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Oncology Research and Treatment 2018Uterine adenosarcoma is a rare malignancy. It is defined as a biphasic tumor composed of both sarcomatous stroma and benign epithelium. While the sarcomatous component... (Review)
Review
Uterine adenosarcoma is a rare malignancy. It is defined as a biphasic tumor composed of both sarcomatous stroma and benign epithelium. While the sarcomatous component usually is a low-grade homologous uterine sarcoma, the epithelium most often consists of endometrium-like cells. If the sarcomatous part occupies more than 25% of the tumor volume, the situation is referred to as sarcomatous overgrowth - accounting for about 10% of cases. While adenosarcoma usually may be considered a tumor of low malignant potential, the sarcomatous overgrowth most often presents as high-grade sarcoma and is associated with aggressive clinical behavior. Adenosarcomas stage I without sarcomatous overgrowth have a rather good prognosis, with a 5-year overall survival up to 80%. For treatment, complete surgical removal is advocated. Adjuvant chemotherapy and radiotherapy are not defined. Recurrences should again be treated surgically, attempting to achieve complete tumor resection. While the optimum medical treatment for relapsed and metastasized adenosarcomas has yet to be found, chemotherapy and endocrine therapy are potential options.
Topics: Adenosarcoma; Chemotherapy, Adjuvant; Female; Humans; Hysterectomy; Neoplasm Recurrence, Local; Palliative Care; Radiotherapy, Adjuvant; Uterine Neoplasms; Uterus
PubMed: 30326467
DOI: 10.1159/000494067 -
Current Oncology Reports Nov 2016Adenosarcomas are rare malignancies of the female genital tract, accounting for approximately 5 % of uterine sarcomas. Occasionally, adenosarcoma occurs in the ovaries... (Review)
Review
Adenosarcomas are rare malignancies of the female genital tract, accounting for approximately 5 % of uterine sarcomas. Occasionally, adenosarcoma occurs in the ovaries or in extra-uterine tissue, which may be related to endometriosis. These tumors are characterized by benign epithelial elements and a malignant mesenchymal component. Pathologic diagnosis is dependent on the identification of the characteristic morphologic features. The most common immunohistochemical markers for adenosarcoma are CD10 and WT1, but these are not specific. The most frequent presenting symptom is abnormal uterine bleeding. The majority of patients present with stage I disease, with a 5-year overall survival of 60 to 80 %. Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth, lymphovascular invasion, necrosis, and the presence of heterologous elements including rhabdomyoblastic differentiation. Patients with sarcomatous overgrowth have significantly increased risk of recurrence 23 versus 77 % and decreased 5-year overall survival 50 to 60 %. Standard of care treatment is total hysterectomy with bilateral salpingo-oophorectomy without lymphadenectomy, as the incidence of lymph node metastasis is rare. Retrospective data does not support the use of adjuvant pelvic radiotherapy in uterine adenosarcomas as no survival benefit is seen. Insufficient data exists to recommend routinely neoadjuvant or adjuvant chemotherapy for uterine adenosarcomas. Limited evidence exists for the role of hormonal therapy in uterine adenosarcomas. The PIK3/AKT/PTEN pathway is mutated in ∼70 % of adenosarcomas, and this may represent a possible therapeutic target. This article reviews the current state of knowledge concerning uterine adenosarcoma and discusses the management of this rare tumor.
Topics: Adenosarcoma; Female; Humans; Prognosis; Uterine Neoplasms
PubMed: 27718181
DOI: 10.1007/s11912-016-0552-7 -
Expert Review of Anticancer Therapy Nov 2018Uterine adenosarcoma is a rare tumor with both epithelial and stromal components. Standard treatment is total abdominal hysterectomy and bilateral salpingo-oophorectomy.... (Review)
Review
Uterine adenosarcoma is a rare tumor with both epithelial and stromal components. Standard treatment is total abdominal hysterectomy and bilateral salpingo-oophorectomy. There is no defined role for adjuvant or neoadjuvant chemotherapy or radiation. There is a misconception that this is an indolent, low-grade sarcoma. In fact, at least 50% of patients will develop disease recurrence. Establishing prognostic factors is of paramount importance. Areas covered: This article reviews the current literature regarding adenosarcoma prognostic factors from case reports, case series, and retrospective series. An extensive review of the literature was undertaken via PubMed and Medline searches, relevant articles are included in this review. Expert commentary: The most important prognostic factors of uterine adenosarcoma are age, presence of sarcomatous overgrowth, presence of myometrial invasion, presence of lymphovascular invasion, and lymph node involvement. These factors can be used to accurately prognosticate for uterine adenosarcoma patients. Patients at low risk of disease recurrence can be identified. These patients require observation alone. Patients at high risk of disease recurrence can be identified and are candidates for aggressive therapy with adjuvant chemotherapy to reduce the risk of disease recurrence.
Topics: Adenosarcoma; Age Factors; Chemotherapy, Adjuvant; Female; Humans; Hysterectomy; Neoplasm Recurrence, Local; Prognosis; Risk Factors; Salpingo-oophorectomy; Uterine Neoplasms
PubMed: 30169984
DOI: 10.1080/14737140.2018.1518136 -
International Journal of Gynaecology... Oct 2018Uterine sarcomas account for approximately 3%-7% of all uterine cancers. Since carcinosarcomas are currently classified as metaplastic carcinomas, leiomyosarcomas remain...
Uterine sarcomas account for approximately 3%-7% of all uterine cancers. Since carcinosarcomas are currently classified as metaplastic carcinomas, leiomyosarcomas remain the most common subtype. Exclusion of several histologic variants of leiomyoma, as well as atypical smooth muscle tumors (so-called "smooth muscle tumors of uncertain malignant potential"), has highlighted that the vast majority of leiomyosarcomas are high-grade tumors associated with poor prognosis even when apparently confined to the uterus. Low-grade endometrial stromal sarcomas are indolent tumors associated with long-term survival. High-grade endometrial stromal sarcomas and undifferentiated endometrial sarcomas behave more aggressively than tumors showing nuclear uniformity. Adenosarcomas have a favorable prognosis except for tumors showing myometrial invasion or sarcomatous overgrowth. The prognosis for carcinosarcomas (which are considered here in a postscript fashion) is usually worse than that for grade 3 endometrial carcinomas. Tumor stage is the single most important prognostic factor for uterine sarcomas.
Topics: Adenosarcoma; Carcinosarcoma; Female; Humans; Leiomyoma; Leiomyosarcoma; Neoplasm Grading; Neoplasm Staging; Prognosis; Sarcoma; Sarcoma, Endometrial Stromal; Smooth Muscle Tumor; Uterine Neoplasms
PubMed: 30306577
DOI: 10.1002/ijgo.12613 -
Archives of Pathology & Laboratory... Mar 2016Müllerian adenosarcoma is an uncommon biphasic tumor composed of malignant stromal and benign epithelial components. Morphologically, adenosarcoma is characterized by a...
Müllerian adenosarcoma is an uncommon biphasic tumor composed of malignant stromal and benign epithelial components. Morphologically, adenosarcoma is characterized by a broad leaflike architecture, reminiscent of phyllodes tumors of the breast. Periglandular cuffing of the stromal cells around the compressed or cystically dilated glands is characteristic. The mesenchymal component is typically a low-grade spindle cell sarcoma, whereas the epithelial counterpart is commonly endometrioid with frequent squamous or mucinous metaplasia and may, in some circumstances, show mild to moderate atypia. In all cases, it is important to assess for the presence of sarcomatous overgrowth and myometrial invasion, which are the prognostic factors. In this brief review, we present the clinical, histopathologic, and immunohistochemical features of adenosarcoma, as well as updates on the molecular biology of this neoplasm.
Topics: Adenosarcoma; DNA Helicases; Diagnosis, Differential; Female; Gene Amplification; Humans; Immunohistochemistry; Mixed Tumor, Mullerian; Molecular Diagnostic Techniques; Mutation; Neoplasm Invasiveness; Neoplasm Proteins; Neoplasm Staging; Nuclear Proteins; Practice Guidelines as Topic; Prognosis; Proto-Oncogene Proteins; Stromal Cells; Trans-Activators; Uterine Neoplasms; Uterus; X-linked Nuclear Protein
PubMed: 26927725
DOI: 10.5858/arpa.2014-0523-RS -
European Journal of Gynaecological... 2014Mullerian adenosarcoma usually originates in the endometrium and grows as a polypoid mass in post-menopausal women presenting as abnormal vaginal bleeding. This report... (Review)
Review
OBJECTIVE
Mullerian adenosarcoma usually originates in the endometrium and grows as a polypoid mass in post-menopausal women presenting as abnormal vaginal bleeding. This report reviewed Miillerian adenosarcoma cases to clarify the clinical and pathologic characteristics.
MATERIALS AND METHODS
Fifteen cases ofMiillerian adenosarcoma in two medical centers covering a 15-year period were reviewed. Their clinical characteristics, pathologic findings, treatment, and outcomes were compared.
RESULTS
Of the 15 cases, three originated from the endometrium, six arose from uterine adenomyosis, three from the adnexa, and three from the cervix. There was only one post-menopausal case. One case was of breast cancer with tamoxifen (TMX) therapy. There were four Miillerian adenosarcoma with sarcomatous overgrowth (MASO) cases, three of which died within one year after surgery. Only the focal MASO case survived.
CONCLUSION
The rare variant of MASO is very aggressive and associated with poor prognosis.
Topics: Adenosarcoma; Adult; Aged; Aged, 80 and over; Female; Humans; Middle Aged; Neoplasm Staging; Uterine Neoplasms
PubMed: 25556263
DOI: No ID Found -
Revista Colombiana de Obstetricia Y... Jun 2019To present the case of a patient diagnosed with high grade adenosarcoma of the endocervix and the endometrial cavity, with a heterologous component, and to conduct a... (Review)
Review
OBJECTIVE
To present the case of a patient diagnosed with high grade adenosarcoma of the endocervix and the endometrial cavity, with a heterologous component, and to conduct a review of the literature focusing on the diagnosis and therapeutic management of this disease condition.
MATERIALS AND METHODS
We present the case of a 31-year-old female patient who came to Virgen Macarena University Hospital of Seville, a Level III regional institution, complaining of genital bleeding arising from an endocervical polypoid mass. The biopsy of the mass revealed a high grade, poorly differentiated leiomyosarcoma of the endocervix. The patient was taken later to total abdominal hysterectomy. The study of the surgical specimen provided the following result: adenosarcoma of the endocervix and endometrial cavity with a heterologous rhabdomyosarcoma component. A search was conducted in the Medline database via Pubmed using the terms "adenosarcoma," "endocervical," "cervix," "uterus," "heterologous." The search in- cluded literature review articles, case reports and clinical case series describing aspects of cervical adenosarcoma and the heterologous rhabdomyosar- coma component, published in English and Spanish since 1974.
RESULTS
Six articles corresponding to literature reviews, case reports or case series in which the most relevant aspects of the diagnosis and treatment of this disease condition are described were retrieved.
CONCLUSIONS
This condition is characterized, on occasions, by rapid and aggresive growth, hence the importance of early diagnosis and optimal treatment based on a combination of surgery, radiation therapy and chemotherapy. However, due to its low prevalence, further studies are needed in order to confirm these data.
Topics: Adenosarcoma; Adult; Endometrial Neoplasms; Female; Humans; Hysterectomy; Neoplasm Grading; Rhabdomyosarcoma; Uterine Cervical Neoplasms
PubMed: 31613077
DOI: 10.18597/rcog.3258 -
International Journal of Gynecological... Jul 2021Müllerian adenosarcoma is an uncommon biphasic malignant tumor most often occurring in the uterine corpus and derived from native surface endometrium. We report a case... (Review)
Review
Müllerian adenosarcoma is an uncommon biphasic malignant tumor most often occurring in the uterine corpus and derived from native surface endometrium. We report a case of intramural uterine adenosarcoma arising in association with adenomyosis, in the absence of tumor involving the surface endometrium. This is an extremely rare phenomenon, with only 8 other published cases of uterine corpus adenosarcoma in the absence of surface endometrial involvement, 5 originating in adenomyosis and 3 in adenomyomas. We review these cases. The current FIGO staging system for uterine adenosarcoma assumes origin from the surface endometrium and does not address the rare occurrence of intramural tumors without a surface endometrial component. Such tumors are problematic to stage and could potentially be overtreated, particularly if there is deep myometrial involvement.
Topics: Adenomyoma; Adenomyosis; Adenosarcoma; Adult; Female; Humans; Hysterectomy; Myometrium; Uterine Neoplasms
PubMed: 32947330
DOI: 10.1097/PGP.0000000000000709 -
Journal of the College of Physicians... Apr 2022Uterine adenosarcoma is a rare tumor composed of benign epithelium in combination with malignant mesenchymal components. Misdiagnosis is common due to the lack of...
Uterine adenosarcoma is a rare tumor composed of benign epithelium in combination with malignant mesenchymal components. Misdiagnosis is common due to the lack of specific clinical manifestations and the rarity of such tumors in young age groups. The mean age at diagnosis is 58.6 years, and <10% of patients are younger than 40 years. Herein, we describe a case of a 19-year girl who presented with worsening menorrhagia. Her ultrasound revealed multiple heterogeneous masses in the uterine cavity. Intraoperatively, a small piece of grayish-white tumor tissue was sent for the frozen section, but the results did not confirm malignancy. She underwent hysteroscopy, laparoscopic hysterectomy, and bilateral salpingo-oophorectomy. Histopathologic examination finally confirmed uterine adenosarcoma. The patient had no evidence of residual tumor or recurrence during six months of follow-up. Key Words: Uterus, Adenosarcoma, Mixed tumor.
Topics: Adenosarcoma; Adolescent; Female; Humans; Hysterectomy; Uterine Neoplasms; Uterus
PubMed: 35633000
DOI: 10.29271/jcpsp.2022.Supp1.S18 -
Arkhiv Patologii 2021Uterine adenosarcoma is an uncommon biphasic tumor with benign epithelial and malignant mesenchymal components, often presenting difficulties for morphological... (Review)
Review
Uterine adenosarcoma is an uncommon biphasic tumor with benign epithelial and malignant mesenchymal components, often presenting difficulties for morphological diagnosis. We describe 5 cases of adenosarcoma of the uterine corpus and cervix, and vaginal stump in patients aged 46-76 years. Clinical data, ultrasound results, morphological data, including immunohistochemical studies with antibodies to CD10, estrogen and progesterone receptors, desmin, smooth muscle actin, and Ki-67 are presented. Large polypoid mass of the epithelial-mesenchymal structure within the uterine cavity in women of peri - and postmenopausal age require the exclusion of malignancy of the mesenchymal component with searchig for diagnostic criteria - periglandular cuffing of the stromal cells and mitoses.
Topics: Adenosarcoma; Aged; Female; Humans; Middle Aged; Polyps; Uterine Neoplasms
PubMed: 33822551
DOI: 10.17116/patol20218302125