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Taiwanese Journal of Obstetrics &... Aug 2018To assess the clinicopathological characteristics and clinical management of patients diagnosed with mullerian adenosarcoma of the uterine cervix. (Review)
Review
OBJECTIVE
To assess the clinicopathological characteristics and clinical management of patients diagnosed with mullerian adenosarcoma of the uterine cervix.
MATERIALS AND METHODS
Records of six patients surgically treated for cervical mullerian adenosarcoma were reviewed.
RESULTS
The median age of the patients was 50 years (range, 17-74). Four patients presented with vaginal bleeding and two of them had watery discharge as the primary symptoms. Three nulliparous patients who hoped to preserve their uterus were included in this study and only one of the three patients received fertility-preservation surgery. Five patients underwent hysterectomy (simple in 3, modified radical in 2) with bilateral salpingo-oophorectomy or bilateral salpingectomy. One patient underwent conization of the cervix to preserve her fertility as there was no sarcomatous overgrowth, heterologous elements, or deep cervical stromal invasion. A recurrence of cervical mullerian adenosarcoma with sarcomatous overgrowth was observed in one patient who underwent simple hysterectomy.
CONCLUSION
The presence of sarcomatous overgrowth and deep cervical stromal invasion are associated with poor prognosis. Along with adequate counseling, fertility-preservation surgery may be an acceptable option for exophytic cervical mullerian adenosarcoma in the cases that do not show sarcomatous overgrowth and deep cervical stromal invasion.
Topics: Adenosarcoma; Adolescent; Adult; Aged; Cervix Uteri; Conization; Female; Fertility Preservation; Humans; Hysterectomy; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Ovariectomy; Salpingectomy; Uterine Cervical Neoplasms; Uterine Neoplasms
PubMed: 30122564
DOI: 10.1016/j.tjog.2018.04.032 -
The Journal of International Medical... Sep 2022Adenofibroma is an extremely rare benign biphasic tumour composed of glandular and fibrous tissues. It occurs more often in the endometrium but it can also occur in the... (Review)
Review
Adenofibroma is an extremely rare benign biphasic tumour composed of glandular and fibrous tissues. It occurs more often in the endometrium but it can also occur in the cervix and extrauterine sites. This case report describes a 39-year-old asymptomatic woman with cervical adenofibroma. The patient was treated successfully with surgical removal of the tumour. As adenofibromas are very rare, the report is presented with a brief review of the literature.
Topics: Adenofibroma; Adult; Female; Humans; Uterine Cervical Neoplasms
PubMed: 36168707
DOI: 10.1177/03000605221125525 -
Diagnostic Pathology Sep 2014Metanephric neoplasms comprised a spectrum of kidney tumors containing renal epithelial or stromal cells or both, including metanephric adenoma, metanephric stromal...
BACKGROUND
Metanephric neoplasms comprised a spectrum of kidney tumors containing renal epithelial or stromal cells or both, including metanephric adenoma, metanephric stromal tumor, and metanephric adenofibroma. The majority of cases were benign; only one case of "metanephric adenosarcoma" had been reported in the English literature.
HISTORY
We present the case of a 69-year-old man who developed a neoplasm composed of renal epithelial component identical to metanephric adenoma combined with malignant spindle-cell stroma. The epithelial component was positive for CD57, AE1/AE3, but negative for WT-1, CD56, SYN, and CgA; whereas the sarcomatous component was negative for epithelial markers, SMA, Caldesmon, MyoD1, Myogenin, and S-100; and positive for vimentin, CD10, and WT1 focally. No specific sarcoma differentiation was apparent in the stroma by immunohistochemistry, and no SYT-SS18 rearrangement or BRAF mutation was detected by molecular analysis.A diagnosis of metanephric adenosarcoma was made because of the morphological features and immunohitochemistry and molecular pathology analysis.
CLINICAL SIGNIFICANCE
We believe that metanephric adenosarcoma should be in the expanded spectrum of metanephric neoplasia as a malignant stromal variant.
CONCLUSIONS
We report a rare case of metanephric adenosarcoma with immunohistochemistry and molecular analysis and emphasize the histopathologic features and differential diagnosis of the rare lesion to promote a better and broader understanding of this less understood subject.
VIRTUAL SLIDES
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_179.
Topics: Adenosarcoma; Aged; Biomarkers, Tumor; Diagnosis, Differential; Fatal Outcome; Humans; Immunohistochemistry; Kidney; Kidney Neoplasms; Male; Stromal Cells
PubMed: 25267074
DOI: 10.1186/s13000-014-0179-7 -
BMJ Case Reports Nov 2020Embryonal rhabdomyosarcoma (ERMS) is a malignant neoplasm mostly affecting the genitourinary system of children and is rarely seen in adults. ERMS in uterine cervix is... (Review)
Review
Embryonal rhabdomyosarcoma (ERMS) is a malignant neoplasm mostly affecting the genitourinary system of children and is rarely seen in adults. ERMS in uterine cervix is rare and can be misdiagnosed in adult as adenosarcoma or carcinosarcoma. The use of immunohistochemical staining is highly recommended to make sure that the correct diagnosis is reached. To the best of our knowledge, only seven cases of cervical ERMS in women above the age of 50 have been reported in English literature. Herein, we report a rare case of cervical ERMS in a 50-year-old woman that was initially misdiagnosed as an adenosarcoma.
Topics: Adenosarcoma; Aged; Diagnosis, Differential; Diagnostic Errors; Female; Humans; Middle Aged; Rhabdomyosarcoma, Embryonal; Uterine Cervical Neoplasms
PubMed: 33257391
DOI: 10.1136/bcr-2020-238596 -
BMC Women's Health Oct 2019Müllerian adenosarcoma is a rare malignancy. These tumors occur mainly in the uterus, but also in extrauterine locations, usually related to endometriosis. Because of...
BACKGROUND
Müllerian adenosarcoma is a rare malignancy. These tumors occur mainly in the uterus, but also in extrauterine locations, usually related to endometriosis. Because of their rarity, there is limited data on optimal management strategies.
CASE PRESENTATION
We present a 44-year-old woman with a history of endometriosis who consults for chronic pelvic pain. In the imaging tests, a heterogeneous mass is observed that impresses endometriosis, encompassing the uterus and left appendage. Surgery is performed by finding an extrauterine adenosarcoma that affected the uterus, ovary and bladder wall.
CONCLUSION
This is a rare case but should be considered in a patient with atypical clinical characteristics or preoperative pathology, so we show the diagnostic and therapeutic strategies carried out for the resolution of the case.
Topics: Adenosarcoma; Adult; Diagnosis, Differential; Endometriosis; Female; Humans; Neoplasm Grading; Ovarian Neoplasms; Pelvic Pain; Uterine Neoplasms
PubMed: 31655582
DOI: 10.1186/s12905-019-0815-1 -
Gynecologic Oncology Reports Apr 2022•Adenosarcoma is a rare tumor of the uterus and cervix occuring mostly in post-menopausal women.•Our patient was a 37-year old nullipara presenting with lower...
•Adenosarcoma is a rare tumor of the uterus and cervix occuring mostly in post-menopausal women.•Our patient was a 37-year old nullipara presenting with lower abdominal pain and backache.•She was diagnosed as a case of multiple leiomyomata and proceeded for myomectomy.•Intraoperative findings raised suspicion of malignancy.•She underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histology confirmed the diagnosis.
PubMed: 35284611
DOI: 10.1016/j.gore.2022.100957 -
Modern Pathology : An Official Journal... Jan 2022A subset of clinically benign uterine polyps shows atypical morphologic features worrisome for, but not diagnostic of, Mullerian adenosarcoma. We report...
A subset of clinically benign uterine polyps shows atypical morphologic features worrisome for, but not diagnostic of, Mullerian adenosarcoma. We report clinicopathologic data for 63 polyps from 58 women with atypical morphologic features including abnormal architecture, abnormal periglandular stroma, stromal atypia, and mitoses >2 per 10 hpf. Four (11%) of 36 women with follow-up tissue sampling had residual/recurrent atypical polyp. Twelve (27%) of 44 women underwent hysterectomy subsequent to a diagnosis of atypical polyp. No patient developed adenosarcoma over median follow-up of 150 months. Twenty-one primary atypical polyps underwent molecular profiling. Five (24%) harbored chr 12q13-15 gain or amplification, 9/20 (45%) harbored chr 6q25.1 gain, and 7/21 (33%) had no significant copy number alterations. Gains of chr 1q, chr 8p12, and chr 10q11.21-23, amplifications of chr 12q24.12-13, chr 15p24.1-26.1, and chr 18q21.33, and loss of chr 7 and chr 11q21 were each seen in a single polyp. Mean tumor mutational burden was 3.1 (range, 0.76-8.365) mutations/Mb. Pathogenic point mutations were identified in 12/20 (60%) primary atypical polyps. We propose the term "atypical uterine polyps" for these lesions, which show biologic overlap with early Mullerian adenosarcoma but lack molecular alterations characteristic of clinically aggressive adenosarcoma and appear to follow a benign clinical course. Conservative management with close clinical follow-up and repeat sampling can be considered for these lesions, when clinically appropriate.
Topics: Adenosarcoma; Adult; Aged; Cohort Studies; Female; Follow-Up Studies; Humans; Middle Aged; Mitosis; Polyps; Uterine Diseases; Young Adult
PubMed: 34675347
DOI: 10.1038/s41379-021-00946-z -
Molecular Oncology Aug 2017Uterine adenosarcomas and breast phyllodes tumors (PTs) are morphologically similar, being composed of stromal projections in a leaf-like fashion lined by epithelial...
Uterine adenosarcomas and breast phyllodes tumors (PTs) are morphologically similar, being composed of stromal projections in a leaf-like fashion lined by epithelial cells. Here, we investigated whether their histologic similarities would be mirrored at the genetic level. The previously reported repertoires of somatic genetic alterations found in 19 adenosarcomas and 22 PTs (six benign, six borderline, and 10 malignant) were compared. PTs significantly more frequently displayed mutations affecting MED12, the TERT gene promoter and bona fide cancer genes, whereas adenosarcomas harbored a higher rate of MDM2/CDK4 and TERT gene amplifications. Pathway analyses based on the genes affected by somatic genetic alterations in these tumors indicated that Wnt signaling likely plays a role in the biology of adenosarcomas and benign/borderline PTs. In conclusion, despite the differences at the gene level, PTs and adenosarcomas share remarkable morphologic similarities and enrichment for somatic genetic alterations affecting Wnt pathway-related genes.
Topics: Adenosarcoma; Breast Neoplasms; Female; Humans; Neoplasm Proteins; Phyllodes Tumor; Uterine Neoplasms; Wnt Signaling Pathway
PubMed: 28267263
DOI: 10.1002/1878-0261.12049 -
Clinical Genitourinary Cancer Dec 2017Müllerian adenosarcoma is a biphasic neoplasm most commonly occurring in the uterus and less frequently of the ovary. It has been rarely described to occur in other...
BACKGROUND
Müllerian adenosarcoma is a biphasic neoplasm most commonly occurring in the uterus and less frequently of the ovary. It has been rarely described to occur in other sites such as peritoneum and liver.
PATIENTS AND METHODS
In this study, we report the clinicopathologic, immunohistochemical and molecular features of a primary Müllerian adenosarcoma of the urinary bladder in a 62-year-old woman. To our knowledge, this is the first report of detailed pathologic characterization of Müllerian adenosarcoma primary to the urinary bladder in the literature.
RESULTS
Light microscopy showed a biphasic epithelial and stromal tumor with benign-appearing glands surrounded by densely cellular endometrial-type stroma that is densely cellular with increased mitotic figures. The stroma surrounding the glands was more cellular than the intervening areas, which were more loose and edematous. Immunohistochemistry staining revealed positive staining for Pax-2/8 within the glands, for positive CD10 and WT-1 within the spindle cell stroma, and for estrogen and progesterone receptors in both. Staining for desmin, GATA3, p63, and human papillomavirus was negative. Molecular analyses identified mutations in protein kinase B E17K, fms related tyrosine kinase 3 D835N, KRAS proto-oncogene, GTPase G12D, and HRAS proto-oncogene, GTPase G12S. These novel molecular aberrations have yet to be reported in the medical literature. X chromosome inactivation analysis revealed a clonal pattern in the stromal component and a nonclonal pattern in the epithelial component. Currently, the patient is disease/recurrence-free after regular follow-up of approximately 84 months.
CONCLUSION
This case represents, to our knowledge, the first reported diagnosis of Müllerian adenosarcoma arising in the urinary bladder with extensive clinicopathologic, immunohistochemical, and molecular analyses.
Topics: Adenosarcoma; Biomarkers, Tumor; Female; Humans; Middle Aged; Mutation; Proto-Oncogene Mas; Proto-Oncogene Proteins c-akt; Uterine Cervical Neoplasms; X Chromosome Inactivation; fms-Like Tyrosine Kinase 3
PubMed: 28690098
DOI: 10.1016/j.clgc.2017.05.020 -
Molecular and Clinical Oncology Mar 2018Uterine sarcomas are some of the most malignant and aggressive tumor types among the gynecologic malignancies, and they are associated with a high rate of recurrence and...
Uterine sarcomas are some of the most malignant and aggressive tumor types among the gynecologic malignancies, and they are associated with a high rate of recurrence and a poor prognosis. Due to their rarity and diversity, the optimal treatment for recurrent uterine sarcomas has not yet been elucidated. The aim of the present study was to investigate the potential of secondary cytoreductive surgery (SCS) for patients with recurrent uterine sarcomas. A total of 18 patients with recurrent uterine sarcomas were retrospectively identified at the Department of Obstetrics and Gynecology, Nagoya University (Nagoya, Japan) between January 2002 and December 2015. This included 8 patients with leiomyosarcoma, 6 with carcinosarcoma, 3 with endometrial stromal sarcoma and 1 with adenosarcoma. All patients underwent primary debulking surgery as the initial treatment. In summary, 9 patients were treated with SCS when they experienced their first recurrence, and the other 9 patients were treated with non-SCS methods, including chemotherapy or radiotherapy. In the SCS group, 5/9 patients had confined pelvic recurrences, 3 patients had extra-pelvic diseases, including pulmonary metastasis, and one patient had intra- and extra-pelvic recurrence. The 3-year overall survival (OS) rates were 77.8 and 11.1% in the SCS and non-SCS groups, respectively. The patients who underwent SCS experienced a significantly longer OS time compared with those in the non-SCS group (P=0.006). In addition, the disease-free survival after second-line therapy was significantly longer in the SCS group than in the non-SCS group (P=0.0496). These findings suggest that resection of recurrent uterine sarcomas may be beneficial for the improvement of patient survival. Prospective studies with sufficient statistical power are warranted for further evaluation of the effect of SCS.
PubMed: 29556383
DOI: 10.3892/mco.2018.1560