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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 -
Asian Journal of Surgery Oct 2023
Topics: Female; Humans; Mesonephroma; Uterine Cervical Neoplasms; Adenocarcinoma
PubMed: 37230815
DOI: 10.1016/j.asjsur.2023.05.057 -
Virchows Archiv : An International... Jan 2024In general, endometrioid-defining features such as squamoid morular metaplasia are not thought to be associated with mesonephric adenocarcinoma (MA) and mesonephric-like...
In general, endometrioid-defining features such as squamoid morular metaplasia are not thought to be associated with mesonephric adenocarcinoma (MA) and mesonephric-like adenocarcinoma (MLA). Here, we report a case of FGFR2-mutated ovarian MLA with squamoid morular metaplasia accompanied by aberrant nuclear and cytoplasmic β-catenin expression and CTNNB1 mutation. Histologically, the tumor showed classical MLA histology, including well-formed glands with intraluminal eosinophilic secretions and cells with papillary thyroid carcinoma-like nuclei. Squamoid morular metaplasia was intimately associated with the tumor. Glandular epithelial elements, including those immediately associated with the squamoid morules, were negative for ER, but positive for both GATA3 and PAX8; aberrant β-catenin expression was limited to the squamoid morules. This case illustrates the ability of mesonephric neoplasia to exhibit histological features previously thought to be restricted to an endometrioid phenotype.
Topics: Female; Humans; Ovary; beta Catenin; Adenocarcinoma; Metaplasia; Mutation; Receptor, Fibroblast Growth Factor, Type 2
PubMed: 36856760
DOI: 10.1007/s00428-023-03522-9 -
International Journal of Gynecological... Sep 2023Mesonephric neoplasms of the lower female genital tract are rare. To date, there are scarce reports of benign biphasic vaginal mesonephric lesions, and none have...
Mesonephric neoplasms of the lower female genital tract are rare. To date, there are scarce reports of benign biphasic vaginal mesonephric lesions, and none have included immunohistochemical and/or molecular analysis. A biphasic neoplasm of mesonephric-type was incidentally identified in the vaginal submucosal tissue of a 55-yr-old woman who underwent a right salpingo-oophorectomy for an ovarian cyst. The well-circumscribed, 5 mm nodule exhibited white-tan, firm homogenous cut surfaces. Microscopic examination showed a lobular arrangement of glands with columnar to the cuboidal epithelium and intraluminal eosinophilic secretions, embedded within a myofibromatous stroma. Cytologic atypia and mitotic activity were absent. Immunohistochemical staining for PAX8 and GATA3 demonstrated diffuse expression in the glandular epithelium, CD10 exhibited a patchy luminal expression pattern, while TTF1, ER, PR, p16, and NKX3.1 were negative. Desmin highlighted a subset of the stromal cells, but myogenin was negative. Whole exome sequencing demonstrated variants of unknown significance in multiple genes including PIK3R1 and NFIA . The morphologic and immunohistochemical profiles are consistent with a benign mesonephric neoplasm. This is the first report describing the immunohistochemical and whole exome sequencing results for a benign biphasic vaginal mesonephric neoplasm. To the best of our knowledge, benign mesonephric adenomyofibroma has not been previously reported in this anatomic location.
Topics: Female; Humans; Epithelium; Neoplasms, Glandular and Epithelial; Ovarian Cysts; Salpingo-oophorectomy
PubMed: 36811844
DOI: 10.1097/PGP.0000000000000945 -
International Journal of Gynecological... Nov 2023Gynecologic carcinomas with RAS mutations may show a wide spectrum of histologic types, including mixed types. We present the case of a 63-yr-old patient diagnosed with...
Ovarian RASoma With Mesonephric-like Adenocarcinoma and Mixed Mullerian Components: A Case Report With Molecular Analysis Demonstrating Multidirectional Mullerian Differentiation.
Gynecologic carcinomas with RAS mutations may show a wide spectrum of histologic types, including mixed types. We present the case of a 63-yr-old patient diagnosed with an ovarian tumor harboring a mesonephric-like adenocarcinoma in a background of mixed mesonephric-like, mucinous, and endometrioid components. Molecular analysis revealed that all 3 components shared the same clonal KRAS mutation (p.G12A) and chromosome 1q gain. Based on shifts in clonality, copy number gains, and acquisition of an additional mutation, our data suggest that the mucinous component likely constituted the substrate from which the mesonephric-like and endometrioid components arose.
Topics: Female; Humans; Adenocarcinoma; Endometrium; Mutation; Ovarian Neoplasms; Middle Aged
PubMed: 36617679
DOI: 10.1097/PGP.0000000000000935