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Glenn W Mccluggage - One of the best experts on this subject based on the ideXlab platform.
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significantly greater prevalence of dicer1 alterations in uterine embryonal rhabdomyosarcoma compared to Adenosarcoma
Modern Pathology, 2020Co-Authors: Leanne De Kock, Juyoon Yoon, Maria Apellanizruiz, Dylan Pelletier, Glenn W Mccluggage, Colin J R Stewart, Brendan C DicksonAbstract:Embryonal rhabdomyosarcomas (ERMS) account for 2–3% of cancers in pediatric and adolescent populations. They are rarer in adults. We and others have reported that ERMS arising in the uterine cervix may harbor mutations in the gene encoding the microRNA biogenesis enzyme, DICER1, but a large series of cases has not been published. In the uterus, distinguishing ERMS from Adenosarcoma can be very challenging, even for expert pathologists, and DICER1 alterations have been identified in a variable subset of uterine Adenosarcomas. We hypothesized that DICER1 genetic testing may be useful in distinguishing between ERMS and Adenosarcoma. We conducted a central pathology review-based study of 64 tumors initially thought to be uterine ERMS or Adenosarcoma; 19 neoplasms had a consensus diagnosis of ERMS, 27 of Adenosarcoma and for 18, no consensus diagnosis was reached. The median age at diagnosis was 30 years (range 2.5–69) for ERMS, 57.5 years (range 27–82) for Adenosarcoma, and 65.5 years (range 32–86) for no consensus cases. In our series, the DICER1 mutation prevalence differed between the three groups: DICER1 alterations were present in 18/19 (95%) ERMS, 7/27 (26%) Adenosarcomas (p < 0.001), and 4/18 (22%) no consensus cases. A germline alteration was present in 6/12 ERMS patients tested versus 0/6 Adenosarcoma patients. Thus, although DICER1 mutations are near ubiquitous in uterine ERMS and are significantly less common in uterine Adenosarcoma, DICER1 testing is only of value in distinguishing between the two neoplasms when a DICER1 mutation is absent, as this is helpful in excluding ERMS. On review of the clinical and radiological features of the single DICER1 wild-type cervical ERMS, this was thought most likely to be of vaginal origin. Given the significant prevalence of DICER1 germline pathogenic variants in uterine ERMS, all patients with this diagnosis should be referred to a genetics service.
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juvenile granulosa cell tumor arising in ovarian Adenosarcoma an unusual form of sarcomatous overgrowth
Human Pathology, 2015Co-Authors: Claire Carleton, Oisin P Houghton, Glenn W MccluggageAbstract:Summary We report 2 ovarian neoplasms in women aged 58 and 69 years composed of an admixture of Adenosarcoma and a predominant stromal component morphologically and immunohistochemically in keeping with juvenile granulosa cell tumor. As far as we are aware, this association has not been reported previously. We speculate that, in both cases, the juvenile granulosa cell tumor component arose from the Adenosarcoma as an unusual form of sarcomatous overgrowth of sex cord elements.
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mullerian Adenosarcoma of the female genital tract
Advances in Anatomic Pathology, 2010Co-Authors: Glenn W MccluggageAbstract:Mullerian Adenosarcoma is an uncommon, but not rare, mixed tumor containing a neoplastic but benign or mildly atypical epithelial element and a sarcomatous, usually low-grade, stromal component. The most common site is the uterine corpus but Adenosarcoma also occurs in the cervix and ovary and more
Nancy L Simon - One of the best experts on this subject based on the ideXlab platform.
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ovarian conservation in a woman of reproductive age with mullerian Adenosarcoma
Gynecologic Oncology, 2001Co-Authors: C M Michener, Nancy L SimonAbstract:BACKGROUND: Total abdominal hysterectomy with bilateral salpingo-oophorectomy is generally considered optimal therapy for patients with uterine sarcomas. Local resection of the tumor or hysterectomy with ovarian conservation has been used in only a small number of patients. Recurrence risk in women undergoing ovarian-sparing surgery for mullerian Adenosarcomas can be difficult to evaluate due to the paucity of literature in this area. We present a reproductive-age woman with a mullerian Adenosarcoma and review the literature on conservative surgical management of this class of tumors. CASE: A 25-year-old nulligravida was diagnosed with a uterine Adenosarcoma and the question of conservative surgical therapy arose. Following a literature review, discussion with the patient led to the decision for ovarian preservation at the time of hysterectomy. The pelvis and abdomen were grossly free of metastatic disease at laparotomy and all tumor was confined to the uterus on pathologic examination. She is free of disease 36 months postoperatively and is now considering in vitro fertilization using a surrogate. CONCLUSION: Ovarian conservation can probably be offered safely in carefully selected women of reproductive age with mullerian Adenosarcomas.
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Ovarian conservation in a woman of reproductive age with müllerian Adenosarcoma.
Gynecologic Oncology, 2001Co-Authors: C M Michener, Nancy L SimonAbstract:Abstract Background. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is generally considered optimal therapy for patients with uterine sarcomas. Local resection of the tumor or hysterectomy with ovarian conservation has been used in only a small number of patients. Recurrence risk in women undergoing ovarian-sparing surgery for mullerian Adenosarcomas can be difficult to evaluate due to the paucity of literature in this area. We present a reproductive-age woman with a mullerian Adenosarcoma and review the literature on conservative surgical management of this class of tumors. Case. A 25-year-old nulligravida was diagnosed with a uterine Adenosarcoma and the question of conservative surgical therapy arose. Following a literature review, discussion with the patient led to the decision for ovarian preservation at the time of hysterectomy. The pelvis and abdomen were grossly free of metastatic disease at laparotomy and all tumor was confined to the uterus on pathologic examination. She is free of disease 36 months postoperatively and is now considering in vitro fertilization using a surrogate. Conclusion. Ovarian conservation can probably be offered safely in carefully selected women of reproductive age with mullerian Adenosarcomas.
Frederic Amant - One of the best experts on this subject based on the ideXlab platform.
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cd10 estrogen and progesterone receptor expression in ovarian Adenosarcoma
Gynecologic Oncology, 2005Co-Authors: Tim Van Mieghem, Vera M Abeler, Philippe Moerman, Lieve Verbist, Ignace Vergote, Frederic AmantAbstract:Abstract Background. The low incidence of ovarian Adenosarcomas explains the paucity of available data regarding their biologic markers. Our aim was to investigate the presence of CD10, estrogen and progesterone receptors (ER and PR) in these tumors. Case. Four cases of primary ovarian Adenosarcoma were retrieved in our pathology databases. We assessed the immunohistochemical stainings for ER, PR and CD10 in 3 ovarian Adenosarcomas without sarcomatous overgrowth (OAS), and 1 ovarian Adenosarcoma with sarcomatous overgrowth (OAS-S). When considering the malignant stromal component, 2 OAS showed the presence of ER and PR, whereas the OAS-S was only ER positive. CD10 positivity was observed in the sarcomatous cells of all 3 OAS but was absent in the OAS-S. Conclusion. The identification of a hormone sensitive disease is the main clinical implication of this study. Moreover, the current results add OAS to the list of CD10 positive ovarian tumors.
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immunohistochemical determination of estrogen and progesterone receptor positivity in uterine Adenosarcoma
Gynecologic Oncology, 2004Co-Authors: Frederic Amant, Vera M Abeler, Philippe Moerman, Lieve Verbist, Katrien Schurmans, Edwin Steenkiste, Gokhan Tulunay, Eric De Jonge, Leon F A G Massuger, Ignace VergoteAbstract:Abstract Background . Given the paucity of data regarding hormone dependency, it was the purpose of this study to screen for the presence of estrogen and progesterone receptors in uterine Adenosarcoma (UAS). Methods . One hundred and five centers were asked to screen their files for uterine Adenosarcomas. A immunohistochemical estrogen and progesterone receptor determination was performed. Results . Twenty-eight primary UAS were stained, including one cervical Adenosarcoma. Sarcomatous overgrowth could be observed in eight. Furthermore, two cases of recurrent UAS, one only consisting of endometrial stromal sarcoma, were stained. UAS lacking sarcomatous overgrowth showed estrogen receptor positivity in 17/20 (85%) and 16/20 (80%) in the epithelial and sarcomatous component, respectively. Progesterone positivity was observed in 13/20 (65%) and 12/20 (60%) in the epithelial and sarcomatous component, respectively. In 18/20 (90%) of the cases, either the estrogen or the progesterone receptor stained positive in the sarcomatous component. UAS with sarcomatous overgrowth showed estrogen receptor positivity in 4/8 (50%) and 0/8 (0%) in the epithelial and sarcomatous component, respectively. Progesterone positivity was observed in 2/8 (25%) and 1/8 (12%) in the epithelial and sarcomatous component, respectively. The stromal component of both recurrent cases stained moderately positive for estrogen receptor whereas progesterone receptor was considered negative. Conclusion . The observation that the sarcomatous component of UAS without sarcomatous overgrowth frequently expresses hormone receptors might be of significant clinical importance.
E Millar - One of the best experts on this subject based on the ideXlab platform.
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extragenital mullerian Adenosarcoma with sarcomatous overgrowth arising in an endometriotic cyst in the pouch of douglas
International Journal of Gynecological Cancer, 2003Co-Authors: A Murugasu, J Miller, A Proietto, E MillarAbstract:Mullerian Adenosarcoma is a neoplasm composed of benign mullerian epithelium and a sarcomatous stroma. This tumor classically occurs in the endometrium of postmenopausal women and less frequently in the extrauterine genital tract. Rare cases of extragenital Adenosarcoma have been reported. We present the case of a 23-year-old female who presented with an extragenital Adenosarcoma arising in an endometriotic cyst in the pouch of Douglas. The patient was treated with local excision, chemotherapy and radiotherapy. At 2 years follow-up she was disease-free. The literature on extragenital Adenosarcoma is reviewed. These tumors are clinically more aggressive than their uterine counterparts. Surgical excision is the initial treatment modality for these patients. Little information is available regarding the efficacy of adjuvant chemotherapy or radiotherapy. This is an area that requires further study.
Ignace Vergote - One of the best experts on this subject based on the ideXlab platform.
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cd10 estrogen and progesterone receptor expression in ovarian Adenosarcoma
Gynecologic Oncology, 2005Co-Authors: Tim Van Mieghem, Vera M Abeler, Philippe Moerman, Lieve Verbist, Ignace Vergote, Frederic AmantAbstract:Abstract Background. The low incidence of ovarian Adenosarcomas explains the paucity of available data regarding their biologic markers. Our aim was to investigate the presence of CD10, estrogen and progesterone receptors (ER and PR) in these tumors. Case. Four cases of primary ovarian Adenosarcoma were retrieved in our pathology databases. We assessed the immunohistochemical stainings for ER, PR and CD10 in 3 ovarian Adenosarcomas without sarcomatous overgrowth (OAS), and 1 ovarian Adenosarcoma with sarcomatous overgrowth (OAS-S). When considering the malignant stromal component, 2 OAS showed the presence of ER and PR, whereas the OAS-S was only ER positive. CD10 positivity was observed in the sarcomatous cells of all 3 OAS but was absent in the OAS-S. Conclusion. The identification of a hormone sensitive disease is the main clinical implication of this study. Moreover, the current results add OAS to the list of CD10 positive ovarian tumors.
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immunohistochemical determination of estrogen and progesterone receptor positivity in uterine Adenosarcoma
Gynecologic Oncology, 2004Co-Authors: Frederic Amant, Vera M Abeler, Philippe Moerman, Lieve Verbist, Katrien Schurmans, Edwin Steenkiste, Gokhan Tulunay, Eric De Jonge, Leon F A G Massuger, Ignace VergoteAbstract:Abstract Background . Given the paucity of data regarding hormone dependency, it was the purpose of this study to screen for the presence of estrogen and progesterone receptors in uterine Adenosarcoma (UAS). Methods . One hundred and five centers were asked to screen their files for uterine Adenosarcomas. A immunohistochemical estrogen and progesterone receptor determination was performed. Results . Twenty-eight primary UAS were stained, including one cervical Adenosarcoma. Sarcomatous overgrowth could be observed in eight. Furthermore, two cases of recurrent UAS, one only consisting of endometrial stromal sarcoma, were stained. UAS lacking sarcomatous overgrowth showed estrogen receptor positivity in 17/20 (85%) and 16/20 (80%) in the epithelial and sarcomatous component, respectively. Progesterone positivity was observed in 13/20 (65%) and 12/20 (60%) in the epithelial and sarcomatous component, respectively. In 18/20 (90%) of the cases, either the estrogen or the progesterone receptor stained positive in the sarcomatous component. UAS with sarcomatous overgrowth showed estrogen receptor positivity in 4/8 (50%) and 0/8 (0%) in the epithelial and sarcomatous component, respectively. Progesterone positivity was observed in 2/8 (25%) and 1/8 (12%) in the epithelial and sarcomatous component, respectively. The stromal component of both recurrent cases stained moderately positive for estrogen receptor whereas progesterone receptor was considered negative. Conclusion . The observation that the sarcomatous component of UAS without sarcomatous overgrowth frequently expresses hormone receptors might be of significant clinical importance.
