Neonatal neuroblastoma
Section snippets
Definition
Tumours arising neonatally are detected either prenatally or during the first 28 days of life. Cancer presenting in the neonatal period is rare, and comprises 2% of all paediatric malignancies [1]. The most common neonatal tumours are neuroblastoma, retinoblastoma, extragonadal malignant germ cell tumours and leukaemia (Fig. 1).
General – all ages
Neuroblastoma originates from the primordial neural crest cells that normally develop into the sympathetic nervous system. It may arise from either the adrenal gland (50%) or anywhere along the sympathetic chain (50%). It is a tumour almost exclusively seen in children, with 90–100 cases diagnosed annually in the U.K. [2]. It is a heterogeneous tumour with wide ranging biological, genetic, morphological and clinical characteristics. 90% secrete catecholamines and take up metaiodobenzylguanidine
Neuroblastoma staging
The International Neuroblastoma Staging System (INSS) 1986 was based on the degree of surgical resection of the tumour [9]. World-wide, surgeons are not uniformly skilled and this system has proved to be subjective and not internationally comparable.
The International Neuroblastoma Risk Group Staging System (INRGSS) system [11], [12] (Table 3) is a recently developed classification system in which pretreatment patient cohorts are defined, which will facilitate the comparison of international
Prenatal
Neuroblastoma may be diagnosed after the incidental finding of a solid or cystic mass on the 20 week anomaly ultrasound scan. In an analysis of prenatally diagnosed suprarenal masses, 58% were subsequently diagnosed as localized neuroblastoma [14]. Other diagnoses that need consideration in this situation include adrenal haemorrhage, cystic renal or bowel lesions, extra lobar pulmonary sequestration and other benign adrenal lesions which undergo spontaneous resolution.
A study looking at cases of
Metastatic tumours
In stage MS, hepatomegaly (sometimes massive) because of infiltration with metastatic neuroblastoma is often the presenting symptom. This hepatomegaly can cause abdominal distension (Fig. 2) and may lead to respiratory compromise, renal failure or bowel dysfunction because of pressure effects. A severe and sometimes rapidly progressive coagulopathy can result from liver infiltration which will need urgent treatment of both the clotting disorder and the underlying neuroblastoma. Stage MS may
Management of an adrenal mass identified in the antenatal period
Routine prenatal scans can pick up masses in a foetus which may suggest a diagnosis of neuroblastoma. More than 90% of these tumours arise in the adrenal gland, suggesting a link between perinatal tumours and the nodular collections of neuroblasts that are part of normal adrenal development [23]. These suprarenal masses should be followed up by further prenatal and subsequently postnatal scans to determine whether the mass is regressing or increasing in size. Urinary catecholamines are
Outcome
Neuroblastoma in the neonatal period has an excellent prognosis, with studies reporting good overall survival rates of 91% [1] and 88% [15]. It is well recognized that the majority of cases of neuroblastoma presenting in this age group have favourable stage (localized) and tumour biology.
In a recently published review of 134 cases of neuroblastoma in the newborn over a 27 year period, Gigliotti et al. [31] reported that 65% had localized and 34% had metastatic disease (all stage 4S except 1 case
Research directions
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Development of a more sophisticated molecular biological techniques (array CGH and MLPA) to detect DNA copy number alterations which will allow more accurate identification of biologically higher risk tumours and therefore achieve improved treatment stratifications for low and intermediate risk tumours.
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Ongoing development of less toxic therapies using biologically based agents that will target genes, proteins and pathways involved in the development of neuroblastoma.
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Continued clinical
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Cited by (16)
Malignant neoplasms in the neonate
2014, Boletin Medico del Hospital Infantil de MexicoNeonatal neuroblastoma
2012, Seminars in Fetal and Neonatal MedicineCitation Excerpt :Neuroblastoma is by far the most prevalent cancer of infancy (age <12 months) with an incidence rate almost double that of leukaemia, the next most prevalent malignancy occurring during the first year of life.3 One study reported that 16% of infant neuroblastomas were diagnosed during the first month of life (i.e. neonatal) and 41% during the first 3 months.4 Data from the UK National Registry of Childhood Tumours found that between 1991 and 2000, 22.6% (43/190) of neonatal cancer cases registered in Great Britain were neuroblastomas (reviewed by Dhir and Wheeler4).
Congenital Sacrococcygeal Neuroblastoma: A Report of Two Cases with Summary of Prior Published Cases
2022, Fetal and Pediatric PathologyNeonatal Neuroblastoma
2019, Neuroblastoma: Clinical and Surgical ManagementAcute shock secondary to congenital neuroblastoma. Neonatal case report
2019, Archivos Argentinos de PediatriaPrenatally detected thoracic neuroblastoma
2018, Obstetrics and Gynecology Science