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.