Report of eight recent cases of locally advanced primary pulmonary artery sarcomas: failure of Doxorubicin-based chemotherapy.
BACKGROUND
Case reports of primary pulmonary artery sarcomas are very rare.


METHODS
We described herein eight new cases diagnosed between December 2000 and December 2004.


RESULTS
There were four men and four women, with median age of 52 years. Presenting symptoms mimicked pulmonary emboli in all cases. There were six "intimal sarcomas" and two leiomyosarcomas. In six cases, we observed initial metastasis in lung (six cases), in bone (two cases), and in brain (two cases), and adrenal gland (one case). The palliative treatments included surgical desobstruction (six cases), conformational radiotherapy (four cases), and chemotherapy (seven cases). Doxorubicin-based regimen failed in seven cases. All patients died (median survival: 8 months, extremes 5-20 months).


CONCLUSIONS
Those eight cases illustrate the high incidence of initial metastasis and the very poor outcome of primary pulmonary artery sarcomas despite classic doxorubicin-based chemotherapy.
Penel
Nicolas
N
Département de Cancérologie Générale, Oscar Lambret Cancer Centre, Lille, France. n-penel@o-lambret.fr
Taieb
Sophie
S
Ceugnart
Luc
L
Dansin
Eric
E
Hoguet
Dominique
D
Vanseymortier
Luc
L
Lartigau
Eric
E
D000310:Adrenal Gland Neoplasms / Q000188:drug therapy / Q000556:secondary; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D001859:Bone Neoplasms / Q000188:drug therapy / Q000556:secondary; D001932:Brain Neoplasms / Q000188:drug therapy / Q000556:secondary; D004317:Doxorubicin / Q000627:therapeutic use*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007890:Leiomyosarcoma / Q000188:drug therapy* / Q000473:pathology; D008175:Lung Neoplasms / Q000188:drug therapy / Q000556:secondary; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011651:Pulmonary Artery / Q000187:drug effects / Q000473:pathology*; D012509:Sarcoma / Q000188:drug therapy* / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000473:pathology; D015996:Survival Rate; D014057:Tomography, X-Ray Computed; D017211:Treatment Failure; D019043:Vascular Neoplasms / Q000188:drug therapy* / Q000473:pathology
D000903:Antibiotics, Antineoplastic; D004317:Doxorubicin