Part 13: Peds Tumors- other tumors

Langerhans Cell Histiocytosis aka

Histiocytosis X

What is Langerhans Cell Histiocytosis

• Several different diseases

• involve abnormal reproduction of the histiocytic immune system’s Langerhans cells

• Debate if its really a malignancy (i.e. cancer)

Why is there debate on whether or not Langerhans Cell Histiocytosis is a malignancy?

Cuz 30-40% of untreated patients survive

Types of lymphomas discussed, rank them in order from most curable/ best prognosis to least

1. HD- fairly curable

2. NHL- less curable

3. Burkitt’s Lymphoma- poorest prognosis of all lymphomas

At what ages are lymphomas most common

More common in adolescents and young adults

less common in children or infants

Osteogenic sarcoma aka

osteosarcoma

Osteosarcoma epidemiology

• most common primary bone cancer

• usually occurs in bones: around the knee

• usually occurs in: teen years

Osteosarcomas have a much better prognosis than __ years ago

20

Treatment of osteosarcomas:

1. surgery

2. RTT

3. Chemo

1. surgery: amputation, not as common as in the past

2. RTT: rare

3. Chemo: used more and more; helps minimize need for amputation

What is Ewing’s Sarcoma

non-osseus tumor
arises from soft tissue near bone (not the actual bone)

Ewing’s sarcoma epidemiology

• peak incidence: teen years

• most common site: femur (on it)

Treatment for Ewing’s Sarcoma

1. Surgery

2. RTT

3. Chemo

1. Surgery: rare

2. RTT: the most radiosensitive of the category of bone/ soft tissue sarcoma (which are usually very radioresistant)

3. Chemo: a common adjunct to RTT

Ewing’s sarcoma 5 year survival

50%

What is a germ cell tumor?

• where does it arise from?

• where does it appear?

• arise from ovum or sperm cell

• can appear on a variety of sites in the body

how common are germ cell tumors

rare

term. teratoma

def. a type of germ cell tumor,

a true neoplasm of many types of tissue

questions about teratomas:

1. when are they formed

2. where are they most often found

3. what are they composed of

1. congenital (present at birth but diagnosed later in life)

2. ovaries & testes

3. different types of tissues arising from all 3 embryonic germ layers

teratoma etymology

from Greek- monstrous tumor

Teratoma etiology

unclear

maybe an abortive attempt at twinning

True or False: Keloids and fibromas are malignant

False

Treatment of Keloids and Fibromas

resection & steroid injections

if the above fails many times, then:

1. resection

2. followed immediately by low dose RTT:

Dose for treating keloids

900-1200 cGy in 3 fractions