MOFFITT ONCOLOGY REPORT PEARLS 1/6/17: Ewing Sarcoma!

Thanks to Nadia and Megan for sharing two interesting oncology cases with us: the first about metastatic pancreatic cancer complicated by bowel ischemia, and the second about Ewing sarcoma with spinal cord compression! Pearls on Ewing sarcoma below.

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Top Pearls:

  1. 30% of Ewing sarcoma cases occur in patients over age 30.
  2. The most common locations are pelvis > femur > ribs.
  3. Fever and elevated LDH are signs of metastatic disease.
  4. Systemic chemotherapy is used even for treatment of localized Ewing sarcoma.

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For those who want more info:

Epidemiology:

  • Peak incidence 10-15 years of age, but 30% occur in patients over age 30
  • Almost exclusively a disease of Caucasian patients! (unknown reason for this)
  • No specific environmental exposures have been associated

Presentation:

  • Most tumors in flat or long bones (pelvis > femur > ribs)
  • Can also arise from other bones or soft tissue
  • Localized pain/swelling is the most common presentation
  • Only 10-20% have constitutional sx at presentation (associated with advanced dz)

Metastasis:

  • Assumed to be present in nearly all patients due to high relapse rate with local therapy alone
  • Overt metastasis present in 25% of patients at initial diagnosis
  • Most common site is lung (70-80%), which is leading cause of death
  • Lymph node, liver, and brain mets are uncommon

Diagnosis:

  • *Pearl: LDH elevation is associated with metastatic disease
  • MRI of entire involved bone is imaging modality of choice (can have skip lesions)
  • CT-guided core needle biopsy is most common method to obtain tissue (FNA insufficient)
  • CT chest and PET/bone scan needed for metastatic workup

Treatment:

  • Systemic chemotherapy even for localized disease!

 

  • VDC/IE is standard regimen (vincristine, doxorubicin, cyclophosphamide with alternating cycles of ifosfomide and etoposide)
  • Ewing sarcoma is radiosensitive, but surgery has better local control than RT

Surveillance:

  • CT imaging of primary site and chest for unspecified duration
  • Most recurrences occur within 10 years

Prognosis:

  • 5-year survival rates are 70% for localized disease and 33% for overtly metastatic disease.

 

Evernote: https://www.evernote.com/shard/s272/sh/6bf7c004-32a1-4a59-81e4-0a883e10bb9f/122b34019b716887ccd3f2d0bdca38f4

 

Have a great day everyone!

Sammy

 

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