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Right Atrial and Right Ventricular Obstruction by Recurrent Stromomyoma
CASE REPORTS
Right Atrial and Right Ventricu lar Obstruct ion by Recurren t Stromom yoma Itzhak Kronzon, MD, Gregory M. Goodkin, BA, Alfred Culliford, MD, John V. Scholes, MD, Fouad Boctor, MD, Robin S. Freedberg, MD, and Paul A. Tunick, MD, New York, New York
A 30-year-old woman had a history of a uterine fibroid 6 years before admission. She had resection of a right atrial mass diagnosed as a leiomyoma 2 years ago and a second cardiac procedure for recurrent tumor l year ago. Pathologic examination at that time indicated that the tumor was a low-grade endometrial stromal sarcoma (stromatosis) with features of benign leiomyoma (intravenous leiomyomatosis) . This time she was admitted with facial and lower extremity swelling as well as ascites. Transthoracic and transesophageal echocardiograph y revealed a large tumor entering the heart from the inferior vena cava and filling the right atrium and ventricle. Lower extremity ischemia from bilateral compartment syndrome due to severe edema developed, and she underwent successful surgical resection of the tumor that filled the right side of the heart, inferior vena cava, and mesenteric and renal veins. (J AM Sac EcHOCARDIOGR 1994;7:528-33.)
Uterine stromomyoma (a mesenchymal turnor of the uterus with features of both leiomyoma and lowgrade endometrial sarcoma) is a rare tumor. We present a case in which a uterine stromomyoma invaded the pelvic veins and grew through the inferior vena cava, obliterating the right-sided heart chambers. This recurrent tumor grew to monstrous dimensions, resulting in a syndrome of acute and severe rightsided heart failure that led to major complications. CASE REPORT A 30-year-old woman was admitted to the hospital with complaints of ankle edema, chest and face puffiness, increasing abdominal girth, progressive shortness of breath, and weakness. Six years before this admission, the patient underwent myomectomy for what was thought to be a uterine fibroid and shortly thereafter required a hysterectomy because of local recurrence of the lesion that was found in veins of the broad ligament. Two years later, the patient complained of ankle swelling. Echocardiography at that time revealed a large mass in the right atrium. The intracardiac portion of a large tumor was resected at another institution. The pathologic diagnosis was intracar-
From the Departments of Medicine, Surgery, and Pathology, New York University School of Medicine. Reprint requests: ltzhak Kronzon, MD, 560 First Ave., New York, NY 10016. Copyright© 1994 by the American Society ofEchocardiography. 0894-7317/94$3. 00 + 0 27/l/54836
528
diac leiomyomatosis. One year later, a follow-up echocardiogram revealed recurrence of the intracardiac tumor and a second, more extensive, resection of the tumor was performed. At that time, new pulmonary lesions were noted on chest x-ray film and chest computed tomography (CT) that were interpreted as pulmonary metastases. After the second operation, a review of the surgical pathology slides by a consultant suggested that the tumor was a low-grade endometrial stromal sarcoma (stromatosis) that also demonstrated features of benign leiomyoma (intravenous leiomyomatosis). This combination has been described before and was termed "stromomyoma."' The patient was followed up at periodic intervals and was noted to have abnormal liver functions with an elevated aspartate aminotransferase level (284 units), lactic dehydrogenase level (828 units), bilirubin value (3.6 mg/dl; direct 1.3 mg/dl), and prolonged prothrombin time (17.2 seconds). One month before the current admission, she noted increasing weight, ankle edema, and increasing abdominal girth. About 1 week before this admission, she noted puffiness of the face and massive swelling of both legs. She was then referred to New York University Medical Center. On admission, physical examination revealed marked jugular venous distention, a puffy face, and engorged conjunctivae. Examination of the heart revealed normal heart sounds and no murmurs. The abdomen was markedly distended with shifting dullness diagnostic of massive ascites. There was marked leg edema. The lower limb pulses below the femoral arteries were not palpable. One day after admission, the patient complained of progressive foot and calf pain.
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Figure 1 Transesophageal echocardiograrn with multiplane probe. A, Fifty-four plane: large tumor (TU) extends from inferior vena cava (IVC) into right atrium (RA). B, Seventy-one plane: large tumor (TU) extends through right ventricle (RV) into right ventricular infundibulum and reaches just 1 cm below pulmonic valve (arrow). PA, Pulmonary artery; IA, left atrium; AO, aorta.
Echocardiography The transthoracic echocardiograrn demonstrated right atrial and right ventricular dilation. A large tumor filled the right atrial and right ventricular chambers. Transesoph-
ageal echocardiography better delineated the tumor (Figure 1). It appeared that this was a smooth-surfaced mass that entered the right atrium through the inferior vena cava, filled most of the right atrial and right ventricular cavities,
530
Kronzon et al.
Journal of the American Society of Echocardiography September-October 1994
Figure 2 Continuous-wave Doppler, transthoracic. Transtricuspid flow velocity reaches 2.8 m/ sec, indicating maximal diastolic gradient of 31 mm Hg between right atrium and right ventricle. and extended into the right ventricular outflow tract 1 cm below the pulmonic valve. Color-flow Doppler revealed a narrow jet of high-velocity diastolic flow across the small space in the right ventricular inflow tract that was not occupied by the tumor. The velocity of this jet was 2.8 m/sec, and the calculated maximal transtricuspid gradient was 31 mm Hg (mean gradient 25 mm Hg) (Figure 2).
Magnetic Resonance Imaging Magnetic resonance imaging of the heart and abdomen showed a tumor that filled the right atrium and right ventricle (Figure 3). The inferior vena cava was markedly dilated and filled with a large mass that extended superiorly from the iliac veins. A large lobular pelvic mass was also noted. The hepatic veins were dilated and there was evidence of venous congestion of the liver.
Doppler Study of the Lower Extremities There were no Doppler signals at the dorsal pedal pulses bilaterally. A venous study showed dilated veins in the lower extremities and no venous flow signals bilaterally. The leg veins were not compressible. The study was interpreted as consistent with bilateral deep venous thrombosis. The absence of arterial pulses was consistent with a compartmental syndrome caused by acute leg edema.
Surgery On the third hospital day the patient underwent surgery. After cardiopulmonary bypass, a huge tumor was re-
moved from the right-sided cardiac chambers and the entire inferior vena cava. The tumor nearly obstructed both the right ventricular infundibulum and the tricuspid valve orifice and filled the inferior vena cava, which was markedly dilated. The tumor extended into the superior mesenteric vein and both renal veins. The femoral veins appeared to contain thrombus. Extensive amounts of fresh, red-stasis thrombus were removed. At the end of the procedure, the lower extremities were well perfused with bilateral palpable dorsal pedal pulses. The retroperitoneal mass was not excised during this procedure.
Pathologic Findings The excised mass, measuring 25 x 5 x 5 cm, was shaped in the form of a cast of the right-sided heart chambers and the entire inferior vena cava (Figure 4) . There was an attached thrombus that filled the femoral veins. Histologic examination confirmed attachment of the tumar to an acute thrombus and a fragment of a large vein. The tumor showed histopathologic and ultrastructural features of a leiomyoma that was intravascular (intravascular leiomyomatosis). Review of the sections from the patient's previous primary uterine tumor and intracardiac recurrence showed a tumor that was similar in many areas to the latest intravascular lesion_ However, particularly in the original uterine specimen, there were areas with the histologic appearance of low-grade endometrial stromal sarcoma (stromatosis and endolymphatic stromal myomosis), some of which was within lymphatics. The tumor was therefore
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Magnetic resonance imaging. A, Large tumor fills right-sided heart cavities. VTU, Ventricular portion of tumor; A TU, atrial portion of tumor. B, Tumor (TU) fills intrahepatic portion of markedly dilated inferior vena cava.
Figure 3
believed to show mixed types of mesenchymal differentiation. Follow-up
Intraoperative transesophageal echocardiography after removal of the mass showed no residual intracardiac tumor. The transtricuspid flow velocity had returned to normal. A lower extremity Doppler study revealed improved arterial pulses and good deep venous flow signals. On the second postoperative day, the patient's renal function started to deteriorate. Within a few days, the blood urea nitrogen level reached 78 mg/dl and the creatinine 3 mg/dl. The serum creatine phosphokinase level was markedly elevated (16,000 mg/dl). The urine appeared to be brown and contained myoglobin. It was believed that the myoglobinuric renal insufficiency was the result of rhabdomyo-
lysis from lower limb ischemia. The urine output remained stable, and both myoglobinuria and renal insufficiency resolved spontaneously within l week. The postoperative liver function also improved and returned to normal limits within l week. The patient gradually recovered. Surgery of the abdominal mass was performed with the removal of two tumor masses from the pelvis.
DISCUSSION
Review of multiple specimens of the tumor removed from the patient showed mixed features of histologically benign intravascular leiomyomatosis and lowgrade endometrial stromal sarcoma, which has been termed "stromomyoma."1 Intracardiac extension of uterine leiomyoma (in-
532 Kronwn et al.
Figure 4 Huge tumor resected appears like cast of rightsided heart chambers and entire inferior vena cava. Extension into renal vein is noted (clamp). Indentation of entry of inferior vena cava into right atrium is marked by arrow. Note the dark thrombus attached to the inferior pole of the tumor. VTU, Right ventricular portion of tumor; A TU, right atrial portion of tumor; CIV, caval portion oftumor.
travenous leiomyomatosis) is quite rare. In 1984 our laboratory reviewed 13 cases and added one more that was diagnosed by echocardiography and resected successfully.2 Since then, several more cases have been reported. 3-6 The tumors, although histologically benign, can extend into the heart and create cavitary obliteration, tricuspid and pulmonic valve obstruction, pulmonary embolization, pulmonary metastasis, right-sided heart failure, and death. 2 - 13 Benign intravenous leiomyoma rarely recurs after excision. However, the rate of recurrence of lowgrade endometrial stromal sarcoma is high (up to 60% recurrence rate extending over many years). 14 Although in most cases the pathologic distinction between these two entities is straightforward, in some cases differentiation is ambiguous. Zalonsek and Norris 15 suggested that combined smooth-muscle and stromal tumors (stromomyoma) may be more common than previously recognized. However, to our knowledge, recurrent cardiac stromomyoma has not been described previously. Interestingly, the histologic appearance of the recurrent intracardiac mass
Journal of the American Society of Echocardiography September-October 1994
in this patient appeared more mature, with more complete smooth-muscle differentiation. "Benign metastasizing leiomyoma" is defined as a spread of benign leiomyoma to remote organs (usually lungs and lymph nodes) . Usually the pulmonary nodules progress slowly, and their clinical course is benign, 16 as was seen with this patient. Low-grade endometrial stromal sarcoma also metastasizes to the lung and lymph nodes and usually has a benign course. 14 The exact histologic results of the pulmonary findings in this patient are not known. Although the tumor histologic findings were fairly benign, the clinical course was stormy and malignant. Inferior vena caval, right atrial, and right ventricular obstruction resulted in severe elevation of venous pressures that led to peripheral edema, ascites, and hepatic failure. Inferior vena caval and iliac vein occlusion by the tumor led to femoral vein thrombosis. As a result, severe, acute leg edema produced a compartmental syndrome, in which increased pressure within a limited space compromised the arterial circulation to the lower limbs. Limb ischemia resulted in rhabdomyolysis, which led to myoglobinuria and renal failure. Rhabdomyolysis, myoglobinuria, and renal failure are rare, previously described complications of compartmental syndromeY Surgical excision of the tumor and femoral vein embolectomy disrupted the vicious cycle, and cardiovascular, liver, and renal function returned to normal. Echocardiography is useful in the diagnosis of intracardiac leiomyomatosis. 2 •18 However, transthoracic echocardiography may provide only limited or suboptimal imaging of atrial masses. 19 Transesophageal echocardiography provides a higher resolution image with better delineation of the tumor. It can also provide better visualization of the great veins and thus better demonstrate the extent of intravenous tumors. 20 Very frequently the caval origin of the cardiac mass cannot be visualized by transthoracic echocardiography. Podolsky et al. 4 described a case in which transthoracic echocardiography gave the false impression of right atrial myxoma in a patient with intracardiac leiomyomatosis. In that case, transesophageal echocardiography performed during anesthesia revealed that the cardiac tumor emerged from the inferior vena cava, and therefore the strategy of the operation was changed. In this report, transesophageal echocardiography better delineated tl1e tumor. It could demonstrate its exact extension from the inferior vena cava to the right ventricular outflow tract, just proximal to the pulmonic valve. The transtricuspid flow could be demonstrated and its velocity measured.
Journal of rhe American Society of Echocardiography Volume 7 Number 5
The list of masses that may reach the right side of the heart through intravenous growth include tumars such as renal cell carcinoma, hepatoma, adrenal carcinoma, thyroid carcinoma, angiosarcoma, benign leiomyoma, and low-grade stromal endometrial sarcoma. Stromomyoma should be considered in the differential diagnosis of secondary, intravenous, and intracardiac tumors in women. It is of interest that in this case the clinical behavior, as well as the pathologic findings, of a stromomyoma were intermediate between intravascular leiomyomatosis and low-grade endometrial stromal sarcoma. REFERENCES l. Tang CK, Toker C, Ances I. Stromomyoma of the uterus. Cancer 1979;43:308-16. 2. Politzer F, Kronzon I, Wicrorek R, et al. Intracardiac leiomyomatosis: diagnosis and treatment. J Am Coli Cardiol 1984;4:629-34. 3. Suginami H, Kaura R, Ochi H, Matsuura S. Intravenous leiomatosis with cardiac extension: successful surgical management and histopathological smdy. Obstet Gynecol 1990;76:527-9. 4. Podolsky LA, Jacobs LE, Ioli A, Kotler MN. TEE in the diagnosis of intravenous leiomyomatosis extending into the right atrium. Am Heart J 1993;125:1462-4. 5. Shida T, Yoshimura M, Chihara H, Nakamura K. Intravenous leiomyomatosis of the pelvis with reextension into the heart. Ann Thorac Surg 1986;42:104-6. 6. Gonzalez-Lavin L, Lee H, Falk L, et al. Tricuspid valve obstruction due to intravenous leiomyomatosis. Am Heart J 1984;108: 1544-6. 7. Garcia FA, Villanueva RA, Narcisco F, Avenmra AP. Intravenous leiomyomatosis of the uterus and pelvis presenting as a cardiac tumor. Ann Thorac Surg 1986;42:S41-3.
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8. Norris HJ, Partnley T. Mesenchymal tumors of the uterus v. intravenous leiomyomatosis: a clinical and pathologic smdy of 14 cases. Cancer 1975;36:2164-78. 9. Tienney WM, Ehrlich CE, Bailey JC, King RD, Roth LM, Wann LS. Intravenous leiomyomatosis of the uterus with extension into the heart. Am J Med 1980;69:471-5. 10. Marcus SG, Krause T, Freedberg RS, Culliford AT, Weinreich DJ, Kronzon I. Pulmonary embolectomy for intravenous uterine leiomatosis. Am Heart J 1994;127:1642-5. ll. Timmis AD, Smallpeice C, Davies AC, MacAuthur AM, Gishen P, Jackson G. Intracardiac spread of intravenous leiomatosis with successful surgical excision. N Engl J Med 1980;303: 1043-4. 12. Bahari CM, Gorodesk.i IG, Nili M, Neri A, Avidor I, Garti IJ. Intravascular leiomyomatosis. Obstet Gynecol 1982;59: 735-7. 13. Rowan DA, Mirchandani H. Intravenous leiomyoma with intracardiac extension causing sudden death. Arch Pathol Lab Med 1987;111:1176-8. 14. Sternberg SS, ed. Diagnostic surgical pathology. New York: Raven Press, 1989:1642-3. 15. Zalonsek C, Norris M. Mesenchymal mmors of the uterus. In: Kurman RJ, ed. Balustein's pathology of the female genital tract. 3rd ed. New York: Springer Verlag, 1987:329-93. 16. Bacchman D, Wolff M. Pulmonary metastases from benignappearing smooth muscle mmors of the uterus. Am J Roentgenol1976;127:441-6. 17. Matsen FA, Kzugmire BR. Compartmental syndromes. Surg Gynecol Obstet 1978;147:943-8. 18. Maurer G, Nanda N. Two dimensional echocardiographic identification of intracardiac leiomyomatosis. Am Heart J 1982;103:915-7. 19. Come PC, Riley MF, Mark.is JE, Malagold M. Limitation of echocardiographic techniques in evaluation of left atrial masses. Am J Cardiol 1981;48:947-53. 20. Reeder G, Khanderia BK, Seward JG, Tajik AJ. Transesophageal echocardiography and cardiac masses. Mayo Clin Proc 1991;66: 1101-9.
Correction
With reference to the abstract entitled "New observations of echocardiography regarding QW3600, an ultrasound contrast agent capable of myocardial opacification by intravenous injection" by O.I. Kwan, A. Tarantal, D. Kessler, B. Cotter, N. Dalton, J. Ross, and A. DeMaria (JAM Soc EcHOCARDIOGR 1994; 7(suppl):S30). Dr. John Ross, Jr., wishes it to be noted that his name should not have appeared on this abstract, since he did not review it prior to its submission and was not aware of its contents prior to publication.
Right Atrial and Right Ventricu lar Obstruct ion by Recurren t Stromom yoma Itzhak Kronzon, MD, Gregory M. Goodkin, BA, Alfred Culliford, MD, John V. Scholes, MD, Fouad Boctor, MD, Robin S. Freedberg, MD, and Paul A. Tunick, MD, New York, New York
A 30-year-old woman had a history of a uterine fibroid 6 years before admission. She had resection of a right atrial mass diagnosed as a leiomyoma 2 years ago and a second cardiac procedure for recurrent tumor l year ago. Pathologic examination at that time indicated that the tumor was a low-grade endometrial stromal sarcoma (stromatosis) with features of benign leiomyoma (intravenous leiomyomatosis) . This time she was admitted with facial and lower extremity swelling as well as ascites. Transthoracic and transesophageal echocardiograph y revealed a large tumor entering the heart from the inferior vena cava and filling the right atrium and ventricle. Lower extremity ischemia from bilateral compartment syndrome due to severe edema developed, and she underwent successful surgical resection of the tumor that filled the right side of the heart, inferior vena cava, and mesenteric and renal veins. (J AM Sac EcHOCARDIOGR 1994;7:528-33.)
Uterine stromomyoma (a mesenchymal turnor of the uterus with features of both leiomyoma and lowgrade endometrial sarcoma) is a rare tumor. We present a case in which a uterine stromomyoma invaded the pelvic veins and grew through the inferior vena cava, obliterating the right-sided heart chambers. This recurrent tumor grew to monstrous dimensions, resulting in a syndrome of acute and severe rightsided heart failure that led to major complications. CASE REPORT A 30-year-old woman was admitted to the hospital with complaints of ankle edema, chest and face puffiness, increasing abdominal girth, progressive shortness of breath, and weakness. Six years before this admission, the patient underwent myomectomy for what was thought to be a uterine fibroid and shortly thereafter required a hysterectomy because of local recurrence of the lesion that was found in veins of the broad ligament. Two years later, the patient complained of ankle swelling. Echocardiography at that time revealed a large mass in the right atrium. The intracardiac portion of a large tumor was resected at another institution. The pathologic diagnosis was intracar-
From the Departments of Medicine, Surgery, and Pathology, New York University School of Medicine. Reprint requests: ltzhak Kronzon, MD, 560 First Ave., New York, NY 10016. Copyright© 1994 by the American Society ofEchocardiography. 0894-7317/94$3. 00 + 0 27/l/54836
528
diac leiomyomatosis. One year later, a follow-up echocardiogram revealed recurrence of the intracardiac tumor and a second, more extensive, resection of the tumor was performed. At that time, new pulmonary lesions were noted on chest x-ray film and chest computed tomography (CT) that were interpreted as pulmonary metastases. After the second operation, a review of the surgical pathology slides by a consultant suggested that the tumor was a low-grade endometrial stromal sarcoma (stromatosis) that also demonstrated features of benign leiomyoma (intravenous leiomyomatosis). This combination has been described before and was termed "stromomyoma."' The patient was followed up at periodic intervals and was noted to have abnormal liver functions with an elevated aspartate aminotransferase level (284 units), lactic dehydrogenase level (828 units), bilirubin value (3.6 mg/dl; direct 1.3 mg/dl), and prolonged prothrombin time (17.2 seconds). One month before the current admission, she noted increasing weight, ankle edema, and increasing abdominal girth. About 1 week before this admission, she noted puffiness of the face and massive swelling of both legs. She was then referred to New York University Medical Center. On admission, physical examination revealed marked jugular venous distention, a puffy face, and engorged conjunctivae. Examination of the heart revealed normal heart sounds and no murmurs. The abdomen was markedly distended with shifting dullness diagnostic of massive ascites. There was marked leg edema. The lower limb pulses below the femoral arteries were not palpable. One day after admission, the patient complained of progressive foot and calf pain.
Journal of the American Society of Echocardiography Volume 7 Number 5
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Figure 1 Transesophageal echocardiograrn with multiplane probe. A, Fifty-four plane: large tumor (TU) extends from inferior vena cava (IVC) into right atrium (RA). B, Seventy-one plane: large tumor (TU) extends through right ventricle (RV) into right ventricular infundibulum and reaches just 1 cm below pulmonic valve (arrow). PA, Pulmonary artery; IA, left atrium; AO, aorta.
Echocardiography The transthoracic echocardiograrn demonstrated right atrial and right ventricular dilation. A large tumor filled the right atrial and right ventricular chambers. Transesoph-
ageal echocardiography better delineated the tumor (Figure 1). It appeared that this was a smooth-surfaced mass that entered the right atrium through the inferior vena cava, filled most of the right atrial and right ventricular cavities,
530
Kronzon et al.
Journal of the American Society of Echocardiography September-October 1994
Figure 2 Continuous-wave Doppler, transthoracic. Transtricuspid flow velocity reaches 2.8 m/ sec, indicating maximal diastolic gradient of 31 mm Hg between right atrium and right ventricle. and extended into the right ventricular outflow tract 1 cm below the pulmonic valve. Color-flow Doppler revealed a narrow jet of high-velocity diastolic flow across the small space in the right ventricular inflow tract that was not occupied by the tumor. The velocity of this jet was 2.8 m/sec, and the calculated maximal transtricuspid gradient was 31 mm Hg (mean gradient 25 mm Hg) (Figure 2).
Magnetic Resonance Imaging Magnetic resonance imaging of the heart and abdomen showed a tumor that filled the right atrium and right ventricle (Figure 3). The inferior vena cava was markedly dilated and filled with a large mass that extended superiorly from the iliac veins. A large lobular pelvic mass was also noted. The hepatic veins were dilated and there was evidence of venous congestion of the liver.
Doppler Study of the Lower Extremities There were no Doppler signals at the dorsal pedal pulses bilaterally. A venous study showed dilated veins in the lower extremities and no venous flow signals bilaterally. The leg veins were not compressible. The study was interpreted as consistent with bilateral deep venous thrombosis. The absence of arterial pulses was consistent with a compartmental syndrome caused by acute leg edema.
Surgery On the third hospital day the patient underwent surgery. After cardiopulmonary bypass, a huge tumor was re-
moved from the right-sided cardiac chambers and the entire inferior vena cava. The tumor nearly obstructed both the right ventricular infundibulum and the tricuspid valve orifice and filled the inferior vena cava, which was markedly dilated. The tumor extended into the superior mesenteric vein and both renal veins. The femoral veins appeared to contain thrombus. Extensive amounts of fresh, red-stasis thrombus were removed. At the end of the procedure, the lower extremities were well perfused with bilateral palpable dorsal pedal pulses. The retroperitoneal mass was not excised during this procedure.
Pathologic Findings The excised mass, measuring 25 x 5 x 5 cm, was shaped in the form of a cast of the right-sided heart chambers and the entire inferior vena cava (Figure 4) . There was an attached thrombus that filled the femoral veins. Histologic examination confirmed attachment of the tumar to an acute thrombus and a fragment of a large vein. The tumor showed histopathologic and ultrastructural features of a leiomyoma that was intravascular (intravascular leiomyomatosis). Review of the sections from the patient's previous primary uterine tumor and intracardiac recurrence showed a tumor that was similar in many areas to the latest intravascular lesion_ However, particularly in the original uterine specimen, there were areas with the histologic appearance of low-grade endometrial stromal sarcoma (stromatosis and endolymphatic stromal myomosis), some of which was within lymphatics. The tumor was therefore
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Magnetic resonance imaging. A, Large tumor fills right-sided heart cavities. VTU, Ventricular portion of tumor; A TU, atrial portion of tumor. B, Tumor (TU) fills intrahepatic portion of markedly dilated inferior vena cava.
Figure 3
believed to show mixed types of mesenchymal differentiation. Follow-up
Intraoperative transesophageal echocardiography after removal of the mass showed no residual intracardiac tumor. The transtricuspid flow velocity had returned to normal. A lower extremity Doppler study revealed improved arterial pulses and good deep venous flow signals. On the second postoperative day, the patient's renal function started to deteriorate. Within a few days, the blood urea nitrogen level reached 78 mg/dl and the creatinine 3 mg/dl. The serum creatine phosphokinase level was markedly elevated (16,000 mg/dl). The urine appeared to be brown and contained myoglobin. It was believed that the myoglobinuric renal insufficiency was the result of rhabdomyo-
lysis from lower limb ischemia. The urine output remained stable, and both myoglobinuria and renal insufficiency resolved spontaneously within l week. The postoperative liver function also improved and returned to normal limits within l week. The patient gradually recovered. Surgery of the abdominal mass was performed with the removal of two tumor masses from the pelvis.
DISCUSSION
Review of multiple specimens of the tumor removed from the patient showed mixed features of histologically benign intravascular leiomyomatosis and lowgrade endometrial stromal sarcoma, which has been termed "stromomyoma."1 Intracardiac extension of uterine leiomyoma (in-
532 Kronwn et al.
Figure 4 Huge tumor resected appears like cast of rightsided heart chambers and entire inferior vena cava. Extension into renal vein is noted (clamp). Indentation of entry of inferior vena cava into right atrium is marked by arrow. Note the dark thrombus attached to the inferior pole of the tumor. VTU, Right ventricular portion of tumor; A TU, right atrial portion of tumor; CIV, caval portion oftumor.
travenous leiomyomatosis) is quite rare. In 1984 our laboratory reviewed 13 cases and added one more that was diagnosed by echocardiography and resected successfully.2 Since then, several more cases have been reported. 3-6 The tumors, although histologically benign, can extend into the heart and create cavitary obliteration, tricuspid and pulmonic valve obstruction, pulmonary embolization, pulmonary metastasis, right-sided heart failure, and death. 2 - 13 Benign intravenous leiomyoma rarely recurs after excision. However, the rate of recurrence of lowgrade endometrial stromal sarcoma is high (up to 60% recurrence rate extending over many years). 14 Although in most cases the pathologic distinction between these two entities is straightforward, in some cases differentiation is ambiguous. Zalonsek and Norris 15 suggested that combined smooth-muscle and stromal tumors (stromomyoma) may be more common than previously recognized. However, to our knowledge, recurrent cardiac stromomyoma has not been described previously. Interestingly, the histologic appearance of the recurrent intracardiac mass
Journal of the American Society of Echocardiography September-October 1994
in this patient appeared more mature, with more complete smooth-muscle differentiation. "Benign metastasizing leiomyoma" is defined as a spread of benign leiomyoma to remote organs (usually lungs and lymph nodes) . Usually the pulmonary nodules progress slowly, and their clinical course is benign, 16 as was seen with this patient. Low-grade endometrial stromal sarcoma also metastasizes to the lung and lymph nodes and usually has a benign course. 14 The exact histologic results of the pulmonary findings in this patient are not known. Although the tumor histologic findings were fairly benign, the clinical course was stormy and malignant. Inferior vena caval, right atrial, and right ventricular obstruction resulted in severe elevation of venous pressures that led to peripheral edema, ascites, and hepatic failure. Inferior vena caval and iliac vein occlusion by the tumor led to femoral vein thrombosis. As a result, severe, acute leg edema produced a compartmental syndrome, in which increased pressure within a limited space compromised the arterial circulation to the lower limbs. Limb ischemia resulted in rhabdomyolysis, which led to myoglobinuria and renal failure. Rhabdomyolysis, myoglobinuria, and renal failure are rare, previously described complications of compartmental syndromeY Surgical excision of the tumor and femoral vein embolectomy disrupted the vicious cycle, and cardiovascular, liver, and renal function returned to normal. Echocardiography is useful in the diagnosis of intracardiac leiomyomatosis. 2 •18 However, transthoracic echocardiography may provide only limited or suboptimal imaging of atrial masses. 19 Transesophageal echocardiography provides a higher resolution image with better delineation of the tumor. It can also provide better visualization of the great veins and thus better demonstrate the extent of intravenous tumors. 20 Very frequently the caval origin of the cardiac mass cannot be visualized by transthoracic echocardiography. Podolsky et al. 4 described a case in which transthoracic echocardiography gave the false impression of right atrial myxoma in a patient with intracardiac leiomyomatosis. In that case, transesophageal echocardiography performed during anesthesia revealed that the cardiac tumor emerged from the inferior vena cava, and therefore the strategy of the operation was changed. In this report, transesophageal echocardiography better delineated tl1e tumor. It could demonstrate its exact extension from the inferior vena cava to the right ventricular outflow tract, just proximal to the pulmonic valve. The transtricuspid flow could be demonstrated and its velocity measured.
Journal of rhe American Society of Echocardiography Volume 7 Number 5
The list of masses that may reach the right side of the heart through intravenous growth include tumars such as renal cell carcinoma, hepatoma, adrenal carcinoma, thyroid carcinoma, angiosarcoma, benign leiomyoma, and low-grade stromal endometrial sarcoma. Stromomyoma should be considered in the differential diagnosis of secondary, intravenous, and intracardiac tumors in women. It is of interest that in this case the clinical behavior, as well as the pathologic findings, of a stromomyoma were intermediate between intravascular leiomyomatosis and low-grade endometrial stromal sarcoma. REFERENCES l. Tang CK, Toker C, Ances I. Stromomyoma of the uterus. Cancer 1979;43:308-16. 2. Politzer F, Kronzon I, Wicrorek R, et al. Intracardiac leiomyomatosis: diagnosis and treatment. J Am Coli Cardiol 1984;4:629-34. 3. Suginami H, Kaura R, Ochi H, Matsuura S. Intravenous leiomatosis with cardiac extension: successful surgical management and histopathological smdy. Obstet Gynecol 1990;76:527-9. 4. Podolsky LA, Jacobs LE, Ioli A, Kotler MN. TEE in the diagnosis of intravenous leiomyomatosis extending into the right atrium. Am Heart J 1993;125:1462-4. 5. Shida T, Yoshimura M, Chihara H, Nakamura K. Intravenous leiomyomatosis of the pelvis with reextension into the heart. Ann Thorac Surg 1986;42:104-6. 6. Gonzalez-Lavin L, Lee H, Falk L, et al. Tricuspid valve obstruction due to intravenous leiomyomatosis. Am Heart J 1984;108: 1544-6. 7. Garcia FA, Villanueva RA, Narcisco F, Avenmra AP. Intravenous leiomyomatosis of the uterus and pelvis presenting as a cardiac tumor. Ann Thorac Surg 1986;42:S41-3.
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Correction
With reference to the abstract entitled "New observations of echocardiography regarding QW3600, an ultrasound contrast agent capable of myocardial opacification by intravenous injection" by O.I. Kwan, A. Tarantal, D. Kessler, B. Cotter, N. Dalton, J. Ross, and A. DeMaria (JAM Soc EcHOCARDIOGR 1994; 7(suppl):S30). Dr. John Ross, Jr., wishes it to be noted that his name should not have appeared on this abstract, since he did not review it prior to its submission and was not aware of its contents prior to publication.