Ann Thorac Surg 2007;84:644-647
© 2007 The Society of Thoracic Surgeons
Case Reports
One-Stage Surgery of Low-Grade Malignant Intravenous Uterine Leiomyosarcomatosis With Right Atrium Extension
Liang Ma, MD,
ShengJun Wu, MD,
Yu Zou, MD,
WeiDong Li, MD,
Liang Gong, MD,
Gabrielle Gerelle, MD,
YiMing Ni, MD*
Department of Cardiothoracic Surgery, 1st Affiliated Hospital, College of Medicine, ZheJiang University, HangZhou, The People’s Republic of China
Accepted for publication March 19, 2007.
* Address correspondence to Dr Ni, Department of Cardiothoracic Surgery, 1st Affiliated Hospital, College of Medicine, ZheJiang University, HangZhou, 310003, The People’s Republic of China (Email: ni_yiming{at}hotmail.com).
 |
Abstract
|
|---|
Uterine leiomyosarcoma is a rare and aggressive malignant tumor arising from the smooth muscle cells of the myometrium, which rarely metastasizes to the heart. We report the case of a 49-year-old Chinese woman who underwent a successful one-stage surgical intervention after an initial diagnosis of intravenous uterine leiomyomatosis. However, her postoperative biopsy showed the presence of low-grade malignant uterine leiomyosarcoma with intravenous and right atrium extension. It is our conclusion that surgical resection is the mainstay treatment for this type of condition.
|
Introduction
|
|---|
Cardiac neoplasms of uterine origin are rare and have seldom been reported in clinical practice. In our report we will present a case of a patient with uterine leiomyosarcoma with metastasis in the right common iliac vein, inferior vena cava, and right atrium. We will describe the combined surgical treatment that we undertook before discussing the rare reports that have described cases of cardiac metastases of uterine origin.
A 49-year-old Chinese woman was admitted with a 1-year history of abdominal distention, post-activity dyspnea, and palpitations. On examination, a large mass was palpated in the lower abdomen, unaccompanied by tenderness. The patient was in New York Heart functional class III with no added sounds or murmurs. Magnetic resonance imaging of the heart and abdomen showed the presence of a Giga tumor (>20 cm x 20 cm) arising from the pelvic cavity (Fig 1A) with abdominal extension and right atrial and inferior vena caval (IVC) involvement (Fig 1B). Intravenous uterine leiomyomatosis was the initial diagnosis of choice and surgery was performed on December 19, 2006.
View larger version (84K):
[in this window]
[in a new window]
|
Fig 1. (A) Magnetic resonance image of the tumors extending into the inferior vena caval and right heart. (IVL = intravenous uterine leiomyomatosis.) (B) Magnetic resonance image of the massive abdominal mass in the abdominal and pelvic cavities (posterior abdominal membrane).
|
|
A 25 x 20 x 10 cm segmented pelvic tumor was detected through a hypogastric meso laparotomy, extending into the right common iliac vein and the IVC. Total lumpectomy was performed, followed by a total abdominal hysterectomy with bilateral salpingo-oophrectomy (Fig 2A). With the IVC and right common iliac vein exposed, a median sternotomy incision was made, and normothermic cardiopulmonary bypass was set up with cannulation of the superior vena cava and left femoral vein, and placement of an arterial line in the ascending aorta that was left unclamped. The right atrium was opened and contiguous tumors were found throughout the atrium, overflowing into the IVC. As deposits were found within the right common iliac vein at the time of surgery, the IVC was opened by microtubule incision and the tumor was excised by section down past the iliac vein until no more tumor deposits were visible. A tissue strip, measuring 20 cm in length and 4 cm in diameter containing the atrial tumors (Fig 2B, 2C) was then gently excised from the right atrium, and a third venous cannula was then inserted into the IVC through the right atrium for the purpose of drainage. Both iliac and pelvic tumors were prepped for histologic examination. Of note, total circulatory bypass time was 45 minutes long.
View larger version (145K):
[in this window]
[in a new window]
|
Fig 2. (A) The excised abdominal mass (25 cm in length and 20 cm in diameter, weighing 3 kg). (B) The tumor after removal from the inferior vena caval (IVC) and right atrium (RA), as well as (C) residual tumor between the inferior vena caval (IVC) and right common iliac vein.
|
|
During the first 2 days after the operation, the patient appeared restless but remained orientated and suffered no further complications. Postoperative histology of the excised tumors confirmed the presence of a low-grade malignant uterine leiomyosarcoma (LMS) with intravenous and right atrial extension (Fig 3). The patient was discharged home 10 days postoperatively. Two months later, the patient remained in good condition and no recurring mass was detected in the heart and abdomen.
|
Comment
|
|---|
Nearly all malignant tumors can metastasize to the heart, but reports of uterine LMS extending through the IVC into the right atrium are extremely rare. We believe that the earliest report discussing leiomyosarcomas of uterine origin with intracardiac invasion was described by Montemezzi [1] in 1966. Since then, only nine reports describing cases of uterine tumors metastasizing to the heart have been published. Most of them describe accounts of uterine intravenous uterine leiomyomatosis with intracardial extension, whereas only two of these actually reported cases of uterine LMS with invasion of the IVC and right atrium [2, 3].
Both uterine leiomyomas and uterine leiomyosarcomas are tumors of smooth muscle origin. However, unlike leiomyomas, which are benign and are usually confined to the pelvis, uterine LMS are malignant tumors that behave in an aggressive manner with a high rate of metastasis to any organ, a high chance of recurrence, and a poor prognosis. If the tumor spreads to the heart, a preoperative heart biopsy may help determine the histologic nature of the tumor before any surgical intervention is undertaken. However, as patients with this type of condition may present with severe symptoms, surgery with a histologic follow-up provides more immediate benefit to the patient than lengthy preoperative invasive procedures.
In terms of surgical technique, it is important to underline that removal of intravenous tumors by sections seemed to ensure that all the tumor deposits were carefully removed. Nevertheless, venule involvement increases surgical difficulty and the risk of recurrence, as tumors growing into these are difficult to excise, even by the most skilled of surgeons.
The two articles that report LMS with intracardiac extension, describe one-stage surgeries by cardiopulmonary bypass with prior removal of the pelvic mass. In the case of our patient, normothemic cardiopulmonary bypass was used, and the aorta was left unclamped. Although no resections of tumors extending into the IVC without the use of cardiopulmonary bypass have been reported [4], cardiopulmonary bypass remains an important adjuvant technique for IVC tumor resection, as the latter may cause a reduction in blood supply and cause hemorrhage and heart failure. Two-stage surgery may also be undertaken, but its practical effects have yet to be evaluated.
Some studies suggest a trend toward improved locoregional control and survival through radiotherapy in patients who receive such treatment after surgical management; however more studies need to be undertaken to verify its practical effectiveness. Furthermore, adjuvant chemotherapy does not seem to improve prognosis with this type of tumor [5]. As research has shown that aggressive surgical cytoreduction of uterine LMS at the time of initial diagnosis offers the possibility of prolonged survival or cure [6], and based on our experience and available data, we believe that complete surgical resection is the mainstay treatment of intravenous uterine leiomyosarcomatosis with intracardiac extension.
|
References
|
|---|
- Montemezzi L. Leiomyosarcoma with extensive venous and cardiac invasion of probable uterine origin Arch De Vecchi Anat Patol 1966;48:37-56.[Medline]
- Moorjani N. Intravenous uterine leiomyosarcomatosis with intracardial extension J Card Surg 2005;20:382-385.[Medline]
- Noedir A, Stolf G, dos Santos GG, Haddad VLS. Unusual abdominal tumors with intra cardiac extention, two cases with successful surgical resection Rev Hosp Clin Fac Med Sao Paulo 1999;54:159-164.[Medline]
- Jibiki M, Inoue Y, Katou T, et al. Tumor thrombectomy without bypass for low-grade malignant tumors extending into the inferior vena cava: report of two cases Surg Today 2006;36:465-469.[Medline]
- Giuntoli RL, Bristow RE. Uterine leiomyosarcoma: present management Curr Opin Oncol 2004;16:324-327.[Medline]
- Gadducci A, Landoni F, Sartori E, et al. Uterine leiomyosarcoma: analysis of treatment failures and survival Gynecol Oncol 1996;62:25-32.[Medline]
Top
Abstract
Introduction
