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Neuroblastoma

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Neuroblastoma Rumey C. Ishizawar April 29, 2005 Neuroblastoma Clinical History CC: Rt leg pain with refusal to bear weight. HPI: a 5 yo male c/o a 4 wk history of Rt ... – PowerPoint PPT presentation

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Title: 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
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.
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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
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
  1. 2nd most common solid childhood cancer.
  2. Develops from neural crest cells. Small round
    blue cell tumors.
  3. 2/3rd arise in abdomen, and 2/3rd in abdomen
    develop from adrenal gland.
  4. Usually presents between 2 mo and 2 yrs, with
    most by age 4.
  5. 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).
  6. Radiographic Features Calcification (75),
    located suprarenally, usually encases vessels
    rather than invasion,
  7. DDX Wilms tumor, Adrenal carcinoma,
    Pheochromocytoma, Hepatoblastoma, Ganglioneuroma,
    Lymphoma (see table in website differentiating
    Wilms vs Neuroblastoma) http//www.med-ed.virgini
    a.edu/courses/rad/peds/index.html
  8. Prognosis Tumor stage Age (better outcome if
    lt1, except neonates) Histology, DNA index, and
    N-myc oncogene amplification (bad)
    (http//www.med-ed.virginia.edu/courses/rad/peds/i
    ndex.html)

9
References
  1. UVA Radiology Website Pediatric Radiology
    Genitourinary Tumors. http//www.meded.virginia.e
    du/courses/rad/peds/index.html
  2. Gay SB, Woodcock RJ. Radiology Recall.
    Lippincott 2000. pg 633-634.
  3. Up to date website - Clinical presentation
    diagnosis and staging evaluation of
    neuroblastoma http//www.utdol.com/application/top
    ic/topicText.asp?fileped_onco/6775typeAselecte
    dTitle125top
  4. http//www-medlib.med.utah.edu/WebPath/ENDOHTML/EN
    DO046.html
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