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.
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References
- Children, teenagers and young adults UK cancer statistics report 2021.
- Children’s cancers incidence statistics. Cancer Research UK.
- Children and young people with cancer. Briefing Paper. National Cancer Intelligence Unit (2013).
Further Reading:
- Thorbinson C et al., An approach to oncological abdominal masses in children. Paediatric and Child Health, Elsevier. VOLUME 31, ISSUE 7, P295-299, 2021.
- Uzunova L, Bailie H, Murray MJ. Fifteen-minute consultation: A general paediatrician’s guide to oncological abdominal masses. Arch Dis Child Educ Pract Ed. 2019 Jun;104(3):129-134.
- Behjati S, Ruffle A, et al., Fifteen-minute consultation: Initial management of suspected acute leukaemia by non-specialists. Arch Dis Child Educ Pract Ed. 2020 Apr;105(2):66-70.
- Howard Scott C et al., The Tumor Lysis Syndrome. N Engl J Med. 2011 May 12; 364(19): 1844–1854.
6 Comments
This topic covered essential points in Paedatic oncology. Explains what clinicians need to know. Thanks.
Important topic, concise and informative
Brilliant brief paeds oncology blog.
Very useful and informative
Great module and good introduction to paediatric oncology. I learnt about paediatric cancer statistics in UK. I am not sure if it is good that more than half of them are diagnosed in ED.
I especially like the summary of blood results interpretation, thank you