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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 -
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 -
Journal of the Korean Society of... May 2023According to the 2020 World Health Organization classification, mesonephric-like adenocarcinoma (MLA) is newly categorized as a subtype of endometrial carcinoma and...
According to the 2020 World Health Organization classification, mesonephric-like adenocarcinoma (MLA) is newly categorized as a subtype of endometrial carcinoma and remains a relatively unknown disease owing to its rarity. To the best of our knowledge, radiological findings of MLA have not been reported in the English literature. The uterine MLAs show a worse clinical prognosis and a more aggressive biological behavior than the usual endometrial carcinoma. Herein, we present the imaging findings of a 65-year-old female with a MLA in the uterine corpus. The tumor was a solid endometrial mass with deep myometrial invasion, poor contrast enhancement, and moderate diffusion restriction.
PubMed: 37324981
DOI: 10.3348/jksr.2022.0119 -
Clinical Case Reports Jun 2023Splenic tissue outside the normal anatomical site can be collectively referred to as ectopic spleen. Clinically, the commonest causes of ectopic spleen include accessory...
Splenic tissue outside the normal anatomical site can be collectively referred to as ectopic spleen. Clinically, the commonest causes of ectopic spleen include accessory spleen, splenic tissue implantation, and splenogonadal fusion (SGF). Accessory spleen is mostly caused by congenital dysplasia, is mostly located near the spleen, and may be supplied by the splenic artery. Splenic implantation is mostly caused by autologous spleen tissue transplantation caused by trauma or surgery. SGF is the abnormal fusion of the spleen with the gonad or with the mesonephric derivatives. As a rare developmental malformation, it is difficult to make a correct diagnosis preoperatively, and easily misdiagnosed as a testicular tumor cause lifelong harm to patients. An 18-year-old male student who developed left testicular pain without obvious cause that radiated to the perineum 4 months prior to presentation. He was diagnosed with cryptorchidism 12 years ago and underwent orchiopexy without intraoperative frozen section examination. An ultrasound was performed, identifying hypoechoic nodules in the left testis, suggestive of seminoma. During surgery, the testicular tumor revealed a dark red tissue and the diagnosis of a pathological ectopic splenic tissue was made. Because the clinical manifestations of SGF are not specific, misdiagnosis and unnecessary orchiectomy may occur. If a complete preoperative examination which includes biopsy or intraoperative frozen section is performed, unnecessary orchiectomy can be effectively avoided and bilateral fertility can be preserved.
PubMed: 37305869
DOI: 10.1002/ccr3.7264