Pediatric Solid Tumors

Question 1

Which tumor is known as a mysterious, unpredictable embryonal tumor?

Answer 1

Neuroblastoma

Question 2

Neuroblastomas originate from:

Answer 2

Neuroblasts

Question 3

Neuroblastomas usually arise in which ages?

Answer 3

1) Infancy ~30%
2) 1–4 years ~50%

Question 4

Which carries a better prognosis: Neuroblastomas in an infant or a 4 year old child?

Answer 4

Infant

Question 5

The most common site of origin for neuroblastomas is:

Answer 5

Adrenal medulla (~50%)

Question 6

What do neuroblastomas look like?

Answer 6

Soft with areas of hemorrhage and necrosis
(Mature areas are firm)

Question 7

What do neuroblastomas look like histologically?

Answer 7

Sheets of dark blue round cells with scanty cytoplasm, embedded in a delicate vascular stroma.

Question 8

How can we differentiate neuroblastomas from other cancers histologically?

Answer 8

Characteristic ring of neuroblasts around
a neurofibrillary core (rosette formation)

Question 9

Neuroblastomas tend to spread with local extension and encasement of:

Answer 9

Major vessels

Question 10

Neuroblastomas mainly metastasize to:

Answer 10

1) Lymph nodes
2) Bones
3) Bone marrow
4) Liver
5) Skin

Question 11

The __ System Classification classifies neuroblastomas into:

Answer 11

Shimada; Favorable vs. Non-favorable

Question 12

The Shimada System Classification is based on the:

Answer 12

1) Mitosis karyorrhexis index (MKI)
2) Age of child
3) Degree of differentiation (towards ganglioneuroma)
4) Stroma-rich or stroma-poor

Question 13

A favorable prognosis based on the Shimada System Classification would be:

Answer 13

1) Low MKI
2) Infants
3) Well-differentiated or intermixed differentiation
4) Stroma-rich

Question 14

___ tell us about the prognosis of neuroblastomas.

Answer 14

Cytogenetics

Question 15

What are the COMMON clinical features of neuroblastomas?

Answer 15

1) Palpable abdominal mass
2) Children often appear sick, lethargic with fatigue
3) Bone pain
4) Weight loss
5) Fever, sweating, and anemia

Question 16

What are the unusual, but CHARACTERISTIC clinical features of neuroblastomas?

Answer 16

1) Racoon eyes (no trauma)
2) Horner’s syndrome
3) SVC Syndrome
4) Hepatomegaly
5) Progressive paraplegia
6) Hypertension
+ other signs of catecholamines

Question 17

What lab tests should you order if you suspect neuroblastoma?

Answer 17

1) ↑↑ Vanillylmandelic acid (VMA) and homovanillic acid (HVA) in urine
2) ↑ ferritin
3) ↑ lactate dehydrogenase (LDH)
4) ↑ Neuron specific enolase (NSE)

Question 18

What are the urinary metabolites of catecholamines?

Answer 18

1) Vanillylmandelic acid (VMA)
2) Homovanillic acid (HVA)

Question 19

What imaging should you order if you suspect neuroblastoma?

Answer 19

1) Abdominal XR
2) Ultrasound
3) CT/MRI
4) Radio-isotopes (MIBG1 scan)

Question 20

What would you see in an abdominal X-Ray for a neuroblastoma?

Answer 20

Tumor calcification (~50%)

Question 21

What would you look for in an ultrasound for a neuroblastoma?

Answer 21

1) Solid vs. Cystic
2) Renal vein and caval involvement

Question 22

What would you look for in a CT/MRI for a neuroblastoma?

Answer 22

1) Anatomy of tumor
2) Metastases
3) Intraspinal extension (“dumb-bell” tumor)

Question 23

What more work up can we do for neuroblastomas?

Answer 23

1) Tissue biopsy
2) Bone marrow biopsy/aspiration

Question 24

The International Neuroblastoma Staging System (INSS) is done:

Answer 24

Post-op

Question 25

What is a Stage 4S tumor?

Answer 25

Neuroblastoma with distant metastasis (skin, liver, bone marrow) in infants

Question 26

What differentiates Stage 4S neuroblastoma from Stage 4?

Answer 26

1) No spread to bones or brain 2) Good prognosis 3) Usually resolve spontaneously

Question 27

Stage 4S neuroblastoma only metastasizes to 3 sites:

Answer 27

1) Skin 2) Liver 3) Bone marrow

Question 28

What are the features of stage 4S neuroblastoma?

Answer 28

1) Hepatosplenomegaly 2) Subcutaneous nodules (‘Blueberry muffin’ spot) 3) Positive bone marrow

Question 29

The International Neuroblastoma Risk Group (INRG) defines risk group by:

Answer 29

1) Pretreatment grade 2) Postsurgery INSS staging 3) Age 4) Tumor biology, histology, and MYCN status

Question 30

How can we manage neuroblastomas?

Answer 30

1) Tumor biopsy (to assess MYCN status → direct Mx plan) 2) Surgical resection alone 3) Neoadjuvant chemotherapy → Surgery 4) Neoadjuvant chemotherapy → Surgery → Adjuvant chemotherapy

Question 31

When should we do surgical resection alone for neuroblastomas?

Answer 31

1) Low risk group: a) Stage 1 b) Stage 2 (<1yr old) c) Stage 4S 2) Absence of Image Derived Risk Factors pre-resection

Question 32

When should we do Neoadjuvant chemotherapy → Surgery for neuroblastomas?

Answer 32

1) Intermediate risk group 2) Intraspinal extension | Apical thoracic tumors

Question 33

When should we do Neoadjuvant chemotherapy → Surgery → Adjuvant chemotherapy for neuroblastomas?

Answer 33

High risk groups

Question 34

Neuroblastoma management for high risk groups should be done in this following order:

Answer 34

1) Induction therapy (Chemotherapy, ALK inhibition, MIBG, monoclonal antibodies) 2) Local control (Surgery, Radio-therapy) 3) Consolidation (Chemotherapy, Autologous hematopoietic stem cell transplantation, RT) 4) Immunotherapy (Dinutuximab & others)

Question 35

What is the aim of surgery for neuroblastomas?

Answer 35

To achieve complete resection

Question 36

What is the aim of second-look procedure for neuroblastomas?

Answer 36

To achieve as complete a debulking as possible

Question 37

What is the possible role for laparoscopic and thoracoscopic surgery for neuroblastomas?

Answer 37

1) Diagnostic 2) Biopsy taking 3) +/- excision of smaller tumors

Question 38

Should screening be done for infants with urinary catecholamines?

Answer 38

No; it does not reduce mortality

Question 39

Nephroblastoma is also called:

Answer 39

Wilms Tumor

Question 40

Nephroblastoma (WT) is:

Answer 40

A highly malignant renal tumor

Question 41

Nephroblastoma (WT) is derived from:

Answer 41

Embryonic tissue

Question 42

What is the prognosis of Nephroblastoma (WT)?

Answer 42

Reasonable prognosis due to successful multimodal therapy

Question 43

What is the most common pediatric renal tumor?

Answer 43

Nephroblastoma (WT)

Question 44

What is the most common intra-abdominal malignancy?

Answer 44

Neuroblastoma

Question 45

Nephroblastoma (WT) is usually ___(solitary/multifocal)

Answer 45

Solitary

Question 46

Nephroblastoma (WT) is usually ___(unilateral/bilateral)

Answer 46

Unilateral

Question 47

Nephroblastoma (WT) is usually __(genetic/sporadic)

Answer 47

Sporadic

Question 48

Nephroblastoma (WT) can be associated with syndromes such as:

Answer 48

1) Beckwith-Wiedemann Syndrome 2) WAGR 3) Denys–Drash syndrome

Question 49

Nephroblastoma (WT) arises from:

Answer 49

Fetal undifferentiated metanephric platesma tissue.

Question 50

Which mutations are associated with Nephroblastoma (WT)?

Answer 50

1) WT1 2) WT2 3) p53 4) FWT1&2

Question 51

The usual presentation of Nephroblastoma (WT) is a small child with:

Answer 51

1) An asymptomatic abdominal mass (80%) 2) Abdominal pain (30-40%) 3) Hematuria (~20%) 4) HTN (25%)

Question 52

Rare features of Nephroblastoma (WT) include:

Answer 52

1) UTI 2) Fever (from tumor necrosis) 3) Anemia 4) Varicocele 5) Acute abdomen with tumor hemorrhage or rupture (avoid vigorous palpation!)

Question 53

What lab investigations should you do if you suspect Nephroblastoma (WT)?

Answer 53

1) Serum Cr & Urine analysis 2) LFT 3) β-FGF 4) Renin 5) Erythropoietin 4) Cytogenetics studies

Question 54

What imaging should you do if you suspect Nephroblastoma (WT)?

Answer 54

1) US 2) CT Scan/MRI staging, extension into renal veins and cava (~40%) 3) Bone and brain scan to identify mets 4) Echocardiogram 5) Arteriography 6) DMSA

Question 55

Why would you do an echo for Nephroblastoma (WT)?

Answer 55

To check for right atrial involvement

Question 56

What are the management options for Nephroblastoma (WT)?

Answer 56

1) Neoadjuvant chemotherapy → Surgery (SIOP) 2) Surgery → adjuvant chemotherapy (COG)

Question 57

What are the surgery options for Nephroblastoma (WT)?

Answer 57

1) Nephrectomy including perinephric fascia and regional lymph nodes 2) Partial Nephrectomy 3) Venous extension venotomy & removal 4) Hepatic or pulmonary metastatectomies

Question 58

When is partial nephrectomy indicated in Wilms Tumor?

Answer 58

1) Bilateral Wilms Tumor 2) Pre-existing abnormality in the contralateral kidney 3) Wilms Tumor in a single kidney 4) Wilms Tumor with nephroblastomatosis

Question 59

What surgical approach is taken if the Nephroblastoma (WT) extends into a vein?

Answer 59

Venotomy and removal of the venous extension

Question 60

What is the favorable histology for Nephroblastoma (WT)?

Answer 60

1) Tubular epithelial 2) blastemal 3) Stromal elements

Question 61

What is the unfavorable histology for Nephroblastoma (WT)?

Answer 61

Anaplasia (focal or diffuse nuclear enlargement)

Question 62

What is the survival rate for Stages I–III Nephroblastoma (WT)?

Answer 62

90%

Question 63

What is the survival rate for Stage IV Nephroblastoma (WT)?

Answer 63

70%

Question 64

What are the most important prognostic factors for Nephroblastoma (WT)?

Answer 64

1) Stage!! 2) Tumor histology 3) Age at diagnosis 4) Recurrence

Question 65

In Nephroblastoma (WT), the survival rate for infants is:

Answer 65

Low

Question 66

Screening for Wilms tumor is performed by:

Answer 66

Serial abdominal ultrasonography

Question 67

When do we screen for Wilms tumor?

Answer 67

In high-risk patients (eg, children with BWS or WAGR syndrome)