Pulmonary artery sarcoma: a clinicopathologic and immunohistochemical study of 12 cases.
We report 12 cases of pulmonary artery sarcoma. The mean age at diagnosis was 48.4 years. Based on histomorphologic features and immunohistochemical findings, 2 tumors were classified as rhabdomyosarcoma, 4 as leiomyosarcoma, 1 as osteogenic sarcoma, 1 as angiosarcoma, and 4 as high-grade sarcoma. All patients underwent surgery. In addition, 7 patients received neoadjuvant or adjuvant therapy. Five patients died 3 to 23 months after surgery. Three patients were still alive at 8, 27, and 68 months at last follow-up. Another 3 patients were alive at 2, 15, and 40 months and then lost to follow-up. The 2 patients with the longest survival (40 months and 68 months) had a diagnosis of leiomyosarcoma. Both patients with rhabdomyosarcoma died at 3 months after surgery. Pulmonary artery sarcoma is an uncommon entity with a poor prognosis. The role of early diagnosis, histologic classification, surgical treatment, and adjuvant therapy in patient outcome is discussed.
Huo
Lei
L
Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston 77030-4009, USA.
Moran
Cesar A
CA
Fuller
Gregory N
GN
Gladish
Gregory
G
Suster
Saul
S
D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor; D003131:Combined Modality Therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000473:pathology*; D011859:Radiography; D012509:Sarcoma / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D019043:Vascular Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy
D014408:Biomarkers, Tumor