Neuroblastoma

Etiology: tumor of neuroblastic origin occurring along sympathetic chain – neck (5%), thoracic (15%), abdominal (60%) – adrenal (50%) + sympathetic chain, pelvic (5%) in organ of Zuckerkandel + presacral
Imaging: solid mass, often grow beyond adrenal, displacing kidney, crossing midline and encasing abdominal vasculature, extends into neural foramina
CT: 80% calcified
DDX: neuroblastoma, ganglioneuroblastoma, ganglioneuroma – differ in malignancy potential with neuroblastoma being the most malignant and ganglioneuroma being essentially benign
Complications: metastases to bone (50% at diagnosis) / lymph nodes / lung / liver, spinal canal invasion
Clinical: urine should be checked for elevated catecholemines (HVA and VMA), 80% before 4 years old, 5% after 10 years old, opsoclonus / myoclonus, associated with Beckwith-Wiedemann syndrome / Neurofibromatosis type 1 / Hirschsprung disease / DiGeorge syndrome

Radiology Cases of Neuroblastoma

Radiology Cases of Thoracic Neuroblastoma

CXR and CT of 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 and MRI of 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 and CT of neuroblastoma — 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 of post radiation scoliosis — 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

CT 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.

CT of adrenal neuroblastoma with bone metastases — 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.

CT of adrenal neuroblastoma — 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.

MRI of neuroblastoma invading the spinal canal — 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.

MRI of neuroblastoma — Sagittal T2 MRI shows a huge left adrenal mass that was noted to cross midline and encase the abdominal vasculature.

CT of neuroblastoma — Axial CT with contrast of the abdomen shows a large midline calcified mass that crosses the midline and encases abdominal vessels.

CT of neuroblastoma with orbital metastasis — 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.

CXR of post radiation scoliosis — 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

BE of presacral neuroblastoma — 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

Radiograph of neuroblastoma bone metastases to the femur — 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.

Radiograph of bone metastases from neuroblastoma — 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.

Surgery Cases of Neuroblastoma

Surgery Cases of Adrenal Neuroblastoma

Surgery Cases of Thoracic Neuroblastoma

Surgery image 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 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 pathology image 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 of 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 pathology image of adrenal neuroblastoma — 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 Pathology Cases of Presacral Neuroblastoma

Gross pathology image 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.