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Case Presentation - Summer, 2010

Post Radiation Sarcoma

Case provided by: Lindsey Towlen, student, Fletcher Allen Health Care, School of Cytotechnology, Burlington, Vermont.

This presentation won the third place award at the ASCT annual meeting in Austin, Texas.


History: 79 year old female

Specimen type: FNA of Right Chest Wall Mass

February 2004:
Patient underwent a partial mastectomy with sentinel and axillary node dissection. Surgery was followed by radiation therapy, and was completed in June 2004.

Patient suffered from complications of Alzheimer's disease and remained cancer free for 4 years and 4 months.

October 2008:
Patient noticed increased pain in upper quadrant of her right breast. Ultrasound showed a hypoechoic mass at the level of the pectoralis muscle; mass was 10 x 5 cm.

Clinical impression was recurrent disease.

November 2008:
FNA of right chest wall mass was performed.

December 2008:
Core biopsy scheduled.

FNA of Right Chest Wall Mass:

Cytology:

Cellular sample, syncytial fragments, and increased single cell population
  • Pleomorphic cells
  • Polygonal cells
  • Bizarre cells
  • Hyperchromatic, coarsely clumped chromatin
  • Hyperchromatic multinucleated giant tumor cells
  • Prominent nucleoli
  • Spindle cells
  • Abundant dense cytoplasm
  • Easily identifiable mitotic figures
Immunocytochemical Stains - Right Chest Wall Mass:

Positive: Vimentin

Negative: MNF116, Keratin AE1-AE3, S-100 protein DAB, CD34, Desmin

Cytologic diagnosis: Positive for Malignancy, favor post-radiation sarcoma

Follow-up Core Biopsy:

Histology:
  • Spindle cell neoplasm
  • Marked cytologic pleomorphism
  • Atypical polygonal cells
  • Atypical giant cells
Immunohistochemical Stains:

Some Positivity: Alpha-smooth muscle actin

Negative: MNF116, Keratin AE1-AE3, Keratin 34BE12, Keratin CAM 5.2, S-100 protein DAB, Desmin, Estrogen Receptor, Progesterone Receptor, P63, CD31

Histologic Diagnosis: Pleomorphic Undifferentiated Sarcoma, High Grade, favor Post Radiation Sarcoma

Discussion:
Post radiation sarcoma (PRS) is rare, occurring in less than 1% of all patients who are treated with radiation therapy and survive 5 years. Studies have noted a larger number of those patients with PRS have a previous history of receiving radiation therapy for breast cancer, lymphoma and Ewing sarcoma. A diagnosis of PRS is based on the following criteria: history of radiation therapy, development of a neoplasm within the irradiated area, a distinct histological difference from the primary tumor, and a latency period of more than 4 years. However, cases have reported latency periods ranging anywhere from 4-55 years.

Etiology:
Post radiation sarcoma can occur from low energy (orthovoltage) or high energy (megavoltage) radiation. Radiation doses range from 40-60 Gy. In cases of low energy radiation, the dosage of radiation is lower and the latency period is longer. PRS can also be attributed to a genetic predisposition for developing a second cancer.

Patients with Stewart-Treves syndrome (associated with lymphedema) have an increase risk of developing PRS. The risk of developing a PRS increases within 5 years after radiation therapy.

Treatment and Prognosis:
The best treatment option for PRS is wide or radical resection. Chemotherapy can be used, but typically has a poor response rate.

Post radiation sarcoma has a very poor prognosis due to the advanced stage or metastasis of the disease at diagnosis. Studies have reported 5 year survival rates ranging from 8.7-22%. Poor survival rates are due to the following: delay in diagnosis, large unresectable lesions, older patient age, anaplastic nature of lesion and lack of effective adjuvant treatment.

References:
  1. Rao, N. et al. Post Radiation Sarcoma. http://emedicine.medscape.com/article/1253714-overview
  2. Towlen, Lindsey. Post-Radiation Sarcoma Presenting in a Chest Wall Mass FNA. ASCT case presentation, ASCT annual meeting, April 2010.