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Diagnostics (Basel, Switzerland) Aug 2022Some ovarian mucinous tumors are thought to originate from Brenner tumors and teratomas; however, data are limited on what could be the origin for the remaining tumors....
Some ovarian mucinous tumors are thought to originate from Brenner tumors and teratomas; however, data are limited on what could be the origin for the remaining tumors. We report a new case of ovarian mucinous borderline tumor/atypical proliferative mucinous tumor (MBT/APMT) co-existing with a mesonephric-like proliferation (MLP)/mesonephric-like hyperplasia (MLH). The patient was a 58-year-old woman who presented with a pelvic mass and abdominal pain. Pathology demonstrated an 11 cm MBT/APMT in the left ovary. In addition, the tumor contained one focal area (<1% of total tumor volume) of MLP/hyperplasia adjacent to, or intimately admixed with, mucinous epithelium, with an immunophenotype of diffuse Pax8 and Gata3 expression and negative TTF-1, ER, and PR staining. Pax8 was also weakly positive in the MBT/APMT component. Some mesonephric-like glands partially exhibited gastrointestinal-type mucinous metaplasia/differentiation. A polymerase chain reaction (PCR)-based Sanger sequencing demonstrated that a KRAS G12V mutation was present in both MLP/MLH and MBT/APMT components, providing further evidence to support their clonal relationship. We previously reported a series of similar cases and demonstrated a novel association between MLP, mesonephric-like adenocarcinoma and ovarian mucinous tumor. It is conceivable that benign MLPs may have ability to differentiate to lineage-specific mucinous lesions, and, as such, they may serve as a possible new origin of some ovarian mucinous neoplasms; in particular, Pax8-positive tumors. The current case provides additional evidence to support this theory.
PubMed: 36010251
DOI: 10.3390/diagnostics12081901 -
Gynecologic Oncology Reports Aug 2022Mesonephric-like adenocarcinoma (MLA) is a recently described histologic tumor subtype of the Müllerian tract. MLA can arise in association with Müllerian lesions that...
Somatic mutation analysis of Mesonephric-Like adenocarcinoma and associated putative precursor Lesions: Insight into pathogenesis and potential molecular treatment targets.
AIMS
Mesonephric-like adenocarcinoma (MLA) is a recently described histologic tumor subtype of the Müllerian tract. MLA can arise in association with Müllerian lesions that share common mutations. We report three MLAs and hypothesize that concurrent endometriosis and cystadenofibroma with focal borderline changes might also carry common mutations.
METHODS AND RESULTS
We searched "mesonephric" in our database from 2015 to mid-2021 to retrieve MLA cases. Somatic mutation analysis was performed on tumors and on associated benign proliferative lesions. All MLAs (2 ovarian and 1 uterine) harbored G12D or G12 V mutations. A alteration (H1047Q) was detected in one MLA and in the associated cystadenofibroma with focal borderline changes. The molecular profile of MLA-associated Müllerian lesions (endometriosis and seromucinous cystadenofibroma with focal borderline changes) was similar to concurrent adenocarcinoma. However, tumor contamination could not be excluded in the endometriotic lesion. Patients presented at various stages, with no evidence of post-operative recurrence after 15 months (FIGO IC) and 33 months (FIGO IIA2). One patient (FIGO IIIA1) died of disease 32 months after surgery.
CONCLUSIONS
mutations commonly characterize MLA. At least some MLA-associated Müllerian lesions show MLA-like genetic profiles, suggesting a precursor role. As far as we are aware, we describe for the first time in MLA the potentially actionable H1047Q variant of .
PubMed: 35880223
DOI: 10.1016/j.gore.2022.101049 -
Frontiers in Oncology 2022Mesonephric-like adenocarcinoma (MLA) is a recently characterized, rare, and aggressive neoplasm that mostly arises in the uterine corpus and ovary. MLA shows...
BACKGROUND
Mesonephric-like adenocarcinoma (MLA) is a recently characterized, rare, and aggressive neoplasm that mostly arises in the uterine corpus and ovary. MLA shows characteristic pathological features similar to mesonephric adenocarcinoma of the cervix. The origin of MLA is still controversial and recognition of it remains challenging for pathologists. The aim of this study was to enrich the clinicopathological features of MLA in the uterine corpus and explore its molecular alterations by targeted next-generation sequencing (NGS).
METHODS
Four cases of MLA were identified among a total of 398 endometrial carcinomas diagnosed in our institution between January 2014 and December 2021. Immunohistochemistry and targeted NGS spanning 437 cancer-relevant genes were performed.
RESULTS
The most common symptom was abnormal vaginal bleeding, and the average age was 68 years. Histologically, the tumors showed a mixture of varied growth patterns including papillary, glandular, tubular, cribriform, solid, and slit-like architectures, which were lined by columnar to cuboidal cells with overlapping vesicular nuclei and sometimes nuclear grooves. Intraluminal eosinophilic colloid-like secretions were focally evident in three of the four cases. Immunohistochemically, the MLAs were positive for GATA3 (4/4), TTF-1 (3/3), luminal CD10 (3/3), calretinin (2/3), and patchy P16 (3/3) and were negative for ER (0/4) and PR (0/4). The expression of P53 was "wild type" (4/4). By targeted NGS, 3/4 (75%), 2/4 (50%), and 1/4 (25%) cases harbored , , and mutations, respectively. None of the tumors had mutations in DNA mismatch repair genes, , , , , or . At the time of diagnosis, three were presented with FIGO IB stage and one with IIIC stage. Two patients received postoperative chemotherapy and radiotherapy and they were alive without evidence of disease at 8 and 56 months follow-up, respectively. One patient developed pulmonary metastasis 13 months after surgery and chemotherapy, and one was dead of the disease 24 months after the operation without adjuvant therapy.
CONCLUSIONS
MLA is a rare and aggressive malignancy, representing approximately 1% of all endometrial carcinomas. It exhibits mixed architectures associated with distinctive immunophenotype and recurrent and mutations, supporting classified as of Müllerian origin with mesonephric differentiation.
PubMed: 35865471
DOI: 10.3389/fonc.2022.911695 -
Annals of Medicine and Surgery (2012) Jul 2022and importance: Zinner syndrome is a rare congenital malformation of the seminal vesicles and the homolateral upper urinary tract. While the majority of patients remain...
INTRODUCTION
and importance: Zinner syndrome is a rare congenital malformation of the seminal vesicles and the homolateral upper urinary tract. While the majority of patients remain asymptomatic and are discovered incidentally, others present symptoms such as micturition or ejaculatory difficulties, or pain. We report a case of Zinner syndrome in a 32-year-old patient with painful ejaculation and discuss the diagnosis and treatment difficulties.
CASE PRESENTATION
A 32-year-old married patient was consulted for pelvic pain associated with painful ejaculation that had been evolving for six months. The clinical examination was normal. Routine laboratory studies of blood and urine were normal. The patient was explored by ultrasound which showed the absence of the right kidney and the presence of a 7 cm right lateral prostatic cystic mass. On MRI, the right kidney was not visualized. Multiple cysts were seen in the right seminal vesicle. Surgical excision of the cyst by laparotomy was performed. The patient had an uneventful recovery and was discharged on the third postoperative day.
CLINICAL DISCUSSION
Congenital malformations of the seminal vesicles are often associated with those of the ipsilateral upper urinary tract, as the ureteral and seminal vesicle buds originate from the mesonephric duct. The syndrome often occurs in the second and third decades of life, especially after the onset of sexual activity. The most common symptoms were dysuria, perineal pain, epididymitis, and painful ejaculation. Diagnostic modalities include ultrasound, MRI, and cystoscopy. In patients with symptoms, the therapeutic management of the cyst includes ultrasound-guided aspiration and laparoscopic or open surgical excision.
CONCLUSION
Seminal vesicle cysts associated with homolateral renal agenesis or hypoplasia are a rare urologic anomaly. The treatment depends on the patient's symptoms. surgical excision of seminal vesicle cysts may be needed for large cysts causing obstructive symptoms.
PubMed: 35860150
DOI: 10.1016/j.amsu.2022.103982 -
Cancer Genomics & Proteomics 2022Uterine mesonephric-like adenocarcinoma (MLA) is a rare malignant tumor of the female genital tract. (Review)
Review
Mesonephric-like Adenocarcinoma of the Uterine Corpus: Comprehensive Analyses of Clinicopathological, Molecular, and Prognostic Characteristics With Retrospective Review of 237 Endometrial Carcinoma Cases.
BACKGROUND/AIM
Uterine mesonephric-like adenocarcinoma (MLA) is a rare malignant tumor of the female genital tract.
PATIENTS AND METHODS
We reviewed 237 endometrial carcinoma cases and investigated the clinicopathological and molecular characteristics of uterine MLA.
RESULTS
We found that 3.0% (7/237) of the endometrial carcinoma cases were MLAs. Compared to endometrial endometrioid carcinoma, MLA showed larger tumor size, deeper myometrial invasion, increasingly advanced-stage disease, and more frequent lymphovascular space invasion. All MLAs exhibited architectural diversity, compactly aggregated small tubules, eosinophilic intraluminal secretions, overlapped and angulated nuclei, scant cytoplasm, and presence of spindle cells. All the MLAs expressed at least two mesonephric markers. All except one MLA harbored activating Kirsten rat sarcoma viral oncogene homolog mutations. All patients with MLA developed postoperative metastases. MLA had the lowest progression-free survival rate among different histological types of endometrial carcinoma.
CONCLUSION
Uterine MLA is a highly aggressive gynecological malignancy, showing unique morphological and molecular features, frequent recurrences and metastases, as well as poor prognosis.
Topics: Adenocarcinoma; Carcinoma, Endometrioid; Endometrial Neoplasms; Female; Humans; Prognosis; Retrospective Studies
PubMed: 35732320
DOI: 10.21873/cgp.20338 -
Urology Case Reports Jul 2022Primary mesonephric adenocarcinoma of the bladder is a very rare lesion. Only 9 cases have been reported since 1968, when it was first described. Due to its...
Primary mesonephric adenocarcinoma of the bladder is a very rare lesion. Only 9 cases have been reported since 1968, when it was first described. Due to its morphological diversity and variable immunohistochemical profile, mesonephric adenocarcinoma presents a diagnostic challenge, especially when seen in male patients, due to the rarity this entity in men. Here we present a rare case of a 63-year-old man who was found to have a bladder tumor and diagnosed with mesonephric adenocarcinoma of the bladder.
PubMed: 35520026
DOI: 10.1016/j.eucr.2022.102096 -
Radiology Case Reports Jun 2022Splenogonadal fusion is a rare, frequently misdiagnosed, congenital anomaly in which the splenic tissue is abnormally attached to the gonadal or mesonephric remnants. It...
Splenogonadal fusion is a rare, frequently misdiagnosed, congenital anomaly in which the splenic tissue is abnormally attached to the gonadal or mesonephric remnants. It is commonly found as an incidental finding at autopsy, during orchiopexy or hernia repair. However, it can present as a testicular mass or as an acute scrotal pathology such as testicular torsion or epididymoorchitis. It poses as a diagnostic challenge preoperatively and often leads to unnecessary orchiectomy. We present a case of a 15-year-old male who presented with a long-standing left testicular mass thought to be a testicular tumor. Resection of the lesion along with partial left orchiectomy was done and histopathologic evaluation revealed splenogonadal fusion.
PubMed: 35496753
DOI: 10.1016/j.radcr.2022.01.065 -
The American Journal of Surgical... Aug 2022The literature indicates that mesonephric carcinoma (MC) and mesonephric-like adenocarcinoma (MLA) typically lack mucinous and squamous features/differentiation. We...
The literature indicates that mesonephric carcinoma (MC) and mesonephric-like adenocarcinoma (MLA) typically lack mucinous and squamous features/differentiation. We report 4 cases of ovarian mucinous tumors (1 mucinous cystadenofibroma and 3 mucinous borderline tumors/atypical proliferative mucinous tumors [MBT/APMT]) co-existing with mesonephric-like lesions which were highlighted by Gata3 and Pax8 expression. All cases contained benign mesonephric-like proliferations (MLP) which focally displayed gastrointestinal-type mucinous metaplasia/differentiation and some were intimately admixed with mucinous glands associated with the mucinous tumor. Metaplastic mucinous epithelium retained expression of Gata3 and Pax8 in some areas while 1 mucinous cystadenofibroma and 1 MBT/APMT were focally positive for Pax8. Along with these mesonephric components, case 1 exhibited features of mesonephric hyperplasia and in 2 cases, 3 and 4, MLA was identified. In case 4, a KRAS c.35G>T (p.Gly12Val) somatic mutation was detected in both the MBT/APMT and the MLA, indicating a clonal origin. This same mutation was also detected in the benign MLP, indicating that it was likely an early genetic event. A CTNNB1 c.98C>T (p.Ser33Phe) somatic mutation, FGFR2 amplification, and CDKN2A/p16 deletion were only detected in the MLA but not in the MBT/APMT. Our result provides evidence to demonstrate the clonal relationship between these morphologically distinct components. Although speculative, we postulate that benign MLPs may give rise to lineage-specific mucinous and mesonephric-like lesions and propose that the MLPs are a new possible origin of some ovarian mucinous tumors. Whether these MLPs arise through transdifferentiation of Müllerian tissue or represent true mesonephric remnants, however, remains largely unknown.
Topics: Adenocarcinoma; Biomarkers, Tumor; Cell Proliferation; Cystadenofibroma; Female; Humans; Ovarian Neoplasms
PubMed: 35405716
DOI: 10.1097/PAS.0000000000001903 -
Journal of Cancer Research and... 2022Mesonephric carcinoma is a rare type of carcinoma seen in the female genital tract. It arises from the mesonephric remnants situated in the broad ligament, lateral wall... (Review)
Review
Mesonephric carcinoma is a rare type of carcinoma seen in the female genital tract. It arises from the mesonephric remnants situated in the broad ligament, lateral wall of the cervix, vagina, and uterine corpus. Very few cases of mesonephric carcinoma have been reported so far in the literature. The sites mentioned in various literatures include the cervix, vagina, or uterus, but we could not find any literature that mentions posthysterectomy vault as a site for mesonephric carcinoma. Here, we report a case of 40-years-old hysterectomised female who presented in the hospital with nodular growth on the vault and complaints of bleeding per vaginum. Microscopy of the lesion did not show typical morphology of mesonephric carcinoma, but immunohistochemistry played a vital role in the diagnosis of this rare tumor.
Topics: Adenocarcinoma; Adult; Carcinoma; Cervix Uteri; Female; Humans; Hysterectomy; Immunohistochemistry; Uterine Cervical Neoplasms
PubMed: 35381800
DOI: 10.4103/jcrt.JCRT_168_19 -
International Journal of Gynecological... Mar 2023Ovarian combined serous borderline tumor/low-grade serous carcinomas (SBT/LGSC) and mesonephric-like adenocarcinomas (MLA) have been previously reported and the presence...
Ovarian combined serous borderline tumor/low-grade serous carcinomas (SBT/LGSC) and mesonephric-like adenocarcinomas (MLA) have been previously reported and the presence of identical oncogenic somatic mutations in both components supports the concept that at least some of MLAs arise from a Müllerian origin. We report 2 cases of ovarian combined SBT/LGSC and mesonephric-like lesion. Case 1 was a 70-yr-old woman presented with a liver lesion and omental carcinomatosis. Histologic examination revealed biphasic tumors in bilateral ovaries consisting of conventional SBT and invasive MLA with extraovarian spread. The right ovary also had a component of cribriform variant of SBT/noninvasive LGSC. The SBT/LGSC component was diffusely positive for Pax8, WT-1, and ER, focally positive for PR, and negative for GATA3, while the MLA component was diffusely positive for GATA3 but negative for WT-1, ER, and PR. Molecular analysis revealed a KRAS G12V mutation in both the SBT/LGSC and MLA components, indicating their clonal origin. Case 2 was a 58-yr-old woman who presented with conventional type SBT in both ovaries. In addition, the left ovarian tumor demonstrated a few areas (each <5 mm) of mesonephric-like differentiation/hyperplasia in close proximity to the serous-type epithelium, with an immunophenotype of focal GATA3 expression, luminal pattern of CD10 staining and negative WT-1, ER, and PR staining. This phenomenon has been reported in endometrioid borderline tumor but not in any serous type lesions. The findings in case 1 provide further evidence to demonstrate the clonal relationship between these morphologically and immunophenotypically distinct components. It also supports the theory that, unlike cervical mesonephric carcinomas originating from mesonephric remnants, MLAs are derived from a Müllerian-type lesion with differentiation into mesonephric lineage. The presence of a hyperplastic mesonephric-like lesion/differentiation in case 2 indicates that a precursor lesion in the same lineage with the potential to develop into MLA exists in the ovary.
Topics: Female; Humans; Ovarian Neoplasms; Carcinoma; Mesonephros; Epithelium; Hyperplasia; Cystadenocarcinoma, Serous
PubMed: 35348533
DOI: 10.1097/PGP.0000000000000868