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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 -
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 -
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 -
BMJ Case Reports Jun 2023Mesonephric remnants (MRs) are embryonic vestiges most commonly found in female pathology specimens from the lateral wall of the cervix. The highly regulated genetic...
Mesonephric remnants (MRs) are embryonic vestiges most commonly found in female pathology specimens from the lateral wall of the cervix. The highly regulated genetic programme of mesonephric duct development has been well characterised in animals based on traditional surgical castration and knockout mouse experiments. However, the process is incompletely understood in humans. MRs are believed to give rise to mesonephric neoplasms, which are rare tumours with uncertain pathophysiology. There is a dearth of molecular studies on mesonephric neoplasms in part due to their rarity. Here, we report the results of next-generation sequencing of MR, which identified amplification of the androgen receptor gene for the first time to the best of our knowledge and discuss the potential implications in the context of the literature.
Topics: Animals; Mice; Humans; Female; Receptors, Androgen; Cervix Uteri; Epithelium; Uterine Cervical Neoplasms
PubMed: 37295813
DOI: 10.1136/bcr-2022-251741 -
Asian Journal of Surgery Oct 2023
Topics: Female; Humans; Mesonephroma; Uterine Cervical Neoplasms; Adenocarcinoma
PubMed: 37230815
DOI: 10.1016/j.asjsur.2023.05.057 -
Histopathology Jun 2023
Topics: Humans; Mesonephros; Carcinosarcoma
PubMed: 37191121
DOI: 10.1111/his.14900 -
Journal of Cancer Research and... Apr 2023Wilms' tumor (or nephroblastoma) is the most common renal malignancy in the pediatric population which consists of blastemal, epithelial, and stromal elements in...
Wilms' tumor (or nephroblastoma) is the most common renal malignancy in the pediatric population which consists of blastemal, epithelial, and stromal elements in variable proportions. The occurrence of renal cysts in children and infants is a rare phenomenon and is possibly an outcome of developmental aberrations in mesonephric blastema. The coincidental association of nephroblastoma with renal cysts is a very rare finding. Here, we describe two cases of Wilms' tumor with an unusual association between glomerulocystic kidney disease and multicystic dysplastic kidney.
Topics: Infant; Child; Humans; Wilms Tumor; Kidney; Kidney Neoplasms; Carcinoma, Renal Cell; Kidney Diseases, Cystic
PubMed: 37147970
DOI: 10.4103/jcrt.jcrt_275_22