JTCS Email Content Delivery
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Hans-Reinhard Zerkowski
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Zerkowski, H.-R.
Right arrow Articles by Knolle, J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Zerkowski, H.-R.
Right arrow Articles by Knolle, J.

J Thorac Cardiovasc Surg 1996;112:1122-1124
© 1996 Mosby, Inc.

BRIEF COMMUNICATIONS

PRIMARY SARCOMA OF PULMONARY ARTERY AND VALVE: MULTIMODALITY TREATMENT BY CHEMOTHERAPY AND HOMOGRAFT REPLACEMENT

Hans-Reinhard Zerkowski, MDa, Hans-Stefan Hofmann, MDa, Iwan Gybels, MDa, Jürgen Knolle, MDb


Halle, Germany

Received for publication Nov. 7, 1995 Accepted for publication Feb. 2, 1996. Primary pulmonary sarcomas are extremely rare tumors. Since the first description by MandelstammGo 1 in 1923, only about 100 case reports have been published, and only a few have dealt with combined treatment of primary sarcoma. We report on a woman with a metastasizing pulmonary sarcoma of the pulmonary valve with infiltration of the pulmonary artery. After complete remission of the pulmonary metastases with polychemotherapy, the patient underwent curative resection of the tumor and insertion of a cryopreserved homograft.

A 49-year-old woman was admitted because of increasing short of breath with exercise. She had pericarditis about 6 months previously. Since then, both chest and back pain occurred frequently, associated with tachycardia and dyspnea. Auscultation revealed a 3/6 systolic ejection murmur in the second left intercostal space and a markedly split second heart sound. There were signs of right ventricular overload on the electrocardiogram. The initial chest radiograph showed several bilateral pulmonary nodular lesions, confirmed by computed tomographic scan. Two-dimensional and transesophageal echocardiography revealed an enlarged right ventricle and an abnormal structure in the main and right pulmonary arteries. Right heart catheterization showed elevated right ventricular and pulmonary arterial pressures, with a ventricular–distal pulmonary artery gradient of 40 mmHg. The main and right pulmonary arteries were seen by cineangiography to be incompletely perfused. Needle biopsy was carried out for cytologic and histologic examination of the pulmonary nodular lesions, which we had suspected and later confirmed to be hemangiosarcoma. Elective resection of the right sided lung metastases was incomplete as a result of life-threatening hemodynamic instability. Histologic examination of one of these tumors revealed granulation and scar tissue without evidence of malignancy.

Because of progression of right heart failure and growth of the pulmonary lesions seen on the computed tomographic scan, chemotherapy was initiated. In the subsequent 7 months, polychemotherapy (etoposide, vincristine, ifosfamide, adriamycin) was administered for six cycles. Response was demonstrated after the second cycle. At the end of this treatment, magnetic resonance imaging revealed complete remission of all pulmonary nodules and reperfusion of both lungs; significant tumor remained in the right ventricular outflow tract and the pulmonary arteries (Fig. 1).



View larger version (175K):
[in this window]
[in a new window]
  		Fig. 1. Preoperative magnetic resonance scan shows a mass in the pulmonary artery.

 
Five months after chemotherapy, with cardiopulmonary bypass and hypothermia (28º C), complete resection of the tumor was performed. The tumor almost completely blocked the lumen of the pulmonary artery and invaded into the anterior wall of the pulmonary artery. In addition, the tumor originated from the pulmonary valve proximally, could not be separated from the anulus, and extended distally into both pulmonary arteries. The pulmonary artery was resected beyond the bifurcation, including the valve and parts of the anterior right ventricular outflow tract, and replaced with a cryopreserved valved bifurcation homograft (Bioimplant Services Foundation, Eurotransplant, Leiden, The Netherlands). The prosthetic valve was placed proximally in the previous plane of the pulmonary valve. The partially resected outflow tract of the ventricle was reconstructed with autologous pericardium. Tumor extending into the arteries was resected as in a pulmonary thromboendarterectomy. Histologic examination of the resected specimen showed no viable tumor tissue. After 17 days, the patient was discharged and was feeling well. At follow-up examination 12 months after the operation, the patient was alive and well without any evidence of recurrence.

Favorable prognosis of primary pulmonary sarcoma depends on early diagnosis. The symptoms are nonspecific; the cause for admission is often a suspicion of a pulmonary embolism. Sixty percentGo 2 of pulmonary sarcomas are diagnosed at postmortem examination, but this is happening with decreasing frequency as a result of newer therapy in the past decade. Currently, surgical resection remains the therapy of choice Go(Table I). The vast majority of the patients in reported cases (70%) were operated upon with the presumed diagnosis of thromboembolism. Simple resection of the tumor without prosthesis or graft substitution is the most frequent operation performed. Resection is often incomplete (ratio 1:2) because of the advanced stage of the tumor. Only a few cases of alloplasticGo Go 3,4 or homograftGo Go 5,6 replacement have been reported. Precise preoperative planning with echographic measurements of the pulmonary valve, pulmonary artery, and branch pulmonary arteries is desirable when doing elective replacement.


View this table:
[in this window]
[in a new window]
  		Table I. Review of patients treated surgically in the past 10 years
 
Adjuvant radiotherapy, chemotherapy, or a combination of bothGo Go 4,5 have so far been carried out only after tumor resection. To our knowledge, multimodal treatment with chemotherapy as pretreatment before operation, such as was used in our case, has not been reported. The prognosis is extremely poor. Only 10% of the patients survive longer than 1 year after diagnosis.Go 7 The mean survival of patients with primary resection (10 months) is significantly longer than that of patients without resection.Go 2 Adjuvant radiotherapy, chemotherapy, and combinations are not regarded as effective by some despite significantly longer survivalGo 2 because survival after the third postoperative year is similar to that of patients who have not received adjuvant treatment. GoTable I, however, shows that patients who undergo combined therapyGo Go 4,5 appear to have the longest survival. When multiple metastases are present, as in our case, chemotherapy after complete or partial remission may allow resection.Go 6

These data suggest that after a chemotherapeutic response extensive resection and reconstructive surgery is justified in patients with pulmonary sarcoma.

Footnotes

From the Department of Cardiothoracic Surgerya and Institute of Pathology,b Martin-Luther-University Halle-Wittenberg, Halle, Germany. Back

J THORAC CARDIOVASC SURG 1996;112:1122-4 Back

References

  1. Mandelstamm M. Über primäre Neubildungen des Herzens. Virchows Arch (Pathol Anat) 1923;245:43-57.
  2. Krüger I, Borowski A, Horst M, de Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg 1990;38:91-5.[Medline]
  3. Tanaka I, Masuda R, Inoue M, et al. Report of a case with complete resection and graft replacement, and review of 47 surgically treated cases reported in the literature. Thorac Cardiovasc Surg 1994;42:64-8.[Medline]
  4. Iversen S, Hake U, Schmiedt W, Jakob H, Ramp U, Gabbert H, et al. Resection of central primary pulmonary sarcoma. Eur J Cardiothorac Surg 1991;5:603-7.[Abstract]
  5. Head HD, Flam MS, John MJ, Lipnik SS, Slater DL, Stewart RD. Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy. Ann Thorac Surg 1992;53:332-4.[Abstract]
  6. Anderson MB, Kriett JM, Kapelanski DP, Tarazi R, Jamieson SW. Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg 1995;59:1478-90.
  7. Bleisch VR, Kraus FT. Polyploid sarcoma of the pulmonary trunk. Cancer 1980;46:314-24.[Medline]
  8. Smet de JM, Dieudonne P, Contu E, Le Clerk JL, Goffin Y, Boelpape de C. Pulmonary artery leiomyosarcoma treated surgically. Thorac Cardiovasc Surg 1986;34:98-9.[Medline]
  9. Nehrlich A, Permanetter W, Ludwig B, Remberger K. Primary leiomyosarcoma of the truncus pulmonalis. Path Res Pract 1990;186:296-9.
  10. Berney CR, Roche B, Kurt AM, Spiliopoulos A, Megevand R. Leiomyosarcoma of the pulmonary hilar vessels. Thorac Cardiovasc Surg 1992;40:48-51.[Medline]
  11. Johansson L, Carlen B. Sarcoma of the pulmonary artery: report of four cases with electron microscopic and immunhistochemical examinations, and review of the literature. Virchows Arch 1994;424:217-24.[Medline]
  12. Kawamura M, Takahasi Y, Sakata Y, et al. A case of malignant fibrous histiocytoma of the pulmonary artery. Nippon Kyobu Geka Gakkai Zasshi 1994;42:456-60.[Medline]



This article has been cited by other articles:


Home page
 Eur. J. Cardiothorac. Surg.Home page
H. K. Kim, Y. S. Choi, K. Kim, Y. M. Shim, K. Sung, Y. T. Lee, P. W. Park, and J. Kim
Surgical treatment for pulmonary artery sarcoma
Eur. J. Cardiothorac. Surg., April 1, 2008; 33(4): 712 - 716.
[Abstract] [Full Text] [PDF]

Home page
 The OncologistHome page
F. Mayer, H. Aebert, M. Rudert, A. Konigsrainer, M. Horger, L. Kanz, M. Bamberg, G. Ziemer, and J. T. Hartmann
Primary Malignant Sarcomas of the Heart and Great Vessels in Adult Patients A Single-Center Experience
Oncologist, September 1, 2007; 12(9): 1134 - 1142.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
M. Totaro, F. Miraldi, C. Ghiribelli, and C. Biscosi
Cardiac Angiosarcoma Arising From Pulmonary Artery: Endovascular Treatment
Ann. Thorac. Surg., October 1, 2004; 78(4): 1468 - 1470.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
H. Tanaka, S. Hasegawa, K. Egi, H. Tachou, F. Saitoh, and M. Sunamori
Successful radical resection of a leiomyosarcoma of the pulmonary trunk
J. Thorac. Cardiovasc. Surg., November 1, 2001; 122(5): 1039 - 1040.
[Full Text] [PDF]

Home page
 Asian Cardiovasc. Thorac. Ann.Home page
S. S. Hu, C. C. Sun, and Z. Zheng
Heart Leiomyosarcoma Mimicking Pulmonary Thromboembolism
Asian Cardiovasc Thorac Ann, March 1, 2001; 9(1): 68 - 70.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
E. Mayer, J. Kriegsmann, A. Gaumann, H. U. Kauczor, M. Dahm, U. Hake, F. X. Schmid, and H. Oelert
Surgical treatment of pulmonary artery sarcoma
J. Thorac. Cardiovasc. Surg., January 1, 2001; 121(1): 0077 - 82.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
T. Kono, T. Takemura, I. Hagino, and G. Matsumura
Complete resection of cardiac leiomyosarcoma extending into the pulmonary trunk and right pulmonary artery
Ann. Thorac. Surg., October 1, 2000; 70(4): 1412 - 1414.
[Abstract] [Full Text] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
T. Shimono, M. Takao, T. Shiraishi, and I. Yada
Reply
J. Thorac. Cardiovasc. Surg., October 1, 1998; 116(4): 666 - 667.
[Full Text]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
T. Shimono, H. Yuasa, U. Yuasa, F. Yasuda, K. Adachi, T. Tokui, M. Takao, S. Namikawa, and I. Yada
Pulmonary leiomyosarcoma extending into left atrium or pulmonarytrunk: complete resection with cardiopulmonary bypass
J. Thorac. Cardiovasc. Surg., February 1, 1998; 115(2): 460 - 461.
[Full Text] [PDF]

This Article
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Hans-Reinhard Zerkowski
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Zerkowski, H.-R.
Right arrow Articles by Knolle, J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Zerkowski, H.-R.
Right arrow Articles by Knolle, J.

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS