Journal of Obstetrics and Gynaecology
ISSN: 0144-3615 (Print) 1364-6893 (Online) Journal homepage: http://www.tandfonline.com/loi/ijog20
Unsuspected diagnosis of uterine leiomyosarcoma after laparoscopic myomectomy G. Cormio, V. Loizzi, O. Ceci, L. Leone, L. Selvaggi & S. Bettocchi To cite this article: G. Cormio, V. Loizzi, O. Ceci, L. Leone, L. Selvaggi & S. Bettocchi (2015) Unsuspected diagnosis of uterine leiomyosarcoma after laparoscopic myomectomy, Journal of Obstetrics and Gynaecology, 35:2, 211-212, DOI: 10.3109/01443615.2014.937332 To link to this article: http://dx.doi.org/10.3109/01443615.2014.937332
Published online: 24 Jul 2014.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ijog20 Download by: [Chinese University of Hong Kong]
Date: 09 November 2015, At: 14:57
Gynaecology Case Reports 211 approximately 4 ⫻ 3 cm in size, located on the right labium minora, not fluctuating, mobile, solid and regular-shaped (Figure 1). Ultrasonographic examination revealed normal internal genitalia. The mass was removed surgically by performing local excision under spinal anaesthesia. Pathological examination was reported as 3.5 cm sized leiomyoma nodules. Immunohistochemical studies revealed that tumour cells were strongly positive for smooth muscle actin (SMA), vimentin, desmin and h-caldesmon. There was negative staining for S100 protein and CD34. The tumour tested positive for both oestrogen and progesterone receptors. Ki-67 proliferation index was 2%. The final histopathological diagnosis therefore, was leiomyoma of vulva with no cellular atypia. She was discharged from the hospital without any complications. During the 10-month clinical follow-up, there was no recurrence of the tumour.
Downloaded by [Chinese University of Hong Kong] at 14:57 09 November 2015
Discussion Although the uterus is the most common site of origin of leiomyomas, the lesions arise as proliferations of smooth muscle cells, and they may develop at any site where such cells are found (Fasih et al. 2008). External genital soft tissue leiomyomas are extremely rare (Reyad et al. 2006). Almost all cases are premenopausal and the mean age at diagnosis is 37 (range 14–71). But, they can occur in women at any age, such as in our patient. The average tumour size varies from 0.5 to 15 cm. Vulvar leiomyomas usually present as an asymptomatic protruding mass and extrauterine leiomyomas may be seen along the labia majora (Nielsen et al. 1996; Reyad et al. 2006). However, in our case, the patient was admitted to our hospital with a protruding mass on the right labium minora, which was causing vulvar pain. Due to its location on the labium minora and the patient having vulvar pain, she was admitted to hospital early, before it became enlarged. The major diagnostic problem with SMTs of the vulva is the distinction between benign and malignant forms, since many vulval lesions have similar appearances, making it difficult to distinguish benign from malignant lesions on gross inspection (Reyad et al. 2006). Vulvar lesions always require complete pathological examination to exclude a malignancy, even in cases with features of benign tumour (Messalli et al. 2012). In our case, immunohistochemical studies revealed accurate histopathological diagnosis of leiomyoma of the vulva. Clinically, vulvar masses are frequently thought to be Bartholin gland cysts or abscesses, although preoperative differential diagnoses include labial cysts, inguinal hernia and mesenchymal tumours. In this case, the leiomyoma was located on a labium minora, which is a very rare location of vulvar leiomyoma and thus we ruled out Bartholin cysts or abscesses. On examination, we identified a mass located on the right labium minora, not fluctuating, mobile and regular in shape, so at first we thought it to be a benign tumour or cyst of the vulva. However, benign and malignant tumours of vulva have similar symptoms. The benign features of a mass should not misguide us (Messalli et al. 2012), as although it may be reported as appearing as a benign vulvar mass, it may indeed be a delayed diagnosis of malignant vulvar mass. The preferred treatment for vulvar fibroids is local wide deep excision to prevent recurrence (Williams et al. 2002). The correct treatment is important because of the aggressive behaviour of other related mesenchymal tumours of the vulva and vagina (Kairi-Vassilatou et al. 2011). Recurrence of vulval leiomyoma is extremely rare and follow-up is seldom required. In our case, the mass was removed surgically by performing local excision. During the 10-month clinical follow-up, there was no recurrence of the tumour. Although smooth muscle cell tumours of the vulva are rarely seen in daily practice, its diagnosis is important in the evaluation of vulvar masses. This case aims to emphasise that the appearance of a leiomyoma should be part of the differential diagnoses of any vulvar mass, even in young women with a lesion involving the labia minora. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Fasih N, Prasad Shanbhogue AK, Macdonald DB et al. 2008. Leiomyomas beyond the uterus: unusual locations, rare manifestations. Radiographics 28: 1931–1948. Kairi-Vassilatou E, Dastamani C, Vouza E et al. 2011. Angiomyofibroblastoma of the vulva: a clinicopathological and immunohistochemical analysis of a rare benign mesenchymal tumour. European Journal of Gynaecological Oncology 32:353–355. Messalli EM, D’Aponte ML, Luise R et al. 2012. An apparently benign vulvar mass: possibly a rare malignancy. European Journal of Gynaecological Oncology 33:441–444. Nielsen GP, Rosenberg AE, Koerner FC et al. 1996. Smooth-muscle tumors of the vulva: a clinicopathological study of 25 cases and review of the literature. American Journal of Surgical Pathology 20:779–793. Reyad MM, Gazvani MR, Khine MM. 2006. A rare case of primary leiomyoma of the vulva. Journal of Obstetrics and Gynaecology 26:73–74. Williams NP, Williams E, Fletcher H. 2002. Smooth muscle tumours of the vulva in Jamaica. West Indian Medical Journal 51:228–231.
Unsuspected diagnosis of uterine leiomyosarcoma after laparoscopic myomectomy G. Cormio1,2, V. Loizzi1,2, O. Ceci1, L. Leone1, L. Selvaggi1 & S. Bettocchi1 1Department of Biomedical Science and Human Oncology,
Obstetrics and Gynecology Unit and 2Division of Gynecology Oncology, IRCCS Bari, Bari, Italy DOI: 10.3109/01443615.2014.937332 Correspondence: V. Loizzi, Dipartimento di Ginecologia, Ostetricia e Neonatologia, Università di Bari, Italy. E-mail: [email protected]
Introduction
Uterine sarcomas are uncommon tumours that account for ⬍ 3% of all female genital tract malignancies and 3–7% of malignant tumours of the uterus (Olah et al. 1991). Diagnosis is often made incidentally at the time of myomectomy or hysterectomy for presumed benign condition. In recent years, with the wide development of endoscopic procedures for surgical treatment of uterine fibroids, the management of an unsuspected sarcoma after laparoscopic myomectomy might become more common. This condition raises the question whether laparoscopic procedure (gas insufflation and morcellement) may increase the risk of tumour dissemination within the abdominal cavity. We report three patients who had laparoscopic myomectomy as primary surgical management of an unsuspected uterine leiomyosarcoma (LMS), and discuss the potential implications of peritoneal dissemination following endoscopic procedures in uterine sarcomas.
Case reports Between January 2000 and December 2010, 588 patients had laparoscopic myomectomy at the Department of Obstetrics and Gynecology, University of Bari, Italy, and among these, three cases (0.5%) of uterine LMS were diagnosed at pathological examination. Two of the women (34 and 37 years) were nulliparous and a 46-year-old woman was gravida 2, para 2. In all three cases, diagnosis at the time of admission was abnormal uterine bleeding due to solitary uterine fibroid. Their past medical history was uneventful. In all cases, pelvic ultrasound demonstrated the presence a subserous myoma with diameter of 4, 5 and 6 cm, respectively. Two of the three patients had received Gn-RH treatment before surgery. Laparoscopic myomectomy was performed with complete resection of the fibroid that was removed with morcellement. In one patient, the resection of the fibroid from the uterus appeared difficult and the surgeon required intraoperative frozen section examination that was inconclusive. All the resected tissue chips were extracted
Downloaded by [Chinese University of Hong Kong] at 14:57 09 November 2015
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Gynaecology Case Reports
without an endobag and sent for pathological examination. No intraoperative or postoperative complications occurred and all patients were discharged on the following day. Diagnosis of leiomyosarcoma was given after H&E stain and immunohistochemistry. Mitotic count was 10 ⫻ 10 HPF in two cases and 5 ⫻ 10 HPF in the other case; coagulative necrosis and vascular-space invasion were found in all patients. Following diagnosis of the malignant condition, all patients were extensively staged with pelvic examination, transvaginal ultrasound, hysteroscopy, Pap smear and total-body computer tomography scan that did not revealed any sign of persistent disease. Within 28 days from primary surgery, all patients, after informed consent, were submitted to a thorough surgical staging, including total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, resection of all trocar ports, pelvic lymphadenectomy and peritoneal washings. In all three cases, pathological examination did not show any residual foci of disease either in the uterus or in peritoneal cytology, adnexae and lymph nodes. Considering the risk associated with the primary procedure, despite the lack of persistent disease, all patients received four courses of adjuvant chemotherapy with doxorubicin (30 mg/m2), hyphosphamide (3 g/m2) plus mesna on days 1, 2 and 3, repeated every 21 days. Two patients had no sign of recurrent disease after 24 and 22 months, while the third patient developed a 3 cm isolated pelvic peritoneal recurrence 42 months after primary surgery. She received surgery and pelvic radiotherapy but the disease progressed and she died 64 months after the laparoscopic procedure.
Discussion The risk of malignancy in leiomyomas is low and there are no specific characteristics to identify malignancy prior to tissue being sent for pathological review. Three large series have tried to answer the question of the frequency of LMS in series of patients undergoing surgery for presumed leiomyoma and found extremely low rates of incidental LMS, ranging from 0.08% to 0.49% (Parker et al. 1994; Leung et al. 2009; Leibsohn et al. 1990). Based on our limited experience on three cases, we showed that laparoscopic myomectomy did not increase the risk of peritoneal or distant dissemination in uterine LMS. However, there are two potential causes of concern regarding laparoscopic resection of a leiomyosarcoma: dissemination of malignant cells at the time of myomectomy and intraperitoneal dissemination of malignant cells after morcellement of the tumour. Whether laparoscopic myomectomy may increase penetration of malignant cells into deeper myometrial tissue, which might facilitate distant metastases through the haematogenous or lymphatic route is uncertain. The intense radiofrequency energy effect on tumour cells and surrounding tissue, including the microvasculature would tend to further reduce the viability of local malignant cells and their potential for intravascular dissemination and subsequent metastatic invasion. On the other hand, the practice of morcellation is a technique consisting of the fragmentation of the tissue into smaller pieces that often prevents the need to enlarge established incisions. During this procedure, it can be difficult to prevent small tissue fragments from being inadvertently dispersed throughout the peritoneal cavity; these fragments may then implant anywhere and cause symptoms and morbidity requiring intervention. Only a few studies to date have evaluated the impact of tumor morcellation on the outcomes of patients with highly malignant tumours (Morice et al. 2003; Buda et al. 2013; Park et al. 2011). Recently, Park et al. (2011) compared treatment outcomes and patterns of recurrence in patients with apparently early uterine LMS who did and did not undergo tumour morcellation during surgery. The authors showed that tumour morcellation during surgery increased the rate of abdomino-pelvic dissemination, in the form of peritoneal sarcomatosis and vaginal apex recurrence, and affected adversely disease-free and overall survivals. To our knowledge, another four studies have confirmed the impact of tumour morcellation on the outcomes of patients with uterine sarcoma (Park et al. 2011; Morice et al. 2003; Seidman et al. 2012).
However, preoperative diagnosis of leiomyosarcoma is difficult since there is no pathognomonic signs, specific symptoms, tumour markers or imaging modalities that would point to the presence of a smooth muscle malignant tumour and make a differentiation from leiomyoma (Park et al. 2011; Seidman et al. 2012). The most common signs, prolonged uterine bleeding and lower abdominal pain, are also characteristic of benign leiomyoma (Leibsohn et al. 1990). Traditionally, a rapidly growing uterus was indicative of malignancy, however the incidence of uterine LMS is extremely low, even in patients who undergo surgery, because of a rapidly growing uterus, as this symptom is not specific to uterine LMS. Among patients operated for ‘rapidly growing’ leiomyoma, Parker et al. (1994) reported that the frequency of incidental uterine LMS was very low of 0.27%. This result was also shown elsewhere (Fukunishi et al. 2007). Laparotomy should be the surgical route whenever there is preoperative suspicion of uterine LMS. However, bearing in mind the lack of specific preoperative context to evoke the diagnosis of LMS and the extremely low rates of this tumour in series of patients operated for presumed leiomyoma, the apprehension of LMS, especially in young women, should not be a criterion to avoid less invasive non-laparotomic surgical routes (Leung and Terzibachian 2012). However, when a uterine malignancy is found incidentally after morcellation, a reoperation for completion surgery and staging is mandatory. For these reasons, a discussion of risks and benefits of a surgery and consideration of reasonable alternatives are essential steps in the preparation for surgery and fundamental to the informed consent process. Therefore, the surgeon who plans to use an electric morcellator should disclose the risks of device-related complications, be explicit about the potential for dissemination of an undetected malignancy and offer the possibility of alternative surgical procedures (Kho and Nezhat 2014). Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Buda A, Marco C, Dolci C et al. 2013. Sentinel node mapping in high risk endometrial cancer after laparoscopic supracervical hysterectomy with morcellation. International Journal of Surgery Case Reports 4:809–12. Fukunishi H, Funaki K, Ikuma K et al. 2007. Unsuspected uterine leiomyosarcoma: magnetic resonance imaging findings before and after focused ultrasound surgery. International Journal of Gynecological Cancer 17:724–728. Kho KA, Nezhat CH. 2014. Evaluating the risks of electric uterine morcellation. Journal of the American Medical Association 311:905–6. Leibsohn S, d’Alblaing G, Mishell DR Jr et al. 1990. Leiomyosarcoma in a series of hysterectomies performed for presumed uterine leiomyomas. American Journal of Obstetrics and Gynecology 162:968–974. Leung F, Terzibachian JJ. 2012. Re: The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecologic Oncology 124:172–173. Leung F, Terzibachian JJ. Gay C et al. 2009. Hysterectomies performed for presumed leiomyomas: should the fear of leiomyosarcoma make us apprehend non laparotomic surgical routes? Gynecologie, Obstetrique et Fertilite 37:109–114. Morice P, Rodriguez A, Rey A et al. 2003. Prognostic value of initial surgical procedure for patients with uterine sarcoma: analysis of 123 patients. European Journal of Gynaecological Oncology 24:237–240. Olah KS, Gree H, Blunt S et al. 1991. Retrospective analysis of 318 cases of uterine sarcoma. European Journal of Cancer 27:1095–1099. Park JY, Kim DY, Kim JH et al. 2011. The impact of tumor morcellation during surgery on the outcomes of patients with apparently early low-grade endometrial stromal sarcoma of the uterus. Annals of Surgical Oncology 18: 3453–61. Park JY, Park SK, Kim DY et al. 2011. The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecologic Oncology 122:255–9. Parker WH, Fu YS, Berek JS. 1994. Uterine sarcoma in patients operated on for presumed leiomyoma. Obstetrics and Gynecology 83:414–8. Seidman MA, Oduyebo T, Muto MG et al. 2012. Peritoneal dissemination complicating morcellation of uterine mesenchymal neoplasms. PLoS ONE 7:e50058.
ISSN: 0144-3615 (Print) 1364-6893 (Online) Journal homepage: http://www.tandfonline.com/loi/ijog20
Unsuspected diagnosis of uterine leiomyosarcoma after laparoscopic myomectomy G. Cormio, V. Loizzi, O. Ceci, L. Leone, L. Selvaggi & S. Bettocchi To cite this article: G. Cormio, V. Loizzi, O. Ceci, L. Leone, L. Selvaggi & S. Bettocchi (2015) Unsuspected diagnosis of uterine leiomyosarcoma after laparoscopic myomectomy, Journal of Obstetrics and Gynaecology, 35:2, 211-212, DOI: 10.3109/01443615.2014.937332 To link to this article: http://dx.doi.org/10.3109/01443615.2014.937332
Published online: 24 Jul 2014.
Submit your article to this journal
Article views: 40
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ijog20 Download by: [Chinese University of Hong Kong]
Date: 09 November 2015, At: 14:57
Gynaecology Case Reports 211 approximately 4 ⫻ 3 cm in size, located on the right labium minora, not fluctuating, mobile, solid and regular-shaped (Figure 1). Ultrasonographic examination revealed normal internal genitalia. The mass was removed surgically by performing local excision under spinal anaesthesia. Pathological examination was reported as 3.5 cm sized leiomyoma nodules. Immunohistochemical studies revealed that tumour cells were strongly positive for smooth muscle actin (SMA), vimentin, desmin and h-caldesmon. There was negative staining for S100 protein and CD34. The tumour tested positive for both oestrogen and progesterone receptors. Ki-67 proliferation index was 2%. The final histopathological diagnosis therefore, was leiomyoma of vulva with no cellular atypia. She was discharged from the hospital without any complications. During the 10-month clinical follow-up, there was no recurrence of the tumour.
Downloaded by [Chinese University of Hong Kong] at 14:57 09 November 2015
Discussion Although the uterus is the most common site of origin of leiomyomas, the lesions arise as proliferations of smooth muscle cells, and they may develop at any site where such cells are found (Fasih et al. 2008). External genital soft tissue leiomyomas are extremely rare (Reyad et al. 2006). Almost all cases are premenopausal and the mean age at diagnosis is 37 (range 14–71). But, they can occur in women at any age, such as in our patient. The average tumour size varies from 0.5 to 15 cm. Vulvar leiomyomas usually present as an asymptomatic protruding mass and extrauterine leiomyomas may be seen along the labia majora (Nielsen et al. 1996; Reyad et al. 2006). However, in our case, the patient was admitted to our hospital with a protruding mass on the right labium minora, which was causing vulvar pain. Due to its location on the labium minora and the patient having vulvar pain, she was admitted to hospital early, before it became enlarged. The major diagnostic problem with SMTs of the vulva is the distinction between benign and malignant forms, since many vulval lesions have similar appearances, making it difficult to distinguish benign from malignant lesions on gross inspection (Reyad et al. 2006). Vulvar lesions always require complete pathological examination to exclude a malignancy, even in cases with features of benign tumour (Messalli et al. 2012). In our case, immunohistochemical studies revealed accurate histopathological diagnosis of leiomyoma of the vulva. Clinically, vulvar masses are frequently thought to be Bartholin gland cysts or abscesses, although preoperative differential diagnoses include labial cysts, inguinal hernia and mesenchymal tumours. In this case, the leiomyoma was located on a labium minora, which is a very rare location of vulvar leiomyoma and thus we ruled out Bartholin cysts or abscesses. On examination, we identified a mass located on the right labium minora, not fluctuating, mobile and regular in shape, so at first we thought it to be a benign tumour or cyst of the vulva. However, benign and malignant tumours of vulva have similar symptoms. The benign features of a mass should not misguide us (Messalli et al. 2012), as although it may be reported as appearing as a benign vulvar mass, it may indeed be a delayed diagnosis of malignant vulvar mass. The preferred treatment for vulvar fibroids is local wide deep excision to prevent recurrence (Williams et al. 2002). The correct treatment is important because of the aggressive behaviour of other related mesenchymal tumours of the vulva and vagina (Kairi-Vassilatou et al. 2011). Recurrence of vulval leiomyoma is extremely rare and follow-up is seldom required. In our case, the mass was removed surgically by performing local excision. During the 10-month clinical follow-up, there was no recurrence of the tumour. Although smooth muscle cell tumours of the vulva are rarely seen in daily practice, its diagnosis is important in the evaluation of vulvar masses. This case aims to emphasise that the appearance of a leiomyoma should be part of the differential diagnoses of any vulvar mass, even in young women with a lesion involving the labia minora. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Fasih N, Prasad Shanbhogue AK, Macdonald DB et al. 2008. Leiomyomas beyond the uterus: unusual locations, rare manifestations. Radiographics 28: 1931–1948. Kairi-Vassilatou E, Dastamani C, Vouza E et al. 2011. Angiomyofibroblastoma of the vulva: a clinicopathological and immunohistochemical analysis of a rare benign mesenchymal tumour. European Journal of Gynaecological Oncology 32:353–355. Messalli EM, D’Aponte ML, Luise R et al. 2012. An apparently benign vulvar mass: possibly a rare malignancy. European Journal of Gynaecological Oncology 33:441–444. Nielsen GP, Rosenberg AE, Koerner FC et al. 1996. Smooth-muscle tumors of the vulva: a clinicopathological study of 25 cases and review of the literature. American Journal of Surgical Pathology 20:779–793. Reyad MM, Gazvani MR, Khine MM. 2006. A rare case of primary leiomyoma of the vulva. Journal of Obstetrics and Gynaecology 26:73–74. Williams NP, Williams E, Fletcher H. 2002. Smooth muscle tumours of the vulva in Jamaica. West Indian Medical Journal 51:228–231.
Unsuspected diagnosis of uterine leiomyosarcoma after laparoscopic myomectomy G. Cormio1,2, V. Loizzi1,2, O. Ceci1, L. Leone1, L. Selvaggi1 & S. Bettocchi1 1Department of Biomedical Science and Human Oncology,
Obstetrics and Gynecology Unit and 2Division of Gynecology Oncology, IRCCS Bari, Bari, Italy DOI: 10.3109/01443615.2014.937332 Correspondence: V. Loizzi, Dipartimento di Ginecologia, Ostetricia e Neonatologia, Università di Bari, Italy. E-mail: [email protected]
Introduction
Uterine sarcomas are uncommon tumours that account for ⬍ 3% of all female genital tract malignancies and 3–7% of malignant tumours of the uterus (Olah et al. 1991). Diagnosis is often made incidentally at the time of myomectomy or hysterectomy for presumed benign condition. In recent years, with the wide development of endoscopic procedures for surgical treatment of uterine fibroids, the management of an unsuspected sarcoma after laparoscopic myomectomy might become more common. This condition raises the question whether laparoscopic procedure (gas insufflation and morcellement) may increase the risk of tumour dissemination within the abdominal cavity. We report three patients who had laparoscopic myomectomy as primary surgical management of an unsuspected uterine leiomyosarcoma (LMS), and discuss the potential implications of peritoneal dissemination following endoscopic procedures in uterine sarcomas.
Case reports Between January 2000 and December 2010, 588 patients had laparoscopic myomectomy at the Department of Obstetrics and Gynecology, University of Bari, Italy, and among these, three cases (0.5%) of uterine LMS were diagnosed at pathological examination. Two of the women (34 and 37 years) were nulliparous and a 46-year-old woman was gravida 2, para 2. In all three cases, diagnosis at the time of admission was abnormal uterine bleeding due to solitary uterine fibroid. Their past medical history was uneventful. In all cases, pelvic ultrasound demonstrated the presence a subserous myoma with diameter of 4, 5 and 6 cm, respectively. Two of the three patients had received Gn-RH treatment before surgery. Laparoscopic myomectomy was performed with complete resection of the fibroid that was removed with morcellement. In one patient, the resection of the fibroid from the uterus appeared difficult and the surgeon required intraoperative frozen section examination that was inconclusive. All the resected tissue chips were extracted
Downloaded by [Chinese University of Hong Kong] at 14:57 09 November 2015
212
Gynaecology Case Reports
without an endobag and sent for pathological examination. No intraoperative or postoperative complications occurred and all patients were discharged on the following day. Diagnosis of leiomyosarcoma was given after H&E stain and immunohistochemistry. Mitotic count was 10 ⫻ 10 HPF in two cases and 5 ⫻ 10 HPF in the other case; coagulative necrosis and vascular-space invasion were found in all patients. Following diagnosis of the malignant condition, all patients were extensively staged with pelvic examination, transvaginal ultrasound, hysteroscopy, Pap smear and total-body computer tomography scan that did not revealed any sign of persistent disease. Within 28 days from primary surgery, all patients, after informed consent, were submitted to a thorough surgical staging, including total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, resection of all trocar ports, pelvic lymphadenectomy and peritoneal washings. In all three cases, pathological examination did not show any residual foci of disease either in the uterus or in peritoneal cytology, adnexae and lymph nodes. Considering the risk associated with the primary procedure, despite the lack of persistent disease, all patients received four courses of adjuvant chemotherapy with doxorubicin (30 mg/m2), hyphosphamide (3 g/m2) plus mesna on days 1, 2 and 3, repeated every 21 days. Two patients had no sign of recurrent disease after 24 and 22 months, while the third patient developed a 3 cm isolated pelvic peritoneal recurrence 42 months after primary surgery. She received surgery and pelvic radiotherapy but the disease progressed and she died 64 months after the laparoscopic procedure.
Discussion The risk of malignancy in leiomyomas is low and there are no specific characteristics to identify malignancy prior to tissue being sent for pathological review. Three large series have tried to answer the question of the frequency of LMS in series of patients undergoing surgery for presumed leiomyoma and found extremely low rates of incidental LMS, ranging from 0.08% to 0.49% (Parker et al. 1994; Leung et al. 2009; Leibsohn et al. 1990). Based on our limited experience on three cases, we showed that laparoscopic myomectomy did not increase the risk of peritoneal or distant dissemination in uterine LMS. However, there are two potential causes of concern regarding laparoscopic resection of a leiomyosarcoma: dissemination of malignant cells at the time of myomectomy and intraperitoneal dissemination of malignant cells after morcellement of the tumour. Whether laparoscopic myomectomy may increase penetration of malignant cells into deeper myometrial tissue, which might facilitate distant metastases through the haematogenous or lymphatic route is uncertain. The intense radiofrequency energy effect on tumour cells and surrounding tissue, including the microvasculature would tend to further reduce the viability of local malignant cells and their potential for intravascular dissemination and subsequent metastatic invasion. On the other hand, the practice of morcellation is a technique consisting of the fragmentation of the tissue into smaller pieces that often prevents the need to enlarge established incisions. During this procedure, it can be difficult to prevent small tissue fragments from being inadvertently dispersed throughout the peritoneal cavity; these fragments may then implant anywhere and cause symptoms and morbidity requiring intervention. Only a few studies to date have evaluated the impact of tumor morcellation on the outcomes of patients with highly malignant tumours (Morice et al. 2003; Buda et al. 2013; Park et al. 2011). Recently, Park et al. (2011) compared treatment outcomes and patterns of recurrence in patients with apparently early uterine LMS who did and did not undergo tumour morcellation during surgery. The authors showed that tumour morcellation during surgery increased the rate of abdomino-pelvic dissemination, in the form of peritoneal sarcomatosis and vaginal apex recurrence, and affected adversely disease-free and overall survivals. To our knowledge, another four studies have confirmed the impact of tumour morcellation on the outcomes of patients with uterine sarcoma (Park et al. 2011; Morice et al. 2003; Seidman et al. 2012).
However, preoperative diagnosis of leiomyosarcoma is difficult since there is no pathognomonic signs, specific symptoms, tumour markers or imaging modalities that would point to the presence of a smooth muscle malignant tumour and make a differentiation from leiomyoma (Park et al. 2011; Seidman et al. 2012). The most common signs, prolonged uterine bleeding and lower abdominal pain, are also characteristic of benign leiomyoma (Leibsohn et al. 1990). Traditionally, a rapidly growing uterus was indicative of malignancy, however the incidence of uterine LMS is extremely low, even in patients who undergo surgery, because of a rapidly growing uterus, as this symptom is not specific to uterine LMS. Among patients operated for ‘rapidly growing’ leiomyoma, Parker et al. (1994) reported that the frequency of incidental uterine LMS was very low of 0.27%. This result was also shown elsewhere (Fukunishi et al. 2007). Laparotomy should be the surgical route whenever there is preoperative suspicion of uterine LMS. However, bearing in mind the lack of specific preoperative context to evoke the diagnosis of LMS and the extremely low rates of this tumour in series of patients operated for presumed leiomyoma, the apprehension of LMS, especially in young women, should not be a criterion to avoid less invasive non-laparotomic surgical routes (Leung and Terzibachian 2012). However, when a uterine malignancy is found incidentally after morcellation, a reoperation for completion surgery and staging is mandatory. For these reasons, a discussion of risks and benefits of a surgery and consideration of reasonable alternatives are essential steps in the preparation for surgery and fundamental to the informed consent process. Therefore, the surgeon who plans to use an electric morcellator should disclose the risks of device-related complications, be explicit about the potential for dissemination of an undetected malignancy and offer the possibility of alternative surgical procedures (Kho and Nezhat 2014). Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
References Buda A, Marco C, Dolci C et al. 2013. Sentinel node mapping in high risk endometrial cancer after laparoscopic supracervical hysterectomy with morcellation. International Journal of Surgery Case Reports 4:809–12. Fukunishi H, Funaki K, Ikuma K et al. 2007. Unsuspected uterine leiomyosarcoma: magnetic resonance imaging findings before and after focused ultrasound surgery. International Journal of Gynecological Cancer 17:724–728. Kho KA, Nezhat CH. 2014. Evaluating the risks of electric uterine morcellation. Journal of the American Medical Association 311:905–6. Leibsohn S, d’Alblaing G, Mishell DR Jr et al. 1990. Leiomyosarcoma in a series of hysterectomies performed for presumed uterine leiomyomas. American Journal of Obstetrics and Gynecology 162:968–974. Leung F, Terzibachian JJ. 2012. Re: The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecologic Oncology 124:172–173. Leung F, Terzibachian JJ. Gay C et al. 2009. Hysterectomies performed for presumed leiomyomas: should the fear of leiomyosarcoma make us apprehend non laparotomic surgical routes? Gynecologie, Obstetrique et Fertilite 37:109–114. Morice P, Rodriguez A, Rey A et al. 2003. Prognostic value of initial surgical procedure for patients with uterine sarcoma: analysis of 123 patients. European Journal of Gynaecological Oncology 24:237–240. Olah KS, Gree H, Blunt S et al. 1991. Retrospective analysis of 318 cases of uterine sarcoma. European Journal of Cancer 27:1095–1099. Park JY, Kim DY, Kim JH et al. 2011. The impact of tumor morcellation during surgery on the outcomes of patients with apparently early low-grade endometrial stromal sarcoma of the uterus. Annals of Surgical Oncology 18: 3453–61. Park JY, Park SK, Kim DY et al. 2011. The impact of tumor morcellation during surgery on the prognosis of patients with apparently early uterine leiomyosarcoma. Gynecologic Oncology 122:255–9. Parker WH, Fu YS, Berek JS. 1994. Uterine sarcoma in patients operated on for presumed leiomyoma. Obstetrics and Gynecology 83:414–8. Seidman MA, Oduyebo T, Muto MG et al. 2012. Peritoneal dissemination complicating morcellation of uterine mesenchymal neoplasms. PLoS ONE 7:e50058.
