A 17-year-old female was admitted after surgical biopsy for a workup and treatment planning for a suspicious lesion in her pelvis diagnosed after a 3 week history of right hip pain.
The past medical history revealed treatment for localized Ewing sarcoma of her right femur at age 9 after presenting with pain and a palpable mass. She received limb salvage surgery, chemotherapy and radiation.
The review of systems showed no sweating, weight loss, fever or other symptoms.
The pertinent physical exam revealed an anxious adolescent with normal vital signs. She had shoddy cervical and inguinal adenopathy and no hepatosplenomegaly.
The right femur showed limb salvage with no swelling, erythema or pain noted in the extremity. She complained of diffuse pain along the right anterior iliac crest but had no point tenderness or specific pain radiation. The area had minor increased warmth but no discernable increased vascularity.
She had no pain over the sacrum or coccyx.
The radiologic evaluation showed an osteolytic lesion of the right anterior iliac crest.
The work-up included a complete blood count with a hemoglobin of 11.8 mg/dl, normal white blood cell count, differential, platelets and smear. She had normal liver function tests.
She was taken to the operating room and
the diagnosis of osteosarcoma was made on the biopsy of the lesion. Her treatment was being planned.
Ewing sarcoma was first described by James Ewing in 1921. It is part of the Ewing sarcoma family of tumors which share a similar cellular physiology and a translocation between chromosomes 11 and 22.
The tumors include: Ewing sarcoma, peripheral primitive neuroectodermal tumor, neuroepithelioma, atypical Ewing sarcoma, and Askin tumor (a chest wall tumor). The tumors are treated based on their clinical presentation rather than histology. Treatment includes surgery, chemotherapy and radiation.
Most cases occur in the first 2 decades of life. Overall survival is 60% and increases to ~70% with localized disease, but survival is < 25% for metastatic disease.
Osteosarcoma is a common solid tumor that occurs often in long bones especially near the growth plates. It occurs in the pelvis 8% of the time. It is a common second primary malignancy especially after radiation treatment.
Overall 5 year survival is 63%. Treatment includes mainly surgery and also chemotherapy.
The incidence of second primary malignancies in the first 20 years after initial diagnosis is 3-12%. The rates vary according to:
- Patient age
- Original neoplasm – e.g. Hodgkin’s disease, retinoblastoma or genetic form of Wilm’s tumor
- Treatment – e.g. radiotherapy, alkylating agents or epipodophyllotoxins
- Genetic conditions that predispose a patient to malignancy – e.g. von Recklinghausen’s neurofibromatosis, Li-Fraumenti family cancer syndrome
Acute nonlymphoblastic leukemia is one of the most common hematopoietic second primary malignancies. It is reported in 10-20% of patients.
Solid tumors are associated with radiotherapy especially bone and soft tissue sarcomas, carcinomas of the skin and thyroid and breast cancer.
It is controversial whether or not Ewing sarcoma has a higher risk of secondary malignancy than other childhood cancers. One study found the risk in Ewing Sarcoma survivors was higher than other childhood and adolescent cancers only if primary treatment included radiotherapy. This study found an overall risk of 12.7% for secondary primary malignancies at 20 years.
Questions for Further Discussion
1. What follow-up screening should be given to long-term survivors of childhood cancers?
2. What are some of the ways primary care physicians, pediatric oncologists and adult oncologists can work together to ensure proper follow-up for long-term survivors of childhood cancers?
3. What is the risk of a third primary malignancy?
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
To view current news articles on this topic check Google News.
Toretsky JA. Ewing Sarcoma and Primitive Neuroectodermal Tumors. eMedicine.
Available from the Internet at http://www.emedicine.com/ped/topic2589.htm (rev. 1/17/2003, cited 11/17/2005).
Cripe TP. Osteosarcoma. eMedicine.
Available from the Internet at http://www.emedicine.com/PED/topic1684.htm (rev. 2.10.2004, cited 11/16/2005).
Bacci G, et.al. Second Malignancy in 597 Patients with Ewing Sarcoma of Bone Treatment at a Single Institution with Adjuvant and Neoadjuvant Chemotherapy Between 1972 and 1999. J. Pediatr Hematol Oncol. 2005;27:517-520.
Pizzo PA, Poplack DG. Principles and Practice of Pediatric Oncology. 4th. Lippincott Williams & Wilkins, Philadelphia, PA, 2001; 1450-2.
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effecively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
7. All medical and invasive procedures considered essential for the area of practice are competency performed.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
Donna M. D’Alessandro, MD
Associate Professor of Pediatrics, Children’s Hospital of Iowa
January 2, 2006