|Case of the Week - Patient Summary 11252|
Peer Reviewed and Certified -
|Demographics: 1 y.o. girl|
|History & Chief complaint:|
| 18 month old female presenting with right facial swelling. |
|Physical exam and Laboratory:|
| Right facial swelling. Otherwise, unremarkable. |
|Summary of Findings:|
|- Ultrasound images of the face demonstrate a heterogeneous 3cm solid mass with internal flow just anterior to the ear.
- MRI demonstrates a T2 bright, enhancing mass in the location of the right parotid gland.
- Tc-99m MDP bone scan shows focal radiotracer accumulation within the left upper quadrant of the abdomen and the right parotid gland.
- Contrast enhanced CT images through the abdomen reveal a large, heterogeneously-enhancing mass within the left suprarenal region causing mass effect on, but not arising from, the left kidney.
- I-123 MIBG scan shows radiotracer accumulation within the left upper quadrant of the abdomen and the right parotid gland.
DDX for a parotid mass in pediatric patient
- salivary gland tumor
| Metastatic neuroblastoma |
|Confirmed by: FNA of the parotid mass.|
|Treatment and Followup:|
|Patient Specific Discussion: (Also Read the Disease Discussion)|
|This case demonstrates an uncommon presentation of neuroblastoma as a parotid mass. The patient initially presented because her mother noted the facial swelling. Initial ultrasound was followed by MRI. An FNA of the mass was performed revealing neuroblastoma. Subsequent work-up included a bone scan, which revealed radiotracer accumulation within the left upper quadrant consistent with the left retroperitoneal mass later seen on CT. There was no evidence of bony metastases. Finally, an MIBG scan was performed confirming the known left adrenal and right parotid masses, with no other areas of abnormal uptake identified.|
|Neuroblastoma is a common childhood malignancy, third to leukemia and primary brain neoplasm. Neuroblastomas arise from primitive neuroblasts in the neural crest of sympathetic ganglia. Thus, neuroblastomas can arise anywhere from the cervical region down to the pelvis. 2/3 of neuroblastomas are in the abdomen. Of, these, 2/3 are adrenal origin. Of the remaining 1/3, most are in the chest.
Neuroblastoma is initially clinically silent. It becomes symptomatic once it encroaches on surrounding structures, once it has metastasized (most commonly to bone), or once it has started to secrete catecholamines (10% present with hypertension/sympathetic overstimulation). It is common for kids to present with an incidental palpable abdominal mass.
The majority of neuroblastoma patients are less than 4 years of age with a preponderance between 2 months and 2 years. The tumor is slightly more common in boys than in girls.
Neuroblastoma is unique among malignant pediatric lesions in that it can spontaneously transform into benign ganglioneuroblastoma.
Syndromes associated with neuroblastoma include Beckwith-Wiedemann and Klippel-Feil.
Two paraneoplastic syndromes have known associations: opsoclonus/myoclonus (2%) and WDHK (Watery diarrhea with hypokalemia)(7%).
In general, the overall survival rate is 72% at less than 1 year of age, 28% at less than 2 years of age, and 12% for more than 2 years of age. Staging is: I - confined to organ of origin, II - extension beyond organ of origin with +/- nodes. No midline crossover. III - midline crossover. IV - distant mets. Stages I,II - 75% two-year survival. Stages III,IV - 25% two-year survival.
Imaging characteristics include inhomogeneous echotexture, tumor encasement of vessels, calcification (2/3) - stippled, amorphous, or diffuse.