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Medicine Nov 2020Mesonephric-like adenocarcinoma (MLA) from ovary is a very rare tumor which derives from mesonephric duct remnant of the female genital tract. Only six cases have been... (Review)
Review
RATIONALE
Mesonephric-like adenocarcinoma (MLA) from ovary is a very rare tumor which derives from mesonephric duct remnant of the female genital tract. Only six cases have been reported so far in the English literature.
PATIENT CONCERNS
A 29-year-old female patient was referred to the local hospital with a 20-day history of abdominal discomfort.
DIAGNOSES
Pelvic ultrasound examination revealed a solid and cystic mass measuring 10 cm in diameter in the right adnexal area and a cystic mass measuring 5 cm in the left adnexal area. Postoperative pathology in the local hospital revealed suspected malignancy of the right ovary, and she was then transferred to our institution for definite diagnosis. The tumor mass was finally diagnosed as a primary MLA arising from the right ovary by histological and immunohistochemical examination in our institution.
INTERVENTIONS
The patient underwent laparoscopic right adnexectomy and removal of left ovarian cyst in the local institution. Then, she underwent a complete staging surgery including a total hysterectomy, left adnexectomy, pelvic plus para-aortic lymphadenectomy, and omentectomy in our hospital. In addition, she received four cycles of combination chemotherapy with carboplatin plus paclitaxel.
OUTCOMES
There is no evidence of recurrence with 13 months of follow-up till now, and we are still following-up this patient.
LESSONS
MLA is an extremely uncommon malignancy with difficult diagnosis, unclear treatment and poor prognosis. Familiarizing with the clinical features and optimal management of this rare tumor may increase awareness of the disease among clinicians and pathologists, thus avoiding the misdiagnosis and mistreatment.
Topics: Adenocarcinoma; Adult; Chemotherapy, Adjuvant; Female; Humans; Ovarian Neoplasms; Rare Diseases; Wolffian Ducts
PubMed: 33235131
DOI: 10.1097/MD.0000000000023450 -
The American Journal of Surgical... Apr 2021Mesonephric adenocarcinoma (MA) and mesonephric-like adenocarcinoma (MLA) are uncommon neoplasms of the gynecologic tract that have until recently been poorly...
Mesonephric adenocarcinoma (MA) and mesonephric-like adenocarcinoma (MLA) are uncommon neoplasms of the gynecologic tract that have until recently been poorly understood. Although their morphologic, immunohistochemical, and molecular profiles have been recently defined, little is known about their clinical behavior. Small studies have demonstrated inconsistent findings and no large studies have examined the clinical behavior of these adenocarcinomas. In this multi-institutional study, representing the largest and most stringently defined cohort of cases to date, we examined the clinicopathologic features of 99 MAs and MLAs (30 MAs of the uterine cervix, 44 MLAs of the endometrium, and 25 MLAs of the ovary). Only tumors with characteristic mesonephric morphology and either immunohistochemical or molecular support were included. Our results demonstrate that the majority of mesonephric neoplasms presented at an advanced stage (II to IV) (15/25 [60%] MA of the cervix, 25/43 [58%] MLA of the endometrium, and 7/18 [39%] MLA of the ovary). The majority (46/89 [52%] overall, 12/24 [50%] MA of the cervix, 24/41 [59%] MLA of the endometrium, and 10/24 [42%] MLA of the ovary) developed recurrences, most commonly distant (9/12 [75%] MA of the cervix, 22/24 [92%] MLA of the endometrium, and 5/9 [56%] MLA of the ovary). The 5-year disease-specific survival was 74% (n=26) for MA of cervix, 72% (n=43) for MLA of endometrium, and 71% (n=23) for MLA of ovary. Our results confirm that mesonephric neoplasms are a clinically aggressive group of gynecologic carcinomas that typically present at an advanced stage, with a predilection for pulmonary recurrence.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Databases, Factual; Endometrial Neoplasms; Female; Humans; Lung Neoplasms; Middle Aged; Neoplasm Recurrence, Local; Neoplasm Staging; North America; Northern Ireland; Ovarian Neoplasms; Progression-Free Survival; Registries; Time Factors; Uterine Cervical Neoplasms; Wolffian Ducts
PubMed: 33165093
DOI: 10.1097/PAS.0000000000001612 -
Journal of Endourology Case Reports 2020A 17-year-old male with Zinner syndrome, a right seminal vesicle cyst, and a solitary left kidney presented with chronic pelvic pain. Previous surgeons had attempted...
A 17-year-old male with Zinner syndrome, a right seminal vesicle cyst, and a solitary left kidney presented with chronic pelvic pain. Previous surgeons had attempted robot-assisted laparoscopic seminal vesicle cyst aspiration and transurethral resection of the ejaculatory duct. Neither surgery provided sustained symptom relief. Abdominal and pelvic MRI showed a cystic structure lodged between the prostate and bladder. The right seminal vesicle, kidney, and ureter were not observed. A robot-assisted laparoscopic seminal vesiculectomy was planned. Dissection distal to the right vas deferens and between the bladder neck and prostate revealed a cystic seminal vesicle-like structure. Attached to this was a tubular structure coursing deep to the vas deferens from the right renal fossa. This was presumed to be a dysplastic ureter. The dysplastic ureter was transected from the seminal vesicle and the seminal vesicle was marsupialized to the deep pelvis. Proximally, the dysplastic ureter was transected and left open. Histologic assessment of the specimen revealed an ∼12.1 cm tubular mesonephric remnant. The postoperative course was uncomplicated. At 6 months follow-up, the patient remains free of symptoms with preserved ejaculatory volume. Mesonephric duct abnormalities and symptoms present on a spectrum. We present a safe and effective resection of a mesonephric duct remnant from a 17-year-old male with Zinner syndrome. A robotic approach localized to the right allowed for excellent observation without compromising left-sided genitourinary anatomy. In males presenting with renal agenesis and pelvic symptoms, clinicians should be suspicious of Zinner syndrome and other mesonephric abnormalities.
PubMed: 33102726
DOI: 10.1089/cren.2020.0020 -
Cancer Genomics & Proteomics 2020Mesonephric carcinoma (MNC) is a rare but notable entity of the female genital tract. While many researchers have acknowledged and studied MNC, much remains unknown on...
BACKGROUND/AIM
Mesonephric carcinoma (MNC) is a rare but notable entity of the female genital tract. While many researchers have acknowledged and studied MNC, much remains unknown on the characteristics of mesonephric remnant (MNR) or hyperplasia (MNH). There has not been any study examining the molecular features of MNR and MNH so far. The aim of this study was to investigate the clinicopathological and molecular characteristics of ten uterine mesonephric lesions, including two MNRs without atypia, four MNHs without atypia, and three MNHs with atypia.
MATERIALS AND METHODS
We reviewed the electronic medical records and all available slides of ten cases from multiple institutions. Targeted sequencing and array comparative genomic hybridization were performed.
RESULTS
Three atypical MNHs displayed nuclear enlargement, mild-to-moderate nuclear pleomorphism, and nuclear membrane irregularity, and harbored pathogenic Kirsten rat sarcoma 2 viral oncogene homolograt sarcoma 2 viral oncogene homolog (KRAS) mutation. Two of those that co-existed with MNC harbored the same sequence alterations as each of their adjacent MNC. One of the three atypical MNHs harbored chromosome 1q gain.
CONCLUSION
Atypical MNH is a potential premalignant lesion in which KRAS mutation and chromosome 1q gain play an important role in the early stage of mesonephric carcinogenesis.
Topics: Adenocarcinoma; Biomarkers, Tumor; Chromosomes, Human, Pair 1; Female; Gain of Function Mutation; Humans; Hyperplasia; Mesonephroma; Middle Aged; Mutation; Precancerous Conditions; Prognosis; Proto-Oncogene Proteins p21(ras); Uterine Cervical Neoplasms
PubMed: 33099482
DOI: 10.21873/cgp.20235 -
Archives of Pathology & Laboratory... Jul 2021While the vast majority of cervical tumors consist of human papillomavirus (HPV)-related squamous cell carcinoma or adenocarcinoma, a subset of rare tumor types,...
CONTEXT.—
While the vast majority of cervical tumors consist of human papillomavirus (HPV)-related squamous cell carcinoma or adenocarcinoma, a subset of rare tumor types, frequently unrelated to HPV, does occur in this location. These tumors vary widely in prognostic and therapeutic implications, and accurate recognition is crucial to providing appropriate treatment. Some are benign or portend a favorable prognosis (adenoid basal carcinoma, ectopic prostate tissue), while others are frankly malignant lesions with a less favorable prognosis (adenoid cystic carcinoma, HPV-negative endocervical adenocarcinoma, mesonephric adenocarcinoma, clear cell carcinoma, small cell carcinoma, and adenosquamous carcinoma).
OBJECTIVE.—
To review the morphologic features of uncommon cervical lesions, the utility of immunohistochemistry for distinction between these entities, and the clinical and prognostic implications of accurate diagnosis.
DATA SOURCES.—
University of Michigan cases and review of the pertinent literature regarding the entities described.
CONCLUSIONS.—
Key morphologic and immunohistochemical features detailed herein will allow for the accurate distinction between these uncommon cervical lesions. Morphology is most useful in discriminating between the entities, as there is frequent immunohistochemical overlap between them; however, in rare instances immunohistochemistry can be useful in resolving the diagnosis.
Topics: Biomarkers, Tumor; Biopsy; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Papillomaviridae; Papillomavirus Infections; Predictive Value of Tests; Reproducibility of Results; Uterine Cervical Neoplasms
PubMed: 33091926
DOI: 10.5858/arpa.2020-0327-RA -
Gynecologic Oncology Reports Nov 2020Mesonephric-like adenocarcinoma (MLA) is a rare malignant gynecologic neoplasm occurring in the uterine corpus and ovary. The morphological and immunohistochemical...
INTRODUCTION
Mesonephric-like adenocarcinoma (MLA) is a rare malignant gynecologic neoplasm occurring in the uterine corpus and ovary. The morphological and immunohistochemical characteristics of MLA closely resemble that of cervical mesonephric adenocarcinomas, but whether they share a common histogenesis remains unclear. Two main theories for histogenesis of MLAs include the origination of these neoplasms from mesonephric remnants, as is the case for cervical mesonephric adenocarcinoma, versus the differentiation along a mesonephric pathway from Mullerian lesions.
CASE
A 67-year-old presented after a right salpingo-oophorectomy for a complex ovarian mass revealed a mesonephric-like adenocarcinoma of the ovary and endometriosis. She underwent a total abdominal hysterectomy, pelvic lymphadenectomy, and infra-colic omentectomy, and diagnosed with Stage IA mesonephric-like adenocarcinoma of the ovary. At 18 months post-operatively, the patient developed flank and abdominal pain and was found to have multiple sites of recurrent disease. She was referred to medical oncology for chemotherapy as she was not a candidate for surgical cytoreduction.
DISCUSSION
This case demonstrates the aggressive nature of ovarian MLA and the need for a multidisciplinary approach when determining the treatment. In addition, this case provides further evidence to support the theory that at least a subset of MLAs arises from a Mullerian lesion which then differentiates down a mesonephric pathway.
PubMed: 33088886
DOI: 10.1016/j.gore.2020.100657 -
Radiologia Brasileira 2020Although secondary involvement of the broad ligament by malignant tumors arising elsewhere in the abdomen and pelvis is common, primary tumors in this location are rare....
Although secondary involvement of the broad ligament by malignant tumors arising elsewhere in the abdomen and pelvis is common, primary tumors in this location are rare. Tumors of the broad ligament can be of mesenchymal and mixed nature, such as leiomyoma, the most common neoplasm; epithelial tumors of Müllerian type, imposing a challenge to differentiate them from other adnexal masses; unique tumors from mesonephric origin; and tumor-like lesions. Most neoplasms in this region, whether benign or malignant, usually present clinically with vague symptoms and are often discovered during a routine gynecological examination. Suspicion of such location and knowledge of the potential range of lesions of this region may allow for planning minimally invasive surgical interventions. To be considered tumor from the broad ligament, it should not be connected with either the uterus or the ovary. Thus, the imaging approach to establish the differential diagnosis includes excluding an ovarian, uterine, or tubal origin by recognizing these separately and by rebutting imaging clues pointing to these origins. This pictorial essay reviews some of the imaging findings that may suggest such location and presents some of the possible differential diagnoses by means of illustrative confirmed cases.
PubMed: 33071380
DOI: 10.1590/0100-3984.2019.0073 -
Fertility and Sterility Feb 2021To describe the treatments of a patient using the laparoscopic Davydov's method for Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and ureteral reimplantation for...
OBJECTIVE
To describe the treatments of a patient using the laparoscopic Davydov's method for Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and ureteral reimplantation for hydronephrosis and hydroureter.
DESIGN
Surgical video article. A consent form from the patient was obtained as appropriate; the nature of the study did not necessitate ethics committee approval. There were no conflicts of interest.
SETTING
University hospital.
PATIENT(S)
A 28-year-old woman who presented at our gynecology department with the symptoms of primary amenorrhea and difficult intercourse. She had repaired congenital rectovestibular fistula and imperforate anus at the age of 8. At physical examination, she had a phenotypically normal vulva with a vaginal small pouch (0.5 cm). Magnetic resonance imaging of the pelvis revealed normal ovaries, a primordial uterus, absence of vaginal canal, and a 4.0 × 4.2 × 4.0 cm cystic structure posterior to the bladder. Magnetic resonance urography showed right to left renal crossed-ectopia with inferior fusion, and hydronephrosis and hydroureter from the superior kidney with Grade Ⅳ vesicoureteral reflux. Karyotype was 46, XX.
INTERVENTION(S)
Saline solution 300 mL was injected into the rectovesical space with an infusion of diluted adrenaline (1:200,000). The goal of this injection was to aid in the identification of tissue planes and reduce blood loss. The space between urethra/bladder and rectum progressively was dissected. Blunt dissection was performed initially with digital separation of tissues. Then, an 8-cm-long neovaginal vault of about 3 cm in diameter was created. The mobilized peritoneum was pulled downward with eight Vicryl sutures and connected to the vaginal epithelium. By cystoscope, we found the left orifice but could not find the right orifice of the hydroureter. Then we ligated the hydroureter by 2-0 absorbable suture near the cyst and cut off the hydroureter, and then incised of all the layers at the top of the bladder to make a bladder flap. We placed the 5 Fr double J stent in the hydroureter and the bladder and anastomozed with the ureteral stump (3-0 Vicryl). Then we removed the cyst laparoscopically. We performed a purse-string stitch to create the apex of the neovagina by taking posterior serosa of the bladder, the pelvic peritoneum between the ovary and rectum, primordial uterus, and anterior rectal serosa.
MAIN OUTCOME MEASURE(S)
Measurement of the final canal length, sexual function (Female Sexual Function Index), and degree of hydronephrosis.
RESULT(S)
Three days later, we started to change the vaginal mold and the patient was advised to wear it day and night for the first postoperative month. The vaginal mold had to be worn each night until normal sexual intercourse was possible. Findings confirmed the cyst was Gartner's duct cyst. One year after the surgery, the final canal length was 9 cm and Female Sexual Function Index score was 28. The ultrasound showed that the degree of hydronephrosis of upper moiety was mild.
CONCLUSION(S)
The distal Wolffian ducts in the female are absorbed but may persist as vestigial remnants (Gartner's duct cysts). A few cases of the combined urogenital-Wolffian anomalies are reported; most of them are associated with the anomalies of müllerian duct fusion, such as Herlyn-Werner-Wunderlich syndrome (uterus didelphys, obstructed hemivagina, and mesonephric duct anomalies). The embryogenesis of the combined anomalies is not completely understood. With comprehensive preoperative assessments, laparoscopic surgery could be a safe and effective treatment to these cases.
Topics: 46, XX Disorders of Sex Development; Adult; Choristoma; Congenital Abnormalities; Cysts; Female; Humans; Kidney; Laparoscopy; Mullerian Ducts; Video-Assisted Surgery; Wolffian Ducts
PubMed: 33069370
DOI: 10.1016/j.fertnstert.2020.08.1433 -
Gynecologic Oncology Reports Nov 2020Mesonephric carcinoma is a rare cancer that most often arises within the cervix, and less frequently, in the ovary and endometrium. A retrospective search of our...
Mesonephric carcinoma is a rare cancer that most often arises within the cervix, and less frequently, in the ovary and endometrium. A retrospective search of our CLIA-certified and CAP-accredited reference molecular laboratory database (Foundation Medicine, Inc.) identified 20 mesonephric or mesonephric-like, cervical (n = 10), endometrial (n = 5), ovarian (n = 4) or peri-bladder (n = 1) carcinomas that had undergone comprehensive genomic profiling via next generation sequencing. Activating mutations were present in 90%, 18 of 20 cases, including G12V (n = 7), G12D (n = 6), G12A (n = 3) and G12C (n = 2). Other recurrent alterations were identified in (25%), (20%), (15%), (10%), (10%) and (10%). One wild-type case had a mutation as the sole alteration, while the second wild-type case had an exon 20 insertion D770_N771insSVD alteration. All tumors were negative for HPV DNA, microsatellite instability, high tumor mutational burden and homologous recombination deficiency. A circulating tumor DNA (ctDNA) liquid biopsy from peripheral blood, which was performed 6 years after original solid tumor resection in one patient with suspected lung metastasis, revealed concordance of alteration, gains of chromosomes 1q, 2, 10, 12 and 20, plus new alterations in the liquid biopsy compared to the original sample. G12 mutation is major driver of mesonephric and mesonephric-like carcinomas, with less frequent contribution by ARID1A and PIK3CA pathways in tumors of non-cervical origin. ctDNA liquid biopsy may be useful in detecting mutations in recurrent or metastatic patients, who may potentially be eligible for trials against emerging targeted therapies.
PubMed: 33024807
DOI: 10.1016/j.gore.2020.100652 -
Diagnostics (Basel, Switzerland) Sep 2020Mesonephric adenocarcinoma is a rare tumor that is considered to develop from mesonephric remnants of the female genital tract. This tumor usually occurs in the lateral...
Mesonephric adenocarcinoma is a rare tumor that is considered to develop from mesonephric remnants of the female genital tract. This tumor usually occurs in the lateral wall of the uterine cervix. Herein, we present an exceptionally rare case of mesonephric adenocarcinoma located in the uterine fundus. The tumor exhibited intense hypermetabolism on F-FDG PET/CT. Based on the characteristic histologic features and immunohistochemical phenotypes, the diagnosis of mesonephric adenocarcinoma was confirmed. The patient underwent hysterectomy with bilateral salpingo-oophorectomy and pelvic lymph node dissection, and no lymph node or distant metastasis was identified. After 20 months of surveillance without adjuvant therapy, she remains free of relapse.
PubMed: 32967381
DOI: 10.3390/diagnostics10090729