Hua Li, MD, PhD, Deming Xu, MD, Zhiqiang Chen, MD, Wenjun Ding, MD, Tao Hong, MD, PhD, Hao Chen, MD, Mengping Shao, MD, PhD, Hao Lai, MD, PhD, Yingyong Hou, MD, PhD, and Chunsheng Wang, MD, PhD Departments of Cardiac Surgery and Pathology, Zhongshan Hospital, Fudan University, Shanghai, China

Background. Primary cardiac sarcomas are rare diseases with a poor prognosis. This study aims to provide a prognostic analysis after different levels of resections of cardiac sarcomas. Methods. Twenty-nine patients undergoing resections of primary cardiac sarcomas at the Zhongshan Hospital from September 1995 to July 2012 were retrospectively reviewed. Results. There were 15 women and 14 men. The mean age was 41.0 years. The most common histologic type was angiosarcoma (28%). The median survival for the entire cohort was 17 months (range, 5 to 216 months). Patients with microscopically negative margin (R0) resections had a better median survival than those with microscopically positive margin (R1) resections (58 months versus 11 months; p < 0.001). The median survival after an R1 resection was not different from that after a partial resection (12 months; p [ 0.81). The median local recurrence-free survival after an R0

resection was longer than that after an R1 resection (36 months versus 6 months; p < 0.001). Five patients who underwent R0 resections and repeated resections of local recurrences or metastases had the longest median survival of 72 months. None of the patients with R0 resections received adjuvant therapy. Multimodality treatment after R1 and partial resections slightly increased the survival. Conclusions. For nonmetastatic and localized primary cardiac sarcoma, an R0 surgical resection of cardiac sarcomas should be performed. Aggressive surgical treatment or radiation therapy for local recurrence or metastasis prolongs the survival. Multimodality treatment is recommended after incomplete resections of cardiac sarcomas. The role of adjuvant chemotherapy after R0 resections is unclear.

P

Some reports ranked the microscopically positive margin (R1) resections as complete procedures [6, 7], but some reports only accepted the microscopically negative margin (R0) resections as complete procedures [5, 8, 9]. The difference between the overall survival times after R0 or R1 resections of cardiac primary sarcoma has not been previously discussed. This article reports a survey of the survival differences after various levels of resections and discusses the role of repeated surgical interventions, chemotherapy, and radiation therapy in the treatment of nonmetastatic and localized primary cardiac sarcoma.

rimary tumors of the heart and great vessels are rare diseases, and their prevalence in autopsies is approximately 0.02% [1]. The majority of primary cardiac tumors are benign, and only 20% of primary cardiac tumors represent various types of sarcomas [2]. Medical therapy alone cannot prevent the dismal outcomes of primary cardiac sarcomas, and the postoperational prognoses of these types of sarcomas are much poorer compared with extracardiac sarcomas [3]. There have been only sporadical long-term (>10 years) survival cases reported [4, 5]. There is consensus by almost all studies that the surgical resection of cardiac sarcoma with curative intention is the foundation of a better prognosis. The roles of adjuvant chemotherapy and radiation therapy remain controversial. Neoadjuvant therapy was thought to be effective for obtaining negative microscopic margins, but there is no direct evidence of this fact [6].

Accepted for publication Dec 9, 2013. Address correspondence to Dr Wang, Department of Cardiac Surgery, Zhongshan Hospital, Fudan University, No. 180 Fenglin Rd, Shanghai, China 200032; e-mail: [email protected].

Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier Inc

(Ann Thorac Surg 2014;97:1379–86) Ó 2014 by The Society of Thoracic Surgeons

Patients and Methods This study was approved by the ethics committees of Zhongshan Hospital, affiliated with Fudan University. The pathology database of the hospital was searched from September 1995 to July 2012, and 33 patients who had previously undergone surgical treatments of primary cardiac sarcomas were identified. Of the 33 total patients, 29 underwent different levels of surgical resections of nonmetastatic localized cardiac sarcomas and were identified to be the study subjects. Three patients who underwent only biopsy owing to wide encroachment and 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.12.030

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Prognostic Analysis for Survival After Resections of Localized Primary Cardiac Sarcomas: A Single-Institution Experience

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1 patient with synchronous lung metastases who received partial resection of a right atrial sarcoma and only biopsy of the lung were excluded from the study. The levels of surgical resections were classified as R0, R1, and partial resection with macroscopic residual (R2). An endocardial resection of a sarcoma misdiagnosed as left atrial (LA) myxoma was classified as an R2 resection. Overall survival times were calculated from the date of the first operation to the date of death or to the present date if the patient was still alive. Local recurrence-free survivals were calculated from the date of operation to the date of radiologic or echocardiographic evidence of a local relapse. All statistical analyses for baseline characteristics were reported as means or as percentages. Survival was estimated using the Kaplan-Meier method, and curves were compared using a log-rank test. All data were calculated using SPSS software (SPSS Inc, Chicago, IL).

Results

Ann Thorac Surg 2014;97:1379–86

Table 1. Histologic Types and Locations Variable Histology Angiosarcoma Undifferentiated pleomorphic sarcoma Synovial sarcoma Intima sarcoma Leiomyosarcoma Myxosarcoma Rhabdomyosarcoma Malignant peripheral nerve sheath tumor Location Left atrium Right atrium Pulmonary artery Right ventricle Left ventricle Superior vena cava

(n ¼ 29) 8 6 5 4 2 2 1 1

(28%) (21%) (17%) (14%) (7%) (7%) (3%) (3%)

12 10 4 1 1 1

(41%) (34%) (14%) (3%) (3%) (3%)

Patients and Presenting Symptoms Between September 1995 and July 2012, 29 patients underwent resections of nonmetastatic and localized primary cardiac sarcomas. The mean age at presentation was 41 years (range, 14 to 66 years). Fifteen patients were men, and 14 were women. The presenting symptoms included dyspnea in 21 patients (72%), cough in 10 (34%), fever or weight loss in 9 (31%), arrhythmias in 6 (21%), chest pain in 4 (14%), syncope in 5 (17%), symptomatic pericardial effusion in 6 (21%), and New York Heart Association functional class III or IV in 16 (55%). The mean duration of symptoms at the time of operation was 1.5 months.

Histology and Location The most common histologic type was angiosarcoma (28%), which was followed by undifferentiated pleomorphic sarcoma (21%). The most common locations of cardiac sarcomas were LA (42%), which was followed by right atrium (34%; Table 1).

Surgery None of the patients had evidence of metastatic disease at the time of initial diagnosis. Thirty-four cardiac surgical procedures were performed in the 29 patients. The surgical details are outlined in Table 2. R0 resections of cardiac sarcoma were performed in 9 patients (cases 1 to 9); 2 of those patients had undergone R1 or endocardial resection before. Nine patients (cases 10 to 18) underwent R1 resections. Extensive resection of the normal cardiac structure surrounding the tumor was the standard pattern in all the R0 and R1 resections. Patch reconstructions or defect closings of cardiac chambers were performed in all R1 resections and in 5 of the 9 R0 resections. A direct closure of the LA defect was performed in 2 R0 resections. Two patients with pulmonary artery (PA) sarcoma received R0 resections that included a right pulmonectomy and resection of the right PA with reconstruction of the main PA. Coronary artery bypass grafting of the resected right coronary artery that was invaded by the tumor was performed in 1 R1 resection.

Eleven patients (cases 19 to 29) underwent R2 resections. Of those patients, 5 patients were misdiagnosed as myxomas during operation and the resections were limited to the endocardial level, and 6 patients underwent R2 resections with visible tumor residue. In an attempt to completely remove the tumor, 2 procedures of patch reconstruction of the right atrium and 2 procedures of mitral valve replacement with a bioprosthetic valve were performed in 4 patients with R2 resections. The surgical treatment of local recurrences included 1 R1 resection, 3 R2 resections, and 1 endarterectomy of a PA sarcoma. There were 1 pulmonary lobectomy and 1 hepatolobectomy performed for distant metastases. Percutaneous PA stenting was performed in 1 patient to alleviate the local progression.

Morbidity and Mortality The 30-day or hospital mortality was zero. The postoperative complications included mild congestive heart failure in 4 patients, pneumonia in 1, delirium in 1, and atrioventricular junctional rhythms treated with medications in 3 and with pacemaker placement in 1. The mean hospital stay was 16 days (range, 7 to 30 days).

Adjuvant Therapy and Radiation Therapy None of the 9 patients with R0 resections received adjuvant therapy. Of the other patients, 7 patients received adjuvant chemotherapy with a single chemotherapeutic regimen: doxorubicin/ifosfamide/cisplatin (n ¼ 4), doxorubicin/paclitaxel (n ¼ 2), and doxorubicin/ifosfamide/ dacarbazine (n ¼ 1). One patient received adjuvant radiation therapy alone, and 1 patient received both types of adjuvant therapies. Five patients received palliative radiation therapy to the local progression or metastasis. One patient died of radiation-related toxicities (Table 2).

Tumor Recurrence Six (67%) patients with R0 resections and 8 (89%) patients with R1 resections experienced local recurrences. The

Case

Histology

Site

Margin Status and Surgical Procedure Details

Adjuvant Therapy

Recurrence (mo)

Synovial sarcoma

LA

R0; patch



Lung (12)

2 3

Undiff Angio

LA, with pedicle RA

R0; direct closure R0; RA reconstruction

– –

Liver (22), local (33) Local (58)

4 5

Angio Undiff

Local (21), liver (21) –

Synovial sarcoma Undiff

– –

Local (36) Local (24)

8

Intimal sarcoma



Local (50)

9

Intimal sarcoma



10

Angio



11

Angio

RA, encroaching SVC and atrioventricular sulcus

12 13 14 15 16 17

Myxosarcoma Myxosarcoma Undiff Synovial sarcoma Angio Leiomyosarcoma

LA RA, protruding into RV LA SVC RA RA, encroaching IVC

18

Angio

RA, encroaching pericardium

19

Rhabdo

LA

R0; RA reconstruction (previous R1 resection) R0; direct closure R0; patch (previous endocardial resection) R0; patch R0; right pulmonectomy; main PA reconstruction R0; right pulmonectomy; main PA reconstruction R1; resection of entire RA free wall and part of atrial septum, RA reconstruction R1; resection including part of atrioventricular sulcus and RCA; RA, SVC reconstruction, SVG to RCA coronary bypass R1; patch R1; RA reconstruction R1; patch R1; SVC reconstruction R1; RA reconstruction R1; extended abdomen incision and IVC, RA reconstruction R1; resection of entire RA free wall and part of pericardium, RA reconstruction R2 (endocardial)

– –

6 7

RA 2 foci, roof and left lateral aspect of LA RA, with pedicle LA

20

MPNST

LA, with pedicle

21 22 23

Undiff Angio Leiomyosarcoma

LA; with pedicle LA, multifoci LA, with pedicle

Tumor in right and main PA, protruding to left PA Main PA, obstructing right PA RA

R2 (endocardial); MV replacement owing to rheumatic disease, R2 (endocardial) R2 (endocardial) R2 (endocardial)

CTx (IAP)

– – XRT – CTx (AT) CTx (IAP), XRT

Left lung upper lobectomy (R0) Hepatolobectomy (R0) R2; recurrence encroached TV annulus and RV – –

NED, 216 D, 47 D, 72

D, 24 NED, 38

Left lung (6)

– R1; recurrence at roof of LA, patch Endarterectomy of recurrence in left PA –

D, 16

Local (6), bone (8)

XRT

AWD, 14

Local (2), lung (16) bone (22)

XRT

D, 29

Local (6) Local (2), lung (3) – Local (4) Local (4), brain (11) Local (6)

D, 42 D, 58 D, 75

– – – – –

D, 10 D, 6 D,a 12 D, 10 D, 11 D, 18



D, 24

D, 10 D, 6 D, 17 D, 8 D, 11

XRT



Local (6), lung (6)



Local (5)



Local (3)

R2; diffuse recurrence in LA –

Local (2) Local (5) Local (4)

R2; recurrence in LA – –

CTx (MAID) – –

Outcome, Survival (mo)

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1

Additional Treatments

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Table 2. Treatment Courses of 29 Patients With Resections of Primary Cardiac Sarcoma

D, 12

D, 5

D, 12 AWD, 54









– – – CTx (IAP)

Brain (2) –

Synovial sarcoma Intimal sarcoma 28 29

Dead of radiation therapy toxicity.

Undiff 27

a

Synovial sarcoma 26

RV Tumor encroached the whole path of PA

R2; debulking, prosthetic valve replacement of MV, residual in the myocardium of RV R2; residual in the myocardium of RV R2; endarterectomy, residuals left in distal PA branches in both lungs

CTx (IAP) R2; debulking, patch

– Intimal sarcoma 25

Ann Thorac Surg 2014;97:1379–86

Angio ¼ angiosarcoma; AT ¼ doxorubicin/paclitaxel; AWD ¼ alive with disease; CTx ¼ chemotherapy; D ¼ dead of disease; IAP ¼ ifosfamide/doxorubicin/cisplatin; IVC ¼ inferior vena cava; LA ¼ left atrium; LV ¼ left ventricular; MAID ¼ mesna/doxorubicin/ifosfamide/dacarbazine; MPNST ¼ malignant peripheral nerve sheath tumor; MV ¼ mitral valve; NED ¼ no evidence of disease; PA ¼ pulmonary artery; patch ¼ incision closed with a bovine or autologous pericardial patch; RA ¼ right atrium; RCA ¼ right coronary artery; Rhabdo ¼ rhabdomyosarcoma; RV ¼ right ventricular; SVC ¼ superior vena cava; SVG ¼ saphenous vein graft; TV ¼ tricuspid valve; Undiff ¼ undifferentiated pleomorphic sarcoma; XRT ¼ radiation therapy.

D, 18 PA stenting –

XRT (lung)

D, 19 Chest (2), brain (18) CTx (AT)

R2; prosthetic valve replacement of MV, residual at atrioventricular sulcus R2; endarterectomy with visible residual, patch

LA, encroaching left pulmonary veins and MV Tumor in the left and main PA, right ventricular outflow RA, encroaching RV, pericardium and diaphragm; LV, encroaching MV Angio 24

Histology Case

Table 2. Continued

Site

Margin Status and Surgical Procedure Details

Adjuvant Therapy

Recurrence (mo)

Additional Treatments

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XRT

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Outcome, Survival (mo)

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median local recurrence-free survival after an R0 resection was 36 months compared with 6 months after an R1 resection (p

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