PediaNotes

Oncological Emergencies other than TLS

7 min read


graph LR
     Main Categories
    MetHem[Metabolic & Hematologic]
    style MetHem fill:#e8f5e9,stroke:#2e7d32,color:#2e7d32

    Struct[Space-Occupying & Structural]
    style Struct fill:#ffebee,stroke:#c62828,color:#c62828

    Organ[Organ-Specific & GI]
    style Organ fill:#fff8e1,stroke:#f57f17,color:#f57f17

    SysTreat[Systemic & Treatment-Related]
    style SysTreat fill:#f3e5f5,stroke:#7b1fa2,color:#7b1fa2

     Sub-categories for Structural
    SVC[SVC / Superior Mediastinal Syndrome]
    style SVC fill:#ffebee,stroke:#c62828,color:#c62828
    Tamponade[Cardiac Tamponade]
    style Tamponade fill:#ffebee,stroke:#c62828,color:#c62828
    SCC[Spinal Cord Compression]
    style SCC fill:#ffebee,stroke:#c62828,color:#c62828
    ICP[Increased Intracranial Pressure]
    style ICP fill:#ffebee,stroke:#c62828,color:#c62828

     Sub-categories for Systemic/Treatment
    FebNeu[Febrile Neutropenia]
    style FebNeu fill:#f3e5f5,stroke:#7b1fa2,color:#7b1fa2
    CRS[Cytokine Release Syndrome]
    style CRS fill:#f3e5f5,stroke:#7b1fa2,color:#7b1fa2
    DiffSyn[Differentiation Syndrome]
    style DiffSyn fill:#f3e5f5,stroke:#7b1fa2,color:#7b1fa2
    Endo[Endocrine: SIADH / Adrenal Insufficiency]
    style Endo fill:#f3e5f5,stroke:#7b1fa2,color:#7b1fa2

    %% Connections
    Root --> MetHem
    Root --> Struct
    Root --> Organ
    Root --> SysTreat

    MetHem --> TLS
    MetHem --> HyperL
    MetHem --> HyperC

    Struct --> SVC
    Struct --> Tamponade
    Struct --> SCC
    Struct --> ICP

    Organ --> Typh
    Organ --> SOS
    Organ --> GI

    SysTreat --> FebNeu
    SysTreat --> CRS
    SysTreat --> DiffSyn
    SysTreat --> Endo

Cardiothoracic Emergencies

Superior Vena Cava Syndrome And Superior Mediastinal Syndrome

  • Results from compression of superior vena cava or trachea by anterior mediastinal masses.
  • Commonly associated malignancies:
    • Non-Hodgkin lymphoma
    • T-cell acute lymphoblastic leukemia (ALL)
    • Hodgkin lymphoma
    • germ cell tumors
    • neuroblastoma
    • thymoma.
  • Intrinsic etiologies: Intravascular tumor thrombosis (Wilms tumor, lymphoma), catheter-related thrombosis.
Clinical ManifestationsDiagnostic EvaluationManagement Strategy
Dyspnea, orthopnea, wheezing, stridor.Chest radiograph (CXR), Computed tomography (CT) scan.Elevate head of bed; provide oxygen.
Facial, neck, upper extremity edema and plethora.Echocardiogram (assess cardiac function, intravascular thrombus).High-dose corticosteroids (IV methylprednisolone 1 mg/kg/dose every 6 hours).
Cyanosis, conjunctival suffusion, engorged collateral neck veins.Determine anesthesia risk. Perform least invasive biopsy technique (local anesthesia).Avoid flat supine positioning (risk of respiratory arrest).
Altered mental status, syncope, headache.Flow cytometry on peripheral blood/pleural fluid.Emergent radiation or tumor-specific chemotherapy if steroid-unresponsive.

Cardiac Tamponade

  • Results from massive pericardial effusion, intracardiac thrombus, tumors, or radiation-induced constrictive pericarditis.
  • Presentation: Chest pain, cough, dyspnea, tachycardia, pulsus paradoxus.
  • Intervention: Oxygenation, hydration, emergent percutaneous pericardial drainage, fluid cytology.

Hematologic And Metabolic Emergencies

Hyperleukocytosis

  • Defined as total white blood cell (WBC) count >100,000/mm³.
  • High blast burden causes microcirculatory sludging, tissue ischemia, local cytokine production.
  • Adhesive reactions between blasts and endothelium worsen leukostasis, leading to thrombosis and secondary hemorrhage.
Disease SubtypeThreshold For RiskClinical Implications
Acute Lymphoblastic Leukemia (ALL) (T-cell, infant, Philadelphia+)>300,000/mm³Extreme risk of metabolic derangements.
Acute Myeloid Leukemia (AML) (FAB M1, M3, M4, M5, infant)>200,000/mm³High risk of intracranial hemorrhage, stroke, pulmonary hemorrhage.
Chronic Myeloid Leukemia (CML) (Blast crisis)>300,000/mm³Leukostasis, neurologic symptoms, hypoxia.

Management Interventions

  • Intravenous (IV) hydration >2 times maintenance rate.
  • Avoid packed red blood cell (RBC) transfusions (increases blood viscosity); transfuse only if hemodynamically unstable using small aliquots (5 cm³/kg).
  • Transfuse platelets if count <20,000/mm³.
  • Correct coagulopathy (vitamin K, fresh frozen plasma).
  • Leukapheresis or exchange transfusion (contraindicated in acute promyelocytic leukemia).

Hypercalcemia Of Malignancy

  • Pathophysiology: Osteolytic bone resorption (leukemia, lymphoma, rhabdomyosarcoma), paraneoplastic parathyroid-like hormone (PTHrP) production, immobilization, renal excretion defects.
  • Presentation: Anorexia, nausea, polyuria, polydipsia, constipation, weakness, bradycardia, shortened QT interval, coma.
  • Management:
    • Stop calcium-containing medications.
    • Aggressive IV hydration with normal saline (2-3 times maintenance).
    • Furosemide diuresis (1-2 mg/kg/dose every 6 hours).
    • Bisphosphonates (Pamidronate 0.5-1 mg/kg IV over 4-6 hours).
    • Corticosteroids (Prednisone 1.5-2.0 mg/kg daily) for lymphoproliferative disorders.
    • Calcitonin.

Neurologic Emergencies

Spinal Cord Compression

  • Occurs in ~5% of pediatric oncology patients.
  • Associated malignancies: Ewing sarcoma, neuroblastoma, lymphomas, epidural chloromas (leukemia), central nervous system (CNS) drop metastases.
  • Pathogenesis: Direct tumor extension via vertebral foramina, vertebral metastasis, or subarachnoid spread.
Clinical EvaluationTherapeutic Modalities
Back pain with localized tenderness (80% of patients).Immediate Dexamethasone (1-2 mg/kg loading dose, then 0.25 mg/kg every 6 hours).
Motor weakness, paraplegia, sensory loss.Emergent chemotherapy (lymphoma, leukemia, neuroblastoma).
Late findings: Incontinence, urinary retention, bowel/bladder dysfunction.Radiation therapy for radiosensitive tumors.
Magnetic Resonance Imaging (MRI) with contrast (modality of choice).Surgical laminectomy for rapid decompression or radioresistant tumors (sarcomas).

Increased Intracranial Pressure (ICP)

  • Etiology: Primary CNS tumors (medulloblastoma, glioma), brain metastasis, hydrocephalus, CNS leukemia, pseudotumor cerebri, hemorrhage.
  • Presentation: Headache, morning emesis, hypertension, bradycardia, papilledema, cranial nerve III and VI palsies, altered mental status, seizures.
  • Interventions: Dexamethasone, ventriculostomy, surgical excision, radiation, specific chemotherapy.

Gastrointestinal And Hepatic Emergencies

Typhlitis (Neutropenic Enterocolitis)

  • Necrotizing colitis involving the cecum; progresses rapidly to bowel infarction and perforation.
  • Associated heavily with severe myelosuppression, acute leukemia, and mucosal injury from cytotoxic chemotherapy.
  • Manifestations: Acute abdomen, right lower quadrant pain, bloody diarrhea, hypotension, fever.
  • Diagnosis: Abdominal radiograph or CT reveals thickened bowel wall, pneumatosis intestinalis, free air.
  • Management: Strict bowel rest, nasogastric suction, surgical consultation, broad-spectrum antibiotics (covering Gram-negatives and anaerobes), antifungals.

Sinusoidal Obstructive Syndrome (Veno-Occlusive Disease)

  • Pathophysiology: Fibrous obliteration of small hepatic vessels; necrosis of zone III hepatocytes.
  • Triggers: Post-hematopoietic stem cell transplant, high-dose cyclophosphamide, total body irradiation, gemtuzumab ozogamicin, inotuzumab ozogamicin, thiopurines.
  • Presentation: Jaundice, tender hepatomegaly, weight gain, ascites, edema.
  • Intervention: Discontinue inciting agents, rigorous fluid management, defibrotide administration.

Bowel Obstruction And Gastrointestinal Hemorrhage

  • Obstruction causes: Tumor masses (lymphoma, neuroblastoma), intussusception (Burkitt lymphoma), adhesions.
  • Hemorrhage causes: Chemotherapy-induced thrombocytopenia, corticosteroid-induced ulcers, tumor masses, prolonged neutropenia.
  • Interventions: Bowel rest, nasogastric suction, blood product transfusions, endoscopic evaluation, surgical consultation.

Infectious Emergencies

Febrile Neutropenia

  • Definition: Absolute neutrophil count (ANC) <500/mm³ (or <1000/mm³ with expected decline) accompanied by fever >38.3°C orally or sustained >38.0°C.
  • Carries immediate risk of life-threatening sepsis, shock, disseminated intravascular coagulation.
  • Pathogens: Gram-negative bacteria, Gram-positive bacteria, fungi (Candida, Aspergillus), opportunistic organisms (Pneumocystis jiroveci).
  • Management Protocol:
    • Mandatory hospitalization.
    • Immediate empiric broad-spectrum IV antimicrobial therapy (e.g., Cefepime, Vancomycin).
    • Add empiric antifungals if fever persists beyond 3-5 days of broad-spectrum antibiotics.
    • Granulocyte colony-stimulating factor (G-CSF) if appropriate.

Endocrine Emergencies

Syndrome Of Inappropriate Antidiuretic Hormone Secretion (SIADH)

  • Pathophysiology: Continuous, unregulated pituitary release of antidiuretic hormone irrespective of plasma osmolality.
  • Triggers: Vincristine, cyclophosphamide, ifosfamide, cisplatin, CNS/pulmonary lesions, severe physiologic stress.
  • Presentation: Oliguria, weight gain, fatigue, nausea. Progression to lethargy, seizures, coma.
  • Laboratory Profile: Hyponatremia (<130 mEq/L), low serum osmolality (<280 mOsm/L), high urine osmolality (>500 mOsm/L), increased urine specific gravity.
  • Management:
    • Strict fluid restriction.
    • Furosemide (1 mg/kg/dose) diuresis.
    • Severe neurologic symptoms: 3% hypertonic saline. Correct cautiously (maximum 2 mEq/L/hour initially) to prevent permanent neurologic sequelae.

Adrenal Insufficiency

  • Triggers: Prior profound corticosteroid exposure, abrupt withdrawal, critical illness, sepsis, tumor infiltration, radiation injury to adrenal glands.
  • Presentation: Fatigue, dizziness, nausea, severe hypotension, profound shock, hyponatremia, hyperkalemia, normal anion gap metabolic acidosis.
  • Management: Immediate glucocorticoid replacement (Hydrocortisone 100 mg/m² loading dose, followed by 25 mg/m² every 6 hours for 7 days without taper). Fludrocortisone. Monitor for hyperglycemia.

Treatment-Associated Emergencies

Cytokine Release Syndrome (CRS)

  • Pathophysiology: Acute systemic inflammation linked to T-cell expansion, macrophage activation, and massive cytokine production (IL-6, IL-10, interferon-gamma).
  • Triggers: Chimeric antigen receptor (CAR) T-cell therapy (tisagenlecleucel), bispecific T-cell engagers (blinatumomab), monoclonal antibodies.
  • Manifestations: Required fever, hypotension, hypoxemia, end-organ dysfunction, capillary leak.
  • Associated Laboratory Findings: Markedly elevated C-reactive protein and ferritin.
  • Interventions:
    • Antipyretics, judicious fluid resuscitation (avoid exacerbating vascular leak), vasoactive agents.
    • Interrupt immunotherapy infusion.
    • Targeted modulation: Tocilizumab (IL-6 receptor antibody) is first-line for CAR T-cell associated severe CRS. Corticosteroids for neurotoxicity or refractory CRS.

Differentiation Syndrome

  • Pathophysiology: Complication of all-trans-retinoic acid (ATRA) and/or arsenic trioxide therapy for acute promyelocytic leukemia. Maturation of leukemic cells produces inflammatory cytokines causing endothelial damage, capillary leak, and microcirculation occlusion.
  • Bimodal Onset: Occurs within 1 week or 3-4 weeks of initiating therapy.
  • Presentation: Respiratory distress, pulmonary infiltrates, peripheral edema, weight gain, hypotension, pleuropericardial effusions, acute renal failure, fever.
  • Interventions: Immediate Dexamethasone (0.5-1 mg/kg every 12 hours). Temporarily halt ATRA/arsenic therapy if symptoms are life-threatening.

Anaphylaxis And Hypersensitivity

  • Implicated Agents: Asparaginase, platinum agents, etoposide, bleomycin, monoclonal antibodies (dinutuximab, rituximab).
  • Presentation: Bronchospasm, laryngospasm, wheezing, hypotension, angioedema, urticaria, flushing.
  • Management: Immediate cessation of infusion. Epinephrine administration. Supportive fluids, oxygen, parenteral corticosteroids (methylprednisolone/hydrocortisone), H1/H2 antagonists. Switch to alternative preparations if available (e.g., Erwinia asparaginase for pegaspargase allergy).

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