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Late Intracaval and Intracardiac Leiomyomatosis Following Hysterectomy for Benign Myomas Treated by Surgery and GnRH Agonist
Gynecologic Oncology 83, 422– 423 (2001) doi:10.1006/gyno.2001.6389, available online at http://www.idealibrary.com on
CASE REPORT Late Intracaval and Intracardiac Leiomyomatosis Following Hysterectomy for Benign Myomas Treated by Surgery and GnRH Agonist Philippe Morice, M.D.,* ,1 Alain Chapelier, M.D.,† Philippe Dartevelle, M.D.,† Damienne Castaigne, M.D.,* and Catherine Lhomme´, M.D.‡ *Department of Gynecologic Surgery and ‡Department of Medical Oncology, Institut Gustave Roussy, 94805 Villejuif Ce´dex, France; and †Department of Cardiothoracic Surgery, Hopital Marie-Lannelongue, Le Plessis-Robinson, France Received June 25, 2001
extension discovered after hysterectomy and successfully treated by single-stage surgery.
Background. The aim of this study was to report an exceptional case of a patient presenting with intracaval and intracardiac leiomyomatosis treated by combined surgical and medical treatment. Case. A 48-year-old presented with intracaval and intracardiac leiomyomatosis (IL) discovered 6 years following a total hysterectomy with ovarian conservation for myomas. Surgical resection of the pelvic myomas and intracaval leiomyomatosis was performed during the same surgical procedure. Given the presence of a small tumor residuum in the pelvic cavity, postoperative medical treatment based on a gonadotropin-releasing hormone (GnRH) agonist was delivered for 1 year. The patient was followed-up using clinical examination and systematic CT scan. Ten months following the end of medical treatment, she is still in good health and the pelvic residuum has stabilized. Conclusions. Patients with pelvic tumor combined with IL could be treated using a one-stage surgical procedure. In cases of incomplete surgical resection, medical treatment based on GnRH agonist could be successfully delivered. © 2001 Academic Press Key Words: leiomyomatosis; intracaval; computed tomography; surgery; GnRH agonist.
CASE REPORT
INTRODUCTION
A 48-year-old nulliparous patient underwent, in 1987, a myomectomy via a laparotomy for benign leiomyomas. In 1993, a hysterectomy with adnexal conservation was performed for recurrent myomas. Six years later, she complained of dyspnea. Cardiomegaly was found on chest X ray. Ultrasonic cardiography revealed caval thrombosis with extension of the thrombus up to the right atrium. A pelvic tumor with extension to the vena cava and pulmonary embolism were found during thoracoabdominopelvic computed tomography (CT) on pulmonary veins. The pelvic tumor was polylobed and the masses were solid and homogeneous. The patient was referred to our institution for treatment of the tumor. Biopsies were performed on the pelvic tumor and on the intracaval thrombus (using an endovascular procedure) to establish the histological diagnosis of the pelvic tumor. The definitive histologic diagnosis was a benign leiomyoma (without mitosis) associated with intracaval and intracardiac leiomyomatosis (IL). A surgical procedure was performed via a laparotomy and a sternotomy. During the surgical exploration we discovered a 15-cm multilobar pelvic tumor involving the right hypogastric vein. Both ovaries were normal. The tumor extended through the right hypogastric vein up to the level of the right atrium. A bilateral salpingo-oophorectomy was carried out and the pelvic tumor was removed with difficulty because of severe adhesions to the rectum and the bladder. A 5-cm residual mass attached to the rectal wall was not removed to avoid bowel resection. The right hypogastric and common iliac veins were resected. These vessels were not replaced. The intracaval and intracar-
Intravenous leiomyomatosis is a rare tumor defined as benign smooth cell neoplasia in the veins. In most cases, only the pelvic veins are implicated but some cases of intracaval and intracardiac involvement have been reported [1–3]. This type of tumor coexists with benign uterine myoma or occurs following hysterectomy for myoma [3]. We present a case of a pelvic tumor (leiomyomatosis) with intracaval and intracardiac 1 To whom reprint requests should be addressed at Institut Gustave Roussy, 39 rue Camille Desmoulins, 94805 Villejuif Ce´dex, France. Fax: 33.1.42.11.52.13. E-mail: [email protected].
0090-8258/01 $35.00 Copyright © 2001 by Academic Press All rights of reproduction in any form reserved.
422
423
CASE REPORT
diac leiomyoma was completely resected using cavotomy and atriotomy under extracorporeal circulation. The postoperative course was uneventful and the patient was discharged with anticoagulant medication. The histology of the resected lesions was that of a benign smooth cell tumor with no mitotic activity nor any histological aspect of malignancy. Given the presence of a small tumor residuum in the pelvic cavity, postoperative medical treatment based on a gonadotropin-releasing hormone analogue (GnRH) (leuproreline, Enantone, 3.75 mg/month, Takeda, Tokyo) was prescribed over 12 months. The patient underwent a clinical examination and CT scan (every 4 months) during follow-up. Twenty-four months after the surgical procedure and 10 months after the interruption of GnRH treatment, she is still in good health and the pelvic residuum has stabilized (6 cm). DISCUSSION This report is original for several reasons. Most of the patients with IL reported in the literature still had their uterus with myomas in place. Only about 15 patients have been reported with IL following a previous history of hysterectomy for benign myomas [3]. In most of these cases, as in ours, ovarian conservation had been performed during the initial hysterectomy. However, the interval between the hysterectomy and IL is often short (⬍4 years). Only a few cases had a long interval (⬎5 years), as in our patient [1, 3]. Several other diagnoses could have been evoked according to the initial presentation of our patient: ovarian tumor with intracaval thrombosis or pelvic sarcoma. In such cases initial treatment with neoadjuvant chemotherapy could be discussed. However, such treatment should only be delivered after histologic confirmation of malignancy. When IL is suspected, initial surgery is mandatory. The diagnosis can be confirmed with this surgical procedure and the pelvic and intracaval tumor can be resected. In the literature, this surgical procedure was often performed in two stages (cardiothoracic surgery followed by abdominal surgery) [4]. Only a few reports have described single-stage surgery [1, 3]. This kind of management avoids the potential risk associated with repeat anesthesia for a second procedure. The one-stage procedure can only be performed by two teams: one trained in cardiothoracic surgery and the other trained to oncologic abdominopelvic surgery. In the case of our patient, we could have performed a bowel resection to achieve a complete resection of the pelvic tumor.
This procedure was not carried out in the present case in order to decrease the risk of infectious complications following sternotomy and atriotomy due to the potential bowel surgery. In such cases, when resection is incomplete or when operative intervention is declined or unfeasible, adjuvant hormonal treatment could be discussed [2, 5–7]. This treatment could be logically proposed because in most cases, leiomyomatosis tumors were estrogen receptor positive. Lo and Lau proposed the introduction of tamoxifen in patients with intracardiac leiomyomatosis who declined surgical treatment [2]. But the risk of thromobosis is increased using such treatment. In our cases we used GnRH agonist. Hameleers et al. and Mitsuhashi et al. also used this treatment in this context [5, 6]. Mitsuhashi et al. proposed this treatment in a patient with pelvic residual mass [5]. But the residual tumor began to enlarge immediately after the cessation of GnRH treatment [6]. In the case reported by Hameleers et al., the patient was treated for a period of 3 years and was in a stable situation [5]. In our case, the patient received agonist treatment for only 1 year. Ten months following the cessation of this treatment, residual disease is asymptomatic and its size is radiologically stable. ACKNOWLEDGMENT We thank Lorna Saint Ange for editing the manuscript.
REFERENCES 1. Cooper MM, Guillem J, Dalton J, Marboe CC, Corwin S, Todd GJ, Rose EA. Recurrent intravenous leiomyomatosis with cardiac extension. Ann Thorac Surg 1992;53:139 – 41. 2. Lo KW, Lau TK. Intracardiac leiomyomatosis. Case report and literature review. Arch Gynecol Obstet 2001;264:209 –10. 3. Le Bouedec G, Bailly C, Penault-Llorca F, Fonck Y, Dauplat J. Intravascular leiomyomatosis of uterine origin. A case of pseudo-metastatic cavocardial thrombus. Presse Med 1999;28:1463–5. 4. Suginami H, Kaura R, Ochi H, Matsuura S. Intravenous leiomyomatosis with cardiac extension: successful surgical management and histopathologic study. Obstet Gynecol 1990;76:527–9. 5. Hameleers JA, Zeebregts CJ, Hamerlijnck RP, Elbers JR, Hameeteman TM. Combined surgical and medical approach to intravenous leiomyomatosis with cardiac extension. Acta Chir Belg 1999;99:92– 4. 6. Mitsuhashi A, Nagai Y, Sugita M, Nakajima N, Sekiya S. GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report. J Reprod Med 1999;44:883– 6. 7. Tresukosol D, Kudelka AP, Malpica A, Varma DG, Edwards CL, Kavanagh JJ. Leuprolide acetate and intravascular leiomyomatosis. Obstet Gynecol 1995;86:688 –92.
CASE REPORT Late Intracaval and Intracardiac Leiomyomatosis Following Hysterectomy for Benign Myomas Treated by Surgery and GnRH Agonist Philippe Morice, M.D.,* ,1 Alain Chapelier, M.D.,† Philippe Dartevelle, M.D.,† Damienne Castaigne, M.D.,* and Catherine Lhomme´, M.D.‡ *Department of Gynecologic Surgery and ‡Department of Medical Oncology, Institut Gustave Roussy, 94805 Villejuif Ce´dex, France; and †Department of Cardiothoracic Surgery, Hopital Marie-Lannelongue, Le Plessis-Robinson, France Received June 25, 2001
extension discovered after hysterectomy and successfully treated by single-stage surgery.
Background. The aim of this study was to report an exceptional case of a patient presenting with intracaval and intracardiac leiomyomatosis treated by combined surgical and medical treatment. Case. A 48-year-old presented with intracaval and intracardiac leiomyomatosis (IL) discovered 6 years following a total hysterectomy with ovarian conservation for myomas. Surgical resection of the pelvic myomas and intracaval leiomyomatosis was performed during the same surgical procedure. Given the presence of a small tumor residuum in the pelvic cavity, postoperative medical treatment based on a gonadotropin-releasing hormone (GnRH) agonist was delivered for 1 year. The patient was followed-up using clinical examination and systematic CT scan. Ten months following the end of medical treatment, she is still in good health and the pelvic residuum has stabilized. Conclusions. Patients with pelvic tumor combined with IL could be treated using a one-stage surgical procedure. In cases of incomplete surgical resection, medical treatment based on GnRH agonist could be successfully delivered. © 2001 Academic Press Key Words: leiomyomatosis; intracaval; computed tomography; surgery; GnRH agonist.
CASE REPORT
INTRODUCTION
A 48-year-old nulliparous patient underwent, in 1987, a myomectomy via a laparotomy for benign leiomyomas. In 1993, a hysterectomy with adnexal conservation was performed for recurrent myomas. Six years later, she complained of dyspnea. Cardiomegaly was found on chest X ray. Ultrasonic cardiography revealed caval thrombosis with extension of the thrombus up to the right atrium. A pelvic tumor with extension to the vena cava and pulmonary embolism were found during thoracoabdominopelvic computed tomography (CT) on pulmonary veins. The pelvic tumor was polylobed and the masses were solid and homogeneous. The patient was referred to our institution for treatment of the tumor. Biopsies were performed on the pelvic tumor and on the intracaval thrombus (using an endovascular procedure) to establish the histological diagnosis of the pelvic tumor. The definitive histologic diagnosis was a benign leiomyoma (without mitosis) associated with intracaval and intracardiac leiomyomatosis (IL). A surgical procedure was performed via a laparotomy and a sternotomy. During the surgical exploration we discovered a 15-cm multilobar pelvic tumor involving the right hypogastric vein. Both ovaries were normal. The tumor extended through the right hypogastric vein up to the level of the right atrium. A bilateral salpingo-oophorectomy was carried out and the pelvic tumor was removed with difficulty because of severe adhesions to the rectum and the bladder. A 5-cm residual mass attached to the rectal wall was not removed to avoid bowel resection. The right hypogastric and common iliac veins were resected. These vessels were not replaced. The intracaval and intracar-
Intravenous leiomyomatosis is a rare tumor defined as benign smooth cell neoplasia in the veins. In most cases, only the pelvic veins are implicated but some cases of intracaval and intracardiac involvement have been reported [1–3]. This type of tumor coexists with benign uterine myoma or occurs following hysterectomy for myoma [3]. We present a case of a pelvic tumor (leiomyomatosis) with intracaval and intracardiac 1 To whom reprint requests should be addressed at Institut Gustave Roussy, 39 rue Camille Desmoulins, 94805 Villejuif Ce´dex, France. Fax: 33.1.42.11.52.13. E-mail: [email protected].
0090-8258/01 $35.00 Copyright © 2001 by Academic Press All rights of reproduction in any form reserved.
422
423
CASE REPORT
diac leiomyoma was completely resected using cavotomy and atriotomy under extracorporeal circulation. The postoperative course was uneventful and the patient was discharged with anticoagulant medication. The histology of the resected lesions was that of a benign smooth cell tumor with no mitotic activity nor any histological aspect of malignancy. Given the presence of a small tumor residuum in the pelvic cavity, postoperative medical treatment based on a gonadotropin-releasing hormone analogue (GnRH) (leuproreline, Enantone, 3.75 mg/month, Takeda, Tokyo) was prescribed over 12 months. The patient underwent a clinical examination and CT scan (every 4 months) during follow-up. Twenty-four months after the surgical procedure and 10 months after the interruption of GnRH treatment, she is still in good health and the pelvic residuum has stabilized (6 cm). DISCUSSION This report is original for several reasons. Most of the patients with IL reported in the literature still had their uterus with myomas in place. Only about 15 patients have been reported with IL following a previous history of hysterectomy for benign myomas [3]. In most of these cases, as in ours, ovarian conservation had been performed during the initial hysterectomy. However, the interval between the hysterectomy and IL is often short (⬍4 years). Only a few cases had a long interval (⬎5 years), as in our patient [1, 3]. Several other diagnoses could have been evoked according to the initial presentation of our patient: ovarian tumor with intracaval thrombosis or pelvic sarcoma. In such cases initial treatment with neoadjuvant chemotherapy could be discussed. However, such treatment should only be delivered after histologic confirmation of malignancy. When IL is suspected, initial surgery is mandatory. The diagnosis can be confirmed with this surgical procedure and the pelvic and intracaval tumor can be resected. In the literature, this surgical procedure was often performed in two stages (cardiothoracic surgery followed by abdominal surgery) [4]. Only a few reports have described single-stage surgery [1, 3]. This kind of management avoids the potential risk associated with repeat anesthesia for a second procedure. The one-stage procedure can only be performed by two teams: one trained in cardiothoracic surgery and the other trained to oncologic abdominopelvic surgery. In the case of our patient, we could have performed a bowel resection to achieve a complete resection of the pelvic tumor.
This procedure was not carried out in the present case in order to decrease the risk of infectious complications following sternotomy and atriotomy due to the potential bowel surgery. In such cases, when resection is incomplete or when operative intervention is declined or unfeasible, adjuvant hormonal treatment could be discussed [2, 5–7]. This treatment could be logically proposed because in most cases, leiomyomatosis tumors were estrogen receptor positive. Lo and Lau proposed the introduction of tamoxifen in patients with intracardiac leiomyomatosis who declined surgical treatment [2]. But the risk of thromobosis is increased using such treatment. In our cases we used GnRH agonist. Hameleers et al. and Mitsuhashi et al. also used this treatment in this context [5, 6]. Mitsuhashi et al. proposed this treatment in a patient with pelvic residual mass [5]. But the residual tumor began to enlarge immediately after the cessation of GnRH treatment [6]. In the case reported by Hameleers et al., the patient was treated for a period of 3 years and was in a stable situation [5]. In our case, the patient received agonist treatment for only 1 year. Ten months following the cessation of this treatment, residual disease is asymptomatic and its size is radiologically stable. ACKNOWLEDGMENT We thank Lorna Saint Ange for editing the manuscript.
REFERENCES 1. Cooper MM, Guillem J, Dalton J, Marboe CC, Corwin S, Todd GJ, Rose EA. Recurrent intravenous leiomyomatosis with cardiac extension. Ann Thorac Surg 1992;53:139 – 41. 2. Lo KW, Lau TK. Intracardiac leiomyomatosis. Case report and literature review. Arch Gynecol Obstet 2001;264:209 –10. 3. Le Bouedec G, Bailly C, Penault-Llorca F, Fonck Y, Dauplat J. Intravascular leiomyomatosis of uterine origin. A case of pseudo-metastatic cavocardial thrombus. Presse Med 1999;28:1463–5. 4. Suginami H, Kaura R, Ochi H, Matsuura S. Intravenous leiomyomatosis with cardiac extension: successful surgical management and histopathologic study. Obstet Gynecol 1990;76:527–9. 5. Hameleers JA, Zeebregts CJ, Hamerlijnck RP, Elbers JR, Hameeteman TM. Combined surgical and medical approach to intravenous leiomyomatosis with cardiac extension. Acta Chir Belg 1999;99:92– 4. 6. Mitsuhashi A, Nagai Y, Sugita M, Nakajima N, Sekiya S. GnRH agonist for intravenous leiomyomatosis with cardiac extension. A case report. J Reprod Med 1999;44:883– 6. 7. Tresukosol D, Kudelka AP, Malpica A, Varma DG, Edwards CL, Kavanagh JJ. Leuprolide acetate and intravascular leiomyomatosis. Obstet Gynecol 1995;86:688 –92.