{"paper_id":"4b1e9ee8-32a1-4fea-a964-950bca84fadd","body_text":"Primary Broad Ligament Endometroid \nAdenocarcinoma: A Case Report and Review of \nLiterature\n1\nMedDocs Publishers\nReceived: Feb 22, 2023\nAccepted: Mar 24, 2023\nPublished Online: Mar 31, 2023\nJournal: Journal of Gynecology Case Reports\nPublisher: MedDocs Publishers LLC\nOnline edition: http://meddocsonline.org/\nCopyright: © Gupta S (2023). This Article is\ndistributed under the terms of Creative Commons \nAttribution 4.0 International License\n*Corresponding Author(s): Sunita Gupta\nConsultant Obstetrics and Gynecology NMC Specialty \nHospital, Abu Dhabi (UAE). \nEmail: drsunitachauhan@gmail.com\nJournal of Gynecology Case Reports\nOpen Access | Case Report\nCite this article: Gupta S, Malik S, Balasubramaniam B, Sambhukumar V. Primary Broad Ligament Endometroid \nAdenocarcinoma: A Case Report and Review of Literature. J Gynecol Case Rep. 2023; 2(1): 1004.\nSunita Gupta1*; Savita Malik1; Balaji Balasubramaniam2; Priya Shambhukumar3\n1Consultant Obstetrics and Gynecology, NMC Specialty Hospital, Abu Dhabi (UAE).\n2Consultant surgical oncology, NMC Specialty Hospital, Abu Dhabi (UAE).\n3Specialist anaesthesiology, NMC Specialty Hospital, Abu Dhabi (UAE).\nIntroduction\nSince the broad ligament is closely related to other struc -\ntures, such as the uterus, fallopian tubes, and ovaries, second -\nary involvement of it due to primary malignancy elsewhere is \ncommon. Primary broad ligament malignancies are rare. Only \n29 cases of primary broad ligament malignancies have been re-\nported in the literature, out of which only 5 cases of endometri-\noid adenocarcinoma [1]. Generally, the primary broad ligament \ntumor diagnosis is made retrospectively, intraoperatively, or on \nhistopathological examination, due to its rarity.\nI have encountered a case where laparotomy and myo -\nmectomy were planned with a preoperative diagnosis of large \nsubserous leiomyoma and intramural leiomyoma. Per opera -\ntively large friable mass was present between 2 leaves of the \nbroad ligament on the right side, and a large adenomyoma was \npresent in the posterior wall of the uterus. Subsequently, ad -\nenomyomectomy and excision of the broad ligament mass was \ndone. On histopathology, the broad ligament mass was diag -\nnosed as endometroid adenocarcinoma, and the uterine mass \nas an adenomyoma.\nCase report \nA 43-year-old nulliparous female presented to the clinic with \ncomplaints of amenorrhea, infertility, and mild pain in the lower \nabdomen for three months. She has been evaluated clinically, \nand on examination, a pelvic mass corresponding to 20 weeks \nof gestation was palpated. On further evaluation by ultrasonog-\nraphy and MRI pelvis: a large subserous fibroid from the uterus \nmeasuring approximately 10.6cm x 8.3 cm x 14 cm and an intra-\nmural fibroid of 7 cm x 6.1 cm indenting endometrium is seen. \nThe right ovary was seen anterior to the subserous fibroid. The \n\nMedDocs Publishers\n2Journal of Gynecology Case Reports\nleft ovary is seen in the pouch of Douglas. The cervix appears \nnormal, and no enlarged lymph node is seen. Mild free fluid \nwas noted in the pelvis. The patient was nulliparous and will -\ning for future pregnancy, so the decision to perform laparotomy \nand myomectomy was taken. Per operatively, mild hemorrhagic \nascites was present. A friable mass protruding from the poste -\nrior leaf of the broad ligament on the right side and an adeno -\nmyoma in the posterior wall of the uterus was present. Broad \nligament mass was excised, and adenomyomectomy of uterine \nmass was done. Broad ligament mass was not attached to the \nuterus, right side ovary, or fallopian tube though they looked \nedematous. The left side tube and ovary were normal. As the \nmass was suspected to be malignant due to physical character \nomental biopsy was taken. On histopathology, the uterine mass \nwas confirmed to be adenomyoma, but the broad ligament \nmass was diagnosed as endometrioid adenocarcinoma. Omen -\ntum was negative for any specified disease. She was referred \nto the oncosurgeon. Computerized Tomography (CT) chest, \nabdomen, and pelvis were performed further to rule out local \nand distant metastasis. In the CT chest -no metastatic foci were \nseen, and CT abdomen pelvis shows no liver metastasis, ascites, \nor abdominal lymph adenopathy. A large hypodense lesion, ap-\nproximately 6.3 cm x 4.8 cm seen in the right parametrium and \nright adnexal region with soft tissue swelling noted, which was \nsuspicious of residual disease. The surgical oncology team did \ntotal abdominal hysterectomy, bilateral salpingo-opherectomy, \npelvic lymphadenectomy, and omental biopsy. On histopathol-\nogy, foci of atypical endometriosis in the background of endo -\nmetriosis in the right ovary and atypical glandular hyperplasia \nin uterine adenomyotic foci without any evidence of invasion \nwere seen. Left ovarian endometriosis was present. Pelvic \nlymph nodes were negative without any evidence of malignan-\ncy in the sent specimens. The final diagnosis of primary broad \nligament endometrioid adenocarcinoma was made.  She re -\nceived postoperative chemotherapy (carboplatin + paclitaxel). \nShe has been doing well to date without recurrence for more \nthan one year.\nDiscussion \nPrimary broad ligament tumors are defined by the proposed \ncriteria given by Gardner et al., Primary location within or on \nthe surface of the broad ligament but completely separated \nfrom the uterus, ipsilateral ovary, and fallopian tube [2]. \nMalignant tumors of the broad ligament are extremely rare; \ntherefore, their etiopathogenesis, optimum management, fol -\nlow-up strategy, and prognosis are still uncertain. \nPatients usually present with vague complaints, pain or \nheaviness in the lower abdomen or a lump abdomen. Some -\ntimes it may be silent and detected incidentally during evalu -\nation for other problems. In our case, the patient came with \ncomplaints of amenorrhea, infertility, and mild pain in the lower \nabdomen. Initially, the decision for laparotomy and myomec -\ntomy was taken due to her willingness for future fertility. \nPrimary endometrioid carcinoma of the broad ligament is \nrare, and only 5 cases have been reported. Most of the epithe -\nlial malignancies of the broad ligament are derived from the \nMullerian remnants, including (i) serous, (ii) mucinous, (iii) clear \ncell, (iv) endometrioid, (v) borderline tumors and (vi) endome -\ntrial stromal sarcomas. Among all the reported carcinomas, \nserous adenocarcinoma, clear cell adenocarcinoma, and en -\ndometrioid adenocarcinoma are the most common histologic \nvariants [2]. Other rare malignancies reported to arise from the \nbroad ligaments are (i) Sex cord-stromal tumors, (ii) Neuroen -\ndocrine carcinomas (small cell and large cell), (iii) Sarcomas and \n(iv) Female Adnexal Tumor of probable Wolffian Origin (FATWO) \n[1]. In our case, evidence of endometriosis in both ovaries with \nfoci of atypical changes and adenomyosis with atypical changes \nhave been seen. The development of primary broad ligament \nendometrioid carcinoma could be hypothetically explained by \nthe malignant transformation of long-standing endometriosis \nof the broad ligament, as endometriotic involvement of the \nbroad ligament is common. Malignant transformation occurs in \n0.7-1% of patients with endometriosis, particularly in the ova -\nries [3]. The most frequent sites of extragonadal involvement \nare the rectovaginal septum, the colon, and the vagina [4]. ova-\nry is the primary site in 79 percent of cases, and extragonadal \nsites are identified in 21 percent [5]. Two cases of endometrioid \ncarcinoma and a case of clear cell carcinoma developed in the \ndiaphragm were reported. Both patients had a history of endo-\nmetriosis/adenomyosis [6]. A rare endometrioid carcinoma de-\nveloped from deep infiltrating endometriosis in the uterosacral \nligament six years after treatment for an atypical proliferative \nendometrioid tumor of the ovary in a 48-year-old woman re -\nported [7].\nManagement and prognosis of broad ligament endometrioid \nadenocarcinoma are still uncertain. Most of the cases patients \nwere managed by total abdominal hysterectomy with bilateral \nsalpingectomy with pelvic lymphadenectomy and/or peritoneal \nand omental biopsies. The role of Postoperative chemotherapy/\nradiotherapy is still not established due to the paucity of cases. \nAs histopathologically they have similarities with ovarian endo-\nmetrioid carcinoma, most authors advise adjuvant platinum-\nbased chemotherapy (paclitaxel and carboplatin) [1]. Our pa -\ntient received a similar regimen. Most cases of broad ligament \ncancer were assumed to be in the early stage (PT1) and have a \nfavorable prognosis; 5-year survival rates of 80-90% were re -\nported [8] aggressive progression is defrayed, and metastasis is \nuncommon because they lack their blood supply, and lymphatic \ndrainage in contrast to ovarian cancers [9].\n Primary broad ligament malignancy should be a differential \ndiagnosis of uterine, adnexal masses. Both clinicians and radi -\nologists require a high index of suspicion to diagnose broad liga-\nment malignancies pre-operatively. In a few cases, CA 125 was \nfound, but this is a non-specific marker raised in several benign \nand malignant gynecological and non-gynecological conditions. \nThere is no specific biomarker has been established for endo -\nmetrioid adenocarcinoma (ovarian or nonovarian origin). Sev -\neral epidemiological studies observed the close association of \nendometriosis with particular histology of endometriosis-asso -\nciated ovarian cancer, endometrioid, and clear cell carcinoma, \nespecially when the diagnosis of endometriosis was more ev -\nidence-based, such as through surgical-pathological diagnosis \n[10]. The overall rate of malignant transformation in endome -\ntriosis has been estimated to be 0.3-0.8%, with a relative risk \nranging from 1.3 to 1.9 [11] and should be suspected if a suspi-\ncious mass is found even at the extragonadal site in a patient \nwith longstanding endometriosis or with symptoms suggestive \nof endometriosis, so that timely diagnosis ensures expeditious \nand efficient management and favorable prognosis.\nReferences\n1. Elangovan A, Dracham CB, Muddabhaktuni MC, Zaidi A. Pri -\nmary broad ligament adenocarcinoma. Autopsy & Case Reports. \n2020; 10. \n\nMedDocs Publishers\n3Journal of Gynecology Case Reports\n2. Gardner, George Henry et al. “Tumors of the broad ligament.” \nAmerican journal of obstetrics and gynaecology. 1957; 73: 536-\n554.\n3. Heaps JM, Nieberg RK, Berek JS. Malignant neoplasms arise in \nendometriosis. Obstet Gynecol. 1990; 75: 1023-1028. \n4. Benoit L, Arnould L, Cheynel N et al. Malignant extra ovarian \nendometriosis: A review. Eur J Surg Oncol. 2006; 32: 6-11\n5. Hoang CD, Boettcher AK, Jessurun J, Pambuccian SE, Bullard KM. \nAn unusual rectosigmoid mass: endometrioid adenocarcinoma \narising in colonic endometriosis: case report and literature re -\nview. Am Surg. 2005.\n6. Okimura H, Tatsumi H, Ito F, Yamashita S, Kokabu T, Kitawaki J. \nEndometrioid carcinoma arising from diaphragmatic endometri-\nosis treated with laparoscopy: A case report. J Obstet Gynaecol \nRes. 2018; 44: 972-977.\n7. Ota Y , Ota K, Takahashi T, Suzki S, Sano R, et al. Primary endome-\ntrioid carcinoma of the uterosacral ligament arising from deep \ninfiltrating endometriosis 6 years after bilateral salpingo-oopho-\nrectomy due to atypical proliferative endometrioid tumor of the \novary: a rare case report. World J Surg Oncol. 2020; 18: 329. \n8. Rojansky N, Ophir E, Sharony A, Spira H, Suprun H. Broad liga -\nment adenocarcinoma—its origin and clinical behavior. A litera-\nture review and report of a case. Obstet Gynecol Surv 1985; 40: \n665-671.\n9. Handa Y , Kato H, Kaneuchi M, Saitoh Y , Yamashita K. High-grade \nbroad ligament cancer of müllerian origin: immunohistochemi -\ncal analysis of a case and review of the literature. Int J Gynecol \nCancer. 2007; 17: 705-709.\n10. Torng P . Clinical implication for endometriosis associated with \novarian cancer. Gynecology and Minimally Invasive Therapy. \n2017; 6: 152-156. \n11. Sayasneh A, Tsivos D, Crawford R. Endometriosis and ovarian \ncancer: a systematic review. ISRN Obstet Gynecol. 2011; 2011: \n140310.","source_license":"CC0","license_restricted":false}