A Pediatric Radiology Textbook and Pediatric Radiology Digital Library
Neuroblastoma
Etiology: — Small round blue cell tumor derived from primordial neural crest cell in sympathetic nervous system — Located anywhere along sympathetic chain – neck 3%, chest in posterior mediastinum 16%, abdomen 75% [adrenal is 50% and extraadrenal is 25%], pelvis 3% in organ of Zuckerkandel and presacral
Imaging: — Heterogenous lobulated tumor along sympathetic chain — Calcifications (greater than or equal to 50%, 85% in abdominal tumors) — Crosses midline, pushes ribs apart, displaces kidney, encases rather than invades vessels, can invade adjacent organs, extends into neural foramina and into spinal canal as extradural lesion that is dumbell-shaped — Metastsizes to liver and bone — Metastases to skull base appear as spiculated periorbital bone expansion centered in diploic space with extraosseous extension
Imaging Radiograph: Paraspinal soft tissue thickening often with calcification
DDX: Neuroblastoma, ganglioneuroblastoma, ganglioneuroma – differ in malignancy potential with neuroblastoma being the most malignant and ganglioneuroma being essentially benign
Complications: — Local spread – to lymph nodes and encases and or displaces vessels and invades and extends into adjacent organs such as liver and kidney and spinal canal — Metastases – 50-60% patients have metastases at presentation most commonly to cortical bone and bone marrow and lymph nodes and liver
Treatment: Chemotherapy then surgical
Clinical: — Most common solid extracranial neoplasm in children (6-10% of all childhood cancers) — Most common cancer diagnosed in first year of life — Median age at diagnosis is 19 months, 90% under 5 years at diagnosis, 40% are infants at diagnosis — 90-95% have elevated catecholamines in urine (HVA and VMA) — Part of a spectrum of tumors including ganglioneuroblastoma and ganglioneuroma — Heterogenous clinical course – silent tumor or regression without treatment or response to treatment or aggressive and lethal disease — Presentation variable with site and size of tumor – antenatal identification of an adrenal mass, abdominal mass – primary mass with liver metastases, metastatic disease – bone pain or raccoon eyes from metastases to skull base (mimics abuse), paraneoplastic syndrome (opsoclonus myoclonus or VIP syndrome), hypertension from catecholamines
Radiology Cases of Neuroblastoma
Radiology Cases of Thoracic Ganglioneuroma
CXR shows a smooth mass in the left superior/posterior mediastinum. Axial CT with contrast of the chest shows a non-calcified solid paraspinal mass.CXR PA and lateral (above) show a large mass in the posterior mediastinum which is deforming the right 6th through 12th ribs. Axial CT without contrast of the chest (below) shows the mass to extend across the midline and to have small areas of calcification within it. At the level of the diaphragm there was extension of tumor into the spinal canal.
Radiology Cases of Thoracic Ganglioneuroblastoma
CXR AP (above left) shows a soft tissue mass in the left superior chest causing spreading and erosion of the left first and second rib, and increased distance between the left C7 and T1 transverse processes. Axial CT without contrast of the chest (above right) shows the mass to be densely calcified, in the posterior mediastinum, and displacing and compressing the trachea. Coronal (below left) and sagittal (below right) T1 MRI without contrast of the chest show a posterior mediastinal mass from C7 to T3 that has intraspinal extension.
Radiology Cases of Thoracic Neuroblastoma
CXR PA and lateral shows a large left posterior mediastinal mass. Coronal and sagittal T1 MRI without contrast (lower left and middle) and coronal T1 MRI with contrast (lower right) of the chest shows an enhancing mass arising from the left adrenal gland extending up into the left thorax.CXR shows a posterior mediastinal mass to the left of the spine that is clearly calcified on the coronal and axial CT with contrast of the chest.CXR AP (left) taken as an infant shows a round left-sided soft tissue mass that was in the posterior mediastinum and found to be neuroblastoma that was subsequently treated in part with radiation therapy. CXR AP (right) taken 17 years later shows a curvature of the upper thoracic spine convex to the right.
Radiology Cases of Adrenal Neuroblastoma
Coronal (above left) and sagittal (above right) CT with contrast of the chest obtained on the first day of life show an oval low density lesion superior to the right kidney. Coronal (below left) and sagittal (below right) CT with contrast of the abdomen obtained 5 weeks later show an increase in the size of the lesion superior to the right kidney. Coronal (left) and axial (above right) CT with contrast of the abdomen show a large low density faintly calcified mass arising from the right adrenal gland. Axial image of the pelvis in bone windows (below) shows multiple lytic bone lesions throughout the iliac wings and sacrum.AXR (above left) shows a distended stomach. Axial (above right), coronal (below left) and sagittal (below right) CT with contrast of the abdomen shows a large calcified left adrenal mass that is compressing and displacing the left kidney inferiorly and laterally.Coronal T1 MRI without contrast of the chest shows a right paraspinal mass in the region of the right adrenal gland that on axial sequences was seen to extend into the spinal canal.Sagittal T2 MRI shows a huge left adrenal mass that was noted to cross midline and encase the abdominal vasculature.Axial CT with contrast of the abdomen shows a large midline calcified mass that crosses the midline and encases abdominal vessels.Axial CT without contrast of the abdomen (above) shows a large mass that has faint calcifications within it that fills the entire left upper quadrant of the abdomen. Axial CT without (lower left) and with (lower right) contrast of the orbit shows a round soft tissue mass around the right pterygoid plate that enhances strongly with contrast.Sagittal US of the left kidney (above left) shows a large, solid, hyperechoic mass superior to the left kidney. Axial CT with contrast of the abdomen (above right) shows an ill-defined solid mass in the region of the left adrenal gland, which (below right) crosses the midline and encases the mesenteric vasculature. Axial CT without contrast of the brain (below left) shows a lytic lesion in the right mandible.Axial CT with contrast of the abdomen shows a large non-calcified low density abdominal mass arising from the right adrenal gland which crosses the midline (above) and displaces the right kidney laterally (middle) and inferiorly (below).CXR AP shows a mild curvature of the thoracolumbar spine convex to the left. Several calcifications are seen projecting over the liver.
Radiology Cases of Presacral Neuroblastoma
Axial CT with contrast of the pelvis (above) shows (from top to bottom in the midline) contrast in the base of the bladder, contrast in the rectum which is deviated to the right, and a solid soft tissue mass anterior to the sacrum. Axial (below left) and sagittal (below right) T2 MRI without contrast of the pelvis shows the presacral mass to have high signal intensity.Lateral image from a barium enema performed after an intravenous pyelogram (below) shows anterior displacement and compression of the bladder (to the left of the image) and rectum (in the middle of the image) by a presacral mass. The scout image (above) showed the mass was not calcified.
Radiology Cases of Neuroblastoma Bone Metastases
AP radiograph of the femurs shows a mottled appearance to both femurs with multiple lytic lesions noted in the femurs with associated periosteal reaction, more prominent on the left than the right.AP radiographs of the humerus (above) and radius and ulna (below) show lesions involving the proximal humeral diaphysis, entire radial diaphysis and proximal ulna diaphysis all of which are lytic in appearance and having a wide zone of transition and associated faint periosteal reaction.Sagittal US of the left kidney (above left) shows a large, solid, hyperechoic mass superior to the left kidney. Axial CT with contrast of the abdomen (above right) show an ill-defined solid mass in the region of the left adrenal gland, which (below right) crosses the midline and encases the mesenteric vasculature. Axial CT without contrast of the brain (below left) shows a lytic lesion in the right mandible.Axial CT without contrast of the abdomen (above) shows a large mass that has faint calcifications within it that fills the entire left upper quadrant of the abdomen. Axial CT without (lower left) and with (lower right) contrast of the orbit shows a round soft tissue mass around the right pterygoid plate that enhances strongly with contrast.Coronal (left) and axial (above right) CT with contrast of the abdomen show a large low density faintly calcified mass arising from the right adrenal gland. Axial image of the pelvis in bone windows (below) shows multiple lytic bone lesions throughout the iliac wings and sacrum.
Surgery Cases of Neuroblastoma
Surgery Cases of Adrenal Neuroblastoma
Surgery Cases of Thoracic Neuroblastoma
Surgical image taken through a left thoracoabdominal incision shows a large tumor adherent posteriorly to the chest wall and spine. The lungs are superior to the tumor. The tumor was seen to be entering the tenth through twelfth neural foramina. The tumor was also adherent to the diaphragm and could be felt going through the diaphragm into the retroperitoneum. The tumor was resected in block.
Surgery Cases of Presacral Neuroblastoma
Surgical image shows a presacral tumor that is being removed through a posterior sagittal trans-sacral approach.
Gross Pathology Cases of Neuroblastoma
Gross Pathology Cases of Thoracic Neuroblastoma
Gross pathological image (left) shows a mass which is tan, measuring 12 x 10 x 5 cm in size, with focal areas of hemorrhage and calcification on its exterior. The cut surface of the mass (right) was whitish gray to hemorrhagic with numerous foci of yellow calcification. The “tails” at the superior aspect of the specimen correspond to tumor infiltration of intervertebral neural foramina.
Gross Pathology Cases of Adrenal Neuroblastoma
Gross pathological image with the aorta in the midline cut open shows a large mass arising from the right adrenal gland, which displaces the right kidney inferiorly and which crosses the midline and encases the aorta.Gross pathological image shows a hemorrhagic tumor mass with a thin rim of residual normal adrenal gland. The yellowish areas are foci of calcification.Gross pathological image (right) shows a mass which is tan with focal areas of hemorrhage and calcification on its exterior. The cut surface of the mass (left) was whitish gray with numerous foci of yellow calcification.
Gross Pathology Cases of Presacral Neuroblastoma
Gross pathological image shows a presacral mass that was 3.0 x 2.5 x 1.5 cm in size, yellow in color and consisted of multiple confluent nodules.
Histopathology Cases of Neuroblastoma
Histopathological image H&E stained slide lower power photomicrograph shows small round blue cells within abundant, eosinophilic, fibrillary stroma and basophilic foci of dystrophic calcification.Histopathological image H&E stained slide higher power photomicrograph shows the neoplastic cells arranged in classic pseudorosettes (often referred to as Homer Wright rosettes).
