Solid tumors

Question 1

What are considered solid tumors and which ones do we see often?

Answer 1

Tumors of organs, bones or tissue. +/- 40% of pediastic tumors. The tumors we see often are Rhabdomyosarcoma’s (soft tissue), Neuroblastoma’s (symphatic NS), Wilms-tumor (kidney’s) and Ewing- and osteosarcoma’s (bone).

Question 2

How can primary tumors be diagnosed?

Answer 2

MRI
Tumormarkers
Biopsy; tissue is the issue.

Question 3

How can tumor staging be performed?

Answer 3

CT-/X-thorax (lungmetastases) 
PET-CT
Lymph node procedure
Lumbal punction
Bone biopsy and punction

Question 4

What is rhabdomyosarcoma, how many cases each year and what can it be divided into?

Answer 4

Malignant tumor of soft tissue; muscle, fat, connective tissue, cartilage. 40 cases each year. Can be divided into head-neck, urogenital and extremities. Head-neck and urogenital is most common in small children, extremities in teenagers.

Question 5

What two types of histology is seen in rhabdomyosarcoma?

Answer 5

Embryonal (80%) and alveolar (20%)

Question 6

What are the risk groups of RMS based on?

Answer 6

Based on age (1-10 yr), size (<5 cm), lymphnode involvement, localisation, histology or molecular.

Question 7

How is RMS treated? It is sensitive for… , unlike non-RMS

Answer 7

It is treated with:
- Chemotherapy –> chemosensitive. For tumorreduction and treatment of (micro)metastases.
- Local therapy: surgery & radiotherapy
Are radio- and chemo sensitive.

Question 8

What are the prognisis rates of; low risk, standard risk, high risk and very high risk?

Answer 8

low risk: 85-90%
Standard risk: 70-80%
high risk: 50-55%
Very high risk: 20-30%

Question 9

What is neuroblastoma and what is the prevalence?

Answer 9

Is tumor of immature sympathic nervous system. Can be benign (and self-limiting) or very malignant. The prevalence is 25/year. You should especially look at renal gland and grensstreng.

Question 10

How can neuroblastoma be staged? and which stage is most prevalent?

Answer 10

Can be staged with MRI. 70% has stage 4 (disseminated to bone or bone marrow)

Question 11

What are symptoms in children with neuroblastoma?

Answer 11

Ill, abdominal distention, high blood pressure, pain, raccoon eyes, opsoclonus myoclonus.

Question 12

Diagnostics in NB?

Answer 12

MRI, bone marrow + bone biopsy, MIGB-tracer.

Question 13

Treatment in NB?

• Localised disease
• Stage 4S
• Stage 4

Answer 13

Localised disease: surgery (OS >85%)
Stage 4S: observation, minimal therapy (OS>85%)
Stage 4: chemotherapy, surgery, radiotherapy, immunotherapy (OR 20-40%)

Question 14

What is Wilms tumor and what is the (peak) prevalence? What is it associated with?

Answer 14

Is tumor from the kidneys. Around 25-30 patients each year, peak incidence at age of 3.5. Is associated with congenital symptoms/ syndroms (such as Beckweth-Wiedeman –> Microcephaly, macroglossia and umbilical hernia)

Question 15

What are symptoms of Wilms tumor?

Answer 15

High blood pressure, abdominal distention, hematuria, fever.

Question 16

What diagnostic tests can be performed in Wilms-tumor?

Answer 16

• Radiology
• Urine test (to distuingish from NB)
• no biopsy!!

Question 17

How can Wilms tumor be treated?

Answer 17

Induction chemotherapy for 4 weeks, surgery and if needed postoperation treatment.

Question 18

What is the prognosis of Wilms tumor?

Answer 18

Overall good, diffuse anaplastic Wilms tumor has lower survival rates.

Question 19

What are two types of bone tumors? Which are seen/found where?

Answer 19

Osteosarcoma: Methaphysis-epiphysis. 10% in humerus, 41% femur, 21% lower leg.
Ewing sarcoma: diaphysis. 26% pelvis, 20% femur.

Question 20

What are the symptoms of the bone tumors?

Answer 20

painful patients (>1 month, during nights -> waking up from pain), swelling, sensibility disturbances, strength, impairment, ilness, fever and weightloss.

Question 21

What is the incidence of ewing and osteosarcoma?

Answer 21

• Osteosarcoma: 13/year, peak incidence 10-30

- Ewingsarcoma: 15/year, peak incidence 5-25

Question 22

How can both bone tumors be treated?

Answer 22

• Ewing sarcoma: is very chemo and radiotherapy sensitive. Systemic therapy is necessary, without the mortality is >90%.
• Osteosarcoma: Not very radio and chemo sensitive. You give chemo + surgery.