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SOLID TUMORS IN CHILDREN (NEUROBLASTOMA (Signs and symptoms (primary tumor…
SOLID TUMORS IN CHILDREN
BRAIN TUMORS
- most common solid tumours in children
- infratentorialy 60%
Early block on liquid circulation
histological types
- astrocytomas 40%
varies from benign to highly malignat
- medulloblastomas/PNET 20%
arises in the midline of the posterior fossa
may seed through the CNS via the CSF
- ependymomas 8%
- brainstem glioma 6%
- craniopharyngioma 4%
arising from squamous remnant of Rathke pouch
locally invasive and grows slowly in the suprasellar region
Signs and symptoms
- Headache (increased ICP) and vomiting
- Depending on tumor’s localization in CNS:
ataxia, visual disorders, endocrinological disturbances, muscle weakness and paralysis, behavioral changes
- Seizures --> coma
Diagnostic examinations
- Imaging: CT, MRI
- Cerebral spinal fluid examination
- Biopsy and histology
Treatment
- Neurosurgery
- Radiotherapy (> 5 yr of age)
- Multimodal chemotherapy - induction & consolidation
- Concomitant therapy:
steroids, anti-seizures medications
ventriculoperitoneal shunt
aim
- to achieve complete remission
- to decrease the side effects
- to improve the quality of life
Prognosis
- depends on:
possibility of complete tumor resection
histological type: medulloblastomas 50-85% (5 year survival) / glioblastoma multiforme poor prognosis
Side effects
Neurological unreversible disorders
Mental retardation, Epilepsy, Endocrine problems
NEPHROBLASTOMA
Wilms tumor
- originates from embryonal renal tissue
- Wilms’ tumor susceptibility: gene WT1, WT2
- the most common malignant tumor of the kidney in children
- Age of diagnosis:
75 % pts between 1- 5 years old
90% pts < 7 years old
- Patients’ condition at diagnosis mostly good
-
clinical features
- Large abdominal tumor
- Urinary infections
- Haematuria intermittens, Hypertension
- Abdominal pain, Digestive disfunction
- Good overall condition
metastases
- Lymph nodes, Liver, Lungs
Diagnostic examinations
- USG, CT, MRI
- According to SIOP protocol histological diagnosis after induction CHT
Treatment
- MULTIMODAL THERAPY:
Chemotherapy (starting without histology)
Surgery
Adjuvant chemotherapy and radiotherapy depend on the stage and histology
prognosis
- Quite good!!!
- even in advanced and metastatic stages more than 80-90% pts survive
NEUROBLASTOMA
- originates from embryonal tissue --> sympathetic part of the neural crest tissue (adrenal medulla and sympathetic nervous system)
- onset:
infants 40% pts
small children up to 5 y of age 90% pts
- Primary tumor: Adrenal glands, Sympatetic trunk (mediastinal or cervical part)
- Metastases: bones, bone marrow, lymph nodes, liver, lungs, CNS
Signs and symptoms
- depend on:
localization of primary tumor
localization of metastases
biologically active substances produced by tumor
primary tumor
- In abdomen - abdominal mass
- In mediastinal region – respiratory disturbances
- In neck region – visible tumor mass
metastases
- to bone marrow – mielosupresion
- to liver – hepatomegaly
- to bones – pain, pathological bone fractures, periorbital bruising
- to mediastinal region – cough and respiratory disturbances
- to skin – blue nodules in the skin
-
Diagnostic examinations
- USG, CT, MRI
- Isotope imaging:
Bone imaging (Technet isotope), Tumor imaging ( MIBG isotope)
- Tumor markers:
catecholamines and their metabolites: VMA, HVA (in serum, in urine)
- Histopathology
clinical stages
- I Localized tumor with complete gross excision
- II Localized tumor with incomplete gross excision
- III Unresectable unilateral tumor infiltrating across the midline
- IV Any primary tumor with dissemination
- IV s Tumor in infant with dissemination limited to skin, liver, and/or bone marrow
-
Prognosis
- most important prognostic factors:
amp MYCN, stage of disease, age of the patient
Treatment
- Surgery in low risk group (with complete resection)
- Multimodal treatment
in aggresive and advanced stages
intensive chemotherapy, surgery, myeloablative chemotherapy, radiotherapy, retinoid acid analogues (differentiating agents), immunotherapy
SOFT TISSUES SARCOMAS
- Heterogenous group (different types of tumors):
rhabdomyosarcoma (RMS)
angiosarcoma, fibrosarcoma
leyomyosarcoma, liposarcoma and others
- Originates from primitive mesenchymal tissue
- In every age
- Localization - variety (in every part of the body)
- Signs and symptomps- depend on the site of tumor mass
Signs and symptoms
- Head and neck:
tumor mass, proptosis, nasal obstruction, bloodstained nasal discharge
- Genitourinary tumors:
dysuria, urinary and stool obstruction, bloodstained vaginal discharge
Metastases
- Bone marrow, Lymph nodes, Lungs
- in 15% patients at diagnosis
Diagnostic examinations
- important role of USG
- CT, MRI also used
- Open biopsy and morphological evaluation
- Needle biopsy is not indicated
Treatment
- CHT+surgery + radiotherapy
- Intensivity of therapy depends on site and extent of disease
- CHT according to CWS protocols:
VCR (Vinkrystin), ACT-D (Daktomycin), CTX (Cyclophosphamide), IFO (Ifosfamide)
Etopozide, Cysplatin in high risk group
BONE TUMORS
- 4,7% of children malignancies
- Pubertal age
- Two main histological types:
Osteosarcoma, Ewing’s sarcoma (in younger children)
Signs and symptoms
- Pain in the tumor region, Tumor mass
OSTEOSARCOMA
Signs and syndromes
- Pain: nocturnal, continuous and intensified
- Bone fractures (not frequent)
- Muscle atrophy
- Signs connected with metastases (lung metastases)
Diagnostic examinations
- primary site: X-ray, CT, MRI
- lung metastases: CT, X-ray
- bone metastases : scyntygraphy
- Laboratory findings: LDH, alkaline fostatase
- Open biopsy, Histological evaluation
EWING’S TUMOR
- Originate from neuroetctodermal tissue
- osseus primary site 87%
- extraosseus primary site 8%
- 62% - boys, 38% girls
- Primary site:
Thigh bone -25%, Flat bones - 20–25%,
Tibia and fibula - 15%, Ribs - 10%
Signs and symptoms
- Pain, Fever, Oedema, Inflamatory symptoms
- Tumor mass in late diagnosis
Diagnostic
- Imaging:
CT - evaluation of bones
MRI - evaluation of tumor extention in soft tissues and bone marrow
Bone scintigraphy (extention in the bones)
- Bone marrow biopsy
-
Treatment
- Multimodal chemotherapy
- Surgery
- Radiotherapy (in Ewing’s sarcoma)
results
- Osteosarcoma 30-40% pts survive 5ys
- Ewing’s sarcoma 50-70% pts survive 5ys