ABSTRACT
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.
Subject(s)
ACTH Syndrome, Ectopic/diagnosis , Broad Ligament/pathology , Fallopian Tube Neoplasms/diagnosis , Pelvic Neoplasms/diagnosis , Sex Cord-Gonadal Stromal Tumors/diagnosis , ACTH Syndrome, Ectopic/etiology , ACTH Syndrome, Ectopic/metabolism , Adolescent , Adrenocorticotropic Hormone/metabolism , Broad Ligament/metabolism , Egypt , Fallopian Tube Neoplasms/complications , Fallopian Tube Neoplasms/metabolism , Fallopian Tube Neoplasms/pathology , Female , Humans , Pelvic Neoplasms/complications , Pelvic Neoplasms/metabolism , Pelvic Neoplasms/pathology , Sex Cord-Gonadal Stromal Tumors/complications , Sex Cord-Gonadal Stromal Tumors/metabolism , Sex Cord-Gonadal Stromal Tumors/pathology , Virilism/diagnosis , Virilism/etiology , Virilism/metabolismABSTRACT
AIMS: Clear cell papillary cystadenoma (CCPC) is associated with von Hippel-Lindau disease (VHLD), but rarely involves mesosalpinx and broad ligament (M/BL). This study provides new data about its behaviour and immunophenotype. METHODS AND RESULTS: We performed an analysis of four benign cases of CCPC of M/BL with either characteristic clinical features or genetic markers [loss of heterozygosity (LOH)] of VHLD in patients ranging from 24 to 36 years and a sporadic case in a 52-year-old presenting with peritoneal metastases. All CCPCs were papillary but had solid and tubular areas. Haemorrhage, thrombosis and scarring were constant features and related to an unusual pattern of sub-epithelial vascularity. All clear or oxyphilic cells co-expressed cytokeratin 7 (CK7), CAM5.2 and vimentin, with strong apical CD10 and nuclear paired box gene 2 (PAX2) immunoreactivity. Three cases also showed positivity for VHL40, epithelial membrane antigen (EMA), Wilms' tumour suppressor gene (WT-1) and cancer antigen 125 (CA125) but only one expressed renal cell carcinoma (RCC) antigen. Vascular plexus overexpressed nuclear and cytoplasmic WT-1. CONCLUSION: The VHLD-associated cases appeared to be benign, but the sporadic case exhibited a low malignant potential. CCPCs show histological and immunophenotypical similarities with the recently reported clear cell papillary RCC, although the previously unreported apical CD10 and nuclear PAX2 expression may be related to their mesonephric origin. CCPC has a distinctive sub-epithelial vascular pattern that is consistent with its pathogenesis.
Subject(s)
Broad Ligament/pathology , Cystadenoma, Papillary/pathology , Fallopian Tube Neoplasms/pathology , Fallopian Tubes/pathology , Uterine Neoplasms/pathology , von Hippel-Lindau Disease/pathology , Adult , Biomarkers, Tumor/metabolism , Broad Ligament/metabolism , Cystadenoma, Papillary/complications , Cystadenoma, Papillary/genetics , Cystadenoma, Papillary/metabolism , Fallopian Tube Neoplasms/complications , Fallopian Tube Neoplasms/genetics , Fallopian Tube Neoplasms/metabolism , Fallopian Tubes/metabolism , Female , Humans , Loss of Heterozygosity , Middle Aged , Neoplasms, Multiple Primary , Uterine Neoplasms/genetics , Uterine Neoplasms/metabolism , Von Hippel-Lindau Tumor Suppressor Protein/genetics , Young Adult , von Hippel-Lindau Disease/complications , von Hippel-Lindau Disease/genetics , von Hippel-Lindau Disease/metabolismABSTRACT
The fascia pelvis parietalis (FPP) or endopelvic fascia is a well-known structure, but few studies described the detailed histological architecture, including the composite fiber directions. We hypothesized a gender-specific fiber architecture corresponding to the functional demand. For the first step to examine this hypothesis, we investigated specimens from 27 adult cadavers (10 males and 17 females) and 11 midterm fetuses (five males and six females) using immunohistochemistry and aldehyde-fuchsin staining. The adult female FPP was a solid, thick monolayered structure that was reinforced by abundant elastic fibers running across the striated muscle fibers, but it contained little or no smooth muscles (SM). In contrast, the male FPP was multilayered with abundant SM. In midterm fetuses, SM originated from the inferior part of the bladder and extended inferiorly along the gender-specific courses. Thus, we found a clear intergender difference in FPP architecture. However, the functional significance remained unknown because the basic architecture was common between nulliparous and multiparous women. Rather than for meeting the likely mechanical demands of pregnancy and vaginal delivery, the intergender difference of the FPP seemed to result from differences in the amount and migration course of bladder-derived SM as well as in hormonal background.
Subject(s)
Broad Ligament/anatomy & histology , Fascia/anatomy & histology , Pelvic Floor/anatomy & histology , Round Ligament of Uterus/anatomy & histology , Actins/metabolism , Aged , Aged, 80 and over , Biomarkers/metabolism , Broad Ligament/metabolism , Cadaver , Elastic Tissue/anatomy & histology , Elastic Tissue/metabolism , Fascia/metabolism , Female , Fetus/anatomy & histology , Gestational Age , Humans , Male , Middle Aged , Muscle, Smooth/anatomy & histology , Muscle, Smooth/metabolism , Round Ligament of Uterus/metabolism , Sex FactorsABSTRACT
The female genital tract is a rare site for angioleiomyoma. Here, we present an unusual case of bilateral large angioleiomyoma in the broad ligaments. A 52-year-old woman underwent total hysterectomy with bilateral salpingo-oophorectomy due to massive tumors in the bilateral adnexal regions. Gross examination revealed a hemangioma-like mass each in the bilateral broad ligaments. The right one extended into the uterine myometrium. Histologically, both tumors had a mixed pattern of prominent thick-walled blood vessels and cellular short spindle myoid cells throughout. The spindle cells often swirled around the vessels, displaying an overlapping feature with angiomyofibroblastoma or myopericytoma. Immunostaining demonstrated that the tumor cells were positive for alpha-smooth muscle actin, desmin, estrogen receptor, and progesterone receptor. We believe that angioleiomyoma should be recognized as a delineated variant of leiomyoma in the female genital tract. This rare benign tumor should be differentiated from other mesenchymal neoplasms with prominent blood vessels.
Subject(s)
Angiomyoma/pathology , Broad Ligament/pathology , Peritoneal Neoplasms/pathology , Angiomyoma/metabolism , Angiomyoma/surgery , Broad Ligament/metabolism , Broad Ligament/surgery , Diabetes Mellitus , Female , Humans , Hypertension/complications , Hysterectomy , Immunohistochemistry , Incidental Findings , Middle Aged , Peritoneal Neoplasms/metabolism , Peritoneal Neoplasms/surgeryABSTRACT
PEComas localized in the region of falciform ligament and broad ligament are exceedingly rare. Most of them are built of spindle neoplastic cells. We report a case of epithelioid PEComa of the falciform ligament and/or broad ligament. There is only one report of such neoplasm in English-language literature. Histologically, the tumor was composed of nests of epithelioid clear cells stained positively for vimentin, HMB45, and SMA. Because of morphological features of the tumour (4 mitoses /20HPF, focal necrosis, and vascular invasion) we assess the neoplasm as potentially malignant.
Subject(s)
Broad Ligament/pathology , Perivascular Epithelioid Cell Neoplasms/pathology , Adult , Biomarkers, Tumor/analysis , Broad Ligament/metabolism , Calmodulin-Binding Proteins/genetics , Female , Gene Rearrangement , Humans , Immunohistochemistry , In Situ Hybridization , Perivascular Epithelioid Cell Neoplasms/genetics , Perivascular Epithelioid Cell Neoplasms/metabolism , RNA-Binding Protein EWS , RNA-Binding Proteins/geneticsABSTRACT
Solitary fibrous tumor (SFT) is a rare mesenchymal tumor that occurs preferentially in the pleura. Although it has been described at some extrathoracic sites, its occurrence in the female genital tract is extremely rare. We are the first to report on an unusual case of a large (14cm in the largest diameter) SFT localized in the broad ligament of the uterus in a 50-year-old woman. The patient underwent surgical tumor extirpation and has remained well without any sign of local tumor recurrence after 6 years of follow-up. We discuss the clinical aspects, the gross macroscopic appearance, the histologic findings, and the differential diagnosis, and provide a review of the literature.
Subject(s)
Broad Ligament/pathology , Neoplasms, Fibrous Tissue/pathology , Broad Ligament/metabolism , Female , Humans , Immunohistochemistry , Middle Aged , Neoplasms, Fibrous Tissue/metabolismABSTRACT
The objective of this study was to demonstrate main bioamine-positive structures in rat mesometrium, to detect their spatial cooperations and to define their catecholamine, serotonin and histamin content during the sexual cycle. The study was performed in 120 female rats of reproductive age, which were divided into groups according to the stages of sexual cycle: early estrus, late estrus, metestrus, early diestrus, late diestrus, proestrus. By using the microspectrofluorometric histological methods, the following bioamine-positive structures were differentiated in rat mesometrium that were cooperated in space: sympathetic nerve fibers (containing catecholamines and serotonin), mast cells and macrophages (containing catecholamines, serotonin and histamine). Regular dynamics of individual bioamine indices was detected during the sexual cycle. The role of mesometrium in the system of uterine extraorganic bioamine supply is discussed.
Subject(s)
Biogenic Monoamines/metabolism , Broad Ligament/cytology , Estrous Cycle/physiology , Adrenergic Fibers/metabolism , Animals , Broad Ligament/innervation , Broad Ligament/metabolism , Catecholamines/metabolism , Female , Histamine/metabolism , Macrophages/cytology , Macrophages/metabolism , Mast Cells/cytology , Mast Cells/metabolism , Rats , Serotonin/metabolismABSTRACT
Luteinizing hormone (LH) and human chorionic gonadotropin (hCG) share common receptors in the uterus of many species. The aim of the present work was to investigate the possibility of LH/hCG binding sites present in the musculature of the porcine broad ligament, and to study the topography of LH/hCG receptors in ligamental blood vessels. Uteri of cyclic pigs were collected during follicular (n = 6) and midluteal (n = 6) phases of the estrous cycle. The horns and broad ligaments were dissected for measurements of numbers and affinities of unoccupied LH/hCG binding sites in crude cell membrane fractions of the myometrium and the smooth muscle layers of the ligament. The tissue samples for contact autoradiography, on film using [125I]hCG as ligand, were collected from different regions of the reproductive tract including: ovary, uterine horn, paraovarian vascular plexus, uterine vein, and uterine artery with supplying vessels and the outer arches of the broad ligament. The kidney and liver were used as negative control tissue. The specific binding of [125I]-labelled hCG in blood vessels was quantified on the base of optical density of the film. Scatchard analysis of data from broad ligamental and myometrial crude membrane preparations gave comparable estimates of the association constants and numbers of LH/hCG receptors. Mean concentrations (+/- SEM) of receptors (nmol/kg protein) were lower in the broad ligament than in the myometrium (p < 0.05) during both follicular and luteal phases (2.2 +/- 0.5 vs. 1.0 +/- 0.3 and 2.5 +/- 0.5 vs 1.7 +/- 0.2; respectively).(ABSTRACT TRUNCATED AT 250 WORDS)
Subject(s)
Broad Ligament/metabolism , Estrus/physiology , Muscle, Smooth, Vascular/metabolism , Receptors, LH/metabolism , Uterus/blood supply , Uterus/metabolism , Animals , Arteries/metabolism , Autoradiography , Corpus Luteum/metabolism , Endometrium/metabolism , Female , Iodine Radioisotopes , Kinetics , Myometrium/metabolism , Swine , Veins/metabolismABSTRACT
This is a report of a paratubal adult granulosa cell tumor (GCT) located within the right broad ligament in a 62-year-old woman. These are rare tumors with only 8 cases reported so far. Because of an overlap of topographic, morphologic, and immunohistochemical features, it is not always possible to differentiate between the broad ligament GCT and female adnexal tumor of probable Wolffian origin (FATWO). Although nuclear grooving is not an exclusive feature of GCT and can be seen in a variety of other neoplasms, in the context of the differential diagnosis between broad ligament GCT and FATWO, the presence of this feature may be very useful in establishing the diagnosis of broad ligament GCT.
Subject(s)
Broad Ligament/pathology , Genital Neoplasms, Female/diagnosis , Granulosa Cell Tumor/diagnosis , Neoplasms, Adnexal and Skin Appendage/diagnosis , Wolffian Ducts/pathology , Biomarkers, Tumor/metabolism , Broad Ligament/metabolism , Broad Ligament/surgery , Cell Nucleus/pathology , Diagnosis, Differential , Female , Genital Neoplasms, Female/metabolism , Genital Neoplasms, Female/surgery , Granulosa Cell Tumor/metabolism , Granulosa Cell Tumor/surgery , Humans , Middle Aged , Neoplasms, Adnexal and Skin Appendage/metabolism , Neoplasms, Adnexal and Skin Appendage/surgery , Treatment Outcome , Wolffian Ducts/metabolismABSTRACT
The aim of the present study was to determine the immunoreactivity of vascular endothelial growth factor (VEGF-A) and its two receptors, viz., Flt-1 (fms-like tyrosine kinase) and Flk-1 (fetal liver kinase), on the surface of endothelial cells of the uterine artery and its branches and of the arcuate arteries in the area of the uterine broad ligament during various phases of the estrous cycle in the pig. We also investigated their expression to determine whether this was phase-related. The highest immunoreactivity for VEGF-A was observed in the uterine artery and arcuate arteries at the early luteal phase and in the branches of the uterine artery during the follicular phase of the estrous cycle. The strongest immunostaining intensity of Flt-1 was found in the uterine artery and its branches at the follicular phase and in arcuate arteries at the mid-luteal phase, whereas Flk-1 immunostaining was at its highest in the uterine artery at the mid-luteal phase and in the branches of the uterine artery and arcuate arteries at the follicular phase. Additionally, VEGF-A expression was assessed by semi-quantitative Western blot analysis, which revealed significantly higher levels of VEGF-A protein during the early luteal and the follicular phase of the estrous cycle (P < 0.001). The phase-related differences in the immunoreactivity and expression of VEGF-A and VEGF receptors suggest that these factors are hormone-dependent during the estrous cycle in the pig.