060-c10, e4060-c11]. Preoperative hormonal evaluation—including urine catecholamines, cortisol, ACTH, and aldosterone—was normal, ruling out functional tumor [e4060-c8, e4060-c9]. Laparoscopic adrenalectomy was performed in modified right lateral decubitus position using 5 mm ports [e6875-c12, e6875-c13]. The surgical approach involved reflecting the pancreas medially off the anterior mass surface, identifying the characteristic golden hue of adrenal tissue, and proceeding with posterior dissection to reflect the mass anteriorly given its size [e6875-c15, e6875-c17, e6875-c18]. Complete circumferential laparoscopic mobilization was achieved before extraction through an extended Pfannenstiel incision [e6875-c20, e6875-c22]. Final pathology confirmed a 14 cm completely encapsulated adrenal cortical neoplasm with adjacent normal adrenal gland [e6875-c23, e6875-c24]. The patient was discharged on postoperative day 4 on regular diet .
- Large pediatric adrenal masses (14 cm) can be successfully resected laparoscopically with extraction through extended Pfannenstiel incision.
- Complete preoperative hormonal evaluation is essential even when adrenal masses appear non-functional on clinical grounds.
- Posterior dissection approach with anterior reflection is preferred for large adrenal masses to optimize visualization and control.
- The golden hue of adrenal tissue serves as a key anatomic landmark during dissection of large retroperitoneal masses.
- Complete encapsulation on final pathology confirms adequate oncologic resection of adrenal cortical neoplasms.
For patients & families
Adrenal tumors in children are uncommon masses that grow in the small glands sitting on top of the kidneys. Doctors sometimes discover these tumors unexpectedly during surgery for other conditions, as happened with a 13-year-old girl who had a large adrenal mass found during a procedure for colon polyps [e4060-c3, e6875-c3]. Before removing an adrenal tumor, physicians check the child's blood and urine to make sure the mass isn't making extra hormones that could cause problems during surgery [e4060-c8, e4060-c9, e6875-c8, e6875-c9]. Imaging scans help doctors see the tumor's size and position—in this case, a 14-centimeter mass that had pushed the kidney downward [e4060-c10, e4060-c11, e6875-c10, e6875-c11]. Surgeons can often remove these tumors using small incisions and a camera, carefully separating the mass from nearby organs like the pancreas, spleen, and kidney [e6875-c14, e6875-c15, e6875-c16, e6875-c19]. The tumor is placed in a special bag and removed through a small opening in the lower belly [e6875-c21, e6875-c22]. After surgery, the tissue is examined under a microscope to determine what type of tumor it was; in this patient's case, it was completely enclosed in its own capsule, which is a reassuring finding [e6875-c23, e6875-c24]. Most children recover well and go home within a few days on a normal diet .
Adrenal tumors in children are uncommon masses that grow in the small glands sitting on top of the kidneys. Doctors sometimes discover these tumors unexpectedly during surgery for other conditions, as happened with a 13-year-old girl who had a large adrenal mass found during a procedure for colon polyps [e4060-c3, e6875-c3]. Before removing an adrenal tumor, physicians check the child's blood and urine to make sure the mass isn't making extra hormones that could cause problems during surgery [e4060-c8, e4060-c9, e6875-c8, e6875-c9]. Imaging scans help doctors see the tumor's size and position—in this case, a 14-centimeter mass that had pushed the kidney downward [e4060-c10, e4060-c11, e6875-c10, e6875-c11]. Surgeons can often remove these tumors using small incisions and a camera, carefully separating the mass from nearby organs like the pancreas, spleen, and kidney [e6875-c14, e6875-c15, e6875-c16, e6875-c19]. The tumor is placed in a special bag and removed through a small opening in the lower belly [e6875-c21, e6875-c22]. After surgery, the tissue is examined under a microscope to determine what type of tumor it was; in this patient's case, it was completely enclosed in its own capsule, which is a reassuring finding [e6875-c23, e6875-c24]. Most children recover well and go home within a few days on a normal diet .
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Thyroid Disorders
Thyroid nodules are less common in children than adults, but when detected in children, they are more likely to be malignant.
epidemiologicalDiana Deason6:23 ↗
When children present with thyroid cancer, they are more likely to have extension outside of the thyroid, regional lymph node involvement, and distant metastasis compared to adults.
clinicalDiana Deason6:31 ↗
Risk factors for thyroid nodules and cancer include previous exposure to radiation or alkylating agents, such as treatment for Hodgkin's lymphoma, leukemia, or CNS tumors.
clinicalDiana Deason4:08 ↗
Some thyroid conditions and thyroid cancers have familial predisposition, including MEN syndromes, PTEN hamartoma tumor syndromes, and APC-associated polyposis syndromes.
clinicalDiana Deason4:27 ↗
If TSH is suppressed, a nuclear thyroid scan should be obtained to identify hyperfunctioning nodules, which do not need to be biopsied if they are going to be resected.
guidelineDiana Deason7:24 ↗
Suspicious ultrasound features for thyroid malignancy include hypoechoic mass, irregular margins, increased blood flow, microcalcifications, or association with abnormal lymph nodes.
clinicalDiana Deason9:57 ↗
In pediatric patients, size cutoffs used in adults (1 cm) cannot be applied for biopsy decisions; ultrasound characteristics and clinical context determine whether FNA is warranted.
guidelineDiana Deason11:21 ↗
Total or near-total thyroidectomy is recommended for papillary thyroid cancer due to risk of bilateral disease (up to 30%), multifocal disease (up to 65%), increased risk of recurrence with lobectomy alone, and ability to optimize for radioactive iodine and use thyroglobulin as a tumor marker.
guidelineDiana Deason15:00 ↗
Nerve monitoring is used routinely during thyroidectomy, and while it does not decrease the risk of nerve injury, it is helpful in identifying the recurrent laryngeal nerve, especially in patients with bulky cervical disease.
opinionDiana Deason17:07 ↗
There is no evidence to support prophylactic lateral neck dissection in thyroid cancer; lateral neck dissection is performed only when lateral nodes are pathologically positive.
guidelineDiana Deason18:33 ↗
After total thyroidectomy, PTH levels less than 10 to 15 in recovery indicate higher risk for hypocalcemia, prompting initiation of calcium replacement or calcitriol.
clinicalDiana Deason19:37 ↗
Low-risk papillary thyroid cancer patients (disease confined to thyroid, no nodal involvement, T1B N0) require only thyroglobulin surveillance with TSH suppression to 0.5-1, ultrasound at 6 months postoperatively, then annually for 5 years.
guidelineDiana Deason21:12 ↗
Intermediate-risk patients (extensive central neck disease or any lateral neck disease) and high-risk patients (extensive regional disease, local invasion, or distant metastases) may require radioactive iodine postoperatively.
guidelineDiana Deason21:48 ↗
Follicular lesions represent about one-third of thyroid FNA results and are indeterminate specimens.
epidemiologicalDiana Deason23:05 ↗
For follicular lesions of undetermined significance, the risk of malignancy in adults is 5-15%, but in pediatric literature it is approximately 28%.
epidemiologicalDiana Deason24:07 ↗
For follicular neoplasms, the reported malignancy rate was 15-30%, but more recent data suggests it is between 50% and 60% in children.
epidemiologicalDiana Deason24:18 ↗
Current ATA recommendations are that all indeterminate (follicular) lesions in children be resected, typically with lobectomy and removal of the isthmus.
guidelineDiana Deason23:50 ↗
Frozen section cannot distinguish follicular adenoma from follicular carcinoma, but can identify papillary components.
clinicalDiana Deason25:04 ↗
For follicular carcinoma with significant vascular invasion or tumor greater than 4 cm, completion thyroidectomy is recommended; smaller tumors with minimal vascular invasion can be monitored.
guidelineDiana Deason26:10 ↗
About 30% of patients who undergo lobectomy may develop hypothyroidism at some point, so thyroid function monitoring is important even after partial thyroidectomy.
epidemiologicalDiana Deason27:01 ↗
TSH suppression goals vary by ATA pediatric risk level: low-risk patients have a TSH goal of 0.5-1, while high-risk patients have a TSH goal of less than 0.1.
guidelineDiana Deason27:31 ↗
For benign thyroid nodules greater than 4 cm, the sensitivity and specificity of FNA is decreased, so it is important to follow these lesions with repeat ultrasound in 6-12 months and repeat biopsy if enlarging or developing suspicious features.
clinicalDiana Deason28:49 ↗
Inadequate FNA specimens occur in roughly 1-3% of cases and should be repeated in 3-6 months to avoid picking up atypia from trauma of the initial FNA.
clinicalDiana Deason29:49 ↗
Sporadic medullary thyroid cancer is unusual in children, so routine calcitonin monitoring is not recommended for every child with a thyroid nodule.
guidelineDiana Deason31:34 ↗
For medullary thyroid cancer, if initial calcitonin level is greater than 500, imaging should be performed to exclude metastatic disease, including CT of neck and chest, MRI or CT of abdomen (looking at liver), and bone scan.
guidelineDiana Deason33:01 ↗
MEN 2A patients can develop medullary thyroid cancer, pheochromocytomas, and hyperparathyroidism.
clinicalDiana Deason33:36 ↗
MEN 2B patients can develop medullary thyroid cancer, pheochromocytomas, mucosal neuromas, and a Marfanoid habitus with elongated features and joint laxity.
clinicalDiana Deason33:47 ↗
MEN 2B patients with RET 918 mutation present with thyroid cancer very early, as young as 3 months of age, and require thyroidectomy before 1 year of age.
guidelineDiana Deason34:11 ↗
MEN 2A high-risk patients (most commonly RET 634 mutation) should have total thyroidectomy before age 5, with surveillance starting at age 3 including calcitonin, CEA, and ultrasounds; if calcitonin or ultrasound abnormalities develop, thyroidectomy should be performed at that time.
guidelineDiana Deason36:03 ↗
If calcitonin levels exceed 40 in MEN patients undergoing surveillance, central neck dissection is recommended at the time of thyroidectomy.
guidelineDiana Deason36:27 ↗
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