Mr imaging of common and uncommon large pelvic masses. Mullerian adenosarcoma with sarcomatous overgrowth of the. 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. Activity of pegylated liposomal doxorubicin for extragenital mullerian adenosarcoma with sarcomatous overgrowth.
It plays an important role if the adenosarcoma shows a sarcomatous overgrowth or not. Uterine sarcomas are rare neoplasms, comprising only 5% of uterine malignancies. The best treatment and oncologic outcome have not been clarified because only a few cases of extrauterine and extraovarian adenosarcoma have. Systemic therapy in advanced uterine adenosarcoma with. In the recurrence group, the percentage of sarcomatous overgrowth presentation was increased in all articles. An aggressive variant of adenosarcoma, mullerian adenosarcoma with sarcomatous overgrowth maso in the cervix is extremely rare. Perivascular epithelioid cell tumors pecomas have received increased attention in recent years as a result of the frequency. Herein, wereported the case of a young patient with advanced uterine asso. We present a fourth maso case derived from uterine cervix in a 72yearold woman with metrorrhagia and a polypoid mass protruding through the cervical ostium. In this report, we describe a case of adenosarcoma of the uterine corpus with overgrown and poorly differentiated sarcoma. Mullerian adenosarcoma with sarcomatous overgrowth was first described in 1989 and suggests that the cluster of adenosarcomas reported herein may be due in part to the current classification of some uterine tumors as adenosarcoma with sarcomatous overgrowth that previously would have been classified as other types of uterine sarcoma. However, extraendometrial uterine adenosarcoma, that is, tumor that has not originated in the eutopic endometrium but in adenomyosis or an adenomyoma, is rare,6,11 and no specific staging guidelines for such tumors are provided in the new figo staging system for uterine sarcomas. Uterine adenosarcoma in a patient with history of breast. Mullerian adenosarcoma with sarcomatous overgrowth maso is a rare variant of uterine sarcomas, associated with postoperative recurrence, metastases and a fatal outcome.
Uterine adenosarcoma is an extremely rare subtype of uterine sarcoma, which represents only 5. The prognosis of uterine adenosarcoma depends on the stage and if sarcomatous overgrowth is present. Mullerian adenosarcoma heterologous of the cervix with sarcomatous overgrowth. Mullerian adenosarcoma with sarcomatous overgrowth in the uterine cervix. The difference between ms and maso is the pure high grade sarcoma features in maso. Most presentations are postmenopausal, and with early stage disease. Uterine adenosarcoma ua is an extremely rare sarcoma subtype. Uterine adenosarcoma with sarcomatous overgrowth asso is a variant form of as. The diagnosis of adenosarcoma and sarcomatous overgrowth was based on the criteria adopted by the world health. Mullerian adenosarcoma with sarcomatous overgrowth maso is a rare variant of uterine adenosarcoma, where the pure sarcoma is similar to. Management of uterine adenosarcomas with and without sarcomatous overgrowth. Mullerian adenosarcoma of the uterine cervix with sarcomatous. Tissue with sarcomatous overgrowth can be detected by immunohistochemical analysis, while the differentiation between adenosarcomas without sarcoma tous overgrowth.
The immune microenvironment of uterine adenosarcomas. Uterine mullerian adenosarcomas are rare malignancies which account for 5% of uterine sarcomas. Sarcomatous overgrowth is defined as the presence of pure sarcoma occupying at least 25% of the tumor, usually high grade in nature and without a benign glandular component 4. Adenosarcoma of the uterus genetic and rare diseases. Mullerian adenosarcoma of uterus with sarcomatous overgrowth and heterologous component associated with stromal deposit in omentum. Socalled adenofibroma has the architectural features of adenosarcoma, but the stroma is typically hypocellular and fibrotic. Pdf mullerian adenosarcoma with sarcomatous overgrowth of. This tumor is composed of a malignant mesenchymal component and a benign epithelial component 4, 5. Uterine adenosarcoma with sarcomatous overgrowth, however, has a malignant potential more akin to that of highgrade sarcoma 2, 3. This sarcoma was figo stage 1a, with no myometrial invasion, sarcomatous overgrowth or lymphovascular invasion. Dec 28, 2017 uterine adenosarcoma is an extremely rare subtype of uterine sarcoma, which represents only 5. Clement and scully provided the first description of uterine adenosarcoma in 1974.
Systemic therapy in advanced uterine adenosarcoma with sarcomatous overgrowth systemic therapy in advanced uterine adenosarcoma with sarcomatous overgrowth schroeder, brett. Treatment of recurrent or metastatic uterine adenosarcoma hindawi. Uterine adenosarcoma ua with sarcomatous overgrowth asso is a rare and aggressive disease. This report highlights these entities diagnostic features, namely having a neoplastic benign or mildly atypical epithelial component, and sarcomatous often lowgrade stromal component. Uterine adenosarcoma is considered to be a less aggressive disease than carcinosarcoma because its malignant component is usually low grade, and often a hysterectomy is curative. Among these patients, we provided a detailed description of the medical history of a 49yearold woman with advanced uterine adenosarcoma with sarcomatous overgrowth who presented a brilliant radiologic and pathologic response after 3 cycles of epirubicin and ifosfamide, ultimately achieving an extraordinary longterm outcome through an. Mullerian adenosarcoma with sarcomatous overgrowth maso is a very. Uterine adenosarcoma with sarcomatous overgrowth asso is a rare variant of uterine sarcoma. Feasibility of uterine preservation in the management of. Uterine mullerian adenosarcoma with sarcomatous overgrowth. Uterine adenosarcoma with sarcomatous overgrowth asso is a variant form of as and exhibits aggressive growth of tumor and the prognosis is relatively poor compared with typical as. Mullerian adenosarcoma heterologous of the cervix with. Click for larger image download as powerpoint slide.
Mullerian adenosarcoma is a rare malignancy composed of benign epithelial and malignant stromal components and usually arises from the uterus. Advanced uterine adenosarcoma with sarcomatous overgrowth in. Endometrial polyps, adenofibroma, and adenomyoma are tumors in which both the epithelial and stromal components are morphologically benign. Mullerian adenosarcoma heterologous of uterine cervix with.
These tumors are broadly classified into three groups by composition. A 37yearold nullipara presented with irregular vaginal bleeding, a normal pelvic examination, and an initially negative ultrasound. Myometrial invasion is seen in 15% of cases and is mainly restricted to the inner layer. There has been limited evaluation of the immune microenvironment in these tumors. Uterine adenosarcoma ua with sarcomatous overgrowth asso is a rare and aggressive d. Oct 21, 2016 it plays an important role if the adenosarcoma shows a sarcomatous overgrowth or not. Uterine adenosarcoma with sarcomatous overgrowth versus.
The term mullerian adenosarcoma with sarcomatous overgrowth maso was first used by clemant and scully4 in 1989 for those tumours in which a pure sarcoma, similar to or of a higher grade than that of the underlying adenosarcoma accounts for atleast onequarter of the tumour after thorough histological sampling. Management of uterine adenosarcomas with and without. Kaplan meier curves for progressionfree survival in patients with uterine adenosarcoma with and without sarcomatous overgrowth. Mullerian adenosarcoma is a rare mixed tumor of low malignant potential that shows an intimate combination of benign glandular epithelium and lowgrade sarcoma, usually of the endometrial stromal type. Lowgrade endometrial stromal sarcoma and uterine adenosarcoma. Differential diagnosis of adenosarcoma without sarcomatous overgrowth. Uterine mullerian adenosarcoma with sarcomatous overgrowth fatal. Adenosarcoma of the uterus is a rare tumor of the uterus that typically originates in the lining of the uterus endometrium.
Adenosarcoma represents 8% of all uterine sarcomas. These behaviours manifest according to the tumours histological characteristics and sites of origin. Typical symptoms in affected individuals may include abnormal vaginal bleeding, an enlarged uterus, and tissue protruding from the external os external. Cervical mullerian adenosarcoma with heterologous sarcomatous. Uterine adenosarcoma as are rare tumors and have more favorable outcomes than the aggressive uterine carcinosarcomas. Mullerian adenosarcoma with sarcomatous overgrowth maso is a very rare variant of uterine sarcomas first described by.
Adenosarcoma is frequently associated with a good outcome if confined to the endometrium. The endocervical biopsy showed undifferentiated carcinoma, and a cytologic examination at that time revealed the presence of cell types ranging from dispersed spindle to round. Mullerian adenosarcoma with sarcomatous overgrowth maso is a rare variant of uterine sarcomas, associated with postoperative recurrence. Uterine adenosarcoma without sarcomatous overgrowth demonstrates a relatively indolent disease course that can be managed with surgery alone. Cd10 and wilms tumour1 positive, with strong immunoreactivity for p53 and ki67 seen in cases with sarcomatous overgrowth, where cd10 and er positivity is often lost. Feb 01, 2014 systemic therapy in advanced uterine adenosarcoma with sarcomatous overgrowth systemic therapy in advanced uterine adenosarcoma with sarcomatous overgrowth schroeder, brett. Mullerian adenosarcoma of the uterus with sarcomatous overgrowth. Clinicopathologic features of mullerian adenosarcoma with sarcomatous overgrowth cases reported in english literature park et al. The relationship of sarcomatous overgrowth in patients with uterine adenosarcoma to poor prognosis was identified by past articles 2, 4, 5, 9, table 5. A case of solitary brain metastasis from uterine mullerian adenosarcoma with sarcomatous overgrowth suk bo hong, 1 min jung kim, 1 ji yeon kwon, 1 seok jin choi, 2 eun young kim, 3 and joohan lim 1 1 department of internal medicine, inha university college of medicine, incheon, korea 2 department of pathology, inha university college of medicine, incheon, korea. Adenosarcoma with sarcomatous overgrowth listed as asso. The objective of this study is to examine and describe the immune infiltrate and pd1pdl1 expression in ua and to correlate these changes in the tumor microenvironment with the overall survival status or the diseasefree survival status dfss.
We present a fourth maso case derived from uterine cervix in a. The presence of heterologous sarcomatous components is associated with aggressive biological behavior. Apr 25, 2018 adenosarcoma of uterine cervix is a rare, lowgrade cancer commonly affecting middleaged women following menopause. The endocervical biopsy showed undifferentiated carcinoma, and a cytologic examination at that time revealed the presence of cell types ranging from dispersed spindle to round atypical cells. Uterine adenosarcoma as are rare tumors and have more favorable outcomes. Uterine adenosarcoma with sarcomatous overgrowth versus uterine. The tumor is classified as an adenosarcoma with sarcomatous overgrowth when at least 25% of the tumor is composed of pure sarcoma. The patient has no evidence of disease, and she is doing well 18 months after the surgery. The most important negative prognostic factors are the location of the lesion, the degree of myometrial invasion and sarcomatous overgrowth. These factors can be used to accurately prognosticate for uterine adenosarcoma patients.
Mullerian adenosarcoma of ovary is generally similar microscopically to uterine adenosarcoma except a few differences like sarcomatous overgrowth is seen in 30% of ovarian cases as compared to 8% in uterine adenosarcoma and scle sex cord like elements were seen in 15% of ovarian cases as compared to 7% in their uterine counterparts. The most common presenting symptom is abnormal vaginal bleeding, and the mean age at presentation is 58 years. This type of tumor is characterized by both benign noncancerous and malignant components lowgrade sarcoma. Mullerian adenosarcoma with sarcomatous overgrowth maso is a rare variant of uterine adenosarcomas first described in 1989 and usually occurs in the sixth decade of life. Using adenosarcoma of the uterus or uterine adenosarcoma as your search term should help you locate articles. This study evaluated the clinicopathologic features of japanese patients with uterine adenosarcoma and their potential treatment challenges. Treatment of recurrent or metastatic uterine adenosarcoma. Oct 01, 2001 read uterine adenosarcoma with sarcomatous overgrowth versus uterine carcinosarcoma. Uterine adenosarcoma is a rare malignancy with limited cohort data in asian countries.
A comparison of clinical manifestations and outcomes. Submucous uterine adenosarcomaminimally invasive treatment. Sep 25, 2007 mullerian adenosarcoma with sarcomatous overgrowth maso is a rare variant of uterine sarcomas, associated with postoperative recurrence, metastases and a fatal outcome. Higher stromal grade or sarcomatous overgrowth worsen the prognosis, whereas an association with endometriosis is protective. We report a rare case of uterine adenosarcoma with low gradeovergrowth and smooth muscle. This variant contains obvious, highgrade sarcoma in addition to a lowgrade form. Compared to uterine adenosarcoma, an extrauterine location confers a higher mortality and recurrence risk. A case of solitary brain metastasis from uterine mullerian. Cervical mullerian adenosarcomas with sarcomatous overgrowth are rare. Pdf uterine sarcomas are relatively rare tumors that account for approximately % of female genital. Oct 12, 2012 adenosarcoma of the uterus is a rare tumor of the uterus that typically originates in the lining of the uterus endometrium. Cluster of uterine mullerian adenosarcoma in the washington. Yanyan zhang, yan li, huifang huang, jiaxin yang, ming wu.
Uterine adenosarcoma with low grade overgrowth and smooth. Largest single institution series of uterine adenosarcoma patients which showed that the presence of sarcomatous overgrowth was associated with a higer risk of recurrence. Read uterine adenosarcoma with sarcomatous overgrowth versus uterine carcinosarcoma. Jun 11, 2011 mullerian adenosarcoma with sarcomatous overgrowth maso is a very aggressive variant, associated with postoperative recurrence, metastases, even when diagnosed in early stage. Uterine adenosarcoma with sarcomatous overgrowth vs. The patient was an 80yearold japanese woman with abnormal genital bleeding. Extrauterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. Uterine sarcomas generally account for less than 4% of all uterine malignancies with the subtype adenosarcoma comprising only 8% of cases, reflecting the rarity of this entity. Gallardo reported that sarcomatous overgrowth was found in 33 % of uterine adenosarcomas.
The presence of sarcomatous overgrowth seems to be. An aggressive variant of adenosarcoma, mullerian adenosarcoma with sarcomatous overgrowth maso is extremely rare. A sarcomatous overgrowth is diagnosed when more than 25 % of the tumour volume is sarcomatous tissue. Uterine adenosarcomas have the highest incidence in perimenopasual and postmenopausal women with a mean age of 50 years, but some incidence among children. Mullerian adenosarcoma with sarcomatous overgrowth maso is a very rare variant of uterine sarcomas first described by clement et al. Mullerian adenosarcoma of uterus with sarcomatous overgrowth. Advanced uterine adenosarcoma with sarcomatous overgrowth. Comparison of treatment and survival, gynecologic oncology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. The best treatment and oncologic outcome have not been clarified because only a few cases of extrauterine and extraovarian adenosarcoma have been. Survival is better compared to other types of uterine sarcomas. Mullerian adenosarcoma with sarcomatous overgrowth maso is a very aggressive variant, associated with postoperative recurrence, metastases, even when diagnosed in early stage.
Uterine adenosarcoma with sarcomatous overgrowth asso is a rare variant of uterine sarcoma first described in 1989. A singlecentre experience on the management of adenosarcoma. This clinicopathologic study was undertaken to compare the treatment and survival of uterine adenosarcoma with sarcomatous overgrowth to that of uterine carcinosarcomas. Uterine mullerian adenosarcoma with sarcomatous overgrowth 2007. It is a malignant tumor that contains benign epithelial components and sarcomatous stroma. Mullerian adenosarcoma of the uterine corpus associated with tamoxifen therapy. Uterine adenosarcomas overgrown by sexcordlike tumour.
Comparison of treatment and survival, gynecologic oncology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at. From the viewpoint of tumour pathogenesis, the massive utrosct component present in our cases should not be considered a phenomenon of dedifferentiation as it occurs in adenosarcomas with sarcomatous overgrowth, but rather as a benign, secondary differentiation associated to the lgess component of adenosarcoma. Uterine adenosarcoma is an extremely rare subtype of uterine sarcoma. Apr 25, 2018 if 25% of the adenosarcoma has sarcomatous features which is highgrade malignancy, then it is called uterine adenosarcoma with sarcomatous overgrowth if the smooth muscle walls myometrium are involved, then sarcomatous overgrowth may be seen. Otherwise deep myometrial invasion, high grade cytomorphology and sarcomatous overgrowth predict a poor prognosis. However, among our 7 cases of adenosarcoma, we have provided a detailed description of the medical history of a 49yearold female patient with advanced uterine adenosarcoma with sarcomatous overgrowth, who presented a brilliant radiologic and pathologic response after 3 cycles of epirubicin and ifosfamide, achieving an extraordinary longterm. A 29yearold woman with the diagnoses of endometrial polyp and adenomyosis underwent hysteroscopic endometrial polypectomy for the giant endometrial polyp. Uterine adenosarcoma with sarcomatous overgrowth versus uterine carcinosarcoma. Mullerian adenosarcoma with sarcomatous overgrowth maso is a very aggressive variant, associated with. Six out of 29 patients with adenosarcoma developed metastasis. Embryonal rhabdomyosarcoma of the uterine cervix is an uncommon presentation of the most common softtissue sarcoma in the first decades of.
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