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doi:10.1510/icvts.2009.226753

Interactive CardioVascular and Thoracic Surgery 10 (2010) 462–464

� 2010 Published by European Association for Cardio-Thoracic Surgery

Case report - Vascular thoracic

The rupture of descending thoracic aorta due to the necrosisof aortic intimal sarcoma

Mutsuo Tanaka*, Minoru Tabata, Tomoki Shimokawa, Shuichiro Takanashi

Department of Cardiovascular Surgery, Sakakibara Heart Institute, 3-16-1 Asahi-cho, Fuchu-si, Tokyo 183-0003, Japan

Received 26 October 2009; received in revised form 27 November 2009; accepted 2 December 2009

Abstract

Aortic intimal sarcoma is rare and the prognosis is very poor. We experienced a case of ruptured aortic intimal sarcoma in the descendingaorta. A 69-year-old man underwent an emergency operation for the rupture of descending aorta. The postoperative course was uneventful.The histological examination of aortic wall showed aortic intimal sarcoma. The patient developed a local recurrence and abdominaldissemination of the tumor three months after surgery. We report the case and discuss about the diagnosis and treatment of thoracic aorticintimal sarcoma.� 2010 Published by European Association for Cardio-Thoracic Surgery. All rights reserved.

Keywords: Aortic diseases; Vascular neoplasms; Aortic rupture

1. Introduction

Primary malignant tumor of the aorta (PMTA) is rarelyseen and the prognosis is poor. The most common growthpattern of PMTA is intimal type, which often forms intra-luminal polyps and develops arterial embolism. However,aortic rupture due to PMTA is extremely rare. We report arupture case of PMTA and discuss about the diagnosis andtreatment of this rare disease.

2. Case history

A 69-year-old man visited a local hospital with two epi-sodes of sharp back pain. His vital signs were stable.However, chest X-ray showed a right pleural effusion, andchest computed tomography (CT) revealed that thedescending aorta ruptured into the right pleural cavity. Afusiform aneurysm with a diameter of 50 mm was found atthe level of the 9th thoracic vertebrae (Fig. 1).

The patient was transferred to our hospital for emergencysurgery. In the operating room, a chest tube was placed inthe right pleural space, and 1400 ml of bloody fluid wasdrained. Then, the patient was positioned in the rightlateral decubitus position. A left thoracotomy was madethrough the 5th intercostal space. A large hematoma wasfound around the descending aorta. The left femoral arteryand left upper pulmonary vein were cannulated for leftheart bypass. The descending aorta was clamped proximallyand distally to the aneurysm, and the aneurysm was

*Corresponding author. Tel.: q8142-314-3111; fax: q8142-314-3199.E-mail address: mutsuoman620@gmail.com (M. Tanaka).

opened. The atherosclerotic finding inside the aneurysmwas minimal. Instead, there was an intimal erosion (about1=1 cm) on the right side of the aorta at the level of the9th thoracic vertebrae, which was suspected as a rupturesite. The aneurysm was resected and reconstructed with awoven polyester straight graft (Hemashield; Boston Scien-tific Corp, Natick, Mass; sizes24 mm). Proximal and distalanastomoses were performed in an end-to-end fashion.

Histological examinations of the aneurysm wall showedmalignant tumor cells in all layers of the aortic wall. Thetumor growth was most evident in the adventitia (Fig. 2).Tumor cells were seen on the surgical margins of bothproximal and distal sides. Immunohistochemical stainingshowed a positive finding for smooth muscle actin andvimentin, and negative finding for smooth muscle myosin,factor VIII, CD31, CD34, D2-40, keratin and symaptophysin.The proliferation marker MIB-1 (Ki-67) stained about 20%of the malignant cells. These findings were compatible withsarcoma of the aortic intima.

The postoperative course was uneventful. We decided notto perform an additional surgical resection considering poorprognosis of the tumor. The patient was discharged home.However, he was re-admitted to our hospital three monthsafter, complaining of appetite loss and fever. The tumormass was palpable on the edge of operative skin incisionsite and a CT showed local recurrence and disseminationin the abdominal cavity. No further treatment was per-formed for this end-stage malignancy. He died four monthsafter surgery.

3. Discussion

PMTA is rarely seen. The maleyfemale ratio is 9:5, andthe mean age is 59.5 years w1x. The favorite site of PMTA is

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Fig. 1. CT showed the rupture of descending aorta. The rupture site wassuspected at the height of the 9th thoracic vertebrae (arrow).

Fig. 2. Hematoxylin and eosin stain of the aortic wall showed that the growthof tumor cells containing tumor necrosis (arrow) and tumor cells extendalong the tunica media (arrow head). (a) Low magnification (=40), (b) highmagnification (=100).

the thoracic descending aorta w1x. PMTA is categorized bynot only histological pattern but also growth pattern. Salm w2xcategorized aortic tumor into three growth pattern; inti-mal, polypoidal (intraluminal), and adventitial (or mural).The intimal and polypoidal types may have a tendency toextend along the intimal surface and frequently formintraluminal polyps, which may cause embolism. The adven-titial type primarily involves media andyor adventitia. Mostof the reported PMTA are the intimal type. In our case,although the tumor was mainly found in the media andadventitia, tumor cells extended along the intimal surfacewith thrombus formation. The histopathological diagnosiswas intimal sarcoma. Some intimal sarcomas have beenreported to have myofibroblastic differential growth pat-tern w3, 4x, which was also seen in our case.

The prognosis of PMTA is poor, and survivals after diagnosisrange from 8 to 14 months w1x. Intimal type sarcoma hasthe most aggressive behavior, leading to death withinmonths w3x. Importantly, PMTA is barely suspected or diag-nosed at the early stage because the symptoms are non-specific and PMTA is a very rare disease. In most cases,PMTA is diagnosed by histological examination followingsurgery for arterial embolism, aneurysm or rupture w1x.Rupture cases are less frequently seen than embolismcases. Cantena and colleagues reported a rupture case w5x.They suspected a PMTA intraoperatively and performedsegmental resection of the descending aorta and a wedgelung resection. There was no recurrence or metastasisduring a 21-month follow-up. Clear surgical margins areimportant for survival after surgical resection of PMTA. Wecould not achieve clear surgical margins since we did notsuspect PMTA when we saw an erosive change inside theaneurysm. Although preoperative diagnosis is not easy onimaging studies, a PMTA should be suspected and an extend-ed resection might be necessary when an atypical lesion orerosion is found inside the aorta during surgery.

References

w1x Akiyama K, Nakata K, Negishi N, Henmi A. Intimal sarcoma of thethoracic aorta; clinical-course and autopsy finding. Ann Thorac Carido-vasc Surg 2005;11:135–138.

w2x Salm R. Primary fibrosarcoma of aorta. Cancer 1972;29:73–83.w3x Burke AP, Virmani R. Sarcoma of the great vessels: a clinicopathologic

study. Cancer 1993;71:1761–1773.w4x Thalheimer A, Fein M, Geissinger E, Franke S. Intimal angiosarcoma of

the aorta: report of a case and review of the literature. J Vasc Surg2004;40:548–553.

w5x Catena F, Bianchi R, Ansaloni L, Pinna AD. Hemothorax caused byrupture of a primitive thoracic leiomyosarcoma of the thoracic aorta:description of a case and literature review. J Thorac Cardiovasc Surg2008;135:688–689.

eComment: Sarcomas of the great vessels. Is there a role forchemotherapy?

Authors: Nikolaos Barbetakis, Department of Thoracic Surgery, Theagen-io Cancer Hospital, A. Simeonidi 2, 54007 Thessaloniki, Greece; ChristosAsteriou, Fani I. Papadopoulou, Eleni Stergiou

doi:10.1510/icvts.2009.226753AWe read with great interest the article by Tanaka et al. regarding a case

of aortic intimal sarcoma w1x. We would like to highlight the role of adjuvantchemotherapy in a setting of multimodality treatment.

Sarcomas of the great vessels previously diagnosed during surgery orautopsy are rare and highly lethal. This represents predominantely femalepatients between the age of 22 and 81. The prognosis is poor with a meansurvival of 12 months after onset of symptoms and one and two yearssurvival rates of 22% and 7%, respectively w2x.

The mainstay of treatment for sarcoma is surgical resection as this remainsthe only potentially curative modality. Adjuvant chemotherapy and radiationcan be considered following surgical excision although their role remainsundefined. An ;20% response rate can be expected with a combinationchemotherapy regimen involving an anthracycline and an alkylating agent;however, the value of this regimen in the adjuvant setting for intimal aorticsarcoma is unclear w3x.

According to the literature, there are two cases with satisfactory survival.The first patient underwent resection followed by adjuvant chemotherapyw4x. The second one was found inoperable during surgery and underwent atwo-drug combination chemotherapy consisting of ifosfamide and epirubicinwith long-term survival w5x.

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In our institute, we had a similar unresectable case of primary pulmonaryartery sarcoma. The patient underwent the same chemotherapeutic schemeand is still alive after six months, with a marked regression of the tumor. Inconclusion, we think that intensive chemotherapy is worth trying in unre-sectable patients.

References

w1x Tanaka M, Tabata M, Shimokawa T, Takanashi S. The rupture of descend-ing thoracic aorta due to the necrosis of aortic intimal sarcoma. InteractCardioVasc Thorac Surg 2010;10:462–464.

w2x Govender D, Pillay SV. Right pulmonary artery sarcoma. Pathology2001;33:243–245.

w3x Keohan ML, Taub RN. Chemotherapy for advanced sarcoma: therapeuticdecisions and modalities. Semin Oncol 1997;24:572–579.

w4x Shuster T, Dall’Olmo C, Spadone D, Silver D. Abdominal aortic sarcoma:report of a case with long-term survival and review of the literature.Ann Vasc Surg 2002;16:545–549.

w5x Uchida A, Tabata M, Kiura K, Tanimoto Y, Kanehiro A, Aoe M, OhoharaN, Ueoka H, Tanimoto M. Successful treatment of pulmonary arterysarcoma by a two-drug combination chemotherapy consisting of ifos-famide and epirubicin. Jpn J Clin Oncol 2005;35:417–419.

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