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;ISleiimyomatosis with intracardiac M. A. Ricci, L. M. Cloutier, S. Mount*, C. Welandert and B. J. Leavitt Ckpa~ents of Suqev, *Patho@y and tOb9et.?s and Gynecology, University of Vermont Coi/eg;r i;“:’ Mediche, Burli@ton, Vermont, USA
Intravenous ieiomyomatosis is a benign smooth muscle tumor of uterine origin characterized by grossly visible masses of smooth muscle cells growing within venous channefs. A case of leiomyomatosis arising in the uterine veins with extension into the right iliac veins, vena cava, right atrium, right ventricle and into the pulmonary oufflow tract i:j described. The tumor was completely and successfully removed by a staged approach with median sternotomy followed by laparotomy several days later. A brief review of the literature and a discussion of’ the operative approach are presented. --
Kqwwds:
leiomyomatosis.vena cavatumor, intracardiac tumor
In 1988, Clement’ summarized 76 reported cases of intravenous leiomyomatosis with extension into the right side of the heart in 22 cases. Since then, five additional caseshave been reported in the literature2-6. Of these 81 reported cases, only two grew into the pulmonary artery 5,7. A case of successful resection of intravenous leiomyomatosis extending the length of the inferior vena cava into the pulmonary artery via a two-stage procedure is reported here.
Case report A 39-year-old gravida 1, para 0 woman was first evaluated in 1989 for menorrhagia. Pelvic ultrasonography and magnetic resonance imaging revealed uterine fibroids. The patient was successfully treated with hormonal therapy (Lupron, TAP Pharmaceuticals, North Chicago, Illinois, USA) for 6 months with regression of the fibroids. Fibroids were not detectable on ultrasonography the following year. In August 1990, the patient was again treated with Lupron and had symptomatic improvement but, by early 1991, the fibroids had regrown and myomectomy was planned. The patient underwent a myomectomy in July 1991, which revealed an enlarged uterus with a multilobulated
Correspondence to: Dr M. A. Ricci, Department of Surgery, FAKC University Health Center Campus, One South Prospect Street, Burlington, VT OS401, USA
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myoma (12 x !i x 9 cm) extending interiorlv to the posterior cervix and along the broad ligament. i’he final pathological dia,gnosis was a cellular lelomyoma. The postoperative course was complicated by left deep venous thrombosis and pulmonary emboii which were treated by anticoagulation. Eight months before admission in August, 1992, the patient experienced episodes of lightheadedness, worsened by bending over, and syncope in addition to continued menorrhagia. A carotid duplex scan and Holter monitoring were negative. The patient was referrred to the University of Vermont in April, 1993 becauseof the sudden onset of chest tightness, lightheadedness, tachycardia and syncope. Physical examination revealed a young woman in severe distress with an irregular hearr rate and a III/VI high-pitched systolic murmur with a famt diastolic flow rumble. Her legs were mildly edematous. Chest radiography showed mild cardiomegaly. An electrocardiogram showed sinus tachycardia with occasional premature ventricular contraction. I’ransesophageal echocardiogram revealed a large intracardiac mass which appeared to originate just proximal to the hepatic veins with extension via the inferior vena cava through the right atrium and right ventricle with a pedunculated extension into the pulmonary artery. The left ventricular chamber size and wall motion were normal. A presumptive diagnosis of pulmonary thromboembolus was made and the patient underwent emergency exploration via a median sternotomy with eardiopulmonary bypass. The aorta was cannulated and a second 693
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cannula was passed into the right atrium and directed into the superior vena cava. The patient was placed on bypass after heparinization. Cooled perfusate and low flows left the heart fibrillating. A transverse right atriotomy revealed a firm, fleshy mass originating in the inferior vena cava. There were no intraluminal attachments, but the mass could not be extricated from the vena cava. The mass was transected at the level of the diaphragm and removed through the atriotomy. A frozen section revealed a spindle-cell neoplasm. As the immediate threatening mass was removed, the patient was closed to allow for the intra-abdominal portion to be fully evaluated. Gross examination of the mass revealed a smooth, tan, cylindrical, focally hemorrhagic mass (9.0 x 3.0-5.0 cm in diameter). Microscopic examination showed a benign smooth muscle neoplasm characterized by interweaving fascicles of uniform spindle cells with an occasional large hyperchromatic nucleus and mitoses averaging one per 10 high-power fields. The specimen was histologically identical to that of the previous myomectomy specimen. Immunoperoxidase staining confirmed smooth muscle differentiation with positive reactivity for a-smooth muscle actin, muscle-specific actin and vimentin. In addition, ultrastructural investingation demonstrated diagnostic features of smooth muscle. Further evaluation after operation included computed tomography of the abdomen which revealed an enlarged inferior vena cava from the diaphragm to the aortic bifurcation with heterogenous enhancement throughout. A contrast study of the inferior vena cava revealed a large filling defect arising in the right internal iliac vein with extension into the common iliac vein, the inferior vena cava and the right atrium (Figure 1). Fluoroscopy revealed movement of the transected tumor end above the level of the diaphragm with each heart beat. Intraoperative transesophageal echocardiography 1 week after the original surgery again demonstrated the mass within the right atrium prolapsing into the right ventricle during diastole. Resection was undertaken via a midline abdominal approach with repeat sternotomy to gain proximal control. The cardiopulmonary bypass team was on standby. The intra-abdominal vena cava and its branches were exposed by medially rotating the colon and abdominal organs. Because preoperative work-up showed tumor thrombus in the heart, a clamp was placed on the right atria1 appendage, which was opened after placing a purse-string suture, to avoid another atria1 incision. The tumor was manually palpated. The cranial portion of the tumor was removed via an incision in the inferior vena cava with digital pressure from above. Because the tumor was adherent in the pelvis, it was divided, with the remainder of the tumor removed from below with ligation of the iliac veins. A total hysterectomy and a bilateral salpingooophorectomy were performed when hemostasis was 694
Figure 1 Venacavagram showing an extensive conical filling defect throughout the length of the inferior’ vena cava. Additional views demonstrated the origin of the mass in the right common and external iliac veins. The right hypogastric vein was occluded
secure. There was grossly visible tumor in the divided uterine veins. In all, including the cardiac portion, a tumor measuring 45 cm in length was removed (Figure 4. The gross and histologic findings of the inferior vena cava tumor matched that removed from the heart. The uterus was slightly enlarged but there was no evidence of uterine leiomyoma. A microscopic focus of tumor within the parametrial uterine vein was demonstrated (Figure 3). The patient did well after surgery and was discharged 8 days after the second procedure. Follow-up echocardiogram revealed normal atria, ventricles and valves and computed tomography has revealed no sign of recurrence 24 months after surgery. CARDIOVASCULAR SURGERYDECEMBER 199sVOL3 NO6
Discussion
Figure 2 Portions of intravenous leiomyomatosis removed from the inferior vena cava and iliac veins at the second operation
Figure 3 Paraffin-embedded section from a hysterectomy specimen showing the spindle-cell tumor filling and expanding a parametrial vessel. (Hematoxylin and eosin stain, original magnification x250)
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Intravenous leimyomatosis with infermr vena cava extension is a rare uterine tumor and growth into the heart or pulmonary arteries is even more unusual. The differential diagnosis for an intracaval and intracardiac mass includes atria1 myxoma, renal, hepatrc, or other intra-abdominal neoplasms, primary inferior vena cavai leiomyoma and thrombus r,s. Of benign tumors of the heart, the majority (reported as JO-#S%)s-- I’) are myxomas, but less than 10% of myxomns are found on the rights-‘I’~ With an extensive tumor burden, such as that seen in the present case, gynecologic symptoms and not cardiac symptoms are the usual presenting complaint of intravenous leiomyomatosis. Clement” found only eight cases that presented with congestive failure or dyspnea. Syncope, the complaint that ultimately Eedto discovery of the tumor in the patient described here, is equally unusual as a presenting complaint’. Typically, definitive preoperative diagnosis is unusual, and \/enous mvolvrment may be notsed only at the time of hysterecxomy’. Echocardiography, abdominal ~~lrr~s~t~o~rap~y and computed tomography are important jn e\rablishing the diagnosis of intravenous leiomyomatosis ;mtl in demon-~ strating the extent of the tumor’,“. ‘C’enacavography may also be important in assessing the extent of caval involvement. Cooper et al.’ also suggested preoperari~~r cardiac catheterization to determine the need for revascularization but, in the present a~thoxs opinion this is unnecessary in this generaljv :“~nger ~~atienr population. A frozen section at the time ot surgrr;, ~.:;x:;l suggest the diagnosis of intravenous leiomyomato~i~~. because dif ferentiation from malignant disease ix \i!~rmately based on the number of mitotic figures ie’:‘:: t)n multiple permanent secticms”, frozen section ~11: ~~rovide only ;I preliminary diag!nosis. This may be sufti~.:enr, however, to guide the surgical treatment by elimlrwrlng from the differential such entities as renal trrll :niinorna and atria1 myxoma. There are two theories for the ortgm ot intravenous leiomyomatosis and the patient descr~bcd here haa evidence to support both. Tumor nb,:\’ be ..g direct extension of a uterine leiomyoma, 3s ~ippearrd to he present in the patient reported here, especially in light of her previous myomectomy. Extrautermr: growth may go undetected, leading to ‘recurrence”. ~~Jternatively, the tumor may develop from the smooth rum& wuhin the wall of the vein”. The absence of leiotnyontn wrthin the uterus and only a microscopic focus withIn a uterine vein might support the argument th:xt TIM prewnt c;tse was a primary venous tumor. Regardless of its origin, however, trc,~tment consists of complete extirpation of the tumor with a total hysterectomy and a bilateral salpingo-oopIaorect(~my ‘. ‘, In the majority of cases reveiwed by ~%mcnt’, venous involvement was limited to the Delvic ‘,~XFIY. %lv c,ne
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Vascukr case reports
case (excluding those with cardiac involvement) had extrauterine spread of the tumor, enveloping the inferior vena cava and retroperitoneum. When the tumor grows into the vena cava to involve the heart, often a staged approach is utilized but, as in the present case, not always by choice. In most instances, the severity of symptoms or misdiagnosis (i.e. atria1 myxoma) leads to removal of the intracardiac portion of the tumor down to the abdominal vena cava followed by later removal of the remaining infra-diaphragmatic tumo?. Although this was attempted in the patient described here, subsequent studies inexplicably showed tumor again extending into the atrium, suggesting the combined approach is probably safest. Ideally, if the preoperative diagnosis and extent of the tumor are known, the authors recommend a single combined procedure, in much the same manner as renal cell carcinoma with extensive intravascular tumor thrombus is treated. Postoperative management should exclude the use of reproductive hormones and may include administration of antiestrogensl. Non-invasive follow-up with echocardiography and abdominal ultrasonography is recommendedl.
References 1. 2.
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Clement PB. Intravenous leiomyomatosis of the uterus. Path01 Annu 1988; 23: 153-83. Cooper MM, Guillem J, Dalton J et al. Recurrent intravenous leiomyomatosis with cardiac extension. Ann Thoruc Surg 1992; 53: 139-41. Fukaya Y, Iida F, Morimoto M, Nobara H, Miwa H, Nakano H. A case report on successful removal of intravenous leiomyomatosis extending in the right ventricle. Surgery 1991; 110: 909-11. Suginami H, Kaura R, Ochi H, Matsuura S. Intravenous leiomyomatosis with cardiac extension: successful surgical management of histopathologic study. Obstet Gynecol 1990; 76: 527-9. Lee P-K, David TE, Sloggett C, Ross JR. Intravenous leiomyomatosis with intracardiac extension: an unusual cause of cardiac syncope. Can Med Assoc J 1990; 142: 1257-9. Kaszar-Seibert D, Gauvin GP. Rogoff PA et al. Intracardiac extension of intraienous leiom;omatosis. Radiology 1988; 168: 409-10. Akatsuka N, Tokunaga K, Isshiki T et al. Intravenous leiomyomatosis of the uterus with continuous extension into the pulmonary artery. Jpn Heart J 1984; 25: 651-9. Bulkley BH, Cooley DA, Frazier OH et al. Atria1 myxoma: a fifty year review. Am HeartJ 1979; 97: 639-46. Mirralles A, Bracamonte L, Soncul H et al. Cardiac tumors: clinical experience and surgical results. Ann Tboruc Surg 1991; 52: 886-95. Blondeau P. Primary cardiac tumors - French studies of 533 cases. Tborac CardiovascSurg 1990; 38: 192-5. Gonzalez-Lavin L, Lee RH, Falk L et al. Tricuspid valve obstruction due to intravenous leiomyomatosis. Am Heart J 1984; 106: 1544-6.
Acknowledgements
11.
The authors thank Drs Robert Battle and Julie Olin for their assistance in the preparation of the manuscript.
Paper accepted 10 October 1994
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