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Intracaval and intracardiac leiomyomatosis of uterine origin
Case reports
Intracaval and intracardiac leiomyomatosis of uterine origin Alessandro MAZZOLA, MD, Renato GREGORINI, MD, Beniamino PROCACCINI, MD, Vincenzo MORETTI, MD, Ricardo LUCANTONI, MD, Wilmo LORENZI, MD, Giuseppe DI EUSANIO, MD, Mauro COLOMBATI, MD, Teramo, Italy
lntracaval leiomyomatosis of uterine origin is a rare disease. Extension to the right heart is exceptional. Based on the review of 11 cases reported in the literature and the case presented herein, which was treated successfully, the diagnostic and therapeutic problems are discussed. Diagnosis should be suggested when a female patient operated on previously for myofibroma of the uterus by hysterectomy, presents with a picture of cardiac myxoma. Diagnosis can be confirmed by iliocavogram and computerized tomography of the abdomen. Excision calls for a cardiac procedure under extracorporeal circulation and caval exploration which may be performed either simultaneously or as a two stage procedure. (Ann Vasc Sufg, 1986, 7,134-138). KEY-WORDS : Vena cava tumor.
- Extracorporeal circulation.
Intracaval leiomyomatosis arising from the uterus is a rare disease. We report here a case of leiomyomatosis of the inferior vena cava (IVC) with cardiac involvement. Its successful surgical management, including excision of the right arterial and caval components, is described. CASE REPORT A 44 year-old woman, gravida 2, para 2, underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy in 1979. During the operation, several paraovarian and parauterine nodules on the pelvic floor were removed. A year later, the patient was readmitted to the hospital because of dyspnea on exertion, edema of the lower extremities and intermittent fever and chills. A presumptive diagnosis of acute bacterial endocarditis was made. Three years later the patient was hospitalized again because of persistent symptoms. An echocardiogram demonstrated an intracardiac mass which was thought to be a right atrial myxoma (Fig.1). She was then admitted to our service where a repeat echocardiogram showed an intra-atrial mass protruding through the tricuspid orifice into the right ventricle during diastole.
From the Departments of Cardiac Surgery and Emergency Surgery, Ospedale Civile, Teramo, ITALY. Reprint requests :M . Colomhati, MD, Department of Emergency Surgery, Ospedale Civile, Piazza dltalia, Teramo, 64100 ITALY.
Fig. 1.
- Echocardiogram
: right intra-atrial tumor (*).
The patient was operated on February 2 , 1984, through a median sternotomy, under moderate hypothermia (28 "C) with extracorporeal circulation between the inferior and superior vena cava and the ascending aorta. The heart was arrested by injection of cardioplegic solution at the root of the aorta. A bilobed cylindrical mass was found to extend into the right ventricle through the tricuspid orifice. The main bulk of the tumor originated from the inferior vena cava (IVC) and was separated from the second
VOLUME1 NO 1 - 1986
INTRACAVAL AND INTRACARDIAC LEIOMYOMATOSIS
Fig. 2. - Operative specimen.
-
lliocaval venogram after removal of the proximal Fig. 3. portion of the tumor.
-
L
part by a stalk 1.5 cm long. The mass was white, firm, elastic and smooth on the surface. There were no adhesions between the tumor and the heart or vena cava. Profound hypothermia (esophageal temperature = 18 C) was achieved. Complete cardiac arrest was obtained and the IVC cannula was removed. The cylinder-shaped tumor occupied the entire vessel. The mass was resected by electrocautery down to 4 cm below the ostium of the IVC (Fig. 2). The IVC was then recannulated and perfusion resumed. The patient was rewarmed and cardiac activity was resumed without problems. The postoperative course was uneventful. An inferior vena cavogram was performed on day 14 (Fig. 3) showing that a neoplasm was present in the left external and common iliac veins as well as in the entire infradiaphragmatic vena cava which was dilated.
On April 12, 1984 she was operated through a midline laparotomy. The infrarenal vena cava, common iliac and external iliac veins were identified. The IVC, which had a diameter of 7 cm, the left common iliac vein, which measured 4 cm in diameter, and the normal right common iliac vein were clamped. A 5 cm venotomy was made in the middle third of the infrarenal IVC and the endocaval portion of the tumor was resected. The neoplasm was found to be free of adhesions to the IVC and presented the same gross appearance of the tumor that had been removed during the first operation. The tumor was divided at the level of the left common iliac vein and the venotomy was closed by lateral venorraphy. An intraoperative venogram was done through the left common iliac vein which showed that the tumor arose from the internal iliac vein. The tumor was removed, the hypogastric vein was divided and ligated, and the left common iliac venotomy was sutured. An incidental cholecystectomy for gallstones was performed. The postoperative course was uneventful except for transient edema of the left leg which resolved during the first week. The. patient remained asymptomatic ten months after the operation when a venogram showed a patent left iliac vein and IVC (Fig. 4 a and b). Pathologic examination of the specimen showed leiomyomatous tissue (Fig. 5).
13.5
136
1NTRACAVAL A N D INTRACARDIAC LEIOMYOMATOSIS
Fig. 5.
ANNALS OF VASCULAR SURGERY
- Microphotograph of the specimen.
DISCUSSION Intracaval leiomyomatosis arising from the uterus represents a unique histologic and clinical entity and must be distinguished from primitive leiomyomas of the IVC, which are rare [l].Leiomyomatosis is characterized by the intraluminal extension of a benign tumor which is believed to arise either from an uterine fibromyoma or from the wall of the uterine veins. The main histologic component of these tumors is smooth muscle tissue, which proliferates into the veins of the uterus. Extension into the veins of the broad ligament has been noted in half of the reported cases [2]. Intravenous spread to the IVC may take place through the internal and common iliac veins o r via the ovarian veins [2]. Occasionally it is difficult to differentiate intravenous leiomyomatosis from intravenous leiomyosarcomas. The presence of immature cells, tumor recurrence as pulmonary [3] o r renal [4] metastases suggest a malignant potential although the histologic appearance can be similar to that of << benign leiomyomatoses. These leiomyomatoses with c malignant potential D have however a better prognosis than classic leiomyosarcomas [ 5 ] . Although known since 1761, when Morgani described a tumor of the uterus with c a thick and oblong polyp extending into the vena cava, the right atrium and ventricle D, there have been few reports of intracaval leiomyomatosis. There are less than 50 cases described in the literature [6]. Intracardiac leiomyomatosis is very rare : only 12 cases, including this observation, have been published [6-161. ))
~ i4 a~ and . b. operation.
- lliocaval venogram 10 months after the
The correct diagnosis of intracardiac leiomyomatosis is difficult and On1 once has been made before operation or autopsy r6]. In all the published re-
VOLUME 1 NO 1 - 1986
131
I N T R A C A V AL A N D INTRA C A R D I A C LEIOM Y O M A TOSIS
ports, right heart failure was the main clinical finding. In those patients who had echocardiograms, the findings were consistent with a tumor of the right atrium which was thought to be a myxoma. In Iverson's case (16), thrombosis of the IVC was diagnosed by CT scan. The vena caval origin of the tumor was suggested, but leiomyomatosis was not suspected. Transient edema of the lower extremities has been described in several instances. The sign, often recognized retrospectively, is the only indication of IVC occlusion. Most cases have been managed by a two-stage procedure. The first operation, conducted under extracorporeal circulation, is the removal of the intracardiac and the upper vena caval portions of the tumor. In a second stage, an abdominal operation is done to remove the remaining portion of the tumor. The literature indicates that this second stage has not been performed routinely [14] and has not always been necessary. The first stage for the excision of intracardiac tumor must adhere to the principles of sur ical management of tumors of the terminal IVC [17f In two cases [11, 151, the removal of the entire tumor was attempted via the transatrial route. In one of these cases, this led to intraoperative death presumably caused by a tear in the IVC at the level of the renal veins [15]. This complication was probably secondary to dense adhesions between the tumor and the wall of the IVC as was observed in the case reported by Jonasson [4]. In such cases, the ve-
notomy should be more generous than the one done in our case in order to remove the tumor safely. Ligation of the hypogastric veins has been performed in all cases and it may avoid recurrence of tumor. Cameron et al. [6] have suggested the possibility of treatment based on the hormonal dependence of leiomyomatosis. According to these authors, management should include either bilateral oophorectomy in cases of intraoperative diagnosis or treatment by tamoxifen if the adnexa are left in place. However, in the case reported here, the previous bilateral oophorectomy did not prevent the growth of the tumor. CONCLUSION In spite of its rarity, the diagnosis of leiomyomatosis of the vena cava with cardiac involvement should be suggested when a female patient with a previous hysterectomy for fibromyoma presents with a clinical picture of intra-atrial myxoma. If leiomyomatosis is suspected, confirmation requires iliocaval venography and computerized tomography of the abdomen. Because of possible adhesions between the tumor and the vena caval wall, leiomyomatosis of the vena cava with cardiac involvement should be treated by a two-staged procedure.
REFERENCES I . MANDELBAUM I., PAULE'ITO F.J., NASSER W.K. -Resection 2. 3. 4.
5.
6. 7. 8. 9. 10. 11.
of a leiomyoma of the inferior vena cava that produced tricuspid valvular obstruction. J Thoruc Curdiovasc Surg, 1974, 67, 561-567. EDWARDS D.L., PEACOCK J.F. - Intravenous lciomyomatosis of the uterus. Obstet Gynecol, 1966,27, 176-181. KAKU K., KAWASHIMA Y., KITAMURA S. et al. - Resection of leiomyosarcoma originating in internal iliac vein and ascending into heart via inferior vena cava. Surgery, 1981, 89, 604-611. JONASSON O., PRITCHARD J., LONG L. - Intraluminal leiomyosarcoma of the inferior vena cava. Cancer, 1966,lY. 1311-1315. KIEFFER E . , BERROD J.L., CHOMETTE G. -Primary tumors of the inferior vena cava. In :BERGAN J.J., YAO J.S.T. -Surgery of the Verns, pp. 422-423. New York, Grune and Stratton, 1985. CAMERON A.E.P., GRAHAM J.C., COTTON L.T. - Intracaval leiomyomatosis. Br J Obster Gynaecol, 1983, 90, 272-275 DURCK H. - Citi. par Edwards [ 2 ] . IIORMANN K. -Cite par Edwards [Z]. PETROWS N. - CitC par Edwards (21. NORRIS H.J., PARMLEY T. - Mesenchymal tumor of the uterus (V). Intravenous leiomyomatosis. Cancer, 1976,36, 2164-2178. BROADBENT J.C., TAJIK A.J., WALLACE R.B. -Thrombus of inferior vena cava presenting as right atrial tumor. J Thorac CardioV R S C Surg, 1976, 72, 422-426.
12. KONO N., TANAKA S . . SHIORI Y. et al. - Removal of a glant
13. 14. 15.
16. 17.
18.
leiomyoma of thc vein propagating into the inferior vena cava. right atrium and right ventricle which led to tricuspid stenosis. J Jpn Asroc Thorac Surg, 1978,26, 1113-1122. TIERNEY W.M., EHRLICH C.E., BAILEY J.C. et al. - Intravenous leiomyomatosis of the uterus with extension into the heart. A m J Med, 1980, 69, 471-475. TINNIS A.D., SMALLPEICE C., DAVIES A.C. et al. - Intracardiac spread of intravenous leiomyomatosis with succcssful surgical excision. N EngiJ Med, 1980.303, 1043-1044. NIL1 M., LIBAN E., LEVY M.J. - Tricuspid stenosis due to intravenous leiomyomatosis : a call for caution. Texas Heart Insr J , 1982. Y, 231-235. IVERSON L.I.G., LEE J . , DREW D. et al. - Intravenous leiomyomatosis with cardiac extension. Texas Heart fnsr J . 1983, 10, 275278. SCHECHTER D.C. - Cardiovascular surgery in the managemcnt of exogenous tumors involving the vena cava. In : BERGAN J.J., YAO J.S.T. - Surgery of rhe Veins, pp. 393-412. New York. Grune and Stratton, 1985. RICHARD T., TAWIL N., BACHET J. et al. - Utilisation de la circulation extra-corporelle dans la chirurgie de la veine cave infericure terminale. In : KIEFFER E. - Chirurgie de la Veine Cuve Infgrieure et de ses brunches, pp. 69-81. Paris, Expansion Scientifiquc Franqaise. 1985.
Spanish summary on the next page
138
INTRACA V A L A N D INTRACARDIAC 1,EIOMYOMATOSIS
MAZZOLA A , , GREGORINI R., PROCACCINI B . , MORETTI V., LUCANTONI R., LOREN21 W . , DI EUSANIO G . , COLOMBATI M. - Leyomioma intracava e intracardiaco de origen uterino. Ann Vasc Surg, 1986, 1, 134.138.
RESUMEN : El leiomiosarcoma intracava de origen uterino es una enfermedad rara, que puede presentar especiales caracteristicas de complejidad y gravedad segun la extension del mismo. Se describe el caso de una mujer de 44 aiios que tras practicarle una histerectomia total con ovariectomia bilateral, present6 a1 ano clinica cardiaca etiquetada de endocarditis bacteriana aguda, y a 10s tres se le diagnostic6 un mixoma en auricula derecha. Con dicho diagnostico se intervino quirurgicamente apreciando en la auricula una formacion blanquecina, cilindrica y consistente, que provenia de la cava inferior y se prolongaba hasta el ventriculo derecho sin adherirse a las paredes vasculares. Se efectuo la reseccion a
ANNALS OF VASCULAR SURGERY
nivel del corazon. En el postoperatorio se practic6 estudio flebografico apreciando el origen de la masa en las venas iliaca externa y primitiva izquierda. A travCs de una laparotomia se realizo la extirpacion del tumor a nivel de la cava inferior y venas iliacas primitiva y externa izquierdas, con ligadura de la hipogastrica. El postoperatorio y seguimiento hasta 10s 10 meses de evolution ha sido correct0 y sin incidentes. El estudio anatomopatoi6gico diagnostic6 un leiomiosarcoma en todos 10s niveles extirpados. Deben diferenciarse 10s leiomiosarcomas intracava de origen uterino de 10s primitivos de cava inferior. Se caracterizan por estar constituidos por fibra muscular lisa originada en las venas uterinas o en fibromas uterinos. La posibilidad de metastasis renales y pulmonares, de recidivas y de poseer celulas inmaduras pueden evocar una cierta malignidad, per0 de hecho el pronostico suele ser benigno.
...
Intracaval and intracardiac leiomyomatosis of uterine origin Alessandro MAZZOLA, MD, Renato GREGORINI, MD, Beniamino PROCACCINI, MD, Vincenzo MORETTI, MD, Ricardo LUCANTONI, MD, Wilmo LORENZI, MD, Giuseppe DI EUSANIO, MD, Mauro COLOMBATI, MD, Teramo, Italy
lntracaval leiomyomatosis of uterine origin is a rare disease. Extension to the right heart is exceptional. Based on the review of 11 cases reported in the literature and the case presented herein, which was treated successfully, the diagnostic and therapeutic problems are discussed. Diagnosis should be suggested when a female patient operated on previously for myofibroma of the uterus by hysterectomy, presents with a picture of cardiac myxoma. Diagnosis can be confirmed by iliocavogram and computerized tomography of the abdomen. Excision calls for a cardiac procedure under extracorporeal circulation and caval exploration which may be performed either simultaneously or as a two stage procedure. (Ann Vasc Sufg, 1986, 7,134-138). KEY-WORDS : Vena cava tumor.
- Extracorporeal circulation.
Intracaval leiomyomatosis arising from the uterus is a rare disease. We report here a case of leiomyomatosis of the inferior vena cava (IVC) with cardiac involvement. Its successful surgical management, including excision of the right arterial and caval components, is described. CASE REPORT A 44 year-old woman, gravida 2, para 2, underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy in 1979. During the operation, several paraovarian and parauterine nodules on the pelvic floor were removed. A year later, the patient was readmitted to the hospital because of dyspnea on exertion, edema of the lower extremities and intermittent fever and chills. A presumptive diagnosis of acute bacterial endocarditis was made. Three years later the patient was hospitalized again because of persistent symptoms. An echocardiogram demonstrated an intracardiac mass which was thought to be a right atrial myxoma (Fig.1). She was then admitted to our service where a repeat echocardiogram showed an intra-atrial mass protruding through the tricuspid orifice into the right ventricle during diastole.
From the Departments of Cardiac Surgery and Emergency Surgery, Ospedale Civile, Teramo, ITALY. Reprint requests :M . Colomhati, MD, Department of Emergency Surgery, Ospedale Civile, Piazza dltalia, Teramo, 64100 ITALY.
Fig. 1.
- Echocardiogram
: right intra-atrial tumor (*).
The patient was operated on February 2 , 1984, through a median sternotomy, under moderate hypothermia (28 "C) with extracorporeal circulation between the inferior and superior vena cava and the ascending aorta. The heart was arrested by injection of cardioplegic solution at the root of the aorta. A bilobed cylindrical mass was found to extend into the right ventricle through the tricuspid orifice. The main bulk of the tumor originated from the inferior vena cava (IVC) and was separated from the second
VOLUME1 NO 1 - 1986
INTRACAVAL AND INTRACARDIAC LEIOMYOMATOSIS
Fig. 2. - Operative specimen.
-
lliocaval venogram after removal of the proximal Fig. 3. portion of the tumor.
-
L
part by a stalk 1.5 cm long. The mass was white, firm, elastic and smooth on the surface. There were no adhesions between the tumor and the heart or vena cava. Profound hypothermia (esophageal temperature = 18 C) was achieved. Complete cardiac arrest was obtained and the IVC cannula was removed. The cylinder-shaped tumor occupied the entire vessel. The mass was resected by electrocautery down to 4 cm below the ostium of the IVC (Fig. 2). The IVC was then recannulated and perfusion resumed. The patient was rewarmed and cardiac activity was resumed without problems. The postoperative course was uneventful. An inferior vena cavogram was performed on day 14 (Fig. 3) showing that a neoplasm was present in the left external and common iliac veins as well as in the entire infradiaphragmatic vena cava which was dilated.
On April 12, 1984 she was operated through a midline laparotomy. The infrarenal vena cava, common iliac and external iliac veins were identified. The IVC, which had a diameter of 7 cm, the left common iliac vein, which measured 4 cm in diameter, and the normal right common iliac vein were clamped. A 5 cm venotomy was made in the middle third of the infrarenal IVC and the endocaval portion of the tumor was resected. The neoplasm was found to be free of adhesions to the IVC and presented the same gross appearance of the tumor that had been removed during the first operation. The tumor was divided at the level of the left common iliac vein and the venotomy was closed by lateral venorraphy. An intraoperative venogram was done through the left common iliac vein which showed that the tumor arose from the internal iliac vein. The tumor was removed, the hypogastric vein was divided and ligated, and the left common iliac venotomy was sutured. An incidental cholecystectomy for gallstones was performed. The postoperative course was uneventful except for transient edema of the left leg which resolved during the first week. The. patient remained asymptomatic ten months after the operation when a venogram showed a patent left iliac vein and IVC (Fig. 4 a and b). Pathologic examination of the specimen showed leiomyomatous tissue (Fig. 5).
13.5
136
1NTRACAVAL A N D INTRACARDIAC LEIOMYOMATOSIS
Fig. 5.
ANNALS OF VASCULAR SURGERY
- Microphotograph of the specimen.
DISCUSSION Intracaval leiomyomatosis arising from the uterus represents a unique histologic and clinical entity and must be distinguished from primitive leiomyomas of the IVC, which are rare [l].Leiomyomatosis is characterized by the intraluminal extension of a benign tumor which is believed to arise either from an uterine fibromyoma or from the wall of the uterine veins. The main histologic component of these tumors is smooth muscle tissue, which proliferates into the veins of the uterus. Extension into the veins of the broad ligament has been noted in half of the reported cases [2]. Intravenous spread to the IVC may take place through the internal and common iliac veins o r via the ovarian veins [2]. Occasionally it is difficult to differentiate intravenous leiomyomatosis from intravenous leiomyosarcomas. The presence of immature cells, tumor recurrence as pulmonary [3] o r renal [4] metastases suggest a malignant potential although the histologic appearance can be similar to that of << benign leiomyomatoses. These leiomyomatoses with c malignant potential D have however a better prognosis than classic leiomyosarcomas [ 5 ] . Although known since 1761, when Morgani described a tumor of the uterus with c a thick and oblong polyp extending into the vena cava, the right atrium and ventricle D, there have been few reports of intracaval leiomyomatosis. There are less than 50 cases described in the literature [6]. Intracardiac leiomyomatosis is very rare : only 12 cases, including this observation, have been published [6-161. ))
~ i4 a~ and . b. operation.
- lliocaval venogram 10 months after the
The correct diagnosis of intracardiac leiomyomatosis is difficult and On1 once has been made before operation or autopsy r6]. In all the published re-
VOLUME 1 NO 1 - 1986
131
I N T R A C A V AL A N D INTRA C A R D I A C LEIOM Y O M A TOSIS
ports, right heart failure was the main clinical finding. In those patients who had echocardiograms, the findings were consistent with a tumor of the right atrium which was thought to be a myxoma. In Iverson's case (16), thrombosis of the IVC was diagnosed by CT scan. The vena caval origin of the tumor was suggested, but leiomyomatosis was not suspected. Transient edema of the lower extremities has been described in several instances. The sign, often recognized retrospectively, is the only indication of IVC occlusion. Most cases have been managed by a two-stage procedure. The first operation, conducted under extracorporeal circulation, is the removal of the intracardiac and the upper vena caval portions of the tumor. In a second stage, an abdominal operation is done to remove the remaining portion of the tumor. The literature indicates that this second stage has not been performed routinely [14] and has not always been necessary. The first stage for the excision of intracardiac tumor must adhere to the principles of sur ical management of tumors of the terminal IVC [17f In two cases [11, 151, the removal of the entire tumor was attempted via the transatrial route. In one of these cases, this led to intraoperative death presumably caused by a tear in the IVC at the level of the renal veins [15]. This complication was probably secondary to dense adhesions between the tumor and the wall of the IVC as was observed in the case reported by Jonasson [4]. In such cases, the ve-
notomy should be more generous than the one done in our case in order to remove the tumor safely. Ligation of the hypogastric veins has been performed in all cases and it may avoid recurrence of tumor. Cameron et al. [6] have suggested the possibility of treatment based on the hormonal dependence of leiomyomatosis. According to these authors, management should include either bilateral oophorectomy in cases of intraoperative diagnosis or treatment by tamoxifen if the adnexa are left in place. However, in the case reported here, the previous bilateral oophorectomy did not prevent the growth of the tumor. CONCLUSION In spite of its rarity, the diagnosis of leiomyomatosis of the vena cava with cardiac involvement should be suggested when a female patient with a previous hysterectomy for fibromyoma presents with a clinical picture of intra-atrial myxoma. If leiomyomatosis is suspected, confirmation requires iliocaval venography and computerized tomography of the abdomen. Because of possible adhesions between the tumor and the vena caval wall, leiomyomatosis of the vena cava with cardiac involvement should be treated by a two-staged procedure.
REFERENCES I . MANDELBAUM I., PAULE'ITO F.J., NASSER W.K. -Resection 2. 3. 4.
5.
6. 7. 8. 9. 10. 11.
of a leiomyoma of the inferior vena cava that produced tricuspid valvular obstruction. J Thoruc Curdiovasc Surg, 1974, 67, 561-567. EDWARDS D.L., PEACOCK J.F. - Intravenous lciomyomatosis of the uterus. Obstet Gynecol, 1966,27, 176-181. KAKU K., KAWASHIMA Y., KITAMURA S. et al. - Resection of leiomyosarcoma originating in internal iliac vein and ascending into heart via inferior vena cava. Surgery, 1981, 89, 604-611. JONASSON O., PRITCHARD J., LONG L. - Intraluminal leiomyosarcoma of the inferior vena cava. Cancer, 1966,lY. 1311-1315. KIEFFER E . , BERROD J.L., CHOMETTE G. -Primary tumors of the inferior vena cava. In :BERGAN J.J., YAO J.S.T. -Surgery of the Verns, pp. 422-423. New York, Grune and Stratton, 1985. CAMERON A.E.P., GRAHAM J.C., COTTON L.T. - Intracaval leiomyomatosis. Br J Obster Gynaecol, 1983, 90, 272-275 DURCK H. - Citi. par Edwards [ 2 ] . IIORMANN K. -Cite par Edwards [Z]. PETROWS N. - CitC par Edwards (21. NORRIS H.J., PARMLEY T. - Mesenchymal tumor of the uterus (V). Intravenous leiomyomatosis. Cancer, 1976,36, 2164-2178. BROADBENT J.C., TAJIK A.J., WALLACE R.B. -Thrombus of inferior vena cava presenting as right atrial tumor. J Thorac CardioV R S C Surg, 1976, 72, 422-426.
12. KONO N., TANAKA S . . SHIORI Y. et al. - Removal of a glant
13. 14. 15.
16. 17.
18.
leiomyoma of thc vein propagating into the inferior vena cava. right atrium and right ventricle which led to tricuspid stenosis. J Jpn Asroc Thorac Surg, 1978,26, 1113-1122. TIERNEY W.M., EHRLICH C.E., BAILEY J.C. et al. - Intravenous leiomyomatosis of the uterus with extension into the heart. A m J Med, 1980, 69, 471-475. TINNIS A.D., SMALLPEICE C., DAVIES A.C. et al. - Intracardiac spread of intravenous leiomyomatosis with succcssful surgical excision. N EngiJ Med, 1980.303, 1043-1044. NIL1 M., LIBAN E., LEVY M.J. - Tricuspid stenosis due to intravenous leiomyomatosis : a call for caution. Texas Heart Insr J , 1982. Y, 231-235. IVERSON L.I.G., LEE J . , DREW D. et al. - Intravenous leiomyomatosis with cardiac extension. Texas Heart fnsr J . 1983, 10, 275278. SCHECHTER D.C. - Cardiovascular surgery in the managemcnt of exogenous tumors involving the vena cava. In : BERGAN J.J., YAO J.S.T. - Surgery of rhe Veins, pp. 393-412. New York. Grune and Stratton, 1985. RICHARD T., TAWIL N., BACHET J. et al. - Utilisation de la circulation extra-corporelle dans la chirurgie de la veine cave infericure terminale. In : KIEFFER E. - Chirurgie de la Veine Cuve Infgrieure et de ses brunches, pp. 69-81. Paris, Expansion Scientifiquc Franqaise. 1985.
Spanish summary on the next page
138
INTRACA V A L A N D INTRACARDIAC 1,EIOMYOMATOSIS
MAZZOLA A , , GREGORINI R., PROCACCINI B . , MORETTI V., LUCANTONI R., LOREN21 W . , DI EUSANIO G . , COLOMBATI M. - Leyomioma intracava e intracardiaco de origen uterino. Ann Vasc Surg, 1986, 1, 134.138.
RESUMEN : El leiomiosarcoma intracava de origen uterino es una enfermedad rara, que puede presentar especiales caracteristicas de complejidad y gravedad segun la extension del mismo. Se describe el caso de una mujer de 44 aiios que tras practicarle una histerectomia total con ovariectomia bilateral, present6 a1 ano clinica cardiaca etiquetada de endocarditis bacteriana aguda, y a 10s tres se le diagnostic6 un mixoma en auricula derecha. Con dicho diagnostico se intervino quirurgicamente apreciando en la auricula una formacion blanquecina, cilindrica y consistente, que provenia de la cava inferior y se prolongaba hasta el ventriculo derecho sin adherirse a las paredes vasculares. Se efectuo la reseccion a
ANNALS OF VASCULAR SURGERY
nivel del corazon. En el postoperatorio se practic6 estudio flebografico apreciando el origen de la masa en las venas iliaca externa y primitiva izquierda. A travCs de una laparotomia se realizo la extirpacion del tumor a nivel de la cava inferior y venas iliacas primitiva y externa izquierdas, con ligadura de la hipogastrica. El postoperatorio y seguimiento hasta 10s 10 meses de evolution ha sido correct0 y sin incidentes. El estudio anatomopatoi6gico diagnostic6 un leiomiosarcoma en todos 10s niveles extirpados. Deben diferenciarse 10s leiomiosarcomas intracava de origen uterino de 10s primitivos de cava inferior. Se caracterizan por estar constituidos por fibra muscular lisa originada en las venas uterinas o en fibromas uterinos. La posibilidad de metastasis renales y pulmonares, de recidivas y de poseer celulas inmaduras pueden evocar una cierta malignidad, per0 de hecho el pronostico suele ser benigno.
...