Signs and Symptoms of Neuroblastoma

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Summary

NBL usually has a wide range of clinical symptoms depending on the primary tumor’s size, location, biological characteristics, and the presence or absence of distant metastatic disease. Symptoms of encephalopathy and paraneoplastic syndromes may be observed in addition to other associated symptoms in NBL patients.

Introduction

Most children with Neuroblastoma (NBL) more than often are between 1 and 5 years old, have an average age of 2 years, and have a large abdominal mass. This may be an incidental finding in a healthy child or in a child who appears to be unwell due to the metastatic spread of the tumor. In fact, NBL usually has a wide range of clinical symptoms depending on the primary tumor’s size, location, biological characteristics, and the presence or absence of distant metastatic disease. There may be non-specific findings of bone marrow involvement, such as general skeletal pain, swelling, or arthritis-type complaints, effects of hormone production and weight loss, malaise, fever, anemia, and irritability. Up to half of the patients with spinal cord enlargement may develop peripheral neurological symptoms and deficits due to compression of the nerve roots or spinal cord​1,2​.

Symptoms and associated syndromes

Symptoms of encephalopathy can occur in children with NBL and are associated with factors such as catecholamine-induced hypertension or an autoimmune response to tumors​3,4​. The spread of cancer to the orbit or compression of the optic nerve can also lead to blindness​5​.

In less than 2% of cases, NBL may indicate paraneoplastic syndromes, such as myoclonic ataxia syndrome or watery diarrhea​2,3​. In fact, Opsoclonus-myoclonus is a twitching of the extremities and eye movements reported as “dancing eyes and legs”, caused by an unknown mechanism. Sometimes cerebral ataxia is also combined along with these factors. It is commonly seen in thoracic tumors and hence is associated with a better prognosis​1​. Markedly, diarrhea is caused by the formation of a tumor of vasoactive intestinal peptides. It’s similar to intestinal malabsorption disease and eventually disappears after the complete removal of the tumor​1,2​.

NBL may be accidentally detected during the scan for other reasons. For instance, tests like Prenatal ultrasound, chest radiography, or pneumonia screening protocol​3​ can reveal NBL. During the year 1973, screening was introduced in Japan to measure the levels of VMA and HVA in the urine of 6-month-old infants​6​.

A common finding in children with NBL during clinical examination is the presence of abdominal mass. Additional findings include renin-associated hypertension (due to compression of the renal arteries), dyspnea (chest mass, large abdominal mass, or infiltrative hepatomegaly that elevates the diaphragm), intestinal or bladder dysfunction (pelvic mass), and umbilical cord obstruction or neurological insufficiency​1,2​. Cervical NBL manifests such as dysphagia, isolated neck mass, or stridor. Horner’s syndrome may appear after surgery due to a rupture of the sympathetic chain in the neck​1​, or it may appear with signs of “harlequin” presenting a small number of hot flushes due to sympathetic vasodilation​7​.

References

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    Lonergan G, Schwab C, Suarez E, Carlson C. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics. 2002;22(4):911-934. doi:10.1148/radiographics.22.4.g02jl15911
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    Hiorns M, Owens C. Radiology of neuroblastoma in children. Eur Radiol. 2001;11(10):2071-2081. doi:10.1007/s003300100931
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    Kushner B. Neuroblastoma: a disease requiring a multitude of imaging studies. J Nucl Med. 2004;45(7):1172-1188. https://www.ncbi.nlm.nih.gov/pubmed/15235064
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    El-Hayek M, Trad O, Hardy D, Islam S. The triad of seizures, hypertension, and neuroblastoma: the first described case. J Pediatr Hematol Oncol. 2004;26(8):523-525. doi:10.1097/01.mph.0000134464.86671.67
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    Lau J, Trobe J, Ruiz R, et al. Metastatic neuroblastoma presenting with binocular blindness from intracranial compression of the optic nerves. J Neuroophthalmol. 2004;24(2):119-124. doi:10.1097/00041327-200406000-00005
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    Okazaki T, Kohno S, Mimaya J, et al. Neuroblastoma detected by mass screening: the Tumor Board’s role in its treatment. Pediatr Surg Int. 2004;20(1):27-32. doi:10.1007/s00383-003-1070-x
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    Yanchar N, Chou S. Pitfalls in the interpretation of MIBG scans in cervical neuroblastoma. Pediatr Surg Int. 2000;16(5-6):451-453. doi:10.1007/s003830050041