67. Pediatric solid tumors Flashcards
brain tumors, soft tissue sarcomas, bone tumors, neuroblastoma (32 cards)
Brain tumors
Most common solid tumors in childhood- 22% of all neoplasms
Brain tumors histological classification
- Glial tumors
- Embryonic & neuronal tumors
- Tumors of choroid plexus
- Other tumors, not originating from CNS
Brain tumors symptoms
Compression of brain structures–> ataxia, dysmetria, diplopia, nystagmus, facial asymmetry, dysphagia
Supratentorial tumors–> motor or sensory deficits, focal epileptic seizures
Originating from hypothalamus or suprasellar region–> endocrine disorders, visual disturbances
Diencephalic involvement–> growth delay, weight loss, decreased appetite
Increased ICP, headache, N/V, papilledema
Brain tumors diagnosis
Head MRI
Spinal cord MRI
CSF liquid examination
tumor markers
Brain tumors treatment
surgery
chemotherapy
radiation therapy
If radiation therapy can’t be applied (<3 yrs of age): high dose chemo & auto HSCT
Brain tumors treatment complications
neurological & mental development delay
auditory & endocrine disorders
Brain tumors survival rates
Operable pilocytic astrocytoma: >90%
Localised forms: 80%
Peripheral neuroectodermal tumor(PNET) & poor differentiated gliomas: 20-30%
Rhabdomyosarcoma
The most common single tumor among the group of soft tissue sarcomas.
4-5% of neoplasms in children
Rhabdomyosarcoma histological classification
Embryonal- less invasive, low metastatic potential, better prognosis (70% of cases)
Alveolar- aggressive, distant metastases in bones & bone marrow with 80% detectable chromosomal translocations
Rhabdomyosarcoma symptoms
Depends on tumor localisation
Parameningeal tumors: asymptomatic tumor mass, epistaxis, rhinorrhoea, upper pharyngeal obstruction, chronic otitis media, cranial nerve palsies, enlarged cervical lymph nodes
Urogenital tumors: palpable testicular mass, polypoid vaginal extrusions, hematuria, urinary retention
Limb tumors: palpable masses, enlarged regional lymph nodes
Eye orbit tumors: eyelid masses, eye protrusion
Hepatobiliary tumors: jaundice, hepatomegaly
Laryngeal tumors: dysphonia, dry cough
Retroperitoneal tumors- asymptomatic masses, GI obstruction, compression of spinal canal
Rhabdomyosarcoma diagnosis
CT
MRI
FDG PET/CT
Bone marrow examination in alveolar type
Rhabdomyosarcoma treatment
surgery
chemotherapy
radiation therapy
Rhabdomyosarcoma survival rates
90% in low risk
60-70% in intermediate risk & localised forms
30% in metastatic embryonal rhabdomyosarcoma
<5% in metastatic alveolar type
Non-rhabdomyosarcoma soft tissue sarcomas
A group of tumors with distinct biological behaviour.
50% of all sarcomas in childhood.
Tendency for local growth
Low metastatic potential
Low/missing response to chemo & radiation therapy
Sx determined by localisation
Non-rhabdomyosarcoma soft tissue sarcomas histological classification
- adipocytic tumors
- fibroblastic tumors
- smooth muscle tumors
- GI stromal tumors
- vascular tumors
- chondrocyte
- originating from peripheral nerves
- of unknown histological origin
- undifferentiated sarcomas
In many of the them characteristic translocations found.
Osteogenic sarcoma
A malignant mesenchymal bone tumor characterised by excessive osteoid production.
Associated with the pubertal growth spike
Commonly diagnosed in 2nd decade of life
Slightly more common in boys
Common tumor sites: distal femur, proximal tibia
Osteogenic sarcoma histological classification
Osteoblastic
Chondroblastic
Fibroblastic
Osteogenic sarcoma symptoms
(nocturnal) pain
swelling of affected area
Osteogenic sarcoma diagnosis
X-ray- shows destruction of bone structure & ossification of surrounding soft tissue
CT- assess tumor size
MRI- assess tumor size
Bone scintigraphy- detects distant bone metastases
Osteogenic sarcoma prognostic factors
Poor prognosis:
initial tumor dissemination/primary metastases
age < 10yrs
male gender
high levels of alkaline phosphatase & lactate dehydrogenase
centrally localised tumors (pelvis, spine)
Osteogenic sarcoma treatment
Preop chemo
Surgery
Postop chemo & advanced complex rehab
Ewing sarcoma
2nd most common tumor after osteogenic sarcoma in adolescents
Average age of onset: 15 yrs
It originates from bone marrow mesenchymal cells &/or neuroectoderm.
Typical chromosomal abnormality t(11;22)
Mainly localised in pelvis, long bones, ribs, vertebrae.
Ewing sarcoma comprises a group of malignant tumors: classical, Askin’s tumor, peripheral neuroectodermal tumor (PNET).
It belongs to a group of “small round blue cell tumors” (including NHL, rhabdomyosarcoma, neuroblastoma)
Ewing sarcoma symptoms
painful tumor with swelling, redness & dysfuntion
Ewing sarcoma diagnosis
increased ESR
high levels of alkaline phosphatase & lactate dehydrogenase
X-ray
CT
MRI