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Bulletin Du Cancer 2023Uterine adenosarcoma is a very rare malignancy defined as a biphasic tumor composed of both benign epithelial component and a malignant sarcoma component. The stage of...
Uterine adenosarcoma is a very rare malignancy defined as a biphasic tumor composed of both benign epithelial component and a malignant sarcoma component. The stage of the disease is determined by the presence of myometrial invasion and the extent of extra-uterine disease. The most important histopronostic factors are the existence of a sarcomatous overgrowth defined by a sarcomatous contingent occupying more than 25 % of the volume of the tumor (directly correlated to the grade of the disease), the presence of a heterologous and/or a high-grade component. Stage I adenosarcomas without sarcomatous overgrowth have a good prognosis, with an overall 5-year survival of up to 80 %. In localized disease, complete surgical removal is recommended. The role of hormone therapy, chemotherapy and adjuvant radiotherapy is not established. If possible, relapses should be re-treated surgically, with the aim of achieving complete resection. In the advanced inoperable or metastatic setting, hormone therapy is an option for low-grade adenosarcomas with estrogen receptor (ER) and progesterone receptor (PR) overexpression. For high-grade tumors, the standard chemotherapies are doxorubicin-based combinations, although an integrated approach of surgery and medical treatment should also be considered in this setting.
Topics: Female; Humans; Genital Neoplasms, Female; Adenosarcoma; Neoplasm Recurrence, Local; Uterine Neoplasms; Hormones
PubMed: 37202293
DOI: 10.1016/j.bulcan.2023.04.017 -
Gynecologic Oncology Dec 2014Uterine adenosarcoma is a rare malignancy with little data on optimal management. We aimed to clarify the impact of adjuvant therapy in patients with uterine...
OBJECTIVES
Uterine adenosarcoma is a rare malignancy with little data on optimal management. We aimed to clarify the impact of adjuvant therapy in patients with uterine adenosarcoma and identify risk factors for recurrence and death.
METHODS
We performed a retrospective review of patients undergoing primary evaluation and treatment for uterine adenosarcoma at a single institution from July 1982 through December 2011. Univariate and multivariate analyses were used to identify prognostic factors for progression-free survival (PFS) and overall survival (OS).
RESULTS
We identified 100 patients with uterine adenosarcoma, and 74 patients met the inclusion criteria. On multivariate analysis, sarcomatous overgrowth (SO) and lymphovascular space invasion (LVSI) were predictors of worse PFS and OS. Median PFS and OS were 29.4 and 55.4 months for patients with SO, compared to 105.9 and 112.4 months for patients without SO (PFS HR 2.58, 95% CI 1.37-4.84, p=0.003; OS HR 2.45, 95% CI 1.26-4.76, p=0.008). Among patients with stage I disease, 17 of 22 patients (77%) with SO and 8 of 37 patients (22%) without SO had a recurrence (p<0.001). Among patients with stage I disease with SO, adjuvant therapy appeared to be associated with longer PFS and OS, but these differences were not statistically significant (PFS, 46.7 vs. 29.4 months, p=0.28; OS, 97.3 vs. 55.4 months, p=0.18).
CONCLUSION
In patients with uterine adenosarcoma, the presence of SO or LVSI confers a higher risk of recurrence. We did not identify an optimal treatment strategy for patients with SO, but adjuvant therapy may be associated with prolonged PFS.
Topics: Adenosarcoma; Adolescent; Adult; Aged; Aged, 80 and over; Cohort Studies; Disease-Free Survival; Humans; Middle Aged; Neoplasm Recurrence, Local; Prognosis; Retrospective Studies; Risk Factors; Treatment Outcome; Uterine Neoplasms; Young Adult
PubMed: 25449308
DOI: 10.1016/j.ygyno.2014.10.022 -
Obstetrics and Gynecology Sep 2006Adenosarcoma arising in inguinal endometriosis is a rare clinical entity.
BACKGROUND
Adenosarcoma arising in inguinal endometriosis is a rare clinical entity.
CASE
A 47-year-old nulligravida presented with a persistent and enlarged right groin mass. Sixteen years earlier she had undergone total abdominal hysterectomy and bilateral salpingo-oophorectomy for endometriosis and was placed on estrogen therapy. Surgical en bloc excision of the 12 x 4 cm inguinal mass revealed adenosarcoma arising in endometriosis.
CONCLUSION
Although a rare entity, adenosarcoma arising in inguinal endometriosis should be included in the differential diagnosis for those patients with a history of endometriosis and chronic unopposed estrogen therapy who present with a groin mass.
Topics: Adenosarcoma; Diagnosis, Differential; Endometriosis; Female; Groin; Humans; Inguinal Canal; Lymph Nodes; Middle Aged; Round Ligament of Uterus
PubMed: 17018491
DOI: 10.1097/01.AOG.0000192550.19403.0f -
Diagnostic Pathology Jan 2023Uterine adenosarcoma is a rare malignant tumor that accounts for 8% of all uterine sarcomas, and less than 0.2% of all uterine malignancies. However, it is frequently...
BACKGROUND
Uterine adenosarcoma is a rare malignant tumor that accounts for 8% of all uterine sarcomas, and less than 0.2% of all uterine malignancies. However, it is frequently misdiagnosed in clinical examinations, including pathological diagnosis, and imaging studies owing to its rare and non-specific nature, which is further compounded by the lack of specific diagnostic markers.
CASE PRESENTATION
We report a case of uterine adenosarcoma for which a comprehensive genomic profiling (CGP) test provided a chance to reach the proper diagnosis. The patient, a woman in her 60s with a history of uterine leiomyoma was diagnosed with an intra-abdominal mass post presentation with abdominal distention and loss of appetite. She was suspected to have gastrointestinal stromal tumor (GIST); the laparotomically excised mass was found to comprise uniform spindle-shaped cells that grew in bundles with a herringbone architecture, and occasional myxomatous stroma. Immunostaining revealed no specific findings, and the tumor was diagnosed as a spindle cell tumor/suspicious adult fibrosarcoma. The tumor relapsed during postoperative follow-up, and showed size reduction with chemotherapy, prior to regrowth. CGP was performed to identify a possible treatment, which resulted in detection of a JAZF1-BCORL1 rearrangement. Since the rearrangement has been reported in uterine sarcomas, we reevaluated specimens of the preceding uterine leiomyoma, which revealed the presence of adenosarcoma components in the corpus uteri. Furthermore, both the uterine adenosarcoma and intra-abdominal mass were partially positive for CD10 and BCOR staining.
CONCLUSION
These results led to the conclusive identification of the abdominal tumor as a metastasis of the uterine adenosarcoma. The JAZF1-BCORL1 rearrangement is predominantly associated with uterine stromal sarcomas; thus far, ours is the second report of the same in an adenosarcoma. Adenosarcomas are rare and difficult to diagnose, especially in atypical cases with scarce glandular epithelial components. Identification of rearrangements involving BCOR or BCORL1, will encourage BCOR staining analysis, thereby potentially resulting in better diagnostic outcomes. Given that platinum-based chemotherapy was proposed as the treatment choice for this patient post diagnosis with adenosarcoma, CGP also indirectly contributed to the designing of the best-suited treatment protocol.
Topics: Female; Humans; Adenosarcoma; Co-Repressor Proteins; Diagnosis, Differential; DNA-Binding Proteins; Genomics; Leiomyoma; Repressor Proteins; Uterine Neoplasms; Aged
PubMed: 36639698
DOI: 10.1186/s13000-022-01279-4 -
International Journal of Surgical... Oct 2011
Topics: Adenosarcoma; Female; Humans; Intestinal Neoplasms; Middle Aged; Uterine Neoplasms
PubMed: 21791483
DOI: 10.1177/1066896911415670 -
BMC Women's Health Dec 2021Adenosarcoma is classified as a mixed epithelial and mesenchymal tumor composed of a benign epithelial component and a malignant stromal component. The stromal component...
BACKGROUND
Adenosarcoma is classified as a mixed epithelial and mesenchymal tumor composed of a benign epithelial component and a malignant stromal component. The stromal component in adenosarcoma is usually low grade, and consequently the prognosis is relatively favorable. While, adenosarcoma with sarcomatous overgrowth (SO) is defined as an adenosarcoma in which the sarcomatous component constitutes more than 25% of the tumor. The stromal component is also high-grade sarcoma showing greater nuclear pleomorphism and mitotic activity, thus, it is associated with worse prognosis. MRI findings of adenosarcoma without SO have been described in previous literatures but the imaging findings in adenosarcoma with SO may be poorly defined. Therefore we present two cases of uterine adenosarcoma with SO.
CASE PRESENTATION
Patient 1 was a 76-year-old woman referred to our hospital with complaint of abdominal distension and postmenopausal bleeding. Patient 2 was a 57-year-old woman with complaint of lower abdominal pain and abnormal uterine bleeding. On magnetic resonance imaging (MRI), T2 weighted imaging showed a large, heterogeneous high-intensity mass with hyperintense tiny cysts that expanded the uterine cavity and extended into the cervical canal for both patients. On diffusion-weighted imaging (DWI), both masses appeared as high signal intensity. Patient 2 also had a right ovarian adult granulosa cell tumor that may have contributed to development of the adenosarcoma. Patient 1 recurred with peritoneal sarcomatosis 6 months after surgery and died of the disease. Patient 2 also recurred with a left upper lung metastasis 3 months after surgery.
CONCLUSIONS
DWI may depict pathological changes produced by SO of adenosarcoma as high signal intensity, even though SO does not seem to change MRI findings of adenosarcoma on other sequences. Therefore, DWI could potentially predict SO in presumptive adenosarcoma on MRI and the patient's prognosis. It is also important for pathologists to know if SO can arise in adenosarcoma because they need to examine the tumor thoroughly to determine the percentage of SO component in the tumor volume when SO is present.
Topics: Adenosarcoma; Aged; Cervix Uteri; Female; Humans; Middle Aged; Neoplasm Recurrence, Local; Uterine Neoplasms
PubMed: 34915866
DOI: 10.1186/s12905-021-01567-z -
Gynecologic Oncology Reports Jun 2024•Non-puerperal uterine inversion can be associated with uterine sarcomas.•Adenosarcoma is a tumor composed of benign epithelium and malignant stroma.•If malignancy...
•Non-puerperal uterine inversion can be associated with uterine sarcomas.•Adenosarcoma is a tumor composed of benign epithelium and malignant stroma.•If malignancy is suspected or confirmed treatment of uterine inversion with hysterectomy is advised.
PubMed: 38681981
DOI: 10.1016/j.gore.2024.101398 -
Balkan Medical Journal Mar 2014Extragenital Müllerian adenosarcomas are extremely rare tumours characterised by a stromal component of low-grade malignancy and by a benign glandular epithelial...
BACKGROUND
Extragenital Müllerian adenosarcomas are extremely rare tumours characterised by a stromal component of low-grade malignancy and by a benign glandular epithelial component.
CASE REPORT
A 26-year-old woman was admitted to our clinic because of lower abdominal distension and left lower quadrant pain. Clinical and radiological examinations suggested an ovarian malignancy. Laparotomy revealed a cystic mass in the pouch of Douglas, originating from the left sacrouterine ligament. A total excision of the tumour was performed and showed low-grade adenosarcoma without sarcomatous overgrowth. Follow-up at 24 months after the surgery showed no evidence of recurrence.
CONCLUSION
Mullerian adenosarcoma located in the pouch of Douglas is rare. For treatment, success may be achieved with only excision of the tumour if there is no sarcomatous overgrowth or spread to adjacent tissues.
PubMed: 25207177
DOI: 10.5152/balkanmedj.2013.9065 -
Pathologica Dec 2020Mullerian adenosarcoma is an uncommon biphasic malignant uterine tumor. It is composed of benign epithelial and malignant stromal elements. We present a case of a...
Mullerian adenosarcoma is an uncommon biphasic malignant uterine tumor. It is composed of benign epithelial and malignant stromal elements. We present a case of a 45-year-old woman who presented with post-menopausal bleeding for three months. She had a significant past medical history of pelvic irradiation for squamous carcinoma of cervix 20 years ago. Pathology revealed adenosarcoma with sarcomatous overgrowth. The patient had a recurrence of pure sarcoma three months later and unfortunately succumbed to her disease. The role of radiation in the pathogenesis of adenosarcoma has been uncommonly described compared to its well established role in the development of carcinosarcoma. Our case fulfils the criteria for a radiation induced sarcoma. We review the salient clinical and pathological features of this uncommon lesion highlighting the importance of sarcomatous overgrowth in these lesions and the possible role of radiation in the development of these tumors.
Topics: Adenosarcoma; Cervix Uteri; Female; Humans; Middle Aged; Mixed Tumor, Mullerian; Neoplasms, Radiation-Induced; Radiotherapy; Uterine Cervical Neoplasms; Uterine Neoplasms
PubMed: 33393526
DOI: 10.32074/1591-951X-114 -
Gynecologic Oncology Jun 2001Mesodermal (müllerian) adenosarcoma arising from the peritoneum is rare and is thought to arise from pluripotent mesothelial and mesenchymal cells of the pelvic cavity... (Review)
Review
BACKGROUND
Mesodermal (müllerian) adenosarcoma arising from the peritoneum is rare and is thought to arise from pluripotent mesothelial and mesenchymal cells of the pelvic cavity or from endometriotic deposits.
CASE
A case of primary peritoneal mesodermal adenosarcoma arising from the omentum is described. A 50-year-old woman presented with sudden abdominal distension. Initial laparotomy revealed a 13-kg mass arising from the omentum, which was determined from frozen and paraffin sections to be serous cystadenofibroma. The tumor recurred within 10 months and weighed 18 kg at a second laparotomy. Histopathology and review of the original tumor established the correct diagnosis of mesodermal adenosarcoma. The patient died from disseminated disease 6 months later.
CONCLUSION
Adenosarcomas are difficult to differentiate from adenofibromas or endometriosis histologically because of the presence of large areas of low cellularity and infrequent mitotic figures. In such cases, stromal nuclear atypia and periglandular stromal cuffing are features that are diagnostic of adenosarcoma.
Topics: Adenosarcoma; Fatal Outcome; Female; Humans; Middle Aged; Peritoneal Neoplasms
PubMed: 11371146
DOI: 10.1006/gyno.2001.6165