Primary Leiomyosarcoma of Broad Ligament: Case Report and Review of Literature

Arch Gynecol Obstet (2009) 279:705–708 DOI 10.1007/s00404-008-0777-2

CASE REPORT

Primary leiomyosarcoma of broad ligament: case report and review of literature

Nirmala Duhan · Sunita Singh · Yogender Singh Kadian · Urmila Duhan · Nidhi Rajotia · Neetu Sangwan

Received: 30 June 2008 / Accepted: 18 August 2008 / Published online: 6 September 2008 © Springer-Verlag 2008

Abstract Gardner et al. only few cases have been reported in litera- Introduction Primary leiomyosarcoma of the broad liga- ture [1–16]. ment (LBL) is a very rare and highly malignant gynecolog- We describe here the 17th case of this rare and highly ical tumor. malignant tumor and the Wrst in hysterectomised woman. Materials and methods A 45 year old hysterectomized The diagnosis and management are also discussed. woman with this rare tumor is presented. Treatment con- sisted of resection of the tumor along with omental and peritoneal sampling and adjuvant chemotherapy. No evidence Case report of metastasis was present after 15 months of follow up. Conclusion Complete surgery along with adjuvant che- A 45 years old, para 4 woman presented to this tertiary care motherapy or radiation and a close follow up for recurrence centre with symptoms of dull pain and lump in lower abdo- are advocated to optimize disease free survival. men for the last 6 months. She had undergone a hysterectomy 4 years ago for heavy menstruation unresponsive to Keywords Leiomyosarcoma · Broad ligament conservative treatment. There was no other signiWcant gynecological or past medical history. Abdominal examination revealed a puckered midline Introduction vertical scar of previous surgery. The lower abdomen was distended with a Wrm to hard, immobile mass of 17 £ 20 cm Primary leiomyosarcomas of the broad ligament (LBL) are size, arising out of the pelvis. There was no ascites. The very rare tumors. According to the strict criteria given by vault was healthy, on speculum examination. Bimanual pelvic examination revealed a large, immobile, lobulated N. Duhan (&) · N. Rajotia · N. Sangwan mass Wlling the pelvis and in continuation with the abdominal Department of Obstetrics and Gynecology, mass. A clinical diagnosis of malignant ovarian tumor was Pt. B.D. Sharma Postgraduate Institute of Medical Sciences, made. 6/9J, Medical Campus, Rohtak, Haryana 124001, India e-mail: [email protected] Ultrasonography documented a 20 cm diameter solid mass Wlling the pelvis and lower abdomen. Computed S. Singh tomography (CT) of the abdomen also suggested a large Department of Pathology, Pt. B.D. Sharma Postgraduate Institute complex mass in lower abdomen and pelvis. Liver and kid- of Medical Sciences, Rohtak, Haryana, India neys were normal and there was no ascites or paraaortic or Y. S. Kadian iliac lymphadenopathy. The serum CA-125 was 35 U/mL. Department of General Surgery, Pt. B.D. Sharma Postgraduate After a complete workup, laparotomy was done which Institute of Medical Sciences, Rohtak, Haryana, India revealed a large (18 £ 24 cm), cherry red coloured, lobu- W U. Duhan lated, rm mass in lower abdomen and pelvis with multiple Department of Medicine, areas of hemorrhage and necrosis. It was adherent to the University of Chicago, Chicago, IL, USA anterior abdominal wall, urinary bladder, gut and omentum. 123 706 Arch Gynecol Obstet (2009) 279:705–708

A tube that was stretched over the tumor and both ovaries, after extensive sampling were free from inWltration. Omental and peritoneal biopsies did not reveal metastasis. In view of the diagnosis of leiomyosarcoma of broad ligament, adjuvant postoperative combination chemotherapy (vincristine, actinomycin D and cyclophosphamide) was given. The woman is disease free after 15 months of follow up.

Discussion

Gardner et al. proposed the deWnition of tumors of the broad ligament, requiring that they “occur in or on the broad ligament, but are completely separated from and in Fig. 1 Microphotograph of the leiomyosarcoma of broad ligament no way connected with either the uterus and the ovary” [1]. showing mitotic activity (H & E, £400) Using these criteria, this is the 17th case of this condition being reported (Table 1). However, features other than ana- On separating these structures, both the ovaries came into tomic connection to uterus or ovaries appear to play a role view and appeared normal. No abnormality was palpable in deWning adjuvant postoperative therapy, follow up and on the liver, undersurface of diaphragm, paraaortic lymph- prognosis. Zaloudek and Norris included mitotic Wgures (5 nodes, gut, omentum and peritoneum. Paracolic washings or more vs. fewer), cellularity and nuclear atypia as impor- were taken for cytology, followed by excision of the tumor tant diVerentiating criteria [17]. Coindre et al. advocated a and bilateral oophorectomy along with omental and perito- three grade system using mitotic Wgures, tumor diVerentia- neal biopsies. The woman fared well postoperatively. tion and tumor necrosis, to be of more prognostic and thera- Though the paracolic washings were negative for malig- peutic value [18]. nant cells, the histology of the tumor revealed a malignant Leiomyoma is the most common primary mesenchymal spindle cell tumor with more than 10 mitosis per 10 high tumor of the broad ligament. Myolipoma, angioleiomyoma power Welds (HPF), suggestive of leiomyosarcoma (Fig. 1). and solitary Wbrous tumor are some other rare benign

Table 1 Leiomyosarcoma of broad ligament Sr. No. Author(s) Age Mitosis/10 HPF Initial treatment Subse-quent Survival therapy after diagnosis

1. Lowell and Karsh 35 0–4 TAH, BSO – >12 months 2. Ullman and Roumell 50 15 TAH, BSO RT, CT 7 months 3. Weed and Podger 50 12 TAH, BSO CT 19 months 4. DiDomenico et al. 48 10.5 TAH, BSO – – 5. Raj Kumar 70 <10 Enucleation – – 6. Herbold et al. 73 21 TAH, BSO – 1 month 7. Shimm et al. 31 08 Enucleation, RT CT >30 months 8. Lee et al. 36 >10 TAH, BSO, CT RT >33 months 9. Lee et al. 65 >10 STAH, BSO, CT – 30 months 10. Cheng et al. 59 >10 TAH, BSO – >12 months 11. T.Pekin et al. 56 14 TAH, BSO RT >12 months 12. Shah et al. 87 30–40 TAH, BSO – 2 months 13. Agarwal et al. 55 >10 TAH, BSO, Resection CT >12 months 14. El Idrissi et al. 52 – TAH, BSO, Resection None 3 months 15. Murialdo et al. 53 <10 TAH, BSO None 13 months 16. Ben et al. 49 – TAH, BSO None 5 months 17. Duhan et al. (Present study) 45 10 Resection CT 15 months, Alive TAH total abdominal hysterectomy, STAH subtotal hysterectomy, BSO bilateral salpingo-oophorectomy, CT chemotherapy, RT radiotherapy, HPF high power Weld 123 Arch Gynecol Obstet (2009) 279:705–708 707 tumors reported in this region [19–21]. The rare malignant survived only for one and two months, respectively [7, 12]. tumors reported in the broad ligament which need to be dis- However, both had undergone only surgical resection, tinguished from leiomyosarcoma include malignant Wbrous without any adjuvant therapy. Conversely, Lee et al. histiocytoma, alveolar soft part sarcoma and hyalinizing reported the maximum survival of more than 33 months in spindle cell tumor. LBL are very rare tumors and most are their case who had >10 mitosis/HPF and was treated by of poor prognosis, specially those with 10 or more mitotic surgery followed by chemotherapy and radiation [9]. How- Wgures per 10 HPFs. A few case reports of malignant trans- ever, the view that multi-modality therapy is better than formation in a pre-existing leiomyoma and of recurrent single modality of treatment also may not hold true for all. leiomyosarcoma in pelvis exist in literature [22, 23]. In the case reported by Ullman and Roumell, a combina- Microscopically, the pattern of growth in a leiomyosarcoma tion of surgery, chemotherapy and radiation did not is predominantly fascicular. The individual cells have elon- improve the survival much as the woman died after gated, blunt nuclei and acidophilic Wbrillary cytoplasm. 7 months of diagnosis, though the tumor had 15 mitosis/10 Areas of hemorrhage and necrosis and high mitotic count HPFs [3]. diVerentiate it from leiomyoma. Immunohistochemically, In view of the unpredictable behaviour of this rare tumor these tumors exhibit reactivity for actin, smooth muscle and the empirical criteria used for its management, com- myosin, desmin and estrogen receptors, thus raising the plete surgery supplemented with adjuvant chemotherapy or possibility of hormone responsiveness [24]. radiation and a close follow up for recurrence would help Management of LBL is considered to be the same as for optimize the chances of disease free survival. that of uterus. Total abdominal hysterectomy and bilateral salpingo-oophorectomy represents the standard surgical treatment for uterine sarcomas. Pelvic and para-aortic lym- References phadenectomy is recommended for carcinosarcomas but V 1. Gardner GH, Greene RR, Peckham R (1957) Tumors of the broad not for leiomyosarcoma and undi erentiated endometrial ligament. Am J Obstet Gynecol 73:536–555 sarcoma. Some of these cases show an incidence of nodal 2. 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Recently, xed-dose gemcitabine (900 mg/m 90(2):450–452. doi:10.1016/S0090-8258(03)00208-7 2 on day 1 and 8) used along with docetaxel (100 mg/m on 13. Agarwal U, Dahiya P, Sangwan K (2003) Leiomyosarcoma of the day 8) has been shown to achieve high objective response broad ligament mimicking as ovarian carcinoma—a case report. rates as a Wrst line therapy in metastatic uterine leiomyosar- Arch Gynecol Obstet 269(1):55–56. doi:10.1007/s00404-002- 0418-0 comas [27]. 14. El Idrissi F, Fadli A (2004) Leiomyosarcoma of the broad liga- Reported survival varies from 4 weeks to 33 weeks ment. Presse Med 33(15):1004–1005. doi:10.1016/S0755-4982 [7, 9]. The two cases with 21 and 30–40 mitosis/10HPF (04)98823-9 123 708 Arch Gynecol Obstet (2009) 279:705–708

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