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Abstract: Extrauterine leiomyomas are rare and often occur in broad ligament or fallopian tube. Diagnosis of broad ligament leiomyoma is challenging for its solid nature and special location. Leiomyomas typically exhibit mild FDG uptake, especially in postmenopausal woman. We here reported a 57-year-old postmenopausal woman with intense FDG-avid broad ligament leiomyoma on 18 F-FDG PET/CT, which might be misdiagnosed as ovarian tumor., Competing Interests: Conflicts of interest and sources of funding: The authors have no conflicts of interest to report. This work was funded by the CAMS innovation fund for medical science (no. CIFMS-2022-I2M-JB-00I)., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Mesonephric lesions in the female genital tract are uncommon, with those arising from the upper tract being much less frequent than those developing in the lower tract (mesonephric hyperplasia and carcinoma). The most common upper tract lesions include rete cyst/cystadenoma and female adnexal tumour of Wolffian origin (FATWO). The integration of morphological, immunohistochemical and molecular studies on FATWOs has enabled recognition of a novel entity, the STK11 adnexal tumour, which is often associated with Peutz-Jeghers syndrome (~50%) and frequently has a salivary gland morphology but an unknown origin. Similarly, 'mesonephric-like' adenocarcinoma, an entity with striking similarities to mesonephric carcinoma but currently favoured to be of Müllerian derivation based on its association with other Müllerian tumours and molecular findings, has also been recently described, and may histologically mimic both FATWOs and STK11 adnexal tumours. In this review, we provide a historical overview of upper female genital tract mesonephric proliferations and discuss mesonephric lesions, STK11 adnexal tumour, mesonephric-like adenocarcinoma, and mimickers, the most common being endometrioid carcinoma., (© 2022 John Wiley & Sons Ltd.)

A novel molecular subset of epithelioid leiomyosarcomas with rhabdoid features harboring PGR gene rearrangements has recently been documented. Herein, we present a unique case of PGR-rearranged smooth muscle tumor with both PGR-NR4A3 and UBR5-PGR gene fusions reported in a 30-year-old woman who had a mass in the broad ligament. The histological examination showed a round/polygonal to spindle cell tumor with abundant myxoid matrix and focal hyalinization, resulting in an epithelioid pattern. Immunohistochemical examination revealed that the tumor had variable staining for desmin, SMA, and h-caldesmon and diffuse nuclear staining of ER, PR, and WT1. Furthermore, targeted RNA sequencing analysis revealed PGR-NR4A3 and UBR5-PGR gene fusions. Our case in addition with the reported cases suggest that myxoid matrix with two types of tumor cells (round/polygonal epithelioid cells and spindle cells) may be significant for the diagnosis of PGR-NR4A3 fusion-positive leiomyosarcoma. UBR5-PGR gene fusion is a novel finding in epithelioid leiomyosarcoma., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Rationale: Female adnexal tumors of probable Wolffian origin (FATWOs) are rare gynecologic neoplasms arising from the mesonephric duct remnants. Less than 90 cases have been reported in the English literature. Although most cases of FATWO are considered benign, recurrence and metastasis may occur in very few cases during the course of the disease. Due to the small number of recurrent and metastatic FATWO cases, there are no clear recommendations regarding optimal treatment., Patient Concerns: A 75-year-old postmenopausal woman, who underwent a mass excision of the right broad ligament three years ago, was found to have a right adnexal mass during a regular postoperative physical examination., Diagnoses: Vaginal ultrasound examination revealed a cystic and solid mass approximately 3.6 × 4.4 × 3.8 cm on the right side of the uterus. Three years ago, the mass of the right broad ligament was diagnosed with FATWO in the local hospital. Following extensive immunohistochemistry analysis and after reviewing the histology slides from the primary tumor, the final diagnosis of the mass on the right side of the uterus was recurrent and metastatic FATWO., Interventions: The patient underwent laparoscopic mass excision, hysterectomy and resection of the metastatic lesion in the small intestine, and then she received 6 cycles of docetaxel and carboplatin-based chemotherapy., Outcomes: The disease has recurred three years after the first surgery in the local hospital. After the second surgery followed by systemic chemotherapy, there is no evidence of recurrence with 24 months of follow-up till now., Lessons: FATWO is considered a benign entity. However, a few FATWOs have been shown to behave aggressively. Due to only a few reported cases, there are no comprehensive recommendations regarding the optimal clinical management of recurrent and metastatic FATWOs. Complete surgical resection followed by combination chemotherapy is considered to be the most effective therapy for recurrent and metastatic FATWOs. Chemotherapy with docetaxel plus carboplatin, which is most commonly used in malignant cases, may be effective in the treatment of recurrent and metastatic FATWOs., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: We sought to assess hernia characteristics and classification through comprehensive review of the literature involving broad ligament herniation., Methods: A literature search via MEDLINE and Embase databases was conducted to identify and select broad ligament herniation studies published between January 1, 2000 and September 30, 2020. Extracted data included previous surgical history, previous obstetric history, diagnostic imaging, herniated organ, hernia classification, and repair performed. The reported data has been compared to a unique case of broad ligament herniation that presented to our institution., Results: A total of 44 articles with 49 cases were identified for the study. Eighteen (36.7%) patients had a history of previous abdominal surgery while 29 (59.2%) had a history of previous childbirth. Type I (51.0%) and Type II (18.4%) defects were most commonly reported with most patients reporting only one defect (85.7%) using the Cilley classification. Twenty-nine patients underwent primary laparoscopic repair of the defect while 19 patients underwent exploratory laparotomy., Conclusions: The analysis of previously reported cases adds to the limited literature on broad ligament hernias and highlights the surgical management of this uncommon pathology. It also highlights the need for a broad differential diagnosis when female patients present with pelvic pain or symptoms of small bowel obstruction. The broad ligament should be fully inspected when mesenteric defects are suspected as multiple defects can be present as evidenced by the attached case study., Competing Interests: Conflict of interests: none., (© 2021 by SLS, Society of Laparoscopic & Robotic Surgeons.)

Study Objective: The objective of this study was to compare the morbidity of vaginal versus laparoscopic hysterectomy when performed with uterosacral ligament suspension., Design: Retrospective propensity-score matched cohort study., Setting: American College of Surgeons National Surgical Quality Improvement Program database., Patients: We included all patients who had undergone uterosacral ligament suspension and concurrent total vaginal hysterectomy (TVH-USLS) or total laparoscopic hysterectomy (TLH-USLS) from 2010 to 2015. We excluded those who underwent laparoscopic-assisted vaginal hysterectomy, abdominal hysterectomy, other surgical procedures for apical pelvic organ prolapse, or had gynecologic malignancy., Interventions: We compared 30-day complication rates in patients who underwent TVH-USLS versus TLH-USLS in both the total study population and a propensity score matched cohort., Measurements and Main Results: The study population consisted of 3,349 patients who underwent TVH-USLS and 484 who underwent TLH-USLS. Patients who underwent TVH-USLS had a significantly higher composite complication rate (11.4% vs 6.4%, odds ratio [OR] 1.9, 1.3-2.8; p <.01) and a higher serious complication rate (5.6% vs 3.1%, OR 1.8, 1.1-3.1; p = .02), which excluded urinary tract infection and superficial surgical site infection. The propensity score analysis was performed, and patients were matched in a 1:1 ratio between the TVH-USLS group and the TLH-USLS group. In the matched cohort, patients who underwent TVH-USLS had a higher composite complication rate than those who underwent TLH-USLS (10.3% vs 6.4%, OR 1.7, 95% confidence interval [CI], 1.1-2.7; p = .04), whereas the rate of serious complications did not differ between the groups (4.3% vs 3.1%, OR 1.4, 95% CI, 0.7-2.8; p = .4). On multivariate logistic regression, TVH-USLS remained an independent predictor of composite complications (adjusted OR 1.6, 95% CI, 1.0-2.6; p = .04) but not serious complications (adjusted OR 1.4, 95% CI, 0.7-2.8; p = .3)., Conclusion: In this large national cohort, TVH-USLS was associated with a higher composite complication rate than TLH-USLS, largely secondary to an increased rate of urinary tract infection. After matching, the groups had similar rates of serious complications. These data suggest that TLH-USLS should be viewed as a safe alternative to TVH-USLS., (Copyright © 2020 AAGL. Published by Elsevier Inc. All rights reserved.)

Study Objective: Recently, there has been a paradigm shift toward uterine conservation during the surgical management of pelvic organ prolapse (POP), specifically uterine prolapse. There are few reports on transvaginal uterosacral ligament hysteropexy (TULH). This study aimed to describe our surgical technique and outcomes., Design: Retrospective review and description of surgical technique. Anatomic outcome has been reported using the POP quantification system. Complications were segregated. A comparison of parametric continuous variables was performed using paired t test. Categoric variables were evaluated using the Pearson χ 2 test and the Fisher exact test. A p-value <.05 was considered significant., Setting: Teaching hospital., Patients: Forty patients who underwent TULH from 2009 to 2017., Interventions: TULH., Measurements and Main Results: A total of 40 patients met the inclusion criteria. Of these, 56.1% had preoperative stage 3 prolapse. The median operative time was 116 minutes. The mean estimated blood loss was 158.5 mL. Transient ureteral obstruction occurred in 2 patients. The mean follow-up time was 17.2 months, and all patients had significant improvement of prolapse (p <.001). There was also an improvement in urinary incontinence and bladder storage symptoms (p <.001). None of the patients were reoperated on for recurrent POP., Conclusion: TULH is an effective uterus-preserving surgical alternative for the treatment of uterovaginal prolapse and provides good apical support. It is also associated with a low short-term recurrence and incidence of reoperation. TULH is a viable option for suitable patients with uterovaginal prolapse who desire uterine conservation., (Copyright © 2020 AAGL. Published by Elsevier Inc. All rights reserved.)

Background: Leiomyosarcoma usually develops in the myometrium and is characterized by a high recurrence rate, frequent hematogenous dissemination, and poor prognosis. Metastasis is usually to lungs, liver, and bone, and occasionally to the brain, but seldom to the head and neck region. Primary leiomyosarcoma very rarely arises in the broad ligament., Case Presentation: A 54-year old woman presented to the otolaryngology department with a mass in the right posterior region of the neck 4 years after surgery for a primary leiomyosarcoma of the right broad ligament. The neck mass was removed and found to be a metastatic leiomyosarcoma. Leiomyosarcoma localizations in lungs and liver were absent. Morphological examination showed both the primary and the secondary leiomyosarcomas to have features of low-grade tumors. One year after excision of the neck mass, the patient presented with tachycardia. Echocardiography detected two intracardiac nodules suggestive of metastatic tumors. Chemotherapy was administered; the disease has been stable since then., Conclusions: We report the first case of broad ligament leiomyosarcoma with the neck subcutaneous region being the first site of secondary involvement. We speculate that the Batson venous plexus might have been the pathway of dissemination.

Steroid cell tumors occur usually in the ovaries with very few reported cases of extra-ovarian origin. Our patient was a fifteen year old female, complaining from secondary amenorrhea and voice deepening. Values of serum cortisol, DHEA, FSH & LH were normal. Serum Testosterone was elevated while ACTH-pm was markedly elevated. MRI described bilateral solid para-ovarian masses. Exploration revealed two bilateral tubal extraluminal cysts & a right broad ligament cyst which were all excised. Pathological examination led to the diagnosis of steroid cell tumor. Serum testosterone & ACTH returned to normal levels after surgery with subsequent regression of the virilizing symptoms. We can conclude that extra-ovarian steroid cell tumors are extremely rare. They are usually presented with virilizing symptoms and hormonal abnormalities. Surgery is the main line of treatment.

Rationale: Uterus-like masses (ULMs) are rare benign lesions that resemble the uterus., Patient Concerns: Here, we describe the case of a woman with a ULM in the right broad ligament. A 51-year-old woman with a 2-month history of irregular vaginal bleeding was found to have a mass in the right broad ligament. Imaging studies revealed a solid-cystic lesion, suggestive of an endometrial cyst with malignant transformation., Interventions: She underwent prompt surgery for the removal of the mass. Intraoperatively, the uterus and ovaries appeared normal, and an 8-cm-long mass was observed in the right broad ligament without any connection to the uterus or ovaries. The mass was successfully excised., Diagnoses: Postoperative histopathological examination showed that the cystic mass was filled with a blackish-brownish fluid and that it had thick walls resembling the uterine myometrium. The cyst center was lined by endometrial glands that were positive for cytokeratin as well as estrogen and progesterone receptors, and by stromal cells that were positive for CD10., Outcomes: The patient recovered well and has had no further symptoms during 2 years of follow-up., Lessons: We have reported a case of ULM in the right broad ligament in a Chinese woman. Although ULMs are rare, they should be considered in the differential diagnosis for pelvic masses.

Background: Adnexal paraovarian cysts are not frequently seen during teen development, their incidence is around 10% and usually benign., Clinical Case: Adolescent female 15 years old with no pathological family and personal history relevant to her current condition. Chief complaint: Six months ago she complained with abdominal pain in meso, hypogastrium and right iliac fossa. Later, she observed an increased volume on her lower quadrant of the abdomen, mostly on her right side. During physical examination an abdominal tumor was palpated. By an abdominal-rectal manouvre, the presence of tumor was confirmed and located in front of the rectum and no implants. Imaging studies confirmed a paraovarian cyst. She underwent on surgical laparatomy and a paraovarian cyst was found. The histological diagnosis was a cystadenoma. The postoperative course was satisfactory., Discussion: Epidemiological data and ultrasonographic findings are examined to confirm the diagnosis of paraovarian cyst. The laparoscopic treatment for adnexal problems is described., (Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.)

Angiomyofibroblastoma (AMF) is a distinctive, rare, benign mesenchymal tumor that often occurs in the lower genital region of women. The most commonly reported location of an AMF is in the vulvovaginal area. We describe a rare case of an AMF located in the broad ligament in a 47-yr-old woman. The patient experienced menorrhagia, dysmenorrhea, and subsequent menstrual spotting. She sought help at the National Cheng Kung University Hospital. Ultrasonography showed an echo-complex mass in the left adnexal area. The patient underwent laparoscopic surgery to remove the soft tissue mass located in the left broad ligament. The final pathology of the mass was reported as an AMF. We reviewed all of the AMF cases reported in the English-language literature found in Pubmed. This case is the first of AMF located in the broad ligament.

Extraovarian sex cord-stromal tumor is an exceedingly uncommon entity that may cause a diagnostic dilemma clinically. We report a case of extraovarian fibroma with minor sex cord elements arising in the left broad ligament. The patient was a 66-year-old woman presenting with an intra-abdominal solid mass near the left ovary on magnetic resonance imaging. The tumor was located in the left broad ligament in contact with the left ovary and fallopian tube based on laparotomy findings. Histological examination revealed that the tumor was a fibroma that contained cell nests with aggregates resembling the Call-Exner bodies of granulosa cell tumors and irregularly shaped cell nests composed of undifferentiated sex cord-type cells. Cellular atypia or mitotic figures were not identified in any of the components. It was speculated that the possible site of origin of this tumor might be a supernumerary ovary in the broad ligament that was thought to be derived from embryonic remnants.

This article describes the case of a 70-year-old woman with an adnexal cystadenocarcinoma located in the right broad ligament and displaying a striking resemblance to a well-differentiated endometrioid adenocarcinoma. The uniqueness of this pseudoendometrioid carcinoma lies in the fact that its mesonephric nature is revealed by the origin from a papillary cystadenoma of the broad ligament, where remnants of the mesonephric duct are seated, and the immunohistochemical profile, particularly a uniform negativity for estrogen and progesterone receptors, apical-luminal positivity for CD10, and strong nuclear positivity for GATA3., (© The Author(s) 2016.)

Malignant primary tumors arising in the uterine broad ligament are extremely rare, and only 26 cases have been reported to date. We describe 2 new cases of primary adenocarcinoma of the broad ligament, and we review the previous literature on such rare tumors. In Case 1, a 71-year-old woman presented with a 2-month history of increased yellow vaginal discharge and lower abdominal pain during bowel movement. Transvaginal sonography revealed a 6.5 cm mass located on the dorsum of the uterus and a 7.0 cm mass (with cystic and solid parts) near the right adnexa. We postoperatively diagnosed the mass as a high-grade serous carcinoma of the broad ligament (pT3cNXM0). The patient is currently receiving adjuvant chemotherapy with paclitaxel and carboplatin. In Case 2, during a complete medical checkup a 43-year-old woman was found to have a pelvic mass indicative of leiomyoma. Transvaginal sonography revealed a 3.8 cm mass located on the dorsum of the uterus. Following surgery, we diagnosed the mass as a clear cell adenocarcinoma of the broad ligament (pT2bN1M0). This patient is also now receiving adjuvant chemotherapy with paclitaxel and carboplatin., (© The Author(s) 2015.)

PEComas are uncommon mesenchymal tumors often involving the pelvic organs. They have an unpredictable behavior. Accurate diagnosis and long-term follow-up is therefore essential in these patients. We report this case of PEComa of uncertain malignant potential in an unusual location with excellent prognosis.

Here we present a report of 61-year-old female patient. Uterus with left appendages was removed together with clinically tagged "tumor of right ovary" and then extensively sampled and routinely processed with Hematoxylin-Eosin (HE) and some additional staining. There was discernible oviduct adherent to grayish, solid, polycyclic 22 cm in diameter focally necrotic tumor to be diagnosed high-grade conventional leiomyosarcoma FNCLCC (Fédération Nationale des Centres de Lutte Contre le Cancer) G3 pT2b, according to 7th edition pTNM, according to World Health Organization (WHO) 2013 International Classification of Diseases for Oncology (ICD-O): 8890÷3, in nearby of right oviduct. Grade of differentiation was given according to FNCLCC classification: grade 3 {point score: 6 = 1 [microscopically necrosis comprised 10% of the tumor] + 3 [high mitotic index eight mitoses÷one high-power field (HPF) in hot spots in HE slides; Ki67 labeled approximately 60% of tumor cells] + 2 [histopathological type: conventional leiomyosarcoma]}. The staging was more appropriate for pT2b (7th edition pTNM) for deeply seated sarcoma of soft tissues, in examined samples, there was no trace of microscopically evident ovarian texture) rather than pT1a for ovarian tumors. The tumor was alpha-smooth muscle actin (α-SMA)-positive. Detected epithelial membrane antigen (EMA) immuno-reactivity indicates a possible change in mesenchymal origin. Next generation sequencing revealed tumor protein p53 (TP53) mutation C275Y (7577114 C>T). Each soft tissue malignancy should be carefully reported with appropriate choice of staging and precisely graded with internationally acknowledged classification.

Study Objective: To demonstrate the importance of being familiar with the anatomy of the endopelvic fascia as seen by laparoscopy to perform safe laparoscopic hysterectomies., Design: Combination of surgical videos and design diagrams., Setting: Compiled high-definition surgical videos from the Galaxy Care Laparoscopy Institute, Pune, India. These videos clearly demonstrate the anatomy of the endopelvic fascia and describe tips to avoid damage to the major structures, including the major vessels, ureter, bowel, bladder, and endopelvic fascia. The laparoscopic view of the anatomy with the current camera system is an excellent tool to demonstrate and teach pelvic anatomy, which can be applied to surgical principles in difficult benign and oncological cases., Intervention: We used a total laparoscopic approach to demonstrate the fasciae that were seen during various types of hysterectomies. The video shows the following: (1) the posterior leaf of the broad ligament was opened until it reached the apex of the uterosacral ligament; (2) the anterior leaf of broad ligament was opened until it reached the vesico-uterine peritoneal reflection; (3) the principles of bladder dissection; (4) the pubocervico-vesical fascia and its relevance to bladder dissection; (5) the relevance of the anatomy of the uterine artery, vein, and ureter with endopelvic fascia within the leaves of the broad ligament; (6) Denonvillier's fascia dissection technique for dissection of the rectum away from the vagina; (7) the anatomy of the vesicocervical ligaments, forming the ureteric tunnel; (8) the dissection principles of lateralizing the ureter in the retrovesical region; (9) the endopelvic fascia reflection, which continued caudally, covering the pelvic floor; and (10) the relevance of the anatomy of the endopelvic fascia and the stress urinary incontinence treatment technique., Conclusion: Understanding the anatomy of the fasciae of the pelvis helps to create avascular planes and is crucial for performing safe hysterectomies., (Copyright © 2015 AAGL. Published by Elsevier Inc. All rights reserved.)

The differential diagnosis of lesions arising in the broad ligament is quite large. Many of these lesions can be clinically interpreted before surgery as adnexal or uterine neoplasms. Although some lesions are similar to those arising in other müllerian sites, there are unique lesions as well. The lesions are uncommon and may prove challenging to clinicians. The purpose was to review the scope of lesions affecting the broad ligament. A literature review was conducted. A Medline search was performed using the terms broad ligament, mesosalpinx, and mesovarium. A review of the scope of broad ligament lesions is presented to assist in developing a differential diagnosis if a patient with such a lesion is encountered., (Copyright © 2015 AAGL. Published by Elsevier Inc. All rights reserved.)

Study Objective: To show the technique of interval laparoscopic isthmocervical cerclage using cotton cardiac tape., Design: Case report (Canadian Task Force Classification III)., Setting: A private practice hospital in Brasília, Brazil., Patient: A 36-year-old female patient with primary infertility for 2 years caused by previous amputation of the cervix because of intraepithelial neoplasia. There was no other suspected factor for infertility. Before undergoing in vitro fertilization, she was referred for interval cerclage because of anticipated cervical insufficiency during an eventual pregnancy. The patient's clinical history was unremarkable, except for the fact that she had developed secondary dysmenorrhea since the amputation, which prompted her to undergo cervical dilatation on 2 occasions. During the physical examination, we noted the absence of the exocervix, a mobile and normal-sized uterus and adnexa, and no pain. Informed consent was obtained from the patient for this case report. The local institutional review board considered this report exempt from approval., Interventions: The procedure was performed according to the technique described by Pereira et al. We incised the visceral peritoneum in the anterior cul-de-sac and developed the vesicouterine space for complete exposure of the uterine isthmus. Then, we incised the posterior leaf of both broad ligaments superiorly to the uterosacral ligaments and medially to the ureter and uterine vessels. We aimed to identify the bifurcation of the uterine artery and to create a "window" between them and the isthmus to place the cerclage tape. For this purpose, we used a 5-mm 36-cm blunt tip retractor for gastric banding (ref 30623G; Karl Storz, Tuttlingen, Germany) to transfix the broad ligament, anteriorly to posteriorly, under direct vision. We tied 2-0 Vicryl (Ethicon, Sommerville, NJ) sutures to the tips of 0.3 × 80 cm cotton cardiac tape (reference FAB-46; Ethicon) and pulled both edges of the tape through the windows in the broad ligament. The final position of the tape was inferior and medial to the main ascending branch of the uterine artery, right over the isthmus, and without any vessels interposed between them. This is to avoid any possible compression and congestion with uterine progressive enlargement as pregnancy proceeds. We then made 6 square knots in the anterior cul-de-sac and adjusted the tension-free tape firmly enough to give the uterine corpus support during pregnancy evolution. Finally, we closed the visceral peritoneum with a 3-0 PDS running suture (Ethicon)., Measurements and Main Results: The surgery lasted 70 minutes, and bleeding was minimal. The patient was discharged the following day. She is currently undergoing her 18th week of pregnancy resulting from in vitro fertilization treatment, and we wait for her obstetric results., Conclusion: Cotton cardiac tape is feasible for laparoscopic cerclage and can be used as an alternative to the commonly used Mersilene tape (Ethicon). Laparoscopy safely allows tape placement medial to the uterine vessels. Additional cases are necessary to establish the obstetric effectiveness of cotton cardiac tape for laparoscopic cerclage., (Copyright © 2015 AAGL. Published by Elsevier Inc. All rights reserved.)

Extrauterine adenomyomas are extremely rare benign tumors of smooth muscles, endometrial glands, and endometrial stroma. Ectopic endometrial glands can undergo malignant change. The ovary is the most common site of malignant change in endometriosis. Cancer arising in extraovarian endometriosis is a rare event with limited cases in the literature. To the best of our knowledge, we present the first case of a clear cell adenocarcinoma arising from foci of ectopic endometrial tissue in an adenomyoma of the broad ligament. It supports the association between endometriomas and clear cell adenocarcinoma. Therefore, patients with a significant history of endometriosis may benefit from close follow-up or definitive surgery., (© The Author(s) 2014.)

Intravenous leiomyomatosis (IVL) is a rare neoplasm of smooth muscle origin with the capacity for distant vascular and/or organ extension. The primary lesion is typically found in the uterus, with the most common extrauterine sites being pelvic/broad ligament vessels, and less often inferior vena cava, right heart chambers, and pulmonary vasculature. Extrauterine involvement can result in direct mass effect or vascular obstruction with associated clinical sequelae. Fourteen cases of IVL were identified in our pathology archives over a 25-yr period. Patient medical records and gross pathologic findings were reviewed. Histologic and related immunohistochemical slides (smooth muscle actin, desmin, CD31, CD34, and CD10) were re-evaluated by 2 gynecologic pathologists to confirm the diagnosis using conventional diagnostic criteria. Patient age at initial diagnosis ranged from 35 to 64 yr (mean: 46.1 yr), with a median age of 45 yr. Four cases demonstrated extrauterine extension, 2 cases were associated with disease recurrence, and 1 case was found to be extrauterine in origin (i.e. arising from the broad ligament). In summary, our study represents the fourth largest IVL cohort in literature to date, and contains the first case of IVL arising from the broad ligament in the absence of a uterine mass. IVL is a rare and potentially underdiagnosed neoplastic condition of smooth muscle origin that may result in significant morbidity when extrauterine vasculature or organs are involved. A high index of suspicion must be maintained, as the histologic diagnosis may be particularly challenging in cases in which the intracaval/intracardiac portion of the tumor is removed before the uterine IVL diagnosis, or a significant interval of time has elapsed since the initial IVL diagnosis was made. Prompt diagnosis combined with surgical excision has been shown to portend an increased survival rate and good overall prognosis in such cases.

Background: The aim of this study was to evaluate and compare the accuracy of diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC) value, and time-intensity curve (TIC) type analysis derived from dynamic contrast-enhanced MR imaging (DCE-MRI) in differentiating benign from malignant adnexal masses., Materials and Methods: 47 patients with 56 adnexal masses (27 malignant and 29 benign) underwent DWI and DCE-MRI examinations, prior to surgery. DWI signal intensity, mean ADC value, and TIC type were determined for all the masses., Results: High signal intensity on DWI and type 3 TIC were helpful in differentiating benign from malignant adnexal masses (p<0.001). The mean ADC value was significantly lower in malignant adnexal masses (p<0.001). An ADC value<1.20?10-3 mm2/s may be the optimal cutoff for differentiating between benign and malignant tumors. The negative predictive value for low signal intensity on DWI, and type 1 TIC were 100%. The pairwise comparison among the receiver operating characteristic (ROC) curves showed that the area under the curve (AUC) of TIC was significantly larger than the AUCs of DWI and ADC (p<0.001 for comparison of TIC and DWI, p<0.02 for comparison of TIC and ADC value)., Conclusions: DWI, ADC value and TIC type derived from DCE-MRI are all sensitive and relatively specific methods for differentiating benign from malignant adnexal masses. By comparing these functional MR techniques, TIC was found to be more accurate than DWI and ADC.

Epithelioid trophoblastic tumor (ETT) is a rare trophoblastic tumor originating from chorionic-type intermediate trophoblasts (ITs). It is usually associated with a prior gestational event. We present a 44-year-old woman who had unusual pregnancy related history. The patient received her second spontaneous abortion at the age of 25 years and had suffered from choriocarcinoma in left board ligament at the age of 29 years. She admitted no more treatment after 3 courses of multiagent chemotherapy when serum β-hCG returned to normal. Then she had Full-term delivery, induced abortion at the ages of 32, 33 years. The patient had high serum levels of beta-human chorionic gonadotropin (6587 IU/L). Microscopically, the tumor was composed of mainly mononuclear tumor cells, grew in cords, nests, and sheets within which were aggregates of hyaline material. Most were with distinct cell borders, eosinophilic cytoplasm. Immunohistochemical staining revealed strong diffuse reactivity for cytokeratins (AE1/AE3, CK18), P63, focal reactivity for beta-human chorionic gonadotropin, human placental lactogen, and inhibin-alpha. The Ki-67 index was 77%. The histological and immunohistochemical features were characteristic of epithelioid trophoblastic tumor. This is the first reported case of these two gestational trophoblastic tumor happened on one person with the intervening normal pregnancy.

Female adnexal tumor of probable Wolffian origin (FATWO) is a rare neoplasm arising from the remnants of the mesonephric duct. We report here a case of FATWO in a 70-year-old woman arising from the broad ligament and confirmed on immunohistochemistry. Most of these tumors behave in a benign fashion but certain histological features like hypercellularity, cellular pleomorphism and nuclear atypia as seen in the index case may increase the rate of recurrence and metastasis. Therefore, close follow-up is recommended for FATWO.

The authors reported a case of small bowel obstruction secondary to internal herniation through a defect of the broad ligament. This is a rare cause of small bowel obstruction. A 42 year-old woman without previous abdominal surgery presented with episodes of abdominal pain, nausea and vomiting. Due to rather unspecific and changing symptoms and the fact that objective findings were normal the patient was discharged and admitted a couple of times before a computed tomography-scan showed small bowel obstruction. Acute diagnostic laparoscopy showed internal herniation of a loop of small bowel trapped in a 2 cm defect in the right side of the broad ligament. The defect was gently expanded bluntly and it was then possible to retract the strangulated part of the small bowel which afterwards was vital. The defect was sutured laparoscopically. The postoperative recovery was uneventful. This case demonstrates that a preoperative diagnosis is difficult and a high index of suspicion is necessary to prevent diagnostic delay. Internal herniation through a defect in the broad ligament should be considered as a differential diagnosis in female patients presenting with symptoms of small bowel obstruction without a history of prior abdominal surgery, especially in parous women.

Tumors of the broad ligament are uncommon. Leiomyoma, which is the commonest female genital neoplasm, is also the most common solid tumor of the broad ligament. Leiomyomas affect 30% of all women of reproductive age but the incidence of broad-ligament leiomyoma is <1%. These benign tumors are usually asymptomatic. A case is being described where a 52 year old presented with gradual abdominal swelling which was clinically and radiologically diagnosed as ovarian malignancy. On abdominal and bimanual palpation a soft cystic mass was noted in the right pelvic region. CA 125 was mildly raised. CEA, CA 19.9 levels were within normal limit. The radiological diagnosis was ovarian cyst with possibility of malignant changes. Staging laparotomy and histopathological examination of the resected specimen revealed a right sided broad ligament leiomyoma with cystic changes. The degenerative changes in the leiomyoma lead to the clinical and radiological diagnostic confusion. Thus, though uncommon, broad ligament leiomyoma should be considered during evaluation of adnexal masses for optimal patient management. The above description of leiomyoma in the broad ligament is a highly unique case and thus deserves appropriate attention.

Massive ovarian edema is a rare disorder in which there is marked accumulation of interstitial fluid in the stroma of the ovary. Grossly, the involved ovary is an enlarged solid mass with a smooth tan-white surface, easily confused with a neoplasm. Microscopically, it features diffuse interstitial edema sparing follicles and outer cortex, dilated lymphatic vessels, thick-walled veins, fibromatosis, and luteinized stromal cells. It is believed that massive ovarian edema arises from interference in lymphatic drainage and venous return of the ovary secondary to partial torsion among other etiologies. Herein we provide the first description of unilateral ovarian edema in association with a large leiomyoma in the ipsilateral broad ligament. It is important to recognize the various presentations of this benign entity and to consider it in the differential diagnosis of an adnexal mass in a reproductive age woman.

Papillary cystadenoma is an uncommon epithelial tumor, originating within the head of the epididymis and broad ligament. When the lesion is bilateral, it is associated with von Hippel-Lindau disease. Its resemblance to metastatic clear cell renal cell carcinoma (RCC) has been noted in the literature. Owing to the emergence of additional markers for RCCs, we have evaluated the immunohistochemical staining patterns of a series of 7 papillary cystadenomas using CK7, RCC, PAX8, carbonic anhydrase IX, and AMACR. Six of the cases involved the epididymis, and 1 involved the broad ligament. The patients ranged in age from 20 to 65 years old. All of the tumors were unilateral and not known to be associated with von Hippel-Lindau disease. The lesions were composed of cystic structures, which focally contained papillary fibrovascular cores lined by cuboidal to columnar bland-appearing cells with clear cytoplasm. Another component was the presence of tubules, which focally had elongated profiles. Reverse polarity, wherein the nuclei are oriented toward the luminal surface with subnuclear vacuoles, was present focally in 4 cases and more extensively in a fifth case. Features associated with malignancy, such as mitotic figures, nuclear pleomorphism, and necrosis, were not identified. All lesions were strongly positive for CK7 and negative for RCC. Carbonic anhydrase IX was positive in all tumors (diffusely positive in 6, patchy in 1) with lack of staining in the apical portion of the cytoplasm (ie, cup-shaped staining). PAX8 showed diffuse positivity in 6 of the 7 lesions, with one of the epididymal cases showing negative staining. AMACR staining was negative in 5 of the 7 cases and showed only focal, weak staining in the remaining 2 cases. The current study more specifically demonstrated that papillary cystadenoma does not resemble clear cell RCC. Rather, papillary cystadenomas of the epididymis and broad ligament have identical morphology and immunohistochemical staining to clear cell papillary RCC. The diagnosis of papillary cystadenoma can be established as clear cell papillary RCC to date has not exhibited metastatic behavior.

Oncocytic adrenocortical neoplasm is characterized by abundant eosinophilic cytoplasm containing mitochondria, occasional nuclear atypia and diffuse growth pattern. Oncocytic adrenocortical neoplasm arising in adrenal rest is, however, extremely rare. We report a case of oncocytic adrenocortical neoplasm arising in adrenal rest of the broad ligament with associated marked lipomatous metaplasia. A well circumscribed tumor was accidentally detected in the pelvic cavity of a 29 year old Japanese woman, adjacent to the broad ligament of the uterus. The tumor was composed of large eosinophilic cells associated with diffuse growth pattern and abundant mature adipose tissue admixed with foci of clear cells. Both steroidgenic factor 1 (SF-1) and alpha-inhibin were immunohistochemically positive in tumor cells. Abundant mitochondria detected by immunohistochemical and electron microscopic examination confirmed the diagnosis of oncocytic adrenocortical neoplasm. The absence of necrosis, capsular and vascular invasion as well as the low mitotic index indicated the benign potential of this tumor. The tumor cells were also positive for dehydroepiandrosteron-sulfotransferase (DHEA-ST), 17β-hydroxysteroid dehydrogenase type 5 (17β-HSD5), 3β-hydroxysteroid dehydrogenase (3β-HSD) and steroid 17α-hydroxylase (P450-c17), suggesting a possible production of testosterone of this tumor. This is the first reported case of oncocytic adrenocortical adenoma arising in adrenal rest of the broad ligament., (© 2014 The Authors. Pathology International © 2014 Japanese Society of Pathology and Wiley Publishing Asia Pty Ltd.)

Primary leiomyosarcoma of the broad ligament is a very rare and highly malignant gynecological tumor. The authors report a 61-year-old postmenopausal woman with signs and symptoms of malignant ovarian tumor. Preoperative magnetic resonance imaging (MRI) was interpreted as being suspicious for malignant tumors, such as an ovarian cancer or a leiomyosarcoma of the broad ligament, so laparotomy was performed. Macroscopically, the tumor was revealed with a 18 x 13.7 x 9.5 cm degenerated, multiple cystic part and solid whitish part arising from broad ligament which on histopathology proved to be leiomyosarcoma. To the best of the authors' knowledge, primary leiomyosarcoma of the broad ligament has been documented in 21 reports or so, and no imaging findings are available. Here the authors present the MRI findings of primary leiomyosarcoma of the broad ligament.

A 22 year-old G1P1 woman presented to the emergency room with clinical impression of "ruptured right adnexal mass" and underwent a right salpingo-oophorectomy to rule out ectopic pregnancy. Instead, gross and microscopic examination revealed a pure choriocarcinoma involving the right fallopian tube and broad ligament. On the basis of the patient's age, recent history of delivery, last menstrual period for 10 weeks, large tumor mass, and possible pelvic lymph node metastasis, the patient promptly started to receive 8 cycles of multiagent chemotherapy regimen with a working diagnosis of high-risk gestational choriocarcinoma. Subsequent DNA genotyping analysis showed that the tumor cells had an identical genetic profile to that of the normal tissue of the patient, therefore establishing a final diagnosis of nongestational choriocarcinoma. Six months after the initial presentation, a second surgery was performed to remove a persistent right para-adnexal mass, which showed only necrotic tissue upon microscopic examination. The patient received 1 additional cycle of multiagent chemotherapy. She was alive without evidence of recurrence 26 months after the initial diagnosis.

Aims: Recent studies have demonstrated that cathepsin K seems to be a powerful marker in identifying renal perivascular epithelioid cell neoplasms (PEComas). However, the expression in extrarenal PEComas has not been well characterized due to their rare incidence. Our aim was to investigate the expression of cathepsin K in a wide spectrum of extrarenal PEComas and evaluate its potential diagnostic usefulness in comparison with other commonly used markers., Methods and Results: Twenty-three cases of PEComa (liver, n = 9; lung, n = 1; broad ligament of uterus, n = 1; vertex subcutaneous soft tissue, n = 1; abdominal wall, n = 1; and kidney, n = 10) were selected for study. All displayed a high percentage of cells with moderately to strongly positive reactions for cathepsin K (mean 91%; range 80-100%). HMB45, Melan-A and smooth muscle actin (SMA) were expressed in 78, 87 and 87% of cases, respectively, with various percentages of positive cells (mean, 34, 40 and 38%; range 0-80, 0-90 and 0-90%). Transcription factor E3 (TFE3) was expressed strongly in only three cases; none exhibited evidence of TFE3 gene fusion or amplification., Conclusions: Cathepsin K appears to be more powerful than other commonly used markers in diagnosing a wide spectrum of PEComas and distinguishing them from the majority of human cancers., (© 2012 Blackwell Publishing Ltd.)