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Cancer in Children (CNS tumors (Low grade astrocytomas (50% of brain…
Cancer in Children
CNS tumors
- more than 50% infratentorial
- 40% supratentorial
- about 5% spinal cord
Low grade astrocytomas
- 50% of brain tumors in children
- cerebellum > hemispheres > midbrain, thalamus > optic nerves
- pilocytic – young children, cerebellum, optic nerves
- fibrillary – older children, brain stem, hemispheres, progression of G - likely
Brain stem tumors
- 10%
- egzophytic or endophytic growth
- craniopharyngioma:
surgery – curative in 60-80%
Radiotherapy after surgery; delivered in case of relapse or partial resection
- Medulloblastoma:
85% before 15 year,
cerebrospinal axis irradiation - 36 Gy,
Unfavourable prognostic factors: resection <75%, cerebrospinal fluid involvement, distant metastases, infiltration of brain stem, age < 4
Wilm’s tumor
- 3-4 years of age
- 95% cases before 7
- initial chemotherapy without pathological examination
- radical surgery
- postoperative chemotherapy
- radiotherapy
stage
- I tumor limited to kidney and completely excised
- II tumor extends beyond kidney but is completely excised
- III residual nonhematogenous tumor confined to abdomen
- IV hematoganous metastases beyond stage III
- V Bilateral renal involvement
Radiotherapy
- Radiotherapy 12-30 Gy
stage III and IV favourable and standard histology
stage II and III unfavourable histology
stage II and III tumor bed with regional lymph nodes
irradiation of whole abdomen and whole lungs
neuroblastoma
- 90% cases before 5 years of age
- cytogenetic disorders are found in 30% of patients deletion of short arm of chromosome 1, DNA hiperploidy, N-myc amplification
- retroperitoneal 60-80%, mediastinum 15%, pelvis 2-5%
- At diagnosis 60-70% patients have distant metastases, 90% increased concentration of katecholamines in urine, IV S - in infants liver, bone marrow and subcutaneous metastases
- I localized tumor with complete gross excision N-
- II A localized tumor with incomplete gross excision N-
- II B localized tumor with or without complete gross excision N+
- III unresectable unilateral tumor infiltrating across the midline
N-/+ or unilateral tumor with contralateral lymph node involvement or midline tumor with bilateral extension
- IV any primary tumor with distant dissemination
treatment
- I - II - surgery with adjuvant chemotherapy
- III induction chemotherapy plus surgery, radiotherapy chemotherapy, high dose chemotherapy with bone marrow transplantation, surgery, radiotherapy
- IVS – no treatment or symptomatic
Bone sarcomas
- 70% osteosarcoma
- Surgical biopsy for diagnosis
- Osteosarcoma - 50% close to knee
- Ewing’s sarcoma – in 40% symptoms of inflammation
Osteosarcoma
- Chemotherapy, Surgery, Radiotherapy
-75% long term survival
Ewing’s sarcoma
- Chemotherapy, Surgery, Radiotherapy
- PTV: Tumor volume at diagnosis + margin
- 50% cure in localized cases
- 20% 5-OS in disseminated disease
-
Soft tissue sarcomas
- 70% RMS
- 10% sarcoma neurogenes, 10% synovioma malignum
- 5% extraosseous Ewing’s sarcoma and PNET
- 5% fibrosarcoma
RMS
- Histologic subtypes of RMS:
Embryonal, Embryonal botryoid, Alveolar, Pleomorphic, undifferentiated
- Superior prognosis: Botryoid, Spindle cell RMS
- Intermediate prognosis: Embryonal RMS
- Poor prognosis: Alveolar RMS, Undifferentiated RMS, Anaplastic RMS
location
Head and neck 43% (orbit4%, parameningeal 26%, Nonparameningeal 13%)
genitourinary 9%
Uterus 7%
Extremity 18%
stage
- I (16%) localized disease, completely resected:
A. confined to organ or muscle of origin
B. infiltration outside of organ or muscle of origin regional nodes not involved
- II (28%) compromised or regional resection
A. Grossly resected tumor with microscopic residual disease.
B. Regional disease completely resected in which nodes may be involved or extension of tumor into adjacent organ may exist.
C. Regional disease with involved nodes, grossly resected, but with evidece of microscopic residual mass
- III (36%) incomplete resection or biopsy with gross residual disease, lymph nodes + or –
- IV (20%) distant metastases at diagnosis
treatment
- I – adjuvant chemotherapy
- II – adjuvant chemotherapy and radiotherapy
- III – neoadjuvant chemotherapy, surgery and/or radiotherapy, adjuvant chemotherapy
- IV – chemotherapy, surgery and/or radiotherapy, adjuvant chemotherapy
radiotherapy
parameningeal
- primary tumor plus 2-5 cm healthy tissue margin treatment planning based on CT or MRI performed before treatment
- 2-3 cm above scull base
- In other locations residual mass with 2 cm margin
- Dose 40-55 Gy
chemotherapy
- Vincristine, Adriamycin, Cyklofosfamide, Actinomycine, Etopozyde, Ifosfamide, Cisplatin, DTIC
lymphomas
NHL
- High grade lymphomas - chemosensitive
- B-cell NHL tumor in abdomen, bones of face, bone marrow infiltration
- T-cell NHL– frequent involvement of mediastinum
- Treatment: multi drug chemotherapy
CNS irradiation
palliative irradiation: SVCS, spinal cord impression
Hodgkin’s disease
- LP 5-15%, NS 40-60%, MC 15-30%, LD<5%
- Combination chemotherapy + radiotherapy
- Doses of irradiation lower than in adults
- cure > 90%
-
Diagnostic work-up
- Anamnesis – special emphasis on family history
- Physical examination
- Lab tests (biochemistry, hormones, immunologic, genetic)
- Imaging
Leukemia
- 80% ALL, 17% AML, 2-5% CML.
- Accompanying genetic disorders: Down syndrome, Fanconi’s anaemia, NF1
- Chemotherapy: induction, consolidation, sustaining of remission
- role of radiotherapy: Irradiation of CNS, Prophylactic – whole brain with medulla oblongata down to the border between C2-C3 vertebrae, dose12-18 Gy in 1,8 Gy fractions, curative – as above or neuraxis 18 Gy po1,8 Gy
- Second leading cause of death in children after accidents
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