Authors: Abishek Ajay Kaul, Ashraf Ali Rasheed Ali / Editor: Liz Herrieven / Codes: GP2, OncP1, SLO5, SLO7 / Published: 22/02/2022

Let’s begin with a case…

You are the ST3 working in the Children’s ED. Your next patient is an 18 month old girl, labelled ‘constipation’ at triage. You immediately notice that the child is grunting. She has had episodic fevers for the last month. She is rather listless, pale, tachycardic and tachypnoeic. The abdomen is very obviously distended and there’s a palpable mass in the left upper quadrant. What is going through your mind at this stage? Abdominal distension, grunting, pallor, fever – that sounds like all kinds of badness…

Problem Statement: It’s all about the numbers

There are an average of 1645 cancer diagnoses in children and young people (0-14 years) in the UK each year1. Leukaemia accounts for 31% of cases, central nervous system (CNS) and miscellaneous intracranial and spinal neoplasms 25%, lymphomas 10%, soft-tissue sarcomas, neuroblastoma (and other peripheral nervous cell tumours) and renal tumours for 6%1 each. Alarmingly, the incidence of cancer in children has increased by 15% since the early 1990s2.

The ED often plays a vital role in making the first diagnosis of cancer. In children aged 0-14 years, 54% of first cancer diagnoses were made in the ED, while this metric stands at 24% in the 15–24-year group3. Therefore emergency physicians must be extremely vigilant for this presentation.

History and Examination: Specific points

 

Investigations

Aim to minimise trauma from the very first episode of cannulation.

Blood Tests  Interpretation
FBC

Cytopaenia in chronic disease or metastatic bone marrow infiltration

Thrombocytosis associated with hepatoblastoma

Venous Blood Gas  Acid-base status, lactate levels
Blood Culture

As indicated, prior to starting antibiotics

Group and save

For potential need for blood products

Blood Film

Lymphoblasts in lymphoma or leukaemia

Coagulation

DIC

Prolonged aPTT in acquired vWD with Wilm’s tumour

U&E

Acute kidney injury

Hyperkalaemia in tumour lysis syndrome (TLS)

LFT

Abnormal in cases of biliary obstruction

Bone Profile

Hypercalcaemia in paraneoplastic syndromes

Hypocalcaemia, hyperphosphataemia in TLS

LDH

Marker of cell turnover, raised in proliferative malignancies such as lymphoma and leukaemia

Uric Acid

Raised in TLS

Urine dipstick: Haematuria, proteinuria in the presence of a renal tumour.

Tumour markers: Alpha-fetoprotein (AFP), Beta-human chorionic gonadotropin (B-hCG), urine catecholamines can be performed as per expert advice.

Imaging: A chest X-ray can be obtained as per clinical need/presentation (e.g. respiratory symptoms) and to look for metastases or mediastinal mass.

An ultrasound of the abdomen is the most appropriate initial imaging.

CT and/or MRI can be considered in an unwell child with a suspected abdominal cause (e.g. bowel obstruction) but may involve sedation.

Management

Consider supportive care including oxygen support, analgesia (use opioids, avoid NSAIDs), fluid and electrolyte management. Other clinical concerns need to be addressed such as correction of anaemia and coagulopathy, with advice from Haematology and Oncology services. Surgical input may be needed in some cases.

Shocked or otherwise critically unwell children require resuscitation as per standard APLS protocols and local guidelines. Secondary sepsis, electrolyte abnormalities, tumour lysis syndrome, bleeding/coagulopathy and hyperleukocytosis are complications to watch out for. Children with suspected leukaemia should be managed as per neutropenic sepsis guidelines, irrespective of their neutrophil count.

Where relevant (clinical instability, very high WCC), a transfer to the tertiary oncology centre needs to be facilitated.

Breaking the bad news…

So, you have done all the medical bits and are feeling rather smug. But what and how much are you going to tell the family? It is important to forewarn them, especially if the child is getting admitted to an oncology ward. The term ‘cancer’ is not just associated with an outpouring of fear and emotion, but also a barrage of questions pertaining to further treatment and prognosis.

Have this discussion (seated!) in a quiet counselling room. Be open, honest and professional. It’s perfectly okay to say ‘I don’t know’. Avoid using medical jargon (eg. ‘cancer of the blood’ instead of leukaemia). Comment only on information that is available to you and to the extent of your knowledge on the matter. Prognostication and more specific treatment information can only be provided after further testing and specialist input. Being honest facilitates trust with future care givers as well. Similarly, children should be given age-appropriate information.

Back to the case…

Blood tests revealed a haemoglobin of just 41gm/dl, a platelet count of 45000/mm3 and a total white cell count of 3,02,000/mm3 (predominantly lymphocytes). Her LDH was 1440. She was admitted under Paediatric Oncology. Subsequent bone marrow analysis was suggestive of Acute Myeloid Leukaemia.

Learning Points:

  • Stick to the basics. A thorough history and examination, coupled with a high index of suspicion, goes a long way in unmasking these cases.
  • Do not hesitate to escalate concerns and obtain timely senior (and specialist) input.
  • Adopt a structured approach to investigations.
  • Practice open communication with the family in order to build trust and prepare the family for what potentially lies ahead.

You can now complete our SBA on Paediatric Oncology. Please log in to access it.

References

  1. Children, teenagers and young adults UK cancer statistics report 2021.
  2. Children’s cancers incidence statistics. Cancer Research UK.
  3. Children and young people with cancer. Briefing Paper. National Cancer Intelligence Unit (2013).

Further Reading: