Common Signs and symptoms of Paediatric Malignancy

1. Common Signs and symptoms of Paediatric Malignancy

2. Childhood Cancer is a rare disease… Health Indicator:

3. Health Indicator: Mortality MORTALITY: TEN LEADING (10) LEADING CAUSES Number and rate/100,000 Population Philippines 5-Year Average (2000-2004) & 2005 Department Of HealthLast Update: June 29, 2009

4. Increasing incidence of malignancy • Many pediatric malignancies are highly curable • With early diagnosis • Associated with better prognosis • Diminished intensity of therapy (treatment stratification) Why increase awareness?

5. Survival Rate (5 years)

6. Event Free Survival of ALL CH PUI, NEJM 1988

7. How the diagnosis is made • Nonspecific signs • Unexplained or prolonged fever (>3 weeks) • Unexplained masses • Weight loss • Pallor • Bleeding • Headache • Eye Changes presenting clinical features

8. Depend on • Type of cancer • Neuroblastoma • Tumor itself • Catecholamine release • diarrhea due to release of vaso-active peptides • Site(s) of disease • Mediastinal -- respiratory • Age of patient Specific Signs and Symptoms

9. Paediatric Malignancy

10. How the diagnosis is made • The presentation depends on the individual malignancy. presenting clinical features

11. Age of Patient • First year of life • Embryonal: Neuroblastoma, Nephroblastoma, Retinoblastoma, Rhabdomyosarcoma, Hepatoblastoma and Medulloblastoma • Two to five years • Acute leukaemia's, Non-Hodgkin’s lymphoma and Gliomas • Greater than 5 years • Bone malignancies, Hodgkin’s lymphoma, Gonadal germ cell malignancies Paediatric Malignancy

12. presenting clinical features • Related Directly to Tumor • Superior Vena Cava Syndrome • Subcutaneous nodules • Leukemoid reaction • Myasthenia gravis • Heterochromia • Not Related Directly to Tumor Growth • Chronic diarrhea • Polymyoclonus-opsoclonus • Failure to thrive • Cushing syndrome • Pseudomuscular dsytrophy

14. Signs and Symptoms of Cancer in Children and Adults

15. ADULTS CHILDREN • Change in bowel or bladder habits    • Blood in stool    • Lump in breast or elsewhere    • Hoarseness or nagging cough    • Difficulty in swallowing    • Sore that will not heal    • Change in wart or mole • Abdominal mass    • Persistent lymphadenopathy    • >1 abnormal hematopoietic lineage    • Specific neurologic deficit    • Increased intracranial pressure    The Most Common Signs and Symptoms of Cancer in Children and Adults

16. ADULTS CHILDREN • Change in bowel or bladder habits    • Blood in stool    • Lump in breast or elsewhere    • Hoarseness or nagging cough    • Difficulty in swallowing    • Sore that will not heal    • Change in wart or mole • Diffuse enlargement of pons    • Proptosis    • White pupillary reflex    • Unilateral knee or shoulder pain/swelling    • Vaginal bleeding or mass The Most Common Signs and Symptoms of Cancer in Children and Adults

21. Genetic predisposition • Down’s Syndrome – leukemia 5-10x at risk • Gardener’s Syndrome – hepatoblastoma • Exposure to radiation/chemicals • Nuclear accidents • Benzenes • Very premature infants • Hepatoblastoma Children at risk

22. Infectious agents • HIV – lymphomas, Kaposi’s sarcoma • Epstein-Barr virus – Lymphomas • Cancer survivors • ALL survivors –2nd AML, • Cranial Irradiation – 2nd CNS tumors • Post transplant • Immunosupressive therapy – lymphoma Children at risk

23. Seen in many forms of childhood illness • But also is one of manifestation of malignancy • Leukemia • Lymphoma • Prolonged fever – (FUO) • Infectious • Connective • Malignancy – 10% 1. Fever

24. When to suspect malignancy • Prolonged • Associated with • Bone pain • Weight loss • Pallor 1. Fever

25. The most important management and therapeutic measures are to determine the underlying cause of the fever • Infection • Inflammation • Neoplasm 1. Fever

26. Normal children Nodes are larger than adults Antigenic exposure Lymphoid tissue increases in volume Lymph nodes palpable during childhood 2. Lymphadenopathy

27. Normal children A nodal mass is not always an indicator for malignancy – more often its due to other causes 2. Lymphadenopathy

28. 2. Lymphadenopathy Common Causes of Lymphadenopathy

29. BENIGN MALIGNANCY • Streptoccocal tonsillitis • URTI • EBV • Kawasaki disease • Toxoplasmosis • Rubella infection • Rosseola • Cat scratch disease • Lymphoma • Hodgkin’s • Non- Hodgkin’s • Neuroblastoma • Rhabdomyosarcoma • Leukaemia 2. Lymphadenopathy

30. 2. Lymphadenopathy Abnormal Lymph nodes Size Location

31. 2. Lymphadenopathy Abnormal Lymph nodes

32. 2. Lymphadenopathy Abnormal Lymph nodes

33. 2. Lymphadenopathy

34. 2. Lymphadenopathy

35. 2. Lymphadenopathy Initial Work-up Definitive • Bone marrow • aspiration • Lymph node biopsy • Complete blood count • Chest X-ray • Skin test for • mycobacterium • ESR • Viral studies

36. When to suspect malignancy • Persistent and painless mass • Enlarged LN • Supraclavicular mass • Presence constitutional symptoms • Fever • Weight loss • Fatigue • Pruritus • Night sweat 2. Lymphadenopathy

37. Indication for LN biopsy • Chronic, persistent, progressive adenopathy • > 6 weeks • Any node which is > 2.5 cm diameter in the absence of signs of infection that warrant antibiotics • Supraclavicular lymphadenopathy • Systemic symptoms • Weight loss, temp of > 38.5o C for 1 week, hepatosplenomegaly, arthralgia 2. Lymphadenopathy Pizzo PA Principles and Practice of Pediatric Oncology,

38. Indication for LN biopsy • Other indications • Anaemia (Hgb lower than 10gm/dl) • Elevated ESR • Abnormal chest radiographic findings • Any persistent and painless mass that does not respond to antibiotics should be investigated 2. Lymphadenopathy

39. Primary tumour • All arises in the mediastinum • Except for pleuropulmonary blastomas, rhabdomyosarcomas • Metastatic tumour • Neuroblastoma • Osteosrcoma 3. Thoracic Masses

40. 3. Mediastinal Mass ANTERIOR POSTERIOR MIDDLE

41. 3. Mediastinal Mass • ANTERIOR • Thymus • Heart • Anterior pericardium • Anterior mediastinal • lymph nodes • POSTERIOR • Descending • aorta • Oesophagus • Sympathetic • chains • MIDDLE • Nodes • Great vessels • Trachea

42. 3. Mediastinal Mass • ANTERIOR/MIDDLE • Compression of • thoracic structures • Superior vena cava • syndrome • Superior mediastinal • syndrome • Pleural/Pericardial • effusion

43. 3. Mediastinal Mass • ANTERIOR/MIDDLE • Superior vena cava syndrome • Distended neck veins • Edema of the neck and face • Orthopnea • Dysphagia • Syncope • Suffusion of the conjunctivae

44. 3. Mediastinal Mass • ANTERIOR/MIDDLE • Superior mediastinal syndrome • Cough • Hoarseness • Dyspnea • Orthopnea • Wheezing • Stridor • Chest pain • Pericardial effusion • Cardiac tamponade

45. 3. Mediastinal Mass • POSTERIOR • Cord Compression • Back pain • Lower extremity pain • Incontinence • Stridor from airway • compression

46. 3. Mediastinal Mass • POSTERIOR • Malignant • Neuroblastoma • Rhabdomyosarcoma • Ewing’s sarcoma • Lymphoma • Schwannoma • Benign • Neurogenic cyst • Thoracic • meningoceles • ANTERIOR • Malignant • Lymphoma • Hodgkin’s • Non Hodgkin’s • Leukaemia • T cell/m B-cell • Germ cell tumours • Neuroblastoma • Ewing’s sarcoma • Thyroid tumours • Thymoma • Benign • Lipoma • Bronchogenic cyst • MIDDLE • Malignant • Hodgkin’s disease • Neuroblastoma • Rhabdomyosarcoma • Germ cell tumour Pizzo PA Principles and Practice of Pediatric Oncology,

47. Differential diagnosis Pizzo PA Principles and Practice of Pediatric Oncology,

48. 3. Mediastinal Mass Initial Work-up Diagnostic Work-up • Biopsy • Bone Marrow • aspiration/biopsy • Ct scan (abdomen) • Gallium scan • PET scan • Bone scan • History and PE • Complete blood count • Imaging • Chest X-ray • CT scan • MRI • Tumour Markers • AFP, BHCG, • Urine VMA

49. Palpable abdominal mass is one of the most common presenting findings of malignant solid tumours in children All abdominal masses require work-up 4. Abdominal Mass

50. Age • History • Detailed • Premature, Low birth weight • Chronic Hepatitis • Physical examination • Not limited to characteristic of abdominal mass • Usually unrewarding • Location 4. Abdominal Mass