EWING SARCOMA Flashcards
DEFINITION
MALIGNANT ROUND CELL TUMOUR OF BONE AND SOFT TISSUE .
PART OF FAMILY OF PNET ( PERIPHERAL NEURO ECTODERMAL TUMOUR )
TRANSLOCATION OF CHROMOSOME 11 AND 22
PATHOLOGY
GROSS =
-LOBULATED AND LARGE
-IS GREY ( BRAIN LIKE ) OR RED ( IF HEMORRHAGE -HAS OCCURED )
-ARISES IN MEDULLARY CAVITY .
MICROSCOPICALLY =
-ROUND CELLS WITH SCANTY CYTOPLASM DUE TO PRESENCE OF GLYCOGEN .
-CYTOPLASM PAS POSITIVE AND DIASTAGE DIGESTABLE .
- HOMER WRIGHT ROSETTE = CELLS ARRANGED AROUND CENTRAL FIBRILLARY SPACE
- PSEUDOROSETTE = CELLS ARRANGED AROUND BLOOD VESSELS
-CELLS EXPRESS MIC 2 GENE IN HIGH AMOUNT ; DISTINGUISHES THEM BETWEEN OTHER ROUND CELL TUMOURS.
AGE
10 TO 20
SEX
MALE
SITE
LONG BONES =
FEMUR , FIBULA > ILLEUM > TIBIA
FLAT BONES = PELVIC , CALCANEUM
LOCATION
DIAPHYSIS
SYMPTOM
PAINFUL
HARD SWELLING
SITE TENDER , WARM AND SWOLLEN
MAY BE PRESENTED WITH PATHOLOGICAL FRACTURE
SIGNS
DIAPHYSEAL SWELLING FIXED TO BONE
SKIN RED , DIALATED VEIN MAY BE PRESENT
INVESTIGATION
X RAY
BIOPSY
OTHERS
X RAY
MOTH EATEN APPEARANCE ( IRREGULAR BONE DESTRUCTION)
ONION PEAL APPEARANCE ( SUBPERIOSTEAL BONE FORMATION IN LAYERS )
CODMANNS TRIANGLE, SUN RAY APPEARANCE ( IF SOFT TISSUE INVADED )
IN FLAT BONE = LYTIC LESION
BIOPSY
SMALL ROUND CELLS WITH SCANTY CYTOPLASM
DEMONSTRATION OF TRANSLOCATION OF CHROMOSOME 11 AND 22 BY RT PCR
OTHER TESTS
-SCREENING FOR MONOCLONAL AB TO MIC 2 GENE TO DIFFERENTIATE BETWEEN OTHER ROUND CELL TUMOUR.
-PAS POSITIVE , RETICULIN NEGATIVE , DIASTASE SENSITIVE
-URINE SHOWS VMA
-ANAEMIA , INCREASE WBC , LDH , ESR
TREATMENT
TUMOUR IN FAVORABLE SITE
TUMOUR IN LESS ACCESSIBLE SITE
TUMOUR IN FAVORABLE SITE
NEO ADJUVANT CHEMOTHERAPY —> WIDE EXCISION / AMPUTATION —> ADJUVANT CHEMOTHERAPY FOR 1 YEAR ( FOR DISTANT METASTASIS)
TUMOUR IN LESS ACCESSIBLE SITE
NEO ADJUVANT CHEMOTHERAPY –> RADIOTHERAPY FOR LOCAL TUMOUR ( 6000 RADS )
–> LOCAL EXCISION –> CHEMOTHERAPY FOR 1 YEAR ( FOR DISTANT METASTASIS)
