- Email: [email protected]
Benign metastasizing leiomyoma – case report and review of literature
[(7421)TD.SARIEM]
240
Letters to the Editor / European Journal of Obstetrics & Gynecology and Reproductive Biology 159 (2011) 230–243
cells and one of plasma was started. Aspiration-drainage in the Douglas was placed. After surgery aspiration-drainage was maintained until the fourth day with a total collection of 800 cc of sero ematic fluid. 5th day pelvic ultrasonography did not show free fluid in the pelvis, uterus and adnexa appeared regular. At discharge on day 5th the patient was in good condition with a value of Hb of 10.1 g/dl. Follow up examinations prove an excellent anatomical and functional outcome: gray scale ultrasonography performed 1 month after surgery showed a regular endometrial line, colour Doppler ultrasonography demonstrated patency of both uterine arteries with regular flow (RI 0.67 on the left and 0.70 on the right) (Fig. 1), moreover hysteroscopy performed 5 months after surgery showed an excellent endometrial cavity without intrauterine adhesions (Fig. 2). In literature there is no uniform clinical approach in the presence of large uterine fibroids. Some Authors report that hysterectomy results in less blood loss, on the other hand leads to a higher complication rate of lower urinary tract [1,2]. Other Authors demonstrate the same safety of conservative treatment even in the presence of large fibroids as blood loss is related to the uterine size but not the procedure performed [3,4]. Therefore if the woman wants to maintain fertility can be tried a conservative procedure as the myomectomy. In this case we preferred to perform B-Lynch suture, a classical obstetric procedure, to manage the massive uterine bleeding instead of the typical conservative treatments such as the hypogastric artery ligation and the uterine artery ligation in view of the severe pelvic adhesions syndrome and the consequent increase in operating time. Furthermore the seriousness of the situation did not allow the planning of intraoperative arterial embolization. Clinical results during follow up of this patient show that BLynch suture may be indicated not only in obstetric post partum massive bleeding but also in gynaecological surgical complications in which conservative treatment is suitable. References [1] Bradley Van Voorhis, MD. A 41-year-old woman with menorrhagia, anemia, and fibroids. [2] Iverson Jr RE, Chelmow D, Strohbehn K, Waldman L, Evantash EG. Relative morbidity of abdominal hysterectomy and myomectomy for management of uterine leiomyomas. Obstet Gynecol 1996;88:415–9. [3] Sawin SW, Pilevsky ND, Berlin JA, Barnhart KT. Comparability of perioperative morbidity between abdominal myomectomy and hysterectomy for women with uterine leiomyomas. Am J Obstet Gynecol 2000;183:1448–55. [4] West S, Ruiz R, Parker WH. Abdominal myomectomy in women with very large uterine size. Fertil Steril 2006;85:36–9.
Marco Mitidieri* Stefano Cosma Paolo Petruzzelli Michela Chiado` Fiorio Tin Tullia Todros Guido Menato Department of Gynecology and Obstetrics University of Turin, Italy *Corresponding
author at: Department of Gynaecology and Obstetrics University of Turin, C.so Spezia 60, Torino, Italy. Tel.: +39 0113134444; fax: +39 0113134859 E-mail address: [email protected] (M. Mitidieri). 6 February 2011
doi:10.1016/j.ejogrb.2011.07.013[(741)TD.ENIM][
Benign metastasizing leiomyoma – case report and review of literature
Dear Editor, Benign metastasizing leiomyoma (BML) is a rare condition affecting females, characterized by multiple soft tissue tumors at distant sites which are histologically similar to uterine leiomyoma. Many cases have been reported in the literature so far [1]. Herein reported is a case of BML with pulmonary and skeletal muscle involvement. A 60-year-old lady, who had undergone hysterectomy 20 years back for fibroid uterus presented with a progressively increasing mass in the abdomen, abdominal pain and weight loss. CECT abdomen revealed a large retroperitoneal mass posterior to stomach, with features suggestive of infiltration of pancreas, encasement of aorta and involvement of left renal artery. CA 125 was 189 U/ml, CEA 3 ng/ml and CA 19-9 77 U/ml. Image guided biopsy from the lesion was reported as leiomyoma. Patient underwent surgery on 01/08/2007. Intra-operatively multiple lesions were noted; a 25 cm 20 cm lobulated, soft to firm, encapsulated mass in the retro peritoneum encasing (but not infiltrating) the aorta and left renal vessels, a 18 cm 15 cm mass in the left iliac fossa abutting the sigmoid mesentery and a 15 cm 15 cm mass anterior to the recto-sigmoid adherent to the urinary bladder and vaginal vault. Complete tumor excision with left nephrectomy and anterior resection was done. Histopathology revealed capsulated neoplasm composed of whorls and fascicles of spindle shaped cells with no evidence of increased mitosis or necrosis. The cells showed positivity with antibodies for Smooth Muscle Antigen (SMA), desmin and estrogen receptors consistent with a diagnosis of leiomyoma. The proliferation index (Ki67) was less than 2–3%. In December 2009, she presented with a localized right calf swelling and further imaging of abdomen and chest revealed recurrence in the retroperitoneum and bilateral pulmonary lesions. The calf swelling was excised and it was reported as leiomyoma. CT-guided tru-cut biopsy of the lung lesion also was reported as leiomyoma. She was put on daily tamoxifen and the thoracic lesions were kept under observation. Since the mediastinal mass was increasing in size, surgical excision of that mass was done in June 2010 and it was confirmed to be benign leiomyoma. She was started on monthly injections of depot GnRH analogues and now after nearly one year of starting the treatment, the retroperitoneal lesion is remaining the same. Several hypotheses have been proposed regarding the etiology of BML: (1) hematogenous spread of a benign uterine tumor, (2) a low-grade leiomyosarcoma metastasizing to the lung; or (3) multifocal proliferations of smooth muscle cells occurring throughout the body in response to hormonal stimulation [2]. Patton et al. [3] on the basis of histologic, immunohistochemical, cytogenetic and molecular evidence concluded that BML cannot be considered as sarcoma. BML usually affects women who have undergone prior uterine surgery for leiomyomas and may appear several years after the surgery [1]. In most reported cases, pulmonary involvement is present. Extrapulmonary lesions have been documented in lymph nodes, deep soft tissues, omentum, mesentery, bone, skull base, spine, heart and skeletal muscle. Patients are commonly asymptomatic, however; symptoms like cough, chest pain, and dyspnoea have been reported. Pulmonary tumors in BML can be treated with bronchoscopic resection, conservative pulmonary surgery and hormonal manipulation [1]. Several facts support the hormonal dependency of BML, viz., presence of estrogen and progesterone receptors and
[(Fig._1)TD$IG]
Letters to the Editor / European Journal of Obstetrics & Gynecology and Reproductive Biology 159 (2011) 230–243
241
regression of metastatic lesions in situations where estrogen levels fall significantly [2]. Hormonal management can be accomplished surgically by bilateral oophorectomy and the medical management includes the use of GnRH analogues, selective estrogen receptor modulators (e.g. tamoxifene, raloxifene) and aromatase inhibitors [1,2]. Recent evidence favours a combination of GnRH analogues with raloxifene to arrest the progression of myomas, without any bone or endometrial adverse effects [4]. In patients with intrapulmonary lesions, a median survival of 94 months has been reported after excision of the lesions [5]. To conclude, BML is a rare entity that usually affects women with history of uterine fibroids. The lungs are the most common site of metastatic involvement and hence BML should be a differential in any patient with multiple pulmonary nodules and a history of uterine leiomyomatosis.
References [1] Pitts S, Oberstein EM, Glassberg MK. Benign metastasizing leiomyoma and lymphangioleiomyomatosis: sex-specific diseases? Clin Chest Med 2004;25:343–60. [2] Rivera JA, Christopoulos S, Small D, Trifiro M. Hormonal manipulation of benign metastasizing leiomyomas: report of two cases and review of the literature. J Clin Endocrinol Metab 2004;89(July (7)):3183–8. [3] Patton KT, Cheng L, Papavero V, et al. Benign metastasizing leiomyoma: clonality, telomere length and clinicopathologic analysis. Modern Pathol 2006;19(January (1)):130–40. [4] Olive DL, Lindheim SR, Pritts EA. Non-surgical management of leiomyoma: impact on fertility. Curr Opin Obstet Gynecol 2004;16(3):239–43. [5] Kayser K, Zink S, Schneider T, et al. Benign metastasizing leiomyoma of the uterus: documentation of clinical, immunohistochemical and lectin-histochemical data of ten cases. Virchows Arch 2000;437:284–92.
Sheikh Zahoor Ahmad R. Anupama* D.K. Vijaykumar Department of Surgical Oncology, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Kochi, Kerala 682041, India *Corresponding author E-mail address: [email protected] (R. Anupama) 3 March 2011 doi:10.1016/j.ejogrb.2011.07.017[(7421)TD.ENIM][
[(742)TD.SARIEM] Misdiagnosed cervical pregnancy following a pregnancy with cotyledonoid leiomyoma Dear editor, We would like to report a case of cervical missed abortion, a G5P4 presented with history of 11 weeks amenorrhea and on and off mild vaginal bleeding for a week. She had three spontaneous vaginal deliveries followed by a cesarean section in her last pregnancy which was complicated by cotyledonoid leiomyoma removal at 5th month.. General and abdominal examinations were unremarkable and pelvic examination revealed uterus of 12 week’s size with closed cervical os. An ultrasound revealed a gestational sac in the lower segment of the uterus (Fig. 1) though in one view the cervix appeared ballooned with the gestational sac. The fetus had a crown rump length equal to 8 weeks + 3 days but no cardiac activity. A diagnosis of missed abortion with the sac in the lower uterine
Fig. 1. Ultrasound scan showing a gestational sac in the lower segment of the uterus with a closed cervical canal.
segment was made and evacuation and curettage was planned. The diagnosis of cesarean scar pregnancy was also entertained but a repeat ultrasound with Doppler study was more suggestive of a cervical abortion. Hemoglobin preoperatively was 12 g/dL. Evacuation of the uterus was carried out after priming the cervix with misoprostol. At the time of evacuation, the cervix was ballooned, admitting one finger, there was severe hemorrhage from the cervical canal which was temporarily controlled with a Foley catheter . Uterine cavity was empty both clinically and by intra operative ultrasound scan. She became hypotensive with tachycardia and 2 units of packed red blood cells were transfused. Since she continued to have moderate vaginal bleeding, uterine artery embolisation (UAE) was performed and the bleeding from the cervix was controlled. A follow up ultrasound scan in 4 days’ time revealed some retained products in the cervix of about 28 mm and a serum beta human chorionic gonadotropin (b-hcg) was 6943 IU. Due to the possibility of further hemorrhage in spite of embolisation, a single dose of 50 mgm methotrexate was given intramuscular and b-hcg became negative in 4 weeks time. Discussion: Cervical pregnancy is a rare form of ectopic pregnancy in which the pregnancy implants in the lining of the endocervical canal with an incidence of 1 in 90001. Neither the speculum examination nor the bimanual examination was suggestive of cervical pregnancy in this case (hourglass shaped uterus)1. The ultrasound findings of classical triad as described by Jurkovic et al 2 were not present. The differential diagnoses in this patient were pregnancy in the uterine scar, cervical abortion and a cervical pregnancy. However the sonographic criteria were not in favor of a pregnancy in the scar as there was no evidence of functional trophoblastic circulation on Doppler examination of the uterus3. The initial diagnosis of cervical abortion was likely, as the ultrasound revealed some blood clot in the uterine cavity, the uterine cavity was enlarged compared to the cervix, the gestational sac was flattened and had no or a minimal echogenic rim and contained a dead embryo. In addition, there was no sign of cardiac activity. Diagnosis of cervical pregnancy was made at the time of evacuation as the products were attached to the cervical canal with brisk hemorrhage. Medical therapy with methotrexate is advised for cervical pregnancy as surgical management carries risk of hemorrhage and hysterectomy4. As the bleeding continued after Foley catheter placement, UAE was performed with gel foam. For fear of delayed hemorrhage with some retained tissue and as the embolisation
240
Letters to the Editor / European Journal of Obstetrics & Gynecology and Reproductive Biology 159 (2011) 230–243
cells and one of plasma was started. Aspiration-drainage in the Douglas was placed. After surgery aspiration-drainage was maintained until the fourth day with a total collection of 800 cc of sero ematic fluid. 5th day pelvic ultrasonography did not show free fluid in the pelvis, uterus and adnexa appeared regular. At discharge on day 5th the patient was in good condition with a value of Hb of 10.1 g/dl. Follow up examinations prove an excellent anatomical and functional outcome: gray scale ultrasonography performed 1 month after surgery showed a regular endometrial line, colour Doppler ultrasonography demonstrated patency of both uterine arteries with regular flow (RI 0.67 on the left and 0.70 on the right) (Fig. 1), moreover hysteroscopy performed 5 months after surgery showed an excellent endometrial cavity without intrauterine adhesions (Fig. 2). In literature there is no uniform clinical approach in the presence of large uterine fibroids. Some Authors report that hysterectomy results in less blood loss, on the other hand leads to a higher complication rate of lower urinary tract [1,2]. Other Authors demonstrate the same safety of conservative treatment even in the presence of large fibroids as blood loss is related to the uterine size but not the procedure performed [3,4]. Therefore if the woman wants to maintain fertility can be tried a conservative procedure as the myomectomy. In this case we preferred to perform B-Lynch suture, a classical obstetric procedure, to manage the massive uterine bleeding instead of the typical conservative treatments such as the hypogastric artery ligation and the uterine artery ligation in view of the severe pelvic adhesions syndrome and the consequent increase in operating time. Furthermore the seriousness of the situation did not allow the planning of intraoperative arterial embolization. Clinical results during follow up of this patient show that BLynch suture may be indicated not only in obstetric post partum massive bleeding but also in gynaecological surgical complications in which conservative treatment is suitable. References [1] Bradley Van Voorhis, MD. A 41-year-old woman with menorrhagia, anemia, and fibroids. [2] Iverson Jr RE, Chelmow D, Strohbehn K, Waldman L, Evantash EG. Relative morbidity of abdominal hysterectomy and myomectomy for management of uterine leiomyomas. Obstet Gynecol 1996;88:415–9. [3] Sawin SW, Pilevsky ND, Berlin JA, Barnhart KT. Comparability of perioperative morbidity between abdominal myomectomy and hysterectomy for women with uterine leiomyomas. Am J Obstet Gynecol 2000;183:1448–55. [4] West S, Ruiz R, Parker WH. Abdominal myomectomy in women with very large uterine size. Fertil Steril 2006;85:36–9.
Marco Mitidieri* Stefano Cosma Paolo Petruzzelli Michela Chiado` Fiorio Tin Tullia Todros Guido Menato Department of Gynecology and Obstetrics University of Turin, Italy *Corresponding
author at: Department of Gynaecology and Obstetrics University of Turin, C.so Spezia 60, Torino, Italy. Tel.: +39 0113134444; fax: +39 0113134859 E-mail address: [email protected] (M. Mitidieri). 6 February 2011
doi:10.1016/j.ejogrb.2011.07.013[(741)TD.ENIM][
Benign metastasizing leiomyoma – case report and review of literature
Dear Editor, Benign metastasizing leiomyoma (BML) is a rare condition affecting females, characterized by multiple soft tissue tumors at distant sites which are histologically similar to uterine leiomyoma. Many cases have been reported in the literature so far [1]. Herein reported is a case of BML with pulmonary and skeletal muscle involvement. A 60-year-old lady, who had undergone hysterectomy 20 years back for fibroid uterus presented with a progressively increasing mass in the abdomen, abdominal pain and weight loss. CECT abdomen revealed a large retroperitoneal mass posterior to stomach, with features suggestive of infiltration of pancreas, encasement of aorta and involvement of left renal artery. CA 125 was 189 U/ml, CEA 3 ng/ml and CA 19-9 77 U/ml. Image guided biopsy from the lesion was reported as leiomyoma. Patient underwent surgery on 01/08/2007. Intra-operatively multiple lesions were noted; a 25 cm 20 cm lobulated, soft to firm, encapsulated mass in the retro peritoneum encasing (but not infiltrating) the aorta and left renal vessels, a 18 cm 15 cm mass in the left iliac fossa abutting the sigmoid mesentery and a 15 cm 15 cm mass anterior to the recto-sigmoid adherent to the urinary bladder and vaginal vault. Complete tumor excision with left nephrectomy and anterior resection was done. Histopathology revealed capsulated neoplasm composed of whorls and fascicles of spindle shaped cells with no evidence of increased mitosis or necrosis. The cells showed positivity with antibodies for Smooth Muscle Antigen (SMA), desmin and estrogen receptors consistent with a diagnosis of leiomyoma. The proliferation index (Ki67) was less than 2–3%. In December 2009, she presented with a localized right calf swelling and further imaging of abdomen and chest revealed recurrence in the retroperitoneum and bilateral pulmonary lesions. The calf swelling was excised and it was reported as leiomyoma. CT-guided tru-cut biopsy of the lung lesion also was reported as leiomyoma. She was put on daily tamoxifen and the thoracic lesions were kept under observation. Since the mediastinal mass was increasing in size, surgical excision of that mass was done in June 2010 and it was confirmed to be benign leiomyoma. She was started on monthly injections of depot GnRH analogues and now after nearly one year of starting the treatment, the retroperitoneal lesion is remaining the same. Several hypotheses have been proposed regarding the etiology of BML: (1) hematogenous spread of a benign uterine tumor, (2) a low-grade leiomyosarcoma metastasizing to the lung; or (3) multifocal proliferations of smooth muscle cells occurring throughout the body in response to hormonal stimulation [2]. Patton et al. [3] on the basis of histologic, immunohistochemical, cytogenetic and molecular evidence concluded that BML cannot be considered as sarcoma. BML usually affects women who have undergone prior uterine surgery for leiomyomas and may appear several years after the surgery [1]. In most reported cases, pulmonary involvement is present. Extrapulmonary lesions have been documented in lymph nodes, deep soft tissues, omentum, mesentery, bone, skull base, spine, heart and skeletal muscle. Patients are commonly asymptomatic, however; symptoms like cough, chest pain, and dyspnoea have been reported. Pulmonary tumors in BML can be treated with bronchoscopic resection, conservative pulmonary surgery and hormonal manipulation [1]. Several facts support the hormonal dependency of BML, viz., presence of estrogen and progesterone receptors and
[(Fig._1)TD$IG]
Letters to the Editor / European Journal of Obstetrics & Gynecology and Reproductive Biology 159 (2011) 230–243
241
regression of metastatic lesions in situations where estrogen levels fall significantly [2]. Hormonal management can be accomplished surgically by bilateral oophorectomy and the medical management includes the use of GnRH analogues, selective estrogen receptor modulators (e.g. tamoxifene, raloxifene) and aromatase inhibitors [1,2]. Recent evidence favours a combination of GnRH analogues with raloxifene to arrest the progression of myomas, without any bone or endometrial adverse effects [4]. In patients with intrapulmonary lesions, a median survival of 94 months has been reported after excision of the lesions [5]. To conclude, BML is a rare entity that usually affects women with history of uterine fibroids. The lungs are the most common site of metastatic involvement and hence BML should be a differential in any patient with multiple pulmonary nodules and a history of uterine leiomyomatosis.
References [1] Pitts S, Oberstein EM, Glassberg MK. Benign metastasizing leiomyoma and lymphangioleiomyomatosis: sex-specific diseases? Clin Chest Med 2004;25:343–60. [2] Rivera JA, Christopoulos S, Small D, Trifiro M. Hormonal manipulation of benign metastasizing leiomyomas: report of two cases and review of the literature. J Clin Endocrinol Metab 2004;89(July (7)):3183–8. [3] Patton KT, Cheng L, Papavero V, et al. Benign metastasizing leiomyoma: clonality, telomere length and clinicopathologic analysis. Modern Pathol 2006;19(January (1)):130–40. [4] Olive DL, Lindheim SR, Pritts EA. Non-surgical management of leiomyoma: impact on fertility. Curr Opin Obstet Gynecol 2004;16(3):239–43. [5] Kayser K, Zink S, Schneider T, et al. Benign metastasizing leiomyoma of the uterus: documentation of clinical, immunohistochemical and lectin-histochemical data of ten cases. Virchows Arch 2000;437:284–92.
Sheikh Zahoor Ahmad R. Anupama* D.K. Vijaykumar Department of Surgical Oncology, Amrita Institute of Medical Sciences and Research Centre, Amrita Vishwa Vidyapeetham, Kochi, Kerala 682041, India *Corresponding author E-mail address: [email protected] (R. Anupama) 3 March 2011 doi:10.1016/j.ejogrb.2011.07.017[(7421)TD.ENIM][
[(742)TD.SARIEM] Misdiagnosed cervical pregnancy following a pregnancy with cotyledonoid leiomyoma Dear editor, We would like to report a case of cervical missed abortion, a G5P4 presented with history of 11 weeks amenorrhea and on and off mild vaginal bleeding for a week. She had three spontaneous vaginal deliveries followed by a cesarean section in her last pregnancy which was complicated by cotyledonoid leiomyoma removal at 5th month.. General and abdominal examinations were unremarkable and pelvic examination revealed uterus of 12 week’s size with closed cervical os. An ultrasound revealed a gestational sac in the lower segment of the uterus (Fig. 1) though in one view the cervix appeared ballooned with the gestational sac. The fetus had a crown rump length equal to 8 weeks + 3 days but no cardiac activity. A diagnosis of missed abortion with the sac in the lower uterine
Fig. 1. Ultrasound scan showing a gestational sac in the lower segment of the uterus with a closed cervical canal.
segment was made and evacuation and curettage was planned. The diagnosis of cesarean scar pregnancy was also entertained but a repeat ultrasound with Doppler study was more suggestive of a cervical abortion. Hemoglobin preoperatively was 12 g/dL. Evacuation of the uterus was carried out after priming the cervix with misoprostol. At the time of evacuation, the cervix was ballooned, admitting one finger, there was severe hemorrhage from the cervical canal which was temporarily controlled with a Foley catheter . Uterine cavity was empty both clinically and by intra operative ultrasound scan. She became hypotensive with tachycardia and 2 units of packed red blood cells were transfused. Since she continued to have moderate vaginal bleeding, uterine artery embolisation (UAE) was performed and the bleeding from the cervix was controlled. A follow up ultrasound scan in 4 days’ time revealed some retained products in the cervix of about 28 mm and a serum beta human chorionic gonadotropin (b-hcg) was 6943 IU. Due to the possibility of further hemorrhage in spite of embolisation, a single dose of 50 mgm methotrexate was given intramuscular and b-hcg became negative in 4 weeks time. Discussion: Cervical pregnancy is a rare form of ectopic pregnancy in which the pregnancy implants in the lining of the endocervical canal with an incidence of 1 in 90001. Neither the speculum examination nor the bimanual examination was suggestive of cervical pregnancy in this case (hourglass shaped uterus)1. The ultrasound findings of classical triad as described by Jurkovic et al 2 were not present. The differential diagnoses in this patient were pregnancy in the uterine scar, cervical abortion and a cervical pregnancy. However the sonographic criteria were not in favor of a pregnancy in the scar as there was no evidence of functional trophoblastic circulation on Doppler examination of the uterus3. The initial diagnosis of cervical abortion was likely, as the ultrasound revealed some blood clot in the uterine cavity, the uterine cavity was enlarged compared to the cervix, the gestational sac was flattened and had no or a minimal echogenic rim and contained a dead embryo. In addition, there was no sign of cardiac activity. Diagnosis of cervical pregnancy was made at the time of evacuation as the products were attached to the cervical canal with brisk hemorrhage. Medical therapy with methotrexate is advised for cervical pregnancy as surgical management carries risk of hemorrhage and hysterectomy4. As the bleeding continued after Foley catheter placement, UAE was performed with gel foam. For fear of delayed hemorrhage with some retained tissue and as the embolisation