Tumors and lesions of childhood lecture 20

Question 1

What is the difference between developmental, benign, and malignant tumors?

Answer 1

DT is an anatomical error of development eg occurs in ectopic location or disorganised normal tissue eg harmatoma ,christoma

BT is a true neoplasm with minimal dysplastic ,localised lesions and do not metastasize

MT : True neoplasm with different degree of differentaition and invade and metastasize

Question 2

Define embryonal tumors

Answer 2

Arises in immature tissue in which the developing area undergoes a neoplastic transformation

Question 3

T/F: Embryonal tumors are only present in embryos

Answer 3

False, it may be present at birth or develop during childhood. More often in children between 3-4

Question 4

T/F: Embryonal tumor rank as a major cause of death in childhood third to accidental death

Answer 4

false ranks as a major cause of death, second to the accident.

Different organs can be affected :

• kidney
• adrenal gland
• retina of the eye
• brain
• liver
• brain
• haematolymphoid tissue

Question 5

What is a Choristoma/heterotopia

Answer 5

*Microscopic normal tissue in abnormal locations eg pancreatic tissue in the stomach

often mistake as a neoplasm

Question 6

What is a hamartoma?

Answer 6

*A noncancerous tissue made up of an abnormal mixture of normal tissue and cells from which it grows. Disrupt / disorganized arrangement of the architecture of the tissue

Question 7

What is a teratoma

Answer 7

Neoplasm of germ cell origin that forms cells representing all 3 germ layers . In their benign form these cellular types are easily recognizable .The tumor may contain teeth ,hair ect. In their malignant form these may appear immature and less recognizable

occur in gonads.

Question 8

T/F: Ovarian teratomas are almost always benign and cystic and in the tests are almost always malignant and cystic. Extragonadal sites for teratomas include mediastinum and sacrococcygeal teratomas

Answer 8

True

Question 9

T/F: benign teratomas are seen in young children and malignant in older children

Answer 9

T

Question 10

What are a naevi

Answer 10

Any developmental blemish of the skin and most described pigmented cellular lesions

Question 11

What are angiomas

Answer 11

Tumors of the blood vessel eg hemangiomas and lymphangiomas

Question 12

Compare the most common affected site by tumors in children and adults

Answer 12

Children: hematopoietic, neural, and soft tissue

Adults: lung, breast, prostate, and colon

Question 13

Discuss leukemia (acute lymphoblastic leukemia ) and causes

Answer 13

• All can be b cell / t cell in origin
• B cell leukemia tends to involve bone marrow
• T cell tends to manifest as mediastinal mass and can involve bone marrow as some stages of the disease

Question 14

Types of groups susceptible to leukemia

Answer 14

All- b cell phenotype affects children -4 years of age
*All -T cell phenotype more common in males between ages of 15-20
ALL makes up 80% of childhood leukemias

Question 15

How to morphologically identify neuroblastoma

Answer 15

1. Morphologically
   * More invading
   * Has a tan grey color of the brain
   * can have necrosis ,cystic softening and hemorrhaged

Question 16

Discuss Neuroblastoma origin, unique features

Answer 16

*Originate in the sympathetic ganglia and adrenal medulla (neural crest cells )

> Unique features

• spontaneous regression
• Spontaneous /therapy induced maturation

Question 17

Discuss the retinoblastoma

Answer 17

*Common intraocular malignancy in children

> Cause
*associated with mutation in RB1 gene (heritable)

> There are familial rsik of developing multiple tumors and at risk of developing osteosarcoma /other soft tissue

Question 18

T/F: The retinosarcoma can disseminate through optic nerve or subarachnoid spaces and distant metastasis to CNS, skull, distal bones, and lymph nodes

Answer 18

true

Question 19

Which 3 groups of congenital malformations are associated with nephroblastoma

Answer 19

> WAGR syndrome : Wilims tumour ,aniridia ,genital abnormalities ,mental retardation

> DENYS : drash syndrome ,gonadal dysgenesis ,renal abnormalities ,

> Beckwith : Wedermann syndrome ,enlargement of organs

Question 20

Embryonal rhabdomyosarcoma

Answer 20

• Common in the neck, head, and genitourinary tract
• Pax3 gene
• Has 3 embryonal subtypes eg embryonal, alveolar and pleomorphic