[76] Radiation-Associated Sarcoma of Soft Tissue and Bone: A Clinicopathologic Study of 70 Cases in a Single Institution

EA Morgan, DE Kozono, JE Butrynski, EH Baldini, CP Raut, AF Nascimento. Brigham and Women's Hospital (BWH), Boston, MA; BWH and Dana-Farber Cancer Institute (DFCI), Boston; DFCI, Boston; BWH, Boston

Background: Development of sarcomas is a rare but serious complication of radiation therapy (RT). Cutaneous angiosarcoma of the breast following radiation for conservative treatment of breast carcinoma is the best described radiation-associated sarcoma (RAS). Sarcomas arising in other sites are poorly characterized.
Design: We reviewed all cases of RAS evaluated at our institution from 1987 to 2008. RT-associated breast angiosarcomas were excluded. Clinicopathologic data were obtained by medical record review. Survival was analyzed by Kaplan-Meier curve.
Results: 70 patients (pts) (51 female, 19 male) were identified, with a mean age at RAS diagnosis of 53 yrs. Primary diseases were invasive breast cancer (18 cases), Hodgkin lymphoma (14), cervical cancer (6), non-small cell lung cancer (3), endometrial cancer (3), head and neck squamous cell cancer (3), DCIS (2), Ewing's sarcoma (2), embryonal rhabdomyosarcoma (2), seminoma (2), non-Hodgkin lymphoma (2), rectal cancer (2), thyroid cancer, prostate cancer, Wilms' tumor, CML, retinoblastoma, endometriosis and desmoid tumor (1 each). Four pts received RT to the same site for two distinct primaries greater than a year apart. Median interval between RT and RAS was 168 mos (range 36 to 552). In 60% of cases, pts noticed symptoms at a median of 3 mos before diagnosis. RAS diagnoses were leiomyosarcoma (10 cases), MPNST (6), osteosarcoma (6), angiosarcoma (2), and unclassified sarcoma (46). The majority of the tumors (68%) were histologically high grade. Cytogenetic analysis (18 cases) demonstrated complex karyotypes without recurrent abnormalities. Surgical resection was performed in 56 cases followed by chemotherapy and/or RT in 21 cases. 21% of pts developed recurrence and 17% developed metastasis (median 8 and 22 mos after RAS, respectively). 49% of pts died of disease and the 5-year survival was 38%.
Conclusions: Most RAS are unclassified, high grade sarcomas. The majority have complex karyotypic abnormalities and sarcomas with recurrent translocations do not typically arise in the setting of RT. The cornerstone of therapy is surgery. Although the median latency period is quite long from initial RT administration, prognosis is poor with a 5-year survival of 38%.
Category: Bone & Soft Tissue

Monday, March 9, 2009 9:30 AM

Poster Session I Stowell-Orbison/Autopsy Award # 17, Monday Morning

 

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