and Mhamed Harif2
(1)
South African Medical Research Council, Cape Town, South Africa
(2)
Université Mohammed VI des Sciences de la Santé Cheikh Khalifa Hospital, Casablanca, Morocco
Keywords
Respiratory emergenciesSuperior vena cava syndromeCentral airway compression syndromePericardial and pleural effusionCardiac tamponadeSpinal cord compressionLeukocytosisTumor lysisHypercalcemiaMetabolicMucositisTyphlitisHemorrhagic cystitisChemotherapy extravasationDefinition
Oncological emergencies are defined as acute and potentially life-threatening events that are directly or indirectly related to the underlying malignancy or occur as sequelae of its treatment. These events may rapidly result in permanent morbidity or the death of a patient if not anticipated, quickly recognized, and effectively treated.
Respiratory Emergencies
Case Presentation 1
A 9-year-old girl, previously well, presented with a 1-month history of shortness of breath which was attributed to a respiratory infection and asthma. The symptoms worsened, she developed chest pain on the left side, and subsequently she was admitted to hospital.
Findings on Examination
The patient did not look acutely ill, but complained of chest pain.
Apart from a respiratory rate of 35/min and a pulse rate of 100/min, her observations were normal. Normal anthropometry was noted. Pallor and mild periorbital edema was present. Her neck and left side of the chest appeared swollen (Fig.5.1); no skin changes were noted. She had significant cervical and submandibular lymphadenopathy, more prominent on the right side, as well as axillary lymphadenopathy. On examination of the chest, decreased air entry was found on the left side with no adventitious sounds or dullness. Hepatosplenomegaly was present, but the rest of the examination was unremarkable. The chest X-ray showed a mediastinal mass (Fig. 5.1).
Fig. 5.1
Swollen neck and left side of chest, cervical and submandibular lymphadenopathy and mediastinal lymphadenopathy on chest X-ray
Which oncological emergency is most likely present?
This is most likely superior vena cava syndrome (SVCS) , due to obstructive lymphadenopathy caused most often by acute (T lymphoblastic) leukemia/lymphoma.
Briefly describe the immediate investigations and management.
Provide supportive oxygen as needed; monitor patient closely.
Monitor intake and urine output.
Full blood and differential count, reticulocyte count and peripheral smear.
Biochemistry (electrolytes, renal function, calcium, magnesium, phosphate, uric acid, lactate dehydrogenase, and other liver enzymes).
Bone marrow aspiration and biopsy.
Choose the diagnostic modality that would confirm a diagnosis in the fastest way: fine needle aspiration for cytology and flow cytometry (if available) or lymph node biopsy and bone marrow biopsy.
Start hyperhydration and allopurinol to prevent/treat tumor lysis syndrome (TLS).
Superior Vena Cava Syndrome
SVCS or superior mediastinal syndrome (SMS) is a clinical phenomenon that develops when a mass lesion compresses and obstructs the great vessels and heart, especially the right ventricular outflow tract and therefore causes obstruction of the blood flow in the SVC. The collateral veins of the thorax, neck, and head become congested and cause the classical symptoms (see below). The term ‘superior mediastinal syndrome’ is used when the respiratory symptoms and compromise predominate the clinical picture.
In children, the most likely cause of SVCS is malignant tumors (Table 5.1). The so-called “terrible Ts”—T-cell lymphoblastic lymphoma, T-cell acute lymphoblastic leukemia, malignant teratoma, thyroid cancer, and thymoma—may all cause this syndrome. Other malignancies associated with SVCS include Kaposi sarcoma, Ewing sarcoma, rhabdomyosarcoma, and peripheral neuroectodermal tumor (PNET). In areas endemic for TB, one should always consider TB adenitis, which can also present with a similar clinical picture.
Table 5.1
Common mediastinal tumors in children
Anterior mediastinum |
• Non-Hodgkin lymphoma |
• Hodgkin disease |
• Teratoma |
• TB/Kaposi sarcoma |
Middle mediastinum |
• Lymphoma |
• TB/Kaposi sarcoma |
Posterior mediastinum |
• Neuroblastoma |
Clinical Presentation
The most common complaints are cough, facial swelling, dyspnea, and orthopnea. Low grade fever and weight loss may or may not be present. Other symptoms include headache, dizziness, visual disturbances, hoarseness, dysphagia, and fatigue. Symptoms worsen in the supine position and patients usually prefer to sit upright. Classical signs include plethora and edema of the head, neck and upper limbs, marked venous distension, laryngeal edema, stridor, wheezing, and anxiety. Significant lymphadenopathy is very often present and may give rise to swelling of the neck, as well as the suprasternal notch. On auscultation of the chest, reduced or absent air entry may be noted.
Diagnosis
The diagnosis of SVCS is usually made clinically. In the majority of the cases, a chest X-ray shows a mediastinal mass or widening of the mediastinum. A pleural or pericardial effusion may be present. Rarely, the chest X-ray may be normal. Computed tomography (CT), if available and if the patient is able to tolerate a supine position, should be used to obtain detailed anatomical information, as well as to evaluate tracheal compression prior to decisions regarding sedation/anesthesia (Fig. 5.2).
Fig. 5.2
CT chest showing a mediastinal mass and tracheal occlusion by the mass
Great caution has to be taken when transporting the patient to the radiology department. Ensure that he/she remains in the upright position. Positioning patients in a supine position for CT scan can be extremely risky and even fatal.
A diagnosis should be confirmed by using the least invasive and fastest method. If significant lymphadenopathy is present, a fine needle aspiration (FNA) may be performed if the local pathologist is experienced in the interpretation thereof. Flow cytometry should also be performed on the same specimen. This is usually adequate to confirm the diagnosis of T cell lymphoblastic lymphoma. Examination of the pleural fluid (chemistry, cytology, and flow cytometry) may also be performed. A bone marrow aspiration and biopsy should follow to make the distinction between T cell lymphoma and leukemia on the grounds of the blast percentage. If FNA cannot be performed, an urgent biopsy of the most accessible lymph node should be performed. If no superficial lymphadenopathy is present, a thoracic surgeon should perform a biopsy of the mediastinal mass.
Management
Patients with SVCS have a high risk of airway compromise and often present with life-threatening respiratory distress and stridor, necessitating admission to an intensive care unit. In some instances immediate emergency treatment may need to be given before a definitive diagnosis has been made.
The main aim is always to secure the airway of the patient. Oxygen should be provided via a face mask or rebreather mask and intravenous access should be obtained. A helpful measure to alleviate the respiratory distress is strict bed rest with the patient sitting in a left lateral decubitus position (this helps to “lift” the mass off the airways and right ventricular outflow tract). Inability to tolerate the supine position is an ominous sign and such patients should definitely be admitted to an intensive care unit.
Treatment
Considering that T cell lymphoblastic lymphoma/leukemia is the commonest cause for a mediastinal mass in a child, treatment should be directed against T lymphoblastic lymphoma/leukemia without waiting for a tissue biopsy if the patient’s airway is compromised.
Empiric chemotherapy (cyclophosphamide, anthracyclines, vincristine, and prednisone as specified in the CHOP, BFM, or COG protocol) may be initiated. An alternative combination may be used, depending on the most probable diagnosis. The use of intravenous steroids (methylprednisolone 50 mg/m2/day in 4 divided doses) may contribute to the reduction of the tumor size and rapidly decrease the severity of the clinical symptoms and signs.
Alternatively, radiotherapy (daily dose of 200–400 cGy) may be commenced. As most lymphomas are radiosensitive, improvement is usually seen within 18 hours. Radiation-induced edema might occur and can result in temporary worsening of symptoms and signs. Note that chemo- or radiotherapy could result in distortion of the histology, thus making it difficult to establish a definitive histological diagnosis.
Anticipate TLS in these patients and start hyperhydration and allopurinol as soon as possible. Monitor intake and urine output closely and repeat biochemistry at least twice a day if possible.
In critical airway compromise, AVOID sedation for procedures unless an anesthesiologist is present and prepared for a very difficult intubation and involve a pulmonologist/intensivist, if available.
Case Presentation 2
A 9-year-old boy presented with anxiety, restlessness, and a sharp pain in his neck, chest, and shoulder. The chest pain worsened with deep breathing or coughing and he was short of breath. He reported that his discomfort was relieved by sitting upright or leaning forward.
Findings on Examination
The boy appeared pale and gray and was tachypnoeic (rate 40/min). His heart rate was 140/min, his peripheral pulses were weak, and pulsus paradoxus was noted. Distended neck veins were present. The apex was found in the 6th intercostal space lateral to the midline. His heart sounds were very soft and a pericardial rub was heard. The chest X-ray is shown in Fig. 5.3.
Fig. 5.3
Chest X-ray showing cardiomegaly with a globular heart shape
Pericardial Effusion/Cardiac Tamponade
Pericardial effusion is an important oncological emergency in children. It is seen with a variety of tumors including lymphomas and metastatic solid tumors . It is often asymptomatic and incidentally observed on a chest X-ray performed to evaluate the lungs or diagnosed as an incidental finding at autopsy.
Inflammation of the pericardium or obstruction of lymphatic drainage from the pericardium of any etiology causes an increase in fluid volume, referred to as a pericardial effusion.
Malignant involvement of the pericardium may be primary (less common) or secondary (spreading from a nearby or distant focus of malignancy). Secondary neoplasms can involve the pericardium by contiguous extension from a mediastinal mass, nodular tumor deposits from hematogenous or lymphatic spread, and diffuse pericardial thickening from tumor infiltration (with and without effusion). In diffuse pericardial thickening the heart may be encased by constrictive pericarditis.
Cardiac Tamponade
Cardiac tamponade is the inability of the ventricle to maintain cardiac output because of extrinsic pressure caused by a mediastinal or an intrinsic mass . It is the most serious consequence of a pericardial effusion, when the accumulation of fluid compresses the heart chambers and results in a reduction of venous return and decrease in cardiac output.
In pediatric patients, cardiac tamponade results from a malignant or reactive pericardial effusion. It is not a common condition and to date only 9 cases have been reported as result of acute myeloid leukemia (3 patients) and one each of acute lymphoblastic leukemia, Hodgkin lymphoma, B-cell lymphoma, medulloblastoma, desmoplastic small round cell tumor, and rhabdomyosarcoma.
Clinical Presentation
Symptoms are similar to those of heart failure and include shortness of breath as the main symptom, cough, palpitations, and chest pain improved by sitting, as well as nonspecific abdominal pain.
On examination, the main findings are jugular venous distension, pulsus paradoxus, soft heart sounds, pericardial rub, and signs of low cardiac output, namely tachycardia with hypotension and poor peripheral perfusion.
Diagnosis
A chest X-ray shows cardiomegaly and an ECG demonstrates low voltage QRS complexes and flattened or inverted T waves. Echocardiography is the best investigation, showing pericardial effusion and atrial or ventricular collapse with hemodynamic compromise.
Treatment
The treatment of choice is percutaneous catheter drainage under electrocardiographic or fluoroscopic guidance. While that is being arranged, an emergency pericardial tap to relieve symptoms can be performed in the ward at the bedside. This has to be done slowly to drain just enough fluid to avoid circulatory collapse. Fluid should be sent for cytology. Once the underlying malignancy is treated, the pericardial effusion will resolve.
Case Presentation 3
An 8-year-old boy, previously treated for T lymphoblastic lymphoma, presented with a 1-month history of shortness of breath and right-sided chest pain .
Findings on Examination
The patient appeared acutely ill. He was tachypnoeic with a respiratory rate of 40/min and tachycardic (pulse rate 110/min). The oxygen saturation in room air was 85 %. There was decreased expansion of the right hemithorax and the trachea was displaced to the left. Stony dullness of the right hemithorax was found, as well as no air entry. The chest X-ray is shown in Fig. 5.4.
Fig. 5.4
Dense, homogenous opacification of right hemithorax with the mediastinum displaced to the left
Malignant Pleural Effusion
Pleural effusions are often seen in children. Pulmonary tuberculosis frequently presents with a pleural effusion and pneumonia is often complicated by the development thereof. It is also often seen in lymphoma, especially non-Hodgkin lymphoma. Pleuropulmonary blastoma, metastatic sarcoma, and nephroblastoma, as well as Kaposi sarcoma, are other examples of malignancies that can cause effusions. Nonmalignant causes include SVCS, cardiac tamponade, chylothorax, empyema, congestive cardiac failure, and hypoalbuminemia among others. Several antineoplastic agents such as methotrexate, procarbazine, cyclophosphamide, and bleomycin have been reported to cause pleural effusion. Although pleural effusion is not life-threatening, sizeable and rapid accumulation of fluid can compress lung parenchyma leading to respiratory insufficiency.
Clinical Presentation
Symptoms are related more to the rate of pleural fluid accumulation rather than to the total volume of fluid. The most common symptoms are dyspnea, orthopnea, cough, and pleuritic chest pain. The presence of fever suggests atelectasis and/or infection.
Diagnosis
A chest X-ray (anteroposterior and lateral), including a lateral decubitus chest film, is useful in confirming the presence of a pleural effusion. Ultrasonography, and CT where available, is useful in differentiating fluid from a solid mass, especially if a pleural tap and/or biopsy is planned.
Treatment
A diagnostic and therapeutic thoracocentesis (pleural tap) is indicated in patients presenting with respiratory distress. Initially drainage can be performed manually using a large bore needle, attached to a three-way stopcock, and a large volume syringe. This is followed by insertion of tube for more complete drainage. Chemotherapy is initiated once a definitive diagnosis is made.
Case Presentation 4
A 10-year-old girl, previously treated for nephroblastoma, presents with shortness of breath for 3 weeks. She also developed noisy breathing for the past few days.
Findings on Examination
On physical examination the patient was apyrexial , but acutely ill and short of breath (respiratory rate of 42/min) with a pulse rate of 133/min and oxygen saturation of 87 % in room air. The trachea was displaced to the left and there was reduced air entry and stony dullness in the right upper zone. Signs of hyperinflation were also noted. The chest X-ray is shown in Fig. 5.5.
Fig. 5.5
Several round areas of opacification in the right lung with displacement of the trachea and mediastinum
Central Airway Compression Syndrome
Airway obstruction is divided into proximal or large airway obstruction (upper airway) or distal (lower) airway obstruction. Mediastinal tumors are a common cause of upper and lower airway obstruction in children with cancer; often also causing SVCS. Kaposi sarcoma is becoming an increasingly common cause of lower airways obstruction.
Clinical Presentation
The clinical manifestations, investigations, and management are similar to that discussed under SVCS.
Treatment
Supportive care (oxygen, positioning, intravenous fluid, monitoring, etc.) should be provided while the diagnosis is being confirmed in the least invasive and fastest way. As in SVCS, treatment may need to be initiated without histological/cytological confirmation of a diagnosis.
Neurological Emergency
Case Presentation
An 8-year-old girl, previously well, presented with a 4-week history of backache and weakness of her legs that led to a fall at school.
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
