Oncological Emergencies: A Must Know Condition for Families With Childhood Cancer

When cells mutate abnormally among children, it results in the occurrence of paediatric or childhood cancer. Affecting children of the age group of 0 to 14 years, childhood cancer is not just a threat to tender lives, but also to their physical dignity. Although childhood cancer is uncommon compared to adults, it still is a significant problem. Majority of the cases, there is no known cause, however in a few children environmental factors like exposure to radiation, parental exposure to smoking and certain chemicals and heredity are known to be primary risk factors for childhood cancer.
 
The consequences of childhood cancer and its treatment on the children and their families are adverse and significant. Besides the cancer itself inflicting pain, discomfort and ailment to the body, the treatment comes with severe impact on both the body and mind of the children. Cancer treatments are observed to alter neural development and delay cognitive, behavioural and emotional functions among children. They experience decreased physical, emotional and social health related quality of life and this interferes with their ability to adjust. This has a direct impact on the families of the children with cancer. Most families develop sustained poor functioning as they face challenges, socially and economically, to take care of the children.
 
Childhood cancers can have varied presentations. Although rare, some paediatric cancers are characterised by a condition called Oncological emergencies which could be life threatening. This needs to be handled urgently.
 
About Oncological emergencies
 
Oncological emergencies are defined as any acute, morbid or life-threatening events among children suffering from cancer which are caused either due to the malignancy or due to the treatment. Such emergencies can develop any time throughout malignancy - from initial symptoms to the end stage of the disease.
 
Causes of Oncological emergencies
 
Characterised with a wide range of life-threatening situations, oncological emergencies may result from functional compromise of the cardiopulmonary or neurologic systems, hematologic abnormalities or a compromised immune system. The situation could arise either due to the disease or due to the consequence of the treatment offered for the disease. Children suffering from cancer are most often immunocompromised secondary to the treatment, thereby, posing several diagnostic and therapeutic challenges.
 
Common kinds of Oncological emergencies and their treatments
 
The various kinds of Oncological emergencies are
 
●      Hyperleukocytosis
●      Tumour lysis syndrome
●      Febrile Neutropenia
●      Superior vena cava syndrome
●      Spinal cord compression
●      Bleeding diathesis
 
Hyperleukocytosis: A condition when the peripheral Leukocyte which is above 100,000/cu mm and is enhancing progressively, is known as hyperleukocytosis. It is more commonly seen among children suffering from Acute Lymphocytic Leukaemia (ALL) and Acute Myeloid Leukaemia (AML). Among those suffering from ALL, patients are likely to get acute tumour lysis syndrome due to increased sensitivity of lymphoblasts to chemotherapy. In case of those with AML, it causes hyperviscosity and haemorrhage in the brains and lungs due to their rigidity and stickiness.
 
Treatment
Patients should be kept hydrated. Steps should be taken to prevent uric acid nephropathy. Red cell transfusion and diuretics should be avoided to prevent hyperviscosity. 
 
Tumour Lysis Syndrome: Characterized by - hyperuricemia, hyperkalemia, and hyperphosphatemia  and hypocalcemia- combine to give rise to Tumour Lysis Syndrome. This occurs due to the rapid release of intracellular metabolites at a rate which is far beyond the kidney’s excretory capacity. This usually develops before or during the first few days of chemotherapy. Those with bulky T-cell or B-cell leukaemia or lymphomas are more prone to get this condition.
Treatment
 
Although prevention is better than cure, there are certain specific ways of treating this kind of an oncological emergency which are reducing uric acid production, enhancing uric acid solubility and curbing the concentration of uric acid in urine.  Hydration with diuretic therapy, urine alkalinisation and medications to bring down uric acid levels are some of the effective ways to treat this.
Febrile Neutropenia :  It is a condition characterized by fever and low blood counts- mainly the neutrophils which are a type of WBC count present in blood. Neutrophils are responsible for immunity and when low increase risk of infections mainly with bacteria, and fungus and sometimes viral too. Febrile neutropenia usually happens when children are receiving chemotherapy for various cancers. It is more common in intense protocols like during Bone marrow transplant- allogenic and autologous, but also in Leukemia, Lymphoma treatment and other solid tumors.
Treatment:  
It is an Oncological emergency and most children would require quick clinical evaluation followed by admission to the hospital unit. Relevant blood tests including blood cultures would be sent and kids need to be started on broad spectrum antibiotics. If managed early and appropriately, most kids would do well.
Bleeding diathesis: It is a condition characterized by bleeding, which may include skin bleeds, mucosal bleeds, Nasal or oral, or through urine or stool. Serious life threatening bleeding may include CNS or brain hemorrhage, gastrointestinal, genitourinary. It may be due to low platelets, most of the times but can also be due to clotting factor deficiency, liver disease or coagulopathy. It can happen either at the time of diagnosis, or during therapy for childhood cancer.
Treatment : 
 
Usually these kids require urgent admission and evaluation. Platelet transfusions and sometimes PRBC transfusions also. If there is problem with clotting factors- then these children may need FFP- fresh frozen plasma and sometimes vitamin K. Other supportive treatment to minimize and control bleeding and to stabilize the child may be needed.
Superior Vena Cava Syndrome : When there are signs that are associated with compression or obstruction to the vena cava - a large vein that carries blood to the heart from other areas of the body, it is called superior vena cava syndrome. When there is also compression of the airway- it is called as Superior Mediastinal compression syndrome (SMS).  It is closely associated with non-Hodgkin's lymphoma (NHL), ALL (T-cell), Hodgkin's disease, Neuroblastoma, and Ewing's sarcoma. Some of the common symptoms include cough, hoarseness, dyspnoea, stridor along with facial puffiness, plethora, cyanosis (facial), and suffusion of conjunctiva, venous engorgement of the neck, chest, and arm with collateral vessels.
Treatment
Tissue diagnosis via least invasive means like from blood tests, Bone marrow examination, lymph node biopsy or rarely direct biopsy from the mediastinal mass under close monitoring.  Sometimes empirical therapy, radiotherapy with steroid for superior vena caval syndrome may be life saving. Improvement is observed within 12 hours of initiating radiotherapy – if indicated. Chemotherapy is useful for children with leukaemia and lymphoma. An urgent thoracotomy (rarely required) is required if no improvement is seen within three to four days.
 
Spinal Cord Compression: Local extension or spread of the tumour to the spinal cord leads to spinal cord compression. Those with Ewing's sarcoma, neuroblastoma, lymphoma, leukaemia, and osteogenic sarcoma are the ones who usually suffer from spinal cord compression. Back pain is the most common sign. Several neurological deficits may also develop.
 
Treatment
 
MRI, CT or PET CT scan and myelography are used for diagnosing spinal cord compression.
Immediate dexamethasone may be required. Local radiotherapy, surgical decompression, and chemotherapy could be used either singly or in combination.
 
Cardiac tamponade and neutropenic enterocolitis are other oncological emergencies that can be seen among children with cancer. These, however, are preventable and treatable if appropriate steps are taken and clinical interventions are made at the right time. It is, therefore, essential to be aware of these conditions and reach out to a doctor on time. The specialists at HCG can be reached at any point of time for detailed consultation on oncological emergencies.
 

Authored by:
Dr. Intezar Mehdi, Director and Head of the Department, Senior Consultant - Paediatric Haeamtology Oncology & BMT at HCG Cancer Centre, Bengaluru