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Paediatrics > 67. Pediatric solid tumors > Flashcards

67. Pediatric solid tumors Flashcards

brain tumors, soft tissue sarcomas, bone tumors, neuroblastoma

1
Q

Brain tumors

A

Most common solid tumors in childhood- 22% of all neoplasms

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2
Q

Brain tumors histological classification

A
  1. Glial tumors
  2. Embryonic & neuronal tumors
  3. Tumors of choroid plexus
  4. Other tumors, not originating from CNS
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3
Q

Brain tumors symptoms

A

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

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4
Q

Brain tumors diagnosis

A

Head MRI
Spinal cord MRI
CSF liquid examination
tumor markers

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5
Q

Brain tumors treatment

A

surgery
chemotherapy
radiation therapy

If radiation therapy can’t be applied (<3 yrs of age): high dose chemo & auto HSCT

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6
Q

Brain tumors treatment complications

A

neurological & mental development delay
auditory & endocrine disorders

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7
Q

Brain tumors survival rates

A

Operable pilocytic astrocytoma: >90%
Localised forms: 80%
Peripheral neuroectodermal tumor(PNET) & poor differentiated gliomas: 20-30%

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8
Q

Rhabdomyosarcoma

A

The most common single tumor among the group of soft tissue sarcomas.
4-5% of neoplasms in children

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9
Q

Rhabdomyosarcoma histological classification

A

Embryonal- less invasive, low metastatic potential, better prognosis (70% of cases)

Alveolar- aggressive, distant metastases in bones & bone marrow with 80% detectable chromosomal translocations

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10
Q

Rhabdomyosarcoma symptoms

A

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

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11
Q

Rhabdomyosarcoma diagnosis

A

CT
MRI
FDG PET/CT

Bone marrow examination in alveolar type

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12
Q

Rhabdomyosarcoma treatment

A

surgery
chemotherapy
radiation therapy

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13
Q

Rhabdomyosarcoma survival rates

A

90% in low risk
60-70% in intermediate risk & localised forms
30% in metastatic embryonal rhabdomyosarcoma
<5% in metastatic alveolar type

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14
Q

Non-rhabdomyosarcoma soft tissue sarcomas

A

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

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15
Q

Non-rhabdomyosarcoma soft tissue sarcomas histological classification

A
  1. adipocytic tumors
  2. fibroblastic tumors
  3. smooth muscle tumors
  4. GI stromal tumors
  5. vascular tumors
  6. chondrocyte
  7. originating from peripheral nerves
  8. of unknown histological origin
  9. undifferentiated sarcomas

In many of the them characteristic translocations found.

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16
Q

Osteogenic sarcoma

A

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

17
Q

Osteogenic sarcoma histological classification

A

Osteoblastic
Chondroblastic
Fibroblastic

18
Q

Osteogenic sarcoma symptoms

A

(nocturnal) pain
swelling of affected area

19
Q

Osteogenic sarcoma diagnosis

A

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

20
Q

Osteogenic sarcoma prognostic factors

A

Poor prognosis:
initial tumor dissemination/primary metastases
age < 10yrs
male gender
high levels of alkaline phosphatase & lactate dehydrogenase
centrally localised tumors (pelvis, spine)

21
Q

Osteogenic sarcoma treatment

A

Preop chemo
Surgery
Postop chemo & advanced complex rehab

22
Q

Ewing sarcoma

A

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)

23
Q

Ewing sarcoma symptoms

A

painful tumor with swelling, redness & dysfuntion

24
Q

Ewing sarcoma diagnosis

A

increased ESR
high levels of alkaline phosphatase & lactate dehydrogenase
X-ray
CT
MRI

25
Q

Ewing sarcoma treatment

A

Preop chemo
Surgery
Postop chemo

ES is radiosensitive.
Combined radio & chemotherapy- used for local control or when surgery is not feasible

26
Q

Ewing sarcoma prognostic factors

A

tumor size
presence of metastases
treatment response

localised forms <200 mL*3 has better survival rate

Myeloablative therapy & auto HSCT improves outcome.
Relapses associated with poor prognosis.

27
Q

Neuroblastoma

A

An embryonic tumor of the SNS.
7-8% of all cancers in childhood
Common primary localisation: abdominal (adrenal medulla), in sympathetic ganglia in neck)

28
Q

Neuroblastoma symptoms

A

palpable tumor mass
abdo pain
arterial HTN
Horner syndrome (unilateral ptosis, myosis & enophthalmos)

Metastases in bone marrow, bones & liver.

29
Q

Neuroblastoma stages

A

Stage I- tumor is localised & surgically resectable
Stage II- tumor is localised but inoperable
Stage III- inoperable tumor crossing midline
Stage IV- tumor with distant metastases

30
Q

Neuroblastoma diagnosis

A

CT
MRT (magnetic resonance tomography)
Scintigraphy with radioactive iodine
PET
Tumor markers: homovanillic & vanillylmandelic acid in urine, lactate dehydrogenase & ferritin in serum.
Histological, immunohistochemical & molecular genetic examination

31
Q

Neuroblastoma prognostic factors

A

Poor prognosis:
older age
less differentiated tumors
MYCN gene amplification
loss of heterozygosity of 1p, 11q, 14q

Good prognosis
age<1.5 yrs
more differentiated tumors
hyperdiploidy

32
Q

Neuroblastoma treatment

A

surgery
chemo

In high risk pts: intensive high dose chemo, surgery, myeloablative chemo & auto HSCT
then immune & maintenance therapy with neuroblastoma differentiating agent (cis-retinoic acid)

Paediatrics (3 decks)

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