3. Discussion
Neuroblastoma is the second most common abdominal mass in children after
Wilms’ tumor [4]. It is a neuroendocrine tumor that originates from
sympathetic ganglion cells. The most common location for neuroblastoma
is on the adrenal glands usually in the adrenal medulla, though it can
arise anywhere in the sympathetic nervous system. Neuroblastoma is
slightly more common in boys than in girls with a sex ratio of
1.2:1[5]. Generally, neuroblastoma has no clinical symptoms unless
they invade surrounding organs or metastasize. Unique features of these
neuroendocrine tumors are the early age of onset, the high frequency of
metastatic disease at diagnosis, and the tendency for spontaneous
regression of tumors in infancy [6]. Common sites of metastases are
lymph nodes, bone, and the liver [7]. Neuroblastoma often presents
late, with non-specific signs including an abdominal mass or pain,
complications of metastasis to orbits such as proptosis or peri-orbital
bruising, unexplained fever and weight loss, anemia, or bone pain
[7]. The most important clue for early diagnosis before biopsy or
resection is the presence of hypertension. However, currently in our
hospital, there is no device to measure blood pressure for young
children so that we did not take blood pressure in this case.
Hypertension is likely because of the combined effects of tumor
secretion of catecholamines, tumor compression of the renal vasculature,
and further activation of the renin-angiotensin aldosterone system.
Checking urine catecholamine levels (vanillylmandelic acid and
homovanillic acid) is very helpful to further correlate these with the
possibility of neuroblastoma [8]. CT Scan often shows a large mass
extending across the midline, engulfing abdominal vessels and
dislocating surrounding structures [4]. In this case, there was a
mass of the adrenal gland with the feature consistent with
neuroblastoma.
The prognosis of neuroblastoma varies depending on whether the tumor has
spread or metastasized (such as to the liver or bone) [4]. Despite
the younger age of onset, which is usually a favorable prognostic
indicator, PTD has a high-risk disease, based on his n-MYC status and
metastasis to cortical bone [9].
Management of high-risk disease includes induction chemotherapy, local
control with surgical resection and radiation, consolidation, and
maintenance phases. It has a poor prognosis, with a 5-year survival
between 40 – 50% [10].
PTD’s case shows the importance of keeping an open mind to a range of
differentials, particularly when these include serious diseases. It also
illustrates the need to examine the entirety of a child’s social
situation to ensure there are no limitations to comprehensive care
during treatment: PTD was presented to the hospital with six hours of
increased work of breathing on a background of two days of rhinorrhea,
cough and bruising around his right eye. His father is a drug addict.
So, our first impression of his condition could be a case of ”child
abuse”. However, after a careful physical examination, we did not detect
any additional bruises on his body. So, we eliminated ”child abuse” in
our diagnosis.
PTD has now completed his induction chemotherapy. He is living well with
his grandmother. Our hospital has to provide financial supports to
ensure he is able to attend future appointments and interventions.