Ann Thorac Surg 2006;82:1517-1519
© 2006 The Society of Thoracic Surgeons
Case Reports
Single-Stage Resection of a Mixed Endometrial Stromal Sarcoma and Smooth Muscle Tumor With Intracardiac and Pulmonary Extension
Maria Coganow, MSIVa,
B. Mohan Das, MDb,
Ellen Chen, MDc,
Juan A. Crestanello, MDa,*
a Division of Cardiothoracic Surgery, The Ohio State University, Columbus, Ohio
b Division of Vascular Surgery, The Ohio State University, Columbus, Ohio
c Division of Cardiology, The Ohio State University, Columbus, Ohio
Accepted for publication February 16, 2006.
* Address correspondence to Dr Crestanello, Division of Cardiothoracic Surgery, 8th Floor North Doan Hall, 410 West 10th Ave, Columbus, OH 43210 (Email: juan.crestanello{at}osumc.edu).
 |
Abstract
|
|---|
A case of mixed endometrial stromal sarcoma and smooth muscle cell tumor of the uterus with intravenous extension into the right heart and pulmonary artery is presented. The current literature and diagnostic and therapeutic strategies of pelvic tumors with intravenous extension are reviewed.
|
Introduction
|
|---|
Mixed endometrial stromal sarcoma and smooth muscle tumors of the uterus with intravascular and intracardiac extension are very rare [1, 2]. We believe that there are only two previously documented cases of such tumors with intracardiac extension [1, 2]. We present the third case of a mixed tumor consisting of intravenous leiomyomatosis and endometrial stromal sarcoma that originated in the uterus and progressed through the iliac vein, inferior vena cava (IVC), right atrium, and right ventricle, into the pulmonary artery.
A 47-year-old woman presented with a 3-month history of dyspnea on exertion, left-sided chest discomfort, and lower extremity edema. Two years prior she had a hysterectomy and left salpingo-oophorectomy for a "benign leiomyoma." At physical examination she had a holosystolic cardiac murmur and mild lower extremity edema. Echocardiogram revealed a long linear mass coming from the IVC extending into the right atrium, right ventricle, and right ventricular outflow tract (Fig 1). Computed tomography and magnetic resonance imaging demonstrated an intraluminal filling defect within the right common iliac vein extending through the IVC, into the right atrium, right ventricle, main pulmonary artery, and right pulmonary artery (Fig 2). There were filling defects in the right lobar pulmonary arteries consistent with emboli. The mass enhanced with gadolinium suggesting a tumor. A 3 x 5 cm right ovarian mass was also identified.
View larger version (60K):
[in this window]
[in a new window]
|
Fig 1. Echocardiogram showing mass (arrows) extending from the inferior vena cava (IVC) into (A) the right atrium (RA), (B) the right ventricle (RV), and (C) the right ventricular outflow tract. (Ao = aorta; LVOT = left ventricular outflow tract.)
|
|
View larger version (82K):
[in this window]
[in a new window]
|
Fig 2. (A) Magnetic resonance imaging scan of the chest and abdomen showing intraluminal filling defect in the inferior vena cava (arrows). (B) Computed tomographic scan of the chest showing filling defect in the pulmonary artery (arrow). (RV = right ventricle.)
|
|
At surgery a median sternotomy was performed. Simultaneously the entire IVC and right iliac veins were exposed by medial reflexion of the right colon and a Kocher maneuver. The superior vena cava, left iliac vein, and ascending aorta were cannulated. The distal end of the mass was removed from the right heart and pulmonary artery through a right atriotomy under cardiopulmonary bypass with beating heart. There were no attachments to the heart. Pulmonary arteriotomies were performed and no residual tumor was identified. The proximal end of the tumor was coming from the IVC, and it was firmly attached. Control of both renal veins, the ovarian vein, and the left common iliac vein was achieved with vessel loops. A venotomy was made in the distal IVC and extended into the right iliac veins. The proximal end of the tumor was identified emanating from a small lateral pelvic wall vein draining into the external iliac vein, which was then excised. The distal tumor was retrieved from the IVC and right iliac vein through the venotomy. An IVC filter was inserted. The IVC and iliac veins were closed primarily by venorraphy. The patient was then weaned from cardiopulmonary bypass. A right salpingo-oophrectomy was performed. The patient recovered well from the surgery and was discharged home 10 days postoperatively.
Grossly the mass appeared to be like a long continuous, white hollow cylinder that extended from the lateral pelvic wall vein to the right pulmonary artery (Fig 3). Pathology revealed a tumor with features of both intravenous leiomyomatosis and low-grade endometrial stroma sarcoma (Fig 3). The fallopian tube showed identical morphology as to the intravenous mass. Review of the previous hysterectomy specimen showed a focus of smooth muscle proliferation with intravascular extension that was the likely origin of the tumor.
View larger version (81K):
[in this window]
[in a new window]
|
Fig 3. (A) Resected specimen and immunohistochemical stains for (B) CD10 (endometrial stromal cells) and (C) H caldesmon (smooth muscle differentiation).
|
|
Nine months after the surgery, the patient is doing well with no signs of recurrence.
|
Comment
|
|---|
Uterine leiomyomatosis is the second most common abdominal tumor with intravascular extension into the IVC and right-sided cardiac chambers [3]. Leiomyomatosis is a neoplastic proliferation of benign smooth muscle originating from either a pre-existing benign uterine leiomyoma or from smooth muscle from the wall of a uterine vein [4]. Endometrial stromal sarcomas are rare uterine tumors composed of cells resembling endometrial stroma [1, 2]. Although they have the propensity for lymphatic and venous invasion, they rarely involve the large vessels or the heart [2, 5]. Mixed tumors are those tumors that contain more than 30% of the other component [1, 2]. Endometrial stromal sarcomas are more aggressive than intravenous leiomyomatosis with greater propensity for local invasion and systemic metastasis [1, 2]. Low-grade endometrial stromal sarcomas are considered to be biologically close to pure smooth muscle tumors [1, 2].
The two previously described cases of this tumor with intracardiac extension were diagnosed at a later stage than the present case, and they had a more aggressive behavior with pelvic, caval, and intracardiac recurrences [1, 2].
Complete resection of the intravascular and extravascular portion of the tumor is mandatory to relieve symptoms and prevent recurrence [5, 6]. The surgical approach should be guided by preoperative determination of the site of origin of the tumor and degree of extension into the IVC, heart, and pulmonary arteries. Single-stage and two-stage operations with cardiopulmonary bypass with or without hypothermic circulatory arrest have been used for resection of the tumors with cava and cardiac extensions [3, 5, 6]. We favor a single-stage operation with simultaneous exposure of the heart, inferior vena cava, and iliac veins. A single-stage operation avoids the risk of tumor embolism, tumor progression, and hemodynamic complications in the interval between the surgical stages. It also avoids the need for a second operation. Control and temporary occlusion of the renal veins, gonadal vein, contralateral iliac vein, and if necessary, a Pringle maneuver are usually sufficient to obtain a bloodless field and allow resection of the caval portion of the tumor. If these measures are not sufficient, hypothermic circulatory arrest is indicated [5]. If there is an invasion of the cava wall by the tumor, partial or complete resection of the vein wall and subsequent reconstruction may be necessary [5].
In the present report, the tumor was a single continuous structure that did not attach nor invade, neither to the inferior vena cava wall nor to the heart. Because of its firm rubbery consistency and structural continuity from the external iliac veins to the pulmonary artery, it may have been possible to remove the tumor on its entirety through an isolated abdominal approach without cardiopulmonary bypass [6]. The presence of pulmonary emboli and the uncertain preoperative diagnosis prevented us from using that approach.
Venous cannulation for cardiopulmonary bypass may be challenging. We believe that superior vena cava cannulation and cannulation of the inferior vena cava system distal to the tumor should be performed. This assures adequate venous drainage for cardiopulmonary bypass.
Long-term surveillance with magnetic resonance imaging and computed tomographic scan of the chest and abdomen, and echocardiography is necessary to detect early recurrence. Anti-estrogen therapy may decrease the likelihood of recurrence [4].
|
References
|
|---|
- Whitlash SP, Meyer RL. Recurrent endometrial stromal sarcoma resembling intravenous leiomyomatosis Gynecol Oncol 1987;28:121-128.[Medline]
- Mikami Y, Demopoulos RI, Boctor F, et al. Low-grade endometrial stromal sarcoma with intracardiac extension Pathol Res Prac 1999;195:501-508.
- Kocica MJ, Vranes MR, Kostic D, et al. Intravenous leiomyomatosis with extension into the heart: rare or underestimated? J Thorac Cardiovasc Surg 2005;130:1724-1726.[Free Full Text]
- Parikh J, Lesseps A. Intravenous leiomyomatosis J Obstet Gynaecol 2000;20:439-440.[Medline]
- Phillips MR, Bower TC, Orszulak TA, Hartman LC. Intracardiac extension of an intracaval sarcoma of endometrial origin Ann Thorac Surg 1995;59:742-744.[Abstract/Free Full Text]
- Moorjani N, Kuo J, Ashley S, Hughes G. Intravenous uterine leiomyosarcomatosis with intracardiac extension J Card Surg 2005;20:382-385.[Medline]
Top
Abstract
Introduction
