Sarcomas & Cancers of the CNS

Question 1

What is the Most Common Adult Soft Tissue Sarcoma

Answer 1

is the GIST

Question 2

Epidemiology for Sarcomas?

Answer 2

Sarcomas Account for <1% of Adult Cancers
Sarcomas Account for ~ 15% of Pediatric Cancers
In Adults, Soft Tissue Sarcomas Predominate
In Children, Sarcomas of Bone are More Common
There are at least 50 Recognized Subtypes

Question 3

What are Sarcomas derived from?

Answer 3

Cancer Derived from Mesodermal Tissue

Question 4

What 4 inherited syndromes predispose people to sarcomas?

Answer 4

Li-Fraumeni Syndrome
Retinoblastoma Syndrome
Neurofibromatosis Type 1
Gardner’s Syndrome

Question 5

4 Environmental Factors Predisposing to Sarcomas:

Answer 5

Iatrogenic Post Radiation Therapy: Angiosarcoma of the Breast
Chemical Carcinogens: Dioxin, Asbestos
Viruses: HIV

Question 6

Presentation of Soft tissue sarcomas

Answer 6

Painless Mass Growing Relatively Rapidly
Tend to Grow Along Soft Tissue Planes
Metastatic Lesions Generally Spread Hematogenously and NOT to Lymph Nodes
Some Soft Tissue Sarcomas have Unusual Metastatic Patterns, e.g. Liposarcomas tend to Spread to Fatty Tissue

Question 7

How to diagnose a soft tissue sarcoma?

Answer 7

Made by Core Needle Biopsy or Excisional Biopsy

Question 8

Prognosis for soft tissue sarcoma?

Answer 8

stage I– 98.8% 5 year survival
stage II– 81.8%
stage III– 51.7%
stage IV– <20%

Question 9

Treatment for individual stages of soft tissue sarcoma

Answer 9

Stage I : Surgery Alone
Stage II : Surgery & Pre or Post Op RT
Stage III : Surgery, Pre or Post Op RT & Sometimes Adjuvant Chemotherapy
Stage IV : Palliative Chemotherapy, Surgery &/ or RT

Question 10

surgery for a soft tissue sarcoma?

Answer 10

Sarcoma Tend to have a Pseudo-capsule
Wide Excision with Negative Margins is Essential for Cure
RT Frequently Used Pre-Op to  Limb Sparing

Question 11

radiations for soft tissue sarcoma

Answer 11

Local Control
Limb Sparing
Pain Control

Question 12

adjuvant chemo for soft tissue sarcomas?

Answer 12

Mainstay of Rx of Rhabdomyosarcoma & Ewing’s Sarcoma of Bone

Rx Usually Incorporates Doxorubicin & Ifosfamide

Question 13

Rhabdomyosarcomas

Answer 13

Most Common Soft Tissue Sarcoma of Infants & Children
Frequently Present as Polypoid Vaginal or Bladder Mass
May Arise in the Orbit
Highly Responsive to Combination Chemotherapy, Radiation and Surgery
Curable Even in Advanced Stages

Question 14

Leiomyosarcoma

Answer 14

The Benign Counterpart is the Leiomyoma.
Leiomyoma may arise in Uterus (Fibroids), GI Tract, Abdominal Cavity or Retroperitoneum
Malignant Tumors Arise in the Same Areas & Have a Poor Prognosis
Primarily Adult Sarcomas

Question 15

Undifferentiated Pleomorphic Sarcoma (UPS)/Malignant Fibrous Histiocytoma (MFH) or Myxofibrosarcoma

Answer 15

Most Common Sarcoma of Adults; Primarily of 6th -8th Decade
Arise in Extremities as a Fairly Rapidly Growing Mass
Surgery & Radiation are the Mainstays of Rx
Metastatic Disease is Not Very Rx’able

Question 16

Liposarcoma

Answer 16

The Benign Counterpart is the Lipoma
Liposarcomas can Occur in the Extremities where Surgery is Highly Successful or
Retroperitoneum Where Excision is Difficult & Prognosis is Poor

Question 17

Osteosarcoma

Answer 17

Presents as a Painful Boney Mass
Peak Incidence 10 – 20 years old & 60 -80 years old
Arises in the Metaphysis of Long Bones (Between the Epiphysis and the Diaphysis)
Presents as a Raised Periosteal Mass

Question 18

Osteosarcoma treatment

Answer 18

Osteosarcomas are Highly Sensitive to Pre Operative and Post Operative Combination Chemotherapy
Limb Sparing Surgery is Frequently Successful
Excision of Pulmonary Mets Can Be Curative
Long Term Survival is 60 – 80%

Question 19

Ewing’s Sarcoma

Answer 19

Sarcoma of Adolescence
Involves the Diaphysis of the Bone with a Classical “Onion Peel” Pattern
Primitive “ Blue Cell” Tumor, Pathologically ~ to Lymphoma, Small Cell Lung Cancer or Embryonal Rhabdomyosarcoma
Always Considered Metastatic at Presentation
ChemoRx is the Mainstay of Rx
Limb Salvage is Usually Possible with RT & Surgery
80% 5 Year Survival

Question 20

Myeloma

Answer 20

decrease in bone density
multiple “punched out” defects
frequently cold on bone scan

Question 21

2 classifications for cancers of the CNS

Answer 21

Gliomas
Astrocytomas
Grade 4 Astrocytomas (Glioblastoma multiforme)
Oligodenrogliomas

Non-gliomas
Meningiomas
Pituitary adenomas

Question 22

Epidemiology of CNS cancers

Answer 22

Most Common Solid Tumor in Children
Second Leading Cause of Cancer Death in Children
Glioblastoma multiforme (Astrocytoma Grade 4) is the most common malignant brain tumor in adults
Incidence: 4.8/105 Children
25/105 Adults

Question 23

Genetic Syndrome’s Predisposing to Brain Tumors (5)

Answer 23

Turcot’s Syndrome 
Li-Fraumini Syndrome
Neurofibromatosis (NF1 Mutations)
Cowden’s Syndrome
MEN 1 (Menin Mutation) Pituitary Adenomas

Question 24

signs/ symptoms of CNS cancers

Answer 24

Headache, N&V, Focal Neurological Symptoms, Change in Personality and Seizure

Question 25

Diagnosis of CNS cancers

Answer 25

Brain CT Scan + Contrast
Brain MRI
Brain Bx Required; Frequently Associated with Excision

Question 26

Grading systems for Astrocytomas

Answer 26

Grade 1: Benign, Localized & Very Indolent
Grade2: Diffusely Infiltrating
Grade 3: Increased Mitoses; Also called Anaplastic Astrocytomas
Grade 4: Diffusely Infiltrating with Mitoses, Tumor Necrosis and Increased Vascularity (Glioblastoma multiforme)

Question 27

Treatment of Low Grade Astrocytomas

Answer 27

Rarely Completely Resected
More Aggressive Resection and Post Op Radiation Offer Better Prognosis
Recurrence Frequently of Higher Grade
Adjuvant Chemotherapy with Temazolamide Helps

Question 28

Treatment of Grade 3 Astocytomas, Anaplastic Astrocytomas

Answer 28

Median Age of Dx 45 y.o.
More Complete Resection  Better Outcome
Rx: Resection, Post –Op RT and Adjuvant PCV Chemotherapy (Procarbazine, CCNU & Vincristine)
Recurrent or Inoperable Disease May Be Rx’d with PCV, Temazolomide & or Bevicizumab
50% will Survive 5 Years

Question 29

Treatment of Grade 4 Astrocytoma or Glioblastoma multiforme (GBM)

Answer 29

Average Age of Presentation 55 y.o.
Average Survival 15 months; 30% Survive 2 years
Excisional Biopsy or Debulking
Rx is Radiation Rx & Concurrent Temazolomide
Recurrent or Progressive Disease May be Rx’d with Temazolomide & or Bevicizumab

Question 30

Oligodendrogliomas & Oligoastrocytomas

Answer 30

Uncommon Tumors Affecting Individuals Age 35 – 45.
Chemotherapeutically Sensitive
Low Grade Lesions
Present with Seizures, Personality Change etc.
Rx’d with ChemoRT
Average Survival 8 – 10 Years

Question 31

Primary CNS Lymphomas

Answer 31

Primarily Disease of Immunosuppressed Individuals
Rx’d with High Dose Methotrexate and RT
Surgery for Dx Only
Average Survival 2 – 3 years

Question 32

Metastatic Cancer to the Brain

Answer 32

Ten Times More Common than All Primary Brain Tumors Put Together
Occur in 20 – 40% of Patients with Advanced Cancer
Most Common in Lung Cancer, Particularly AdenoCa and Small Cell, Breast Cancer & Melanoma.
Identified with Multiple Lesions at the Junction of the Gray & White Matter
Solitary Lesions May Be Rx’d with Surgery & Post Op RT
Multiple Lesions Rx’d Whole Brain RT
ChemoRx of Limited Benefit Except in Lymphomas and Germ Cell Cancers
Average Survival < 6 months Regardless of Rx