Germ Cell Tumors with Associated Vascular Neoplasia: Report of 14 Cases
NP Agaram, TM Ulbright. Indiana University-Perdue University Indianapolis, Indianapolis, IN
Background: Germ cell tumors rarely develop sarcomas, most commonly rhabdomyosarcoma. Vascular neoplasia in a germ cell tumor is rare and may range from relatively benign appearing proliferations to frank angiosarcomas. In reviewing our experience, we attempt to define the spectrum of vascular neoplasia arising in germ cell tumors.
Design: We searched our pathology information system for germ cell tumors with associated vascular lesions. In addition, personal consultation files of one of us were searched for similar cases. The slides were reviewed to confirm the diagnosis and the medical records were reviewed for additional follow up information.
Results: Seventeen cases from 14 patients were identified from 2000 -2008. All were males and the age range was 24 46 years, with a median of 29 years. Eleven (79%) were primary mediastinal germ cell tumors and 3 (21%) were primary testicular germ cell tumors. After review, 7 cases (41%) were diagnosed as angiosarcoma, 9 (64%) as atypical vascular proliferation and 1 (5%) as prominent vascular proliferation. Angiosarcoma was the only sarcomatous differentiation diagnosed in all cases except for 1 case that had other sarcomatous elements. Histologically, many cases demonstrated a myxoid angioblastic mesenchyme wherein neoplastic endothelium appeared to derive from atypical stromal cells and gradually coalesced into recognizable vessels. Follow up from 1 month to 5 years was available in 12 patients. 8/12 (67%) developed metastasis, confirmed as angiosarcoma in 3cases (all of which had an initial diagnosis of atypical vascular proliferation). Two patients with died of postoperative complications and 2 patients are disease free.
Conclusions: Vascular neoplasia in germ cell tumors occurs predominantly in the mediastinum. They typically range from dysplastic-appearing vessels in myxomatous stroma to angiosarcoma. Germ cell tumors with angiosarcoma have increased rates of metastases and a poor prognosis.
Monday, March 9, 2009 1:00 PM
Poster Session II # 217, Monday Afternoon