Sarcomas & Cancers of the CNS Flashcards
What is the Most Common Adult Soft Tissue Sarcoma
is the GIST
Epidemiology for Sarcomas?
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
What are Sarcomas derived from?
Cancer Derived from Mesodermal Tissue
What 4 inherited syndromes predispose people to sarcomas?
Li-Fraumeni Syndrome
Retinoblastoma Syndrome
Neurofibromatosis Type 1
Gardner’s Syndrome
4 Environmental Factors Predisposing to Sarcomas:
Iatrogenic Post Radiation Therapy: Angiosarcoma of the Breast
Chemical Carcinogens: Dioxin, Asbestos
Viruses: HIV
Presentation of Soft tissue sarcomas
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
How to diagnose a soft tissue sarcoma?
Made by Core Needle Biopsy or Excisional Biopsy
Prognosis for soft tissue sarcoma?
stage I– 98.8% 5 year survival
stage II– 81.8%
stage III– 51.7%
stage IV– <20%
Treatment for individual stages of soft tissue sarcoma
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
surgery for a soft tissue sarcoma?
Sarcoma Tend to have a Pseudo-capsule
Wide Excision with Negative Margins is Essential for Cure
RT Frequently Used Pre-Op to Limb Sparing
radiations for soft tissue sarcoma
Local Control
Limb Sparing
Pain Control
adjuvant chemo for soft tissue sarcomas?
Mainstay of Rx of Rhabdomyosarcoma & Ewing’s Sarcoma of Bone
Rx Usually Incorporates Doxorubicin & Ifosfamide
Rhabdomyosarcomas
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
Leiomyosarcoma
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
Undifferentiated Pleomorphic Sarcoma (UPS)/Malignant Fibrous Histiocytoma (MFH) or Myxofibrosarcoma
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
Liposarcoma
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
Osteosarcoma
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
Osteosarcoma treatment
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%
Ewing’s Sarcoma
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
Myeloma
decrease in bone density
multiple “punched out” defects
frequently cold on bone scan
2 classifications for cancers of the CNS
Gliomas
Astrocytomas
Grade 4 Astrocytomas (Glioblastoma multiforme)
Oligodenrogliomas
Non-gliomas
Meningiomas
Pituitary adenomas
Epidemiology of CNS cancers
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
Genetic Syndrome’s Predisposing to Brain Tumors (5)
Turcot’s Syndrome Li-Fraumini Syndrome Neurofibromatosis (NF1 Mutations) Cowden’s Syndrome MEN 1 (Menin Mutation) Pituitary Adenomas
signs/ symptoms of CNS cancers
Headache, N&V, Focal Neurological Symptoms, Change in Personality and Seizure
Diagnosis of CNS cancers
Brain CT Scan + Contrast
Brain MRI
Brain Bx Required; Frequently Associated with Excision
Grading systems for Astrocytomas
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)
Treatment of Low Grade Astrocytomas
Rarely Completely Resected
More Aggressive Resection and Post Op Radiation Offer Better Prognosis
Recurrence Frequently of Higher Grade
Adjuvant Chemotherapy with Temazolamide Helps
Treatment of Grade 3 Astocytomas, Anaplastic Astrocytomas
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
Treatment of Grade 4 Astrocytoma or Glioblastoma multiforme (GBM)
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
Oligodendrogliomas & Oligoastrocytomas
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
Primary CNS Lymphomas
Primarily Disease of Immunosuppressed Individuals
Rx’d with High Dose Methotrexate and RT
Surgery for Dx Only
Average Survival 2 – 3 years
Metastatic Cancer to the Brain
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
