Neuroblastoma

1. Neuroblastoma Rumey C. Ishizawar April 29, 2005

2. Clinical History CC: Rt leg pain with refusal to bear weight. HPI: a 5 yo male c/o a 4 wk history of Rt flank pain with progression to Rt hip and leg prior to admission on 04/11/05. Reported a history of falling during onset of pain with initial improvement with NSAIDs. However, pain continued to worsen with Pt refusing to bear weight one week prior to admission. Initial evaluation with a pelvis and right hip x-ray on 04/06/05 showed no recognizable abnormalities. Further outpatient work-up by MRI located a right suprarenal mass with abnormal lesions in the right acetabulum and vertebral bodies. ROS: Low-grade fever and intermittent abdominal pain over past several weeks. PMH/PSH: none Allergies: none Meds: Motrin SH: Lives with mother and 9 yo sister in Albemarle county. PE: VS stable. Positive for wide-based gait secondary to pain. Negative lymphadenopathy or palpable masses. Labs: ESR 51 (H), CRP 3.4 (H), CMP WNL, WBC 7.05, Hgb 11, Hct 31.6 (L), MCV 77.8 (L), Ferritin 278 (N), Plt 541

3. Calcification CT Report: 4.5 x 2.8 x 5.0 cm heterogeneous, partially calcified mass within the right suprarenal space. There is poor margination with the right hepatic lobe. There is good margination of the right kidney. No intraspinal extension and the mass does not cross the midline. This likely represents a neuroblastoma. Other differential considerationsinclude adrenal cortical carcinoma, ganglioneuroma.

6. Nuclear Bone Scan Report: Numerous areas of abnormal radiotracer uptake involving multiple thoracolumbar vertebral bodies, ribs, the skull, the bilateral femurs and bilateral tibiae compatible with bone metastases

7. This neonate had a congenital neuroblastoma of the right adrenal. This neoplasm (marked by the white arrow) is displacing the liver to the left of the body. http://www-medlib.med.utah.edu/WebPath/ENDOHTML/ENDO045.html Cont. Clinical History 04/14/05 - Pt underwent resection of Rt adrenalectomy including involved tumor, central venous line placement, lymph node biopsies, bilateral bone marrow aspiration and biopsy. Pt has Neuroblastoma – Stage 4 Pathology Results: 7 x 4.5 x 3 cm nodular mass with a red-tan, smooth surface composed predominantly of a differentiating neuroblastoma cells with some ganglioneuromatous appearance and calcifications. Lymph node biopsies and bone marrow aspiration were positive for metastasis. 04/24/05 – Discharged home and is being followed by Hem/Onc. Started on Chemotherapy. Radiation therapy maybe considered in high-risk disease.

8. Neuroblastoma Features and Differential Dx • 2nd most common solid childhood cancer. • Develops from neural crest cells. Small round blue cell tumors. • 2/3rd arise in abdomen, and 2/3rd in abdomen develop from adrenal gland. • Usually presents between 2 mo and 2 yrs, with most by age 4. • Imaging – U/S often initial study, but requires CT or MRI F/U. Nuclear bone scan helpful for evaluating metastasis to cortical bone (tumor uptake 75%). • Radiographic Features – Calcification (75%), located suprarenally, usually encases vessels rather than invasion, • DDX – Wilm’s tumor, Adrenal carcinoma, Pheochromocytoma, Hepatoblastoma, Ganglioneuroma, Lymphoma (see table in website differentiating Wilm’s vs Neuroblastoma) http://www.med-ed.virginia.edu/courses/rad/peds/index.html • Prognosis – Tumor stage; Age (better outcome if <1, except neonates); Histology, DNA index, and N-myc oncogene amplification (bad) (http://www.med-ed.virginia.edu/courses/rad/peds/index.html)

9. References • UVA Radiology Website – Pediatric Radiology: Genitourinary Tumors. http://www.meded.virginia.edu/courses/rad/peds/index.html • Gay SB, Woodcock RJ. Radiology Recall. Lippincott 2000. pg 633-634. • Up to date website - Clinical presentation; diagnosis; and staging evaluation of neuroblastomahttp://www.utdol.com/application/topic/topicText.asp?file=ped_onco/6775&type=A&selectedTitle=1~25#top • http://www-medlib.med.utah.edu/WebPath/ENDOHTML/ENDO046.html