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Case Reports in Women's Health Sep 2023Mesonephric-like adenocarcinomas are newly classified rare neoplasms of the upper genital tract. They share identical features with mesonephric adenocarcinomas, with the...
Mesonephric-like adenocarcinomas are newly classified rare neoplasms of the upper genital tract. They share identical features with mesonephric adenocarcinomas, with the exception of location. There is ongoing discussion on whether mesonephric-like adenocarcinomas arise from mesonephric remnants or are of Müllerian origin. A 65-year-old woman (G2P1) presented with pelvic pain. Transvaginal ultrasound revealed multiple fibroids, and a robotic total laparoscopic hysterectomy with bilateral salpingo-oophorectomy was planned. Intraoperatively, a complex mass with cystic and solid components was found on the left ovary, which also adhered to the rectosigmoid colon. Pathologic reports documented mesonephric-like adenocarcinoma that appeared to be arising in association with an endometrioid adenofibroma. This case is notable due to the patient's unique background of in-utero exposure to diethylstilbestrol (DES) and multiple gynecological malignancies within her mother, as well as the associated endometrioid adenofibroma. This case contributes evidence to two seemingly opposing theories of mesonephric-like adenocarcinoma histogenesis: the mesonephric remnant theory (supported by the patient's DES exposure), and the Müllerian theory (supported by the associated endometrioid adenofibroma).
PubMed: 37560022
DOI: 10.1016/j.crwh.2023.e00531 -
Scientific Reports Aug 2023Cadherin-16 (CDH16) plays a role in the embryonal development in kidney and thyroid. Downregulation of CDH16 RNA was found in papillary carcinomas of the thyroid. To...
Cadherin-16 (CDH16) plays a role in the embryonal development in kidney and thyroid. Downregulation of CDH16 RNA was found in papillary carcinomas of the thyroid. To determine the expression of CDH16 in tumors and to assess the diagnostic utility a tissue microarray containing 15,584 samples from 152 different tumor types as well as 608 samples of 76 different normal tissue types was analyzed. A membranous CDH16 immunostaining was predominantly seen in thyroid, kidney, cauda epididymis, and mesonephric remnants. In the thyroid, CDH16 staining was seen in 100% of normal samples, 86% of follicular adenomas, 60% of follicular carcinomas, but only 7% of papillary carcinomas (p < 0.0001). CDH16 positivity was frequent in nephrogenic adenomas (100%), oncocytomas (98%), chromophobe (97%), clear cell (85%), and papillary (76%) renal cell carcinomas (RCCs), various subtypes of carcinoma of the ovary (16-56%), various subtyped of carcinomas of the uterus (18-40%), as well as in various subtypes of neuroendocrine neoplasms (4-26%). Nineteen further tumor entities showed a weak to moderate CDH16 staining in up to 8% of cases. Our data suggest CDH16 as a potential diagnostic marker-as a part of a panel-for the identification of papillary carcinomas of the thyroid, nephrogenic adenomas, and the distinction of renal cell tumors from other neoplasms.
Topics: Male; Female; Humans; Carcinoma, Renal Cell; Immunohistochemistry; Thyroid Gland; Carcinoma, Papillary; Kidney Neoplasms; Carcinoma; Cadherins; Biomarkers, Tumor; Thyroid Neoplasms
PubMed: 37558687
DOI: 10.1038/s41598-023-39945-2 -
Journal of Clinical Medicine Jul 2023Mesonephric adenocarcinoma (MA) of the vagina is a rare tumor that arises from mesonephric remnants (Wolffian) in the female genital tract. It is a neoplasm with no... (Review)
Review
BACKGROUND
Mesonephric adenocarcinoma (MA) of the vagina is a rare tumor that arises from mesonephric remnants (Wolffian) in the female genital tract. It is a neoplasm with no significant evidence about its diagnosis, treatment, follow-up and prognosis.
METHODS
Systematic research of the literature was conducted in Scopus, PubMed/MEDLINE, ScienceDirect and the Cochrane Library, including observational prospective and retrospective studies, case series and case reports. We collected data regarding studies related to diagnosis and treatment options evaluating the following aspects: study design, population, treatment type, rate of surgical complications and fertility outcome. We further included a case report of laparoscopic management of MA with pictorial assays.
RESULTS
Thirteen cases of MA of the vagina are available in the literature, including our case report. The median age at diagnosis was 52 years old; the majority of patients reported vaginal bleeding as a symptom (38%); and ultrasound, followed by a magnetic resonance and CT scan were the diagnostic tools most used. In 54% of the cases, a surgical biopsy was performed, and 92% of the patients underwent upfront surgery with an open access or vaginal resection except one case fully managed by minimally invasive surgery. Most of the patients (68%) received adjuvant treatment with chemotherapy or radiotherapy or a combination of them. The mean follow-up period was 6 years.
CONCLUSIONS
Despite the rarity of this cancer and bizarre location, a minimally invasive approach seems feasible after multidisciplinary evaluation. According to the rarity of this tumor, any future case and follow-up data must be reported in the literature in order to enlarge the knowledge about it.
PubMed: 37510961
DOI: 10.3390/jcm12144846 -
Annales de Pathologie Nov 2023Mesonephric lesions in the female genital tract are uncommon and heterogeneous. Those deriving from the upper tract differ from those developing in the lower tract,... (Review)
Review
Mesonephric lesions in the female genital tract are uncommon and heterogeneous. Those deriving from the upper tract differ from those developing in the lower tract, based on their morphology and immunohistochemical profile. Carcinomas of mullerian origine may display the morphology, the immunoprofile and even the molecular abnormalities of those deriving from mesonephric remnants and are designated mesonephric-like carcinomas. These are high-grade lesions despite their well-differentiated glandular morphology (wolf in sheep's clothing). New entities, such as STK11 adnexal tumors, have merged recently and should not be confused with adnexal tumors of wolffian origin (FATWO), which have a better prognostic and outcome. In this review, we provide an overview of these lesions and their mimickers, in order to help pathologists in the diagnostic approach of these complex and rare neoplasms.
Topics: Female; Humans; Carcinoma; Genitalia, Female; Epithelium; Adenoma; Skin Neoplasms
PubMed: 37481413
DOI: 10.1016/j.annpat.2023.07.001 -
Development (Cambridge, England) Jul 2023Temporal transcription profiles of fetal testes with Sertoli cell ablation were examined in 4-day culture using a diphtheria toxin (DT)-dependent cell knockout system in...
Temporal transcription profiles of fetal testes with Sertoli cell ablation were examined in 4-day culture using a diphtheria toxin (DT)-dependent cell knockout system in AMH-TRECK transgenic (Tg) mice. RNA analysis revealed that ovarian-specific genes, including Foxl2, were ectopically expressed in DT-treated Tg testis explants initiated at embryonic days 12.5-13.5. FOXL2-positive cells were ectopically observed in two testicular regions: near the testicular surface epithelia and around its adjacent mesonephros. The surface FOXL2-positive cells, together with ectopic expression of Lgr5 and Gng13 (markers of ovarian cords), were derived from the testis epithelia/subepithelia, whereas another FOXL2-positive population was the 3βHSD-negative stroma near the mesonephros. In addition to high expression of Fgfr1/Fgfr2 and heparan sulfate proteoglycan (a reservoir for FGF ligand) in these two sites, exogenous FGF9 additives repressed DT-dependent Foxl2 upregulation in Tg testes. These findings imply retention of Foxl2 inducibility in the surface epithelia and peri-mesonephric stroma of the testicular parenchyma, in which certain paracrine signals, including FGF9 derived from fetal Sertoli cells, repress feminization in these two sites of the early fetal testis.
Topics: Mice; Animals; Male; Female; Sertoli Cells; Testis; Mice, Transgenic; Ovary; Fetus
PubMed: 37376880
DOI: 10.1242/dev.201660 -
Annals of Medicine and Surgery (2012) Jun 2023Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome also known as the Herlyn-Werner-Wunderlich Syndrome is a rare embryological disorder associated...
UNLABELLED
Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome also known as the Herlyn-Werner-Wunderlich Syndrome is a rare embryological disorder associated with Mullerian and mesonephric duct abnormality.
CASE PRESENTATION
The cases presented describe the imaging (ultrasound and MRI) findings of four young females who presented with dysmenorrhea and urinary complaints. All of them had solitary kidneys with a didelphic uterus and unilateral hematometrocolpos. A proximally blind-ending ureter with distal ectopic insertion, transverse vaginal septum, and left-sided endometrioma was seen.
CLINICAL DISCUSSION
OHVIRA syndrome is associated with duplicated uterovaginal structure with OHVIRA. Ultrasound is the first line of investigation; however, MRI better delineates the anatomy and assists in preoperative planning.
CONCLUSION
This report highlights that earlier clinical suspicion and imaging diagnosis of OHVIRA is crucial to prevent adverse outcomes and treating complications.
PubMed: 37363484
DOI: 10.1097/MS9.0000000000000904 -
International Journal of Surgical... May 2024Ovarian mesonephric-like adenocarcinoma (MLA) is a rare cancer subtype. We describe a patient with ovarian MLA wherein liver metastases developed 1 month after surgery.... (Review)
Review
Ovarian mesonephric-like adenocarcinoma (MLA) is a rare cancer subtype. We describe a patient with ovarian MLA wherein liver metastases developed 1 month after surgery. A phenotypic analysis of the tumor was performed to identify molecular therapeutic targets. A 53-year-old woman, without any symptoms, underwent uterine cancer screening. Transvaginal ultrasonography revealed an ovarian mass, and subsequent pelvic magnetic resonance imaging showed a 13 × 10 cm multicystic ovarian lesion with a solid part. No extra ovarian lesions were observed and a staging laparotomy was performed. Pathological examination revealed an MLA of the left ovary (stage IC1). The tumor comprised tumor cells in a tubular pattern with intraluminal eosinophilic material, as well as mixed glandular and papillary, cord-like, and solid patterns. Endometriosis was also observed. Immunohistochemically, the tumor cells were positive for PAX8, GATA3 (focal), TTF1 (focal), and CD10 (luminal) and negative for the estrogen receptor, progesterone receptor, and WT1. One month after surgery, computed tomography revealed multiple liver metastases. Additional immunohistochemistry for therapeutic targets revealed that the tumor cells were weakly positive for human epidermal growth factor receptor 2 (focal; score 1+), pan-tropomyosin receptor kinase-negative, programmed death-ligand 1-negative, and PMS2 and MSH6 intact. The companion homologous recombination deficiency test (MyChoice) showed homologous recombination repair proficiency. These findings suggest that poly(ADP-ribose) polymerase inhibitors and immune checkpoint inhibitors may not be effective treatment options. A literature review revealed that data on therapeutic targets in MLA are scarce. In summary, we report a patient with ovarian MLA showing an aggressive clinical course and the phenotypic analysis of the tumor may contribute to the identification of therapeutic targets for MLA.
Topics: Female; Humans; Middle Aged; Ovarian Cysts; Ovarian Neoplasms; Adenocarcinoma; Liver Neoplasms
PubMed: 37345348
DOI: 10.1177/10668969231183631