epidemiology, clinical Cancers in childhood: epidemiology, clinical approach to diagnosis And Lymphadenopathy

leading cause of disease-related death in children Children cancers • leading cause of disease-related death in children in developed countries and remains an important public health concern because of its great physical and psychological impact on the affected children and their families the causes of childhood cancer are mostly unknown •

treatment is an essential Children cancers • short latency periods and often grow rapidly are very invasive more responsive to treatment than adult tumors timely diagnosis immediately followed by effective treatment is an essential

Hereditary diseases and cancers Primary Disease Phakomatoses neurofibromatosis tuber sclerosis Genetics Type of cancers AD AD AD CNS tu, sarcomas syndrome glikogenosis type galactosemia IV AR AR AR AR AR Cirrhosis hypermetioninemia AD – autosomal dominant AR – autosomal recesive AS – sex linked

Hereditary diseases and cancers Genetics Primary Disease Type of cancer disorders SR ? AD? Leukemia, Leukemia, , lymphoma syndrome AR

Diagnostic criteria for neurofibromatosis type 1 (NF 1) - Memorandum WHO (1992)   

ocular skin CNS NF1 signs and symptoms others bones cancers

NF1 - summary Disease: chronic progressive unpredictable

, „cafe au 4.4% Half body overgrowth 2.9% 2.9% Aniridia 1.1%

that Chief complaints Chronic drainge from ear Brain tumor lymphomas Proptosis

Leukemia; lymphoma Bone pain Hodgkin’s lymphoma

Headaches  only few of the headaches are caused by intracranial tumors tu tu : the headaches

 Presence or onset of neurologic abnormality   repeatedly awaken child from 

Abdomen – tumor examination examination

Bone pain  Bone  intermittent, and it increases in  (no bone involvement)

1. Newborns 1. 2. Damage during delivery 3. Newborns diathesis, thrombocytopenia

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• unexplained fever, fatigue, and arthralgia - ??

• – unexplained fever, fatigue, and arthralgia – collagen vascular disease or serum sickness •

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Lymphadenitis (inflammation of lymph nodes) • Lymphadenopathy: – regional – generalised (>= 2 lymph nodes groups) Lymphadenitis (inflammation of lymph nodes) • – acute: – chronic: < 6 weeks > 6 weeks • Lymphocutaneous syndromes - regional lymphadenitis associated with a characteristic skin lesion at the site of inoculation. Lymphangitis is an inflammation of subcutaneous lymphatic channels (acute bacterial infecton - Staphylococcus aureus and group A streptococcus. •

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A. C. 1 1. 2. 3. 1. 2. B. D. 1. 2. 3. 1. 2. 3. 4.

lymphadenopathy: no treatment observation • lymphadenopathy: no treatment observation acute bacterial cervical lymphadenitis without a known primary infectious source – antibiotics: adequate coverage for both S. aureus and group A β-hemolytic streptococci appropriate oral antibiotics: cloxacillin, cephalexin, or clindamycin – • acute bacterial cervical lymphadenitis with a known primary infectious source empirical antibiotics: against the microorganism most frequently associated with that source, pending the results of the culture and sensitivity tests cervical lymphadenopathy and periodontal or dental disease: treatment - penicillin V or clindamycin • •

• cervical lymphadenopathy and periodontal – treatment - penicillin V or clindamycin indications for intravenous treatment: or dental disease: • – toxic or immunocompromised children those who do not tolerate, will not take, or fail to respond to an oral medication • fluctuant lymph node: – incision and drainage supportive care: – oral analgesia – e.g. acetaminophen •

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Examination: mouth, throat, Cervical LN Epitrochlear LN Examination: mouth, throat, teeth, gums, ears Skin examination: melanoma exclusion Skin examination: melanoma exclusion Exploratory laparotomy Abdominal LN biopsy/excision LN Skin examination: melanoma exclusion Thoracotomy Mediastinoscopy Inguinal LN Mediastinal LN

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• cutaneous papule or conjunctival granuloma, lymphadenopathy localized to the draining regional nodes site of initial infection: •

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weeks of antibiotics, it will require incision and drainage • LN: diameter < 1 cm = normal range – patient (pts) can be reassured and discharged from clinic LN: diameter 1–3 cm • – pts should be treated with an antibiotic with good anti- staphylococcal and streptococcal cover for 2 weeks – no improvement - investigation by ultrasound LN: diameter > 3 cm pts should be assessed initially with ultrasound • – inflammatory nodes - treat with antibiotics for 2 weeks If an abscess develops they usually resolve with intravenous (IV) antibiotics however if the abscess is pointing on presentation, or does not resolve with 2 weeks of antibiotics, it will require incision and drainage

• a node that is chronically present, but not enlarging, should ultrasound be assessed initially with • any progressively enlarging cervical node should be referred for urgent fine needle aspiration biopsy failure of regression • after 4 to 6 weeks might be an indication for a diagnostic biopsy

• pts is suspected of having serious disease undiagnosed LN persisting longer than 2months worrisome features such as firm, matted, or rapidly enlarging LN • the presence of night sweats, weight loss, bone pain, hepatosplenomegaly, fever of unknown etiology, or failure to thrive a positive PPD and/or an abnormal chest radiograph •