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42 Cards in this Set
- Front
- Back
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How was Ewing's sarcoma first defined in 1921 by James Ewing?
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An undifferentiated bone tumor that differs from osteosarcoma b/c of its radiosensitivity
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Initially Ewings sarcoma tumor was thought to be of endothelial origin. What origin was later identified?
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Neural crest origin
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Where does Ewing's sarcoma most frequently occur in the body?
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The bone
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What other neoplasms of neuroectodermal origin have similar histolgic & immunohistochemical characteristics?
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1. EES (Extraosseous Ewing's Sarcoma)
2. PNET (peripheral neuroectodermal tumor) 3. Askin's tumor (malignant small-cell tumor of the thoracopulmonary region) |
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What does ESFT stand for?
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Ewing's sarcoma family of tumors
( = EES, PNET, Askin's tumor) |
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How is PNET different from the other tumors in the ESFT?
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PNET is the most highly differentiated tumor on the spectrum
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What is Ewing's sarcoma basic pathophysiology?
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tumor of small, round cells with blue granules
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What characteristic chromosomal abnormalities does a Ewing's sarcoma tumor have?
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90% of tumors have EWS-FLI1 t(11;22; q24;q12)
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If an Ewing's sarcoma tumor lacks the EWS-FLI1 translocation, what translocation do 5-10% of those tumors have?
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EWS-ERG t(21;22;q22;q12)
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What is the most commonly occurring cytogenetic alteration in the ESFT?
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Trisomy 8 (55%)
Trisomy 12 (33%) 9q21 LOH (p16;INK4) (33%) |
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What other well known mutation occurs in 5-20% of ESFT?
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p53 gene mutation (Li Fraumini)
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What special stain is needed to differentiate them from other tumors?
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Stain w/vimentin & express CD 99
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T or F: The ESFT are the second most common bone malignancy in childhood & adolescence
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True; in US - 200 kids & teens <20yrs of age are diagnosed annually
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What percentage of bone cancers are ESFT?
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34%
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What ancestry is ESFT exceedingly rare?
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Asian & African decent
(most common in European or Hispanic ancestry) |
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Review page 41
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Table 2-11: Causes of Ewing's Sarcoma
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How does ES typically present?
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Intermittent pain present from weeks to months that was mild at first and has increased over time
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What can potentially intensify the pain associated with a newly presenting ES patient?
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Exercise can intensify pain
Night time pain is often worse |
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What is present among 10-15% of patients that present with a ES?
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Pathological fractures
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What is the distribution of primary sites for disease in ES?
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53% = extremities
47% = central axis (pelvis, chest wall, spine/paravertebral region, head & neck) |
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What percentage of patients have overt metastatic disease at initial diagnosis?
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25% (lung, bone, bone marrow)
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If a soft tissue mass is present, what must you consider at time of diagnosis?
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Is the primary tumor bone w/an associated soft-tissue mass
OR is the it soft-tissue mass that invades the bone |
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What is assumed as a result of the 80-90% relapse rate when local therapy is used alone for treatment?
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believed that metastatic disease is sub clinically present in most cases
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What radiographic appearance does ESFT have?
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- a loss of normal sharp, dense, cortical bone
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How is the radiologic abnormality typically described d/t the presence of finely destructive lesions?
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"Moth-eaten appearance"
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How else is ESFT describe as a result of the periosteal reaction within the layers of reactive bone?
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"Onion-skin appearance"
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What is an MRI necessary at time of diagnosis?
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can reveal soft tissue, nerve, & vessel involvement & tumor boundaries
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What other procedure is done at time of diagnosis as part of the workup?
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Bilateral BMA/Bx
Bone Scan Biopsy of tumor at pedi onc center |
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What is the most significant prognostic indicator at time of diagnosis?
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Detectable metastatic disease
= poor outcomes |
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What other specifics are considered to be a poor prognostic factor at diagnosis?
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- Primary lesion in the pelvis
- Older age - p53 mutation - del of short arm of chromosome 1p - homozygous deletions of CDKN2A and p16/p14ARF - Higher level of LDH |
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What primary tumor sites are considered to fare best?
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primary lesions of distal extremity or the rib
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What chemotherapy is typically given for ESFT?
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- Ifosfamide (Ifex)
- Cyclophosphamide (Cytoxan) - Vincristine (Oncovin) - Etoposide (VP-16) - Doxorubicin (Adriamycin) - Dactinomycin (Actinomycin D) |
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What does of radiation is typically used for ESFT? And does it respond?
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Radiosensitive yes
Total tumor dose = 55-60Gy |
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Why is it important that local control of a tumor not compromise systemic therapy?
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B/c metastatic disease = poor prognosis (they assume most have a subclinical form)
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What treatment is used for lung metastases?
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Lung irradiation
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What are the cure rates for localized ESFT?
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60-70% (distal more easily cured than axial)
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What is the cure rate for metastatic ESFT?
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18-30%
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With disease recurrence what is the chance of survival related to?
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extent of recurrence, biology of tumor cells, aggressiveness of previous treatment, length of time btwn treatment, recurrence & age
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What age group has improved outcomes of survival rates after recurrence of disease?
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younger than 11yrs
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What patients are being given high-dose therapy followed by Auto SCR?
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- metastatic disease
- refractory or relapsed disease - large inoperable tumors |
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What is the risk with High dose chemo and AUto SCR?
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Secondary malignancy = 30%
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What is the overall survival rate for those patients with recurrent disease?
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less than 20%
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