Case 1.

5
HISTORY: A 1-year-old child with an abdominal mass, focal swelling of the right temple, anemia, and
­elevated urinary catecholamines

FIGURE 1.5.1 FIGURE 1.5.2

FIGURE 1.5.3 FIGURE 1.5.4

1 / PEDIATRICS 9

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 Case 1.5  (Continued)
FINDINGS: IV contrast-enhanced CT images
(Figs.  1.5.1 and 1.5.2) through the abdomen
demonstrate a calcified right paraspinal mass; a
­ vessel encasement, retrocrural adenopathy, and
large ­calcified retroperitoneal mass that crosses
the midline, encasing the aorta and SMA; and left
hydronephrosis. A delayed image from an meta-
iodobenzylguanidine (MIBG) scan (Fig. 1.5.3) dem-
onstrates increased uptake in the midabdomen
corresponding to the retroperitoneal mass on CT.
Head CT with IV contrast (Fig. 1.5.4) reveals a soft-
tissue mass, with an epicenter in the right temporal
bone, associated with bone destruction and a sun-
burst periosteal reaction.
DIAGNOSIS: Retroperitoneal neuroblastoma with
skull metastases
DISCUSSION: Neuroblastoma is the most common
solid extra-cranial malignant tumor of childhood.
It is derived from primitive neural crest cells and,
therefore, originates in the sympathetic chain gan-
glia and adrenal medulla. Two-thirds of cases arise in
the abdomen, and two-thirds of abdominal tumors
arise in the adrenal medulla. The most common
sites of origin are adrenal medulla (35%), extra-
adrenal retroperitoneum (30%–35%), and posterior
mediastinum (20%). Tumors in the neck and pelvis
(3%–8%) are much less common. Peak age of pre-
sentation is 22 months. At diagnosis, 60% to 70%
of patients have metastatic disease with spread to
cortical bone (in particular the skull), bone marrow,
liver, and lymph nodes. The main challenge in im-
aging these tumors is differentiating neuroblastoma
10 AUNT MINNIE’S ATLAS AND IMAGING-SPECIFIC DIAGNOSIS
(c) 2015 Wolters Kluwer. All Rights Reserved.
from Wilms tumor. Calcification, suprarenal loca-
tion with a displaced but normal ipsilateral kidney,
­extension across the midline are features that allow
a confident diagnosis of neuroblastoma. Paraspi-
nal tumor may invade the spinal canal via exten-
sion through adjacent neural foramina and is best
evaluated with CT or magnetic resonance imaging
(MRI). Although initial diagnosis is suspected when
a calcified adrenal or paraspinal mass is identified
on plain films and ultrasonography, CT of chest,
abdomen, and pelvis, bone scans, MIBG scans,
±  MRI are required for complete staging. MIBG
(labeled with iodine-123) scintigraphy is sensitive
and specific for catecholamine-secreting tumors;
however, only 70% of neuroblastomas are MIBG-
positive; therefore, normal results of MIBG do not
exclude the diagnosis of neuroblastoma. Age and
stage at diagnosis, N-myc oncogene amplification,
DNA content, and Shimada histology are important
prognostically and are used to stratify patients into
high-, ­intermediate-, and low-risk groups. Treat-
ment consists of chemotherapy and surgical debulk-
ing. Despite continued advancements in therapy,
the prognosis remains poor (7).
Aunt Minnie’s Pearl
A childhood suprarenal mass with calcification that
crosses the midline and encases the mesenteric vas-
culature and/or invades the neural foramina is almost
certainly a neuroblastoma.