Incidental Neuroblastoma in 5-month-old
A 5-month-old boy was taken to the pulmonology clinic after complaining of coughing for two months. There were no other related symptoms. The vital signs and physical exam were also normal. A fusiform mass in the left paraspinal area established an obtuse angle with the spine on an anteroposterior chest radiograph (Panel A, asterisk). A later computed tomographic imaging of the chest revealed a posterior mediastinal mass (Panel B); radiologic characteristics of the mass included calcifications, heterogeneous enhancement, and expansion into the intervertebral foramina (not visible in this picture). A surgical biopsy material was histopathologically examined and indicated neuroblastoma.
Neuroblastoma is a tumour that develops in sympathetic nervous system tissues from neural crest cells. If the tumour arises from the paravertebral sympathetic ganglia in the chest, it may be discovered by chance on a chest scan. The mass’s compression of the lung tissue was assumed to be the source of the patient’s cough in this case. Due to the patient’s young age at the time of diagnosis, genetic testing for familial neuroblastoma was undertaken and found to be negative. The tumour was partially removed surgically, and was referred for chemotherapy. The patient was in remission and had no tumour recurrence at the 1-year follow-up appointment.
The tumour is usually found in the adrenal glands
Neuroblastoma is most usually found in the adrenal gland(s), but it can develop anywhere there is sympathetic nerve tissue, including the paraspinal sympathetic ganglia in the abdomen and chest. The term neuroblastoma refers to a group of neuroblastic tumours that include neuroblastomas (the most frequent kind), ganglioneuroblastomas, and ganglioneuromas. The vast majority of neuroblastomas are diagnosed in children under the age of five, and virtually all patients are diagnosed by the age of ten. Similarly, the average age of diagnosis is about 18 months.nNeuroblastoma is the most frequent solid tumour in children that does not arise from the brain, accounting for roughly 8% of all childhood malignancies. This is a common occurrence in developed countries.
Given the disease’s rarity, exact correlations are difficult to establish, and no single environmental exposure has been linked to the development of incidental neuroblastoma
Neuroblastoma is a type of embryological cancer that arises from neural crest cells. During foetal development, the neural crest is a collection of neuronal cells that migrate from the spinal cord to generate a variety of structures, including the sympathetic nervous system. As a result, the cancer is discovered to have originated from sympathetic ganglia near the spinal cord.
Tumours develop from primitive neuroectodermal cells or neural crest cells (precursors of the adrenal medulla). The histology is comparable to that of tiny round blue cell tumours 3. The majority of them had 1p deletion and N-myc amplification on chromosome. Macroscopically, they are huge grey-tan coloured soft lesions with or without a fibrous pseudocapsule; thus, some are highly defined, while others are infiltrative. Necrosis, haemorrhage, and, in particular, calcification are all highly common.They produce Homer Wright rosettes under the microscope. The majority of them secrete catecholamines, specifically vanillylmandelic acid (VMA) and homovanillic acid (HVA).
Treatment and prognosis
Treatment is determined by the patient’s stage. Localised tumours deemed ‘low-risk’ are surgically removed or sent for minimally invasive surgery (MIS), and patients typically fare well (see below). In ‘high-risk’ tumours, a combination of surgery, chemotherapy, and/or bone marrow transplantation is used, with disappointing overall results. In some circumstances, where tumours are exceedingly large, presurgical chemotherapy may be used to try to downstage the tumour.
Patients in stages one, two, or four have a better prognosis. Unfortunately, 40-60% of patients arrive with illnesses at stages 3 or 4. The age of the child is the most critical factor in advanced sickness.
Source: NEJM
