Epithelioid leiomyosarcoma of broad ligament harboring PGR-NR4A3 and UBR5-PGR gene fusions: a unique case report.

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.

Malignant clear-cell myomelanocytic tumor of broad ligament--a case report.

Clear-cell myomelanocytic tumors (CCMT) of the perivascular epithelioid cell tumor (PEComa) family have been recently reported. We report a case involving a 12-year-old girl. The tumor (9 x 7.5 x 7 cm) was a firm, tan-gray mass with heavily dark pigmentation, massive hemorrhage, and necrosis, and was located in the right broad ligament attached to the right ovary. Histologically, the tumor was composed of polygonal cells exhibiting diffuse hemorrhage, multifocal necroses, and vascular invasion. Most of the tumor cells contained melanin pigments with Fontana-Masson positivity and ultrastructurally suspicious, membrane-bound premelanosomes. Immunohistochemical staining was positive against HMB-45 and focally positive for smooth muscle actin. The tumor recurred in the form of multiple conglomerated masses of the right iliac fossa, with the greatest measuring up to 3.8 cm in dimension, within 1 year. Most CCMT are believed to originate from falciform ligament/ligamentum teres. To the best of our knowledge, this is the second report of a CCMT arising in the broad ligament with typical morphology and contributory ancillary results. Further study for proper subclassification of the PEComa family should be validated, not by anatomic site but by clinical behavior.

Primary extraneural myxopapillary ependymoma of the broad ligament.

Primary extraneural ependymomas are rare tumors that arise in ectopic sites, including pulmonary, sacrococcygeal region, ovarian, and paraovarian tissues. Four such ependymomas reported in the literature involve the paraovarian tissues, including 2 broad ligament ependymomas. Here we describe a myxopapillary ependymoma of the broad ligament in a 22-year-old woman, which may be the first tumor of this type to be reported in this location. Cytology, histology, cytochemistry, immunohistochemistry, and flow cytometry ploidy analysis are studied and described. Identification of perivascular ependymal rosettes, ependymal canals, vimentin and glial fibrillary acidic protein immunoreactivity, cytochemical staining of blepharoplasts or terminal bars by phosphotungstic acid hematoxylin, and presence of multiple foci of myxoid degeneration among the ependymal rosettes characterized a myxopapillary ependymoma.

Primary Ewing's sarcoma family of tumors arising from the broad ligament.

We report a case of a primary Ewing's sarcoma family of tumors (ESFT) that was an incidental finding in the broad ligament of a 53-year-old woman. ESFT now includes tumors previously described as Askin tumor, neuroepithelioma, extraskeletal Ewing's sarcoma, and peripheral primitive neuroectodermal tumor. This is because of the discovery that all of the above tumors contain a specific gene rearrangement involving chromosome 22q12. On microscopic examination, the tumor was composed of small, round cells with mild nuclear pleomorphism and scant eosinophilic cytoplasm. There were large areas of tumor necrosis and numerous mitoses. Immunohistochemically, there was strong membrane staining for CD99 and weak focal staining for CD56 and neuron specific enolase. Fluorescence in situ hybridization revealed a separation of the breakapart probe on chromosome 22q12 consistent with the presence of a gene rearrangement, supporting the diagnosis of ESFT. We believe the importance of recognizing the existence of a primary ESFT in the broad ligament as some primary tumors of the gynecological tract and of other systems may resemble it histologically. To the best of our knowledge, our case is the second case of extraskeletal Ewings sarcoma arising from the broad ligament but the first case that is confirmed by strong CD99 positivity and supported by fluorescence in situ hybridization.

Primary broad ligament cystadenocarcinoma with mucinous component: a case report with immunohistochemical study.

Primary cystadenocarcinoma that arises in the broad ligament is extremely rare, especially when it is mucinous. We report the case of a 59-year-old woman with a cystic mass of the right broad ligament who underwent a complete excision of the mass (7 x 7 x 3 cm) with hysterectomy, right salpingo-oophorectomy, omentectomy, appendicectomy, and peritoneal biopsies. Pathologic examination showed a low-grade cystadenocarcinoma with a mucinous component limited to the broad ligament. Despite the chemotherapy (cisplatinum and cyclophosphamide) performed, early tumor recurrence occurred after approximately 6 months. Our observation revealed an abundant mucin production with pools of mucin similar to those of pseudomyxoma peritonei and an inflammatory infiltrate with prominent lipid phagocytosis. Immunohistochemical analysis demonstrated a strong and diffuse positivity for both cytokeratin 7 and epithelial membrane antigen. A less extensive staining with carcinoembryonic antigen and a focal unequivocal positivity with cytokeratin 20, particularly in mucin-secreting cells, were also observed. This finding could indicate a metaplastic process toward colonic phenotype similar to primary ovarian tumors.

Epithelioid trophoblastic tumor of the broad ligament: a case report and review of the literature.

We report an epithelioid trophoblastic tumor, a recently delineated type of gestational trophoblastic tumor (GTT), discovered in the right broad ligament of a 41-year-old woman. The patient had gestational trophoblastic disease and was treated with methotrexate regimen 15 times 10 years earlier. The yellowish, spongy tumor with tiny hemorrhage spots was located in the right broad ligament, adherent to the right ovary. Microscopically, the tumor was circumscribed, with a pushing border, and the epithelial-differentiated tumor cells grew in cords, nests, and sheets within which were aggregates of hyaline material and necrotic debris. Most tumor cells were mononuclear with distinct cell borders, eosinophilic cytoplasm, and had nuclei with occasional indistinct nucleoli. Scattered multinucleated cells consistent with syncytiotrophoblastic cells were also present. Immunohistochemical staining revealed strong diffuse reactivity for cytokeratins (AE1/AE3, CAM 5.2, CK18), and focal reactivity, mainly in syncytiotrophoblastic cells, for beta-human chorionic gonadotropin, human placental lactogen, and inhibin-alpha. The histologic and immunohistochemical features were characteristic of epithelioid trophoblastic tumor and helped to distinguish the tumor from other trophoblastic tumors and squamous cell carcinoma. Our unusual findings in this case included a high level of serum beta-human chorionic gonadotropin, an unusual tumor location, and a higher Ki-67 proliferative index of 47.2%.

Analysis of type I collagen in the parametrium of women with and without uterine prolapse, according to hormonal status.

The content and quality of type I collagen in the parametrium of women with and without uterine prolapse was evaluated. Forty-four consecutive patients were selected and divided into two groups: A, 21 women without uterine prolapse, and B, 23 with uterine prolapse. Patients in group A had uterine leiomyoma and were submitted to abdominal hysterectomy; in those from group B, vaginal hysterectomy was performed for correction of the uterine prolapse. During surgery, fragments of the parametrium were removed and processed for immunohistochemical analysis using polyclonal antibodies for type I collagen. A system of computerized digital imaging analysis was used for the quantification of collagen fibers. There was no difference between collagen content in patients either with or without prolapse, nor between pre- and postmenopausal women with prolapse. A modification of the quality of the collagen fiber was observed, it being longer and more compact in the group without uterine prolapse. In contrast, in the group with prolapse, the fibers were shorter and thinner and areas with large spaces between fibers were found at several points of the parametrium. The conclusion was that patients with uterine prolapse have the same type I collagen content as those without, but the quality of the fiber is modified. The hormonal status also did not affect collagen content.

Immunohistochemical localisation of ET-1 and eNOS in lymphatic stomata of the porcine broad ligament of the uterus.

Immunohistochemical localization and distribution of endothelin (ET-1) and nitric oxide synthase (eNOS) were investigated in lymphatic stomata in areas of their special accumulation in the porcine broad ligament during the estrous cycle. The study was performed using polyclonal antibody for ET-1 and monoclonal antibody for eNOS. ET-1 and eNOS immunoreactivities were demonstrated in some thin endothelial lymphatic lacuna walls throughout the estrous cycle. In the mesothelial cell layer, ET-1 and eNOS were detected only in stomata-related cuboidal mesothelial cells, however, the intensity of the immunostaining and distribution of the positive cells varied during the cycle. These results suggest that ET-1 and eNOS can play a role in mechanisms regulating the tone of lymphatic stomata during the absorption and passage of fluids, particles and cells from the peritoneal cavity to lymphatic vessels in the porcine broad ligament.

Tumor of the broad ligament in von Hippel-Lindau disease of probable mullerian origin.

A rare neoplasm seen in women with von Hippel-Lindau disease is the papillary cystadenoma of the broad ligament, only three cases of which have been reported. All three exhibited characteristic histologic features identical to those of epididymal tumors that occur in affected patients, and were presumed to be of wolffian origin. This is the case of a broad ligament tumor in a woman with von Hippel-Lindau disease that had features more consistent with a mullerian rather than wolffian origin. This is the first report of a broad ligament tumor of probable mullerian origin in von Hippel-Lindau disease.

Undifferentiated carcinoma of the broad ligament in a 28-year-old woman--a case report and results of immunohistochemical and electron-microscopic studies.

A case of undifferentiated carcinoma of the broad ligament is reported. The patient was a 28-year-old Japanese woman who underwent laparotomy for a cystic and solid tumor of the left broad ligament. The tumor measured 9 cm in diameter and was unattached to the uterus, ipsilateral ovary, or salpinx. Histological examination revealed tumor tissue consisting of solid nests of irregular configuration and rather small to medium-sized undifferentiated epithelial cells. The tumor cells were positive for cytokeratin, but negative for CA125, epithelial membrane antigen, S-100 protein, amylase, carcinoembryonic antigen, chromogranin A, synaptophysin, HHF35, desmin, and vimentin. Electron-microscopic examination revealed gap junctions and primitive cellular junctions, but no intracytoplasmic mucous granules or microvilli on the surface of the tumor cells. This is the first report of undifferentiated carcinoma of the broad ligament as far as we have been able to determine from a search of the literature.