Neuroblastoma

Case contributed by Dr Candice Norris

Presentation

4 day history of fevers, pallor, lethargy with associated generalized abdominal pain and distension. Normal birthing and developmental milestones and nil prior history of similar episodes or presentations. Nil haematuria, nausea, vomiting, weight loss or other constitutional symptoms. On examination vitals were within normal parameters but the patient appeared pale. Abdomen was moderately distended and taut at rest with a palpable left flank mass. Baseline blood results showed a microcytic anaemia (Hb 66, MCV 58).

Patient Data

Age: 2-year-old
Gender: Female
X-ray

Chest radiograph

A large soft tissue opacification is noted in the left upper quadrant which is displacing adjacent bowel and raising the left hemidiaphragm. 

CT

CT Abdomen and pelvis

There is a large heterogeneous mass occupying the left flank and upper quadrant. It measures 14cm craniocaudally, 10cm anteroposteriorly and 11.5cm transversely. No obvious calcification in the lesion is identified. A claw sign is present with some normal appearing renal parenchyma in the left renal lower pole at the inferior edge of the lesion, suggestive of a mass arising from the left kidney. Extrinsic compression of the left renal vein but no thrombus within the left renal vein or within the inferior vena cava. No active bleeding within the tumour mass is seen. There is mild hydronephrosis of the lower pole of the left kidney. 

There is soft tissue in the left para-aortic position, displacing and partially compressing the abdominal aorta, including some soft tissue at the anterior, posterior and left side of the abdominal aorta, with the lower half of the abdominal aorta being displaced to the right. The splenic vessels and pancreas are displaced anteriorly due to the left abdominal mass. No involvement of the spleen and no splenomegaly. No focal liver lesions. The gallbladder is normal in appearance. The right kidney is normal.

A small amount of free fluid is present in the pelvis, posterior left upper quadrant and posterior to the spleen. No free gas.  

Case Discussion

Neuroblastomas (NBL) and nephroblastomas/Wilm's tumours (WT) represent two of the most commonly detected solid tumours in childhood 1. These are the two crucial differential diagnoses to be considered with any renal mass identified in a child of this age group 2.

Features strongly predictive of neuroblastoma on CT have also been seen in a significant number of patients with Wilm's tumours and this is highlighted by the case outlined above. Their disease sequelae, prognosis, management and potential complications are however vastly different and therefore not confusing or misdiagnosing one for the other is important.  This case, due to the overlap in similar radiological features of both WT and NBL, demonstrates how CT imaging, as important as its role is, only constitutes one part of the investigative spectrum and is not a gold standard modality in establishing a correct diagnosis 2

The mass identified on CT is heterogeneous in appearance which favours both diagnoses but with an irregular margin is more consistent with an NBL. A WT is typically well circumscribed, with smooth margins and often forms a pseudocapsule. No calcification is present within the mass. Calcifications and low attenuation necrotic areas are more common in 80-90% of NBLs whereas WTs present with calcifications in only 10-15% of cases3

A claw sign is present and favoured to represent a WT. This refers to the delineated, sharp angles and margins on either side of a mass which the normal surrounding parenchymal tissue forms when the mass originates from the parenchyma 4.  

There is soft tissue, likely tumour extension into the left para-aortic position, displacing and partially compressing and encasing the abdominal aorta without invading it. There is extrinsic compression of the left renal vein but nil extension and nil invasion of the inferior vena cava. These features are typical of an NBL over a WT. WTs tends to extend into the renal vein and IVC whereas a NBLs often encases and displaces vessels 5.  

Additionally, the age group of 2 years is more suggestive of a likely NBL whereas WT affects slightly older children often aged between 3-4 yrs. 

Overall, features are mostly suggestive to represent an NBL but does have overlapping radiological features consistent with the presence of a WT. 

The patient was admitted overnight, received a blood transfusion and was transferred to an children's hospital under a Paediatric Oncology team for further investigations and arrangement of a biopsy to obtain a histopathological confirmatory diagnosis. 

She was found to have a high-risk neuroblastoma due to age, Nmyc amplication and extension of tumour and was commenced on chemotherapy per ANBL0532 Induction. Microscopic histopathology reports showed a small blue cell tumour growing as poorly formed nodules with a sheet-like architecture, with poor to moderate cohesion. The cells are hyperchromatic with fine chromatin and scant cytoplasm. There are focal areas of tumour necrosis. No calcification is present. Mitotic activity is easily identified. A bone marrow biopsy showed no disease present.

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Case information

rID: 50955
Case created: 30th Jan 2017
Last edited: 30th May 2017
Inclusion in quiz mode: Included

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