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Intimal Sarcoma of the Thoracic Aorta: a Case Report
Eur J Vasc Endovasc Surg 18, 181–182 (1999) Article No. ejvs.1999.0888
CASE REPORT
Intimal Sarcoma of the Thoracic Aorta: a Case Report J. P. Le Rochais1, P. Icard1, O. Coffin1, F. Galateau2, M. Khalil1 and D. Maiza∗1 1
Service de Chirurgie Thoracique et Cardiovasculaire, and 2Service d’Anatomopathologie, CHRU de Caen, France
Introduction Sarcomas of the aorta are extremely rare, most of the cases being discovered at postmortem autopsy.1–5 We report a case that presented with bilateral peripheral tumour emboli.
Case Report A 78-year-old female patient without any significant past medical history underwent a bilateral embolectomy of both femoral arteries because of sudden bilateral lower limb ischaemia. The removed material was unusually white, friable, with ann ‘‘encephaloı¨d’’ aspect, and microscopic examination revealed sarcoma cells. Physical examination was normal. There were no intracardiac abnormalities on transoesophageal echocardiography. CT scan revealed an intraluminal lesion, approximately 2.5 cm in length, located at the left posterior wall of the descending thoracic aorta (Fig. 1). No other sources of embolism were discovered and a diagnosis of an intraluminal sarcoma with peripheral tumour embolisation was made. Through a left thoracotomy, the diseased aorta was resected and reconstructed with a 4-cm long prosthetic graft. The limits of resection were free of tumour on frozen sections. Histological examination showed an intraluminal aortic sarcoma, sessile and polypoid, that measured 3 cm×2 cm, reducing the cross-section of the aorta (Fig. 2). The media was normal, and a necrotic polypoid intraluminal lesion surrounded by a rim of large neoplastic cells arose from the intima. These cells ∗ Please address all correspondence to: D. Maiza, Service de Chirurgie Thoracique et Cardiovasculaire, CHU Coˆte de Nacre, 14033 Caen Cedex, France.
were positive to vimentin and to CD 31, negative to CD 34. Less than 10% of cells were positively stained with KL1, whereas all other immunohistochemical markers (EMA, CLA, PS 100, HMB 45, AFP, HCG, ACE, CD15) were negative. These various elements were consistent with a haemangioendotheliosarcoma. Postoperatively a CT scan was performed because of abdominal distention and this showed occlusion of the mesenteric superior artery. An open embolectomy was successfully performed and intestinal resection was not necessary. Microscopy showed a similar picture to the aortic primary sarcoma. Postoperative recovery was thereafter uneventful. No complementary treatment was administered. Five months later, the patient died of profound cachexia, with clinical evidence of generalised metastases. No autopsy was performed.
Discussion Since the initial report made by Brodowski in 1873,1 Seelig et al.2 have reported 87 cases of aortic sarcomas, almost 60% of them only discovered at autopsy. Wright et al.3 distinguished two types of aortic sarcomas, intimal and mural forms. Intraluminal sarcomas give signs of peripheral emboli as in our patient, and/ or vascular signs of aortic obstruction. Because of embolisation, distant metastases may ocur early in various sites throughout the body. Intimal sarcoma may also grow along the lumen, obstructing all branches of the aorta.2–4 Mural aortic sarcoma originates from the media or from the adventitia,3,4 and has a tendency to extend extramurally, involving paraaortic tissues and lymph nodes. It has a lower risk of producing emboli and early distant metastases. As in
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J. P. Le Rochais et al.
Fig. 2. Macroscopic aspect of the intraluminal aortic sarcoma.
with adherent thrombus. With various immunohistochemical stains, it is often possible to define the subtype of sarcoma, a F VIII positive presentation suggesting an endothelial origin.3–5 Aortic sarcomas generally have a poor prognosis. The 1-year survival rate has been reported as only 13%,3,4 and Seelig et al.2 calculated that the median survival of patients was 7 months (0.25 to 168 months); 12 months for mural forms and 6 months for intimal forms. References
Fig. 1. CT scan with vertical reconstruction showing the intraluminal thoracic aortic tumour.
our patient, diagnosis of aortic sarcomas can sometimes be made before autopsy. Transoesophageal echography, CT scan or MRI scanning may localise an intraluminal defect, aneurysmal disease or para-aortic mass, but an intraluminal aortic tumour may be mistaken for an intraluminal atheroslerotic plaque
Eur J Vasc Endovasc Surg Vol 18, August 1999
1 Brodowski W. Prima¨res Sarkom der Aorta thoracica mit Verbreitung des Neugebildes in der unteren Korpehalfte. Jahresb Leistung Fortschr Ges Med 1873; 8: 243–246. 2 Burke A, Virmani R. Sarcomas of the great vessels. Cancer 1993; 71: 1761–1773. 3 Seelig MH, Kingler PJ, Oldenburg WA, Blackshear JL. Angiosarcoma of the aorta: report of a case and a review of the literature. J Vasc Surg 1998; 28: 732–737. 4 Wright EP, Glick AD, Virmani R, Page DL. Aortic intimal sarcoma with embolic metastases. Am J Surg Pathol 1985; 9: 890–897. 5 Haber LM, Truong L. Immunohistochemical demonstration of the endothelial nature of aortic intimal sarcoma. Am J Surg Pathol 1988; 12: 798–802. Accepted 26 March 1999
CASE REPORT
Intimal Sarcoma of the Thoracic Aorta: a Case Report J. P. Le Rochais1, P. Icard1, O. Coffin1, F. Galateau2, M. Khalil1 and D. Maiza∗1 1
Service de Chirurgie Thoracique et Cardiovasculaire, and 2Service d’Anatomopathologie, CHRU de Caen, France
Introduction Sarcomas of the aorta are extremely rare, most of the cases being discovered at postmortem autopsy.1–5 We report a case that presented with bilateral peripheral tumour emboli.
Case Report A 78-year-old female patient without any significant past medical history underwent a bilateral embolectomy of both femoral arteries because of sudden bilateral lower limb ischaemia. The removed material was unusually white, friable, with ann ‘‘encephaloı¨d’’ aspect, and microscopic examination revealed sarcoma cells. Physical examination was normal. There were no intracardiac abnormalities on transoesophageal echocardiography. CT scan revealed an intraluminal lesion, approximately 2.5 cm in length, located at the left posterior wall of the descending thoracic aorta (Fig. 1). No other sources of embolism were discovered and a diagnosis of an intraluminal sarcoma with peripheral tumour embolisation was made. Through a left thoracotomy, the diseased aorta was resected and reconstructed with a 4-cm long prosthetic graft. The limits of resection were free of tumour on frozen sections. Histological examination showed an intraluminal aortic sarcoma, sessile and polypoid, that measured 3 cm×2 cm, reducing the cross-section of the aorta (Fig. 2). The media was normal, and a necrotic polypoid intraluminal lesion surrounded by a rim of large neoplastic cells arose from the intima. These cells ∗ Please address all correspondence to: D. Maiza, Service de Chirurgie Thoracique et Cardiovasculaire, CHU Coˆte de Nacre, 14033 Caen Cedex, France.
were positive to vimentin and to CD 31, negative to CD 34. Less than 10% of cells were positively stained with KL1, whereas all other immunohistochemical markers (EMA, CLA, PS 100, HMB 45, AFP, HCG, ACE, CD15) were negative. These various elements were consistent with a haemangioendotheliosarcoma. Postoperatively a CT scan was performed because of abdominal distention and this showed occlusion of the mesenteric superior artery. An open embolectomy was successfully performed and intestinal resection was not necessary. Microscopy showed a similar picture to the aortic primary sarcoma. Postoperative recovery was thereafter uneventful. No complementary treatment was administered. Five months later, the patient died of profound cachexia, with clinical evidence of generalised metastases. No autopsy was performed.
Discussion Since the initial report made by Brodowski in 1873,1 Seelig et al.2 have reported 87 cases of aortic sarcomas, almost 60% of them only discovered at autopsy. Wright et al.3 distinguished two types of aortic sarcomas, intimal and mural forms. Intraluminal sarcomas give signs of peripheral emboli as in our patient, and/ or vascular signs of aortic obstruction. Because of embolisation, distant metastases may ocur early in various sites throughout the body. Intimal sarcoma may also grow along the lumen, obstructing all branches of the aorta.2–4 Mural aortic sarcoma originates from the media or from the adventitia,3,4 and has a tendency to extend extramurally, involving paraaortic tissues and lymph nodes. It has a lower risk of producing emboli and early distant metastases. As in
1078–5884/99/080181+02 $12.00/0 1999 W.B. Saunders Company Ltd.
182
J. P. Le Rochais et al.
Fig. 2. Macroscopic aspect of the intraluminal aortic sarcoma.
with adherent thrombus. With various immunohistochemical stains, it is often possible to define the subtype of sarcoma, a F VIII positive presentation suggesting an endothelial origin.3–5 Aortic sarcomas generally have a poor prognosis. The 1-year survival rate has been reported as only 13%,3,4 and Seelig et al.2 calculated that the median survival of patients was 7 months (0.25 to 168 months); 12 months for mural forms and 6 months for intimal forms. References
Fig. 1. CT scan with vertical reconstruction showing the intraluminal thoracic aortic tumour.
our patient, diagnosis of aortic sarcomas can sometimes be made before autopsy. Transoesophageal echography, CT scan or MRI scanning may localise an intraluminal defect, aneurysmal disease or para-aortic mass, but an intraluminal aortic tumour may be mistaken for an intraluminal atheroslerotic plaque
Eur J Vasc Endovasc Surg Vol 18, August 1999
1 Brodowski W. Prima¨res Sarkom der Aorta thoracica mit Verbreitung des Neugebildes in der unteren Korpehalfte. Jahresb Leistung Fortschr Ges Med 1873; 8: 243–246. 2 Burke A, Virmani R. Sarcomas of the great vessels. Cancer 1993; 71: 1761–1773. 3 Seelig MH, Kingler PJ, Oldenburg WA, Blackshear JL. Angiosarcoma of the aorta: report of a case and a review of the literature. J Vasc Surg 1998; 28: 732–737. 4 Wright EP, Glick AD, Virmani R, Page DL. Aortic intimal sarcoma with embolic metastases. Am J Surg Pathol 1985; 9: 890–897. 5 Haber LM, Truong L. Immunohistochemical demonstration of the endothelial nature of aortic intimal sarcoma. Am J Surg Pathol 1988; 12: 798–802. Accepted 26 March 1999