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1.
Women Health ; 62(5): 439-443, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35655370

RESUMO

Non-puerperal uterine inversion is an extremely uncommon condition, and its occurrence due to malignant mixed Mullerian tumor (MMMT) of the uterus is quite exceptional. We report one such case of acute non-puerperal uterine inversion ascribed to MMMT in a 77-year-old postmenopausal woman. Such a case poses a diagnostic and management dilemma, and prior knowledge may result in a successful outcome.


Assuntos
Tumor Mulleriano Misto , Inversão Uterina , Neoplasias Uterinas , Idoso , Feminino , Humanos , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/patologia , Tumor Mulleriano Misto/cirurgia , Inversão Uterina/diagnóstico , Inversão Uterina/etiologia , Inversão Uterina/cirurgia , Neoplasias Uterinas/complicações , Neoplasias Uterinas/diagnóstico , Neoplasias Uterinas/cirurgia , Útero/patologia , Útero/cirurgia
2.
J Obstet Gynaecol Can ; 43(1): 34-42, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33041218

RESUMO

OBJECTIVE: To determine the impact of adjuvant therapy and other factors associated with the recurrence and survival of patients with uterine carcinosarcoma (UCS). METHODS: A total of 102 patients who underwhent surgery for UCS from 1998 to 2017 were included in the analysis. Data were analyzed using Kaplan-Meier methods and Cox proportional hazards regression. RESULTS: At 240 months, the actuarial recurrence rate was 34.3%. Distant recurrence was the most common recurrence pattern. Patients with higher CA 125 levels, sarcoma dominance, cervical involvement, advanced stage, no lymphadenectomy, and residual tumour had a significiantly higher risk of recurrence. Five-year disease-free survival (DFS) and overall survival (OS) were 67% and 77%, respectively. FIGO stage was found to be an independent prognostic factor for DFS and OS. Sarcoma dominance was independently associated with decreased OS. CONCLUSION: Sarcoma dominance is associated with poor survival in UCS. Adjuvant treatment was not found to affect recurrence or survival. Given this finding, more effective postoperative strategies are needed.


Assuntos
Carcinossarcoma/terapia , Quimioterapia Adjuvante/métodos , Tumor Mulleriano Misto/terapia , Radioterapia Adjuvante/métodos , Neoplasias Uterinas/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinossarcoma/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Tumor Mulleriano Misto/patologia , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Resultado do Tratamento , Neoplasias Uterinas/patologia
3.
J Oncol Pharm Pract ; 26(3): 688-691, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31474213

RESUMO

Malignant mixed Müllerian tumor remains an important contributor to morbidity and mortality in women with uterine cancer. Surgery is the primary treatment modality, followed by chemotherapy and/or radiation for advanced disease or high-risk patients. Clinico-epidemiologic characteristics and outcomes in older versus younger women with Malignant mixed Müllerian tumor may differ. We analyzed and now report on 15 consecutive patients with uterine Malignant mixed Müllerian tumor treated at our institution from 2000 to 2018. The mean age at diagnosis was 65 years; 60% (9/15) patients were overweight/obese. Forty-six percent (7/15) had hypercholesterolemia, an association not previously linked with Malignant mixed Müllerian tumor in the literature. All patients but one had surgical excision of the tumor. A third of patients received adjuvant radiation therapy. A majority of patients received chemotherapy; the preferred regimen was carboplatin-paclitaxel. The patients older than 70 had a tendency towards a more advanced disease stage at diagnosis and a significantly shorter cancer-specific survival than their younger counterparts (6 months vs. 102 months (hazard ratio 1.32, p = 0.02)). Our study's conclusions are restricted due to its relatively small size, retrospective design, and some variation in the chemotherapy doses administered in individual patients. Larger studies are needed to confirm the significance of our findings.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Tumor Mulleriano Misto/terapia , Neoplasias Uterinas/terapia , Idoso , Carboplatina/administração & dosagem , Feminino , Humanos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Paclitaxel/administração & dosagem , Estudos Retrospectivos
4.
Pathologica ; 112(4): 219-223, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33393526

RESUMO

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.


Assuntos
Adenossarcoma , Radioterapia/efeitos adversos , Adenossarcoma/etiologia , Adenossarcoma/patologia , Colo do Útero/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Tumor Mulleriano Misto/patologia , Neoplasias Induzidas por Radiação/patologia , Neoplasias do Colo do Útero/patologia , Neoplasias Uterinas/patologia
5.
BMC Cancer ; 18(1): 134, 2018 02 05.
Artigo em Inglês | MEDLINE | ID: mdl-29402239

RESUMO

BACKGROUND: Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours' histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes. CASE PRESENTATION: A 79-year-old woman was referred to our Department with an abdominal mass resembling a fibroid with a haemorrhage. Her gynaecological history was negative. A transvaginal and transabdominal ultrasound examination revealed a multicystic mass resembling an ovarian tumour arising from the pelvis and extending up to the abdomen. At laparotomy a peritoneal mass arising from Douglas peritoneum was resected. The uterus and adnexa appeared normal, and a supra-cervical hysterectomy with bilateral salpingo-oophorectomy was performed. No macroscopic residual disease was present. Final pathology diagnosed a malignant peripheral nerve sheath tumors with divergent differentiation. Four weeks later a new, multicystic mass was found. Due to the progressive poor condition, the patient died four months after diagnosis. Histological slides were reviewed by external expert pathologists and the final diagnosis was of extra-genital adenosarcoma with sarcomatous overgrowth. Furthermore, we also collected and analysed articles written in English regarding extra-uterine and extra-ovarian adenosarcomas published between January 1974 and October 2016. PubMed was used as a database for this search. Clinical and pathological characteristics, treatments and outcomes were assessed. CONCLUSIONS: Only 41 cases has been reported in literature. Previous endometriosis and sarcomatous overgrowth showed an inverse effect on prognosis. Endometriosis was confirmed to have a positive effect on disease free survival Complete surgical resection is the mainstay of treatment. A worldwide registry is urgently required to collect data to standardize treatment and to obtain reliable data on prognosis.


Assuntos
Adenossarcoma/diagnóstico , Tumor Mulleriano Misto/diagnóstico , Neoplasias Ovarianas/diagnóstico , Neoplasias Uterinas/diagnóstico , Adenossarcoma/cirurgia , Idoso , Feminino , Humanos , Leiomioma/diagnóstico , Leiomioma/cirurgia , Tumor Mulleriano Misto/cirurgia , Neoplasias Ovarianas/cirurgia , Neoplasias Uterinas/cirurgia
6.
Int J Gynecol Pathol ; 37(1): 22-26, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28319572

RESUMO

Uterine carcinosarcomas, also known as malignant-mixed mullerian tumors, are rare and highly aggressive tumors whose prognostic factors remain controversial. The stage at the time of presentation is the most important prognostic factor thus far, but little information exists on the prognostic impact of the sarcomatous component (SC) in these tumors. We reviewed 21 cases of uterine carcinosarcomas and estimated the volume of the SC in each case. This information was correlated with the stage of the tumor at presentation. The percentage of the SC was also used to stratify the patients into 2 cohorts (high percentage of SC and low percentage of SC), and the 2 patient cohorts were compared based on the available follow-up data to identify prognostic differences. Patients with a lower concentration of SC (<30%) typically presented with low stage of disease when compared with their counterparts. Although not statistically significant (P=0.1966), our data suggest a correlation between a lower concentration of SC with longer follow-up and longer survival rates when compared with those of patients presenting with higher volumes of the SC (≥30%). Greater volume of the SC is seen in advanced stage tumors, which could serve as an indicator of prognosis.


Assuntos
Carcinossarcoma/patologia , Tumor Mulleriano Misto/patologia , Neoplasias Uterinas/patologia , Idoso , Idoso de 80 Anos ou mais , Carcinossarcoma/diagnóstico , Estudos de Coortes , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Tumor Mulleriano Misto/diagnóstico , Prognóstico , Estudos Retrospectivos , Neoplasias Uterinas/diagnóstico
7.
J Pak Med Assoc ; 68(8): 1263-1266, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30108400

RESUMO

Mullerian adenosarcoma (MA) of ovary with sarcomatous (rhabdomyoblastic) overgrowth is an extremely rare malignant type of female genital tract neoplasm. These tumours are highly aggressive and presence of heterologous elements is associated with worse prognosis. A 44 year old female presented with lower abdominal pain and distension. She had history of removal of tumour from pouch of Douglas in 2006 for which she did not receive any additional treatment nor did she keep continuous follow up. Current preoperative radiological examination revealed bilateral ovarian masses. She underwent abdominal hysterectomy with bilateral oophorectomy. Microscopic examination revealed biphasic tumours exhibiting sarcomatous overgrowth with rhabdomyoblastic differentiation. Review of the previous biopsy revealed low grade Mullerian adenosarcoma without sarcomatous overgrowth. Hence the current tumour was considered recurrent. This report highlights the aggressive nature of MA even with low grade morphological features and emphasizes the importance of continuous follow up and additional treatment.


Assuntos
Adenossarcoma/diagnóstico , Escavação Retouterina , Tumor Mulleriano Misto/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Neoplasias Ovarianas/diagnóstico , Neoplasias Peritoneais/diagnóstico , Rabdomiossarcoma/diagnóstico , Neoplasias Uterinas/diagnóstico , Adenossarcoma/patologia , Adulto , Escavação Retouterina/patologia , Feminino , Humanos , Tumor Mulleriano Misto/patologia , Segunda Neoplasia Primária/patologia , Neoplasias Ovarianas/patologia , Neoplasias Peritoneais/patologia , Rabdomiossarcoma/patologia , Neoplasias Uterinas/patologia
8.
Orv Hetil ; 159(19): 741-747, 2018 May.
Artigo em Húngaro | MEDLINE | ID: mdl-29730944

RESUMO

INTRODUCTION AND AIM: The aim of our study was to evaluate the prognostic factors and treatment options of a very rare and highly aggressive type of uterine neoplasms, the malignant mixed Müllerian tumor, known as carcinosarcoma. METHOD: Between 2009 and 2017, 29 patients were treated with malignant mixed Müllerian tumor. At stage I, surgery and postoperative radiotherapy were performed. At stages II-IV, trimodal treatment (surgery, chemotherapy and radiotherapy) was administered. RESULTS: The average age of patients was 68.51 (49-90) years, mean body mass index was 30.22 (20.90-37.22). We have experienced recurrence of disease after complete resection in 6 cases (4 of 6 patients did not accept radiation therapy). Local recurrence has occurred after an average 15.52 (6-36) months, distant metastasis with an average 19.2 (8-32) months. Overall survival was 11.92 (1-75) months. Six patients are free of tumours at the moment. CONCLUSIONS: As overall survival has not increased in recent decades by using combined chemotherapy, there is no congruent consensus associated with the optimal treatment. The standard surgical treatment is total abdominal hysterectomy with bilateral oophorectomy, although due to high rates of recurrence and metastases, the necessity of lymphadenectomy and postoperative treatment is in the focus of recent studies. Though postoperative irradiation improves local control, the beneficial effect on overall survival is still not proven. Adjuvant chemotherapy decreases the rate of both pelvic and extrapelvic recurrence at the same time, although there is no recommendation for the optimal chemoterapeutic agent. Multimodal therapy should lead to better outcomes. Recently there are many ongoing studies with biologic and target therapies to improve efficiency, however, the relevant results will be disclosed in many years only, due to the small number of patients. Orv Hetil. 2018; 159(19): 741-7747.


Assuntos
Tumor Misto Maligno/mortalidade , Tumor Mulleriano Misto/mortalidade , Neoplasias Uterinas/mortalidade , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumor Misto Maligno/tratamento farmacológico , Tumor Misto Maligno/radioterapia , Tumor Misto Maligno/cirurgia , Tumor Mulleriano Misto/tratamento farmacológico , Tumor Mulleriano Misto/radioterapia , Tumor Mulleriano Misto/cirurgia , Prognóstico , Resultado do Tratamento , Neoplasias Uterinas/tratamento farmacológico , Neoplasias Uterinas/radioterapia , Neoplasias Uterinas/cirurgia
9.
Rev Med Brux ; 39(3): 146-149, 2018.
Artigo em Francês | MEDLINE | ID: mdl-29964388

RESUMO

Primary retroperitoneal carcinosarcoma or mixed malignant mullerian tumor (MMMT) is an extremely rare clinical entity. These aggressive tumors arise most commonly from genital tract. The retroperitoneal location is exceptional. Here we report the case of a 63-years old female diagnosed with heterologous, extra-genital, retroperitoneal carcinosarcoma, with malignant cells in the ascitic fluid and extra-ovarian metastatic implants. She was treated with complete radical surgical treatment consisting of resection of the retroperitoneal tumor, with omentectomy, hysterectomy, bilateral salpingooophorectomy and lumbo-aortic and pelvic lymphadenectomy. She received adjuvant chemotherapy with 6 cycles of Carboplatin and Paclitaxel. She is in complete clinical and radiological remission since the end of chemotherapy, for a total of 113 months. To our knowledge, this is the longest reported disease free survival of the extra-genital retroperitoneal MMMT. This case and the review of the literature illustrate the importance of surgical treatment. However, there are no evidence-based guidelines for the systemic management of these tumors.


Le carcinosarcome rétropéritonéal ou tumeur mullérienne mixte maligne (TMMM) est une entité clinique extrêmement rare. Ce sont des tumeurs agressives du tractus génital provenant des tissus mullériens. Les localisations rétropéritonéale et extra-génitale sont exceptionnelles. Ce cas clinique concerne une patiente de 63 ans atteinte d'un carcinosarcome extra-génital, retropéritonéal, hétérologue associé à des cellules malignes dans le liquide péritonéal et des implants métastatiques extra-ovariens. Elle a bénéficié d'une laparotomie de résection de la masse rétropéritonéale avec hystérectomie, annexectomie bilatérale, omentectomie et lymphadénectomie pelvienne et lombo-aortique, et a reçu une chimiothérapie adjuvante par 6 cycles de Carboplatine et Paclitaxel. Elle est en rémission clinique et radiologique complète depuis la fin de sa chimiothérapie, soit une durée de 113 mois. A notre connaissance, il s'agit de la durée de suivi sans récidive la plus longue rapportée dans la littérature. Ce cas, ainsi que la revue de littérature, mettent en évidence l'importance du traitement chirurgical. Par contre, il n'existe pas de standard thérapeutique pour la prise en charge adjuvante ou systémique de ces tumeurs.


Assuntos
Tumor Mulleriano Misto/diagnóstico , Neoplasias Retroperitoneais/diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade , Tumor Mulleriano Misto/patologia , Prognóstico , Neoplasias Retroperitoneais/patologia
10.
Oncology ; 92(4): 197-204, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28092915

RESUMO

OBJECTIVES: The aim of our study was to evaluate the clinicopathological features and prognostic factors of uterine carcinosarcoma. PATIENTS AND METHODS: In this retrospective study, the clinical characteristics of 44 patients with uterine MMMT were evaluated. Survival curves were estimated by the Kaplan-Meier method and compared by the log-rank test. RESULTS: Forty-four patients with uterine carcinosarcoma were referred to our unit between 1995 and 2015. Their median age was 66.5 years. All women underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Twenty-five percent had omental resection. Pelvic lymphadenectomy was performed in 18.2% of the cases. Twenty-six of the patients presented with stage I/II disease, 17 with advanced stages. In 20.5% of the cases there were metastases at diagnosis. Forty women received adjuvant chemotherapy, with complete remission in 67.9% of the cases. Recurrences were observed in 27.3% of the women. Disease-free and overall survival was 27 and 103 months, respectively. The FIGO stage, histological type, tumour size, chemotherapy regimen, pelvic lymphadenectomy, and myometrial invasion did not affect survival. CONCLUSIONS: Uterine MMMT is an aggressive tumour, often diagnosed at an advanced stage and with a high rate of metastases or recurrences. Because of its rarity, its management is controversial and fixed prognostic factors cannot be defined.


Assuntos
Tumor Mulleriano Misto/mortalidade , Tumor Mulleriano Misto/terapia , Neoplasias Uterinas/mortalidade , Neoplasias Uterinas/terapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinossarcoma/mortalidade , Carcinossarcoma/patologia , Carcinossarcoma/terapia , Quimioterapia Adjuvante , Intervalo Livre de Doença , Feminino , Humanos , Histerectomia/métodos , Estimativa de Kaplan-Meier , Excisão de Linfonodo , Pessoa de Meia-Idade , Tumor Mulleriano Misto/patologia , Recidiva Local de Neoplasia/patologia , Ovariectomia/métodos , Estudos Retrospectivos , Neoplasias Uterinas/patologia
11.
Int J Gynecol Pathol ; 36(1): 24-30, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26974998

RESUMO

Müllerian adenosarcoma (MA) is an uncommon biphasic neoplasm of the female genital tract, composed of malignant stroma and benign epithelium. Little is known about the molecular and cytogenetic aberrations in MA pathogenesis, including those with progression to sarcomatous overgrowth (SO). Herein, we report all cases of MA in which karyotyping was attempted at our institution. Twenty-one samples from 20 subjects consisted of 15 primary (7 without SO, 8 with SO) and 6 metastatic MA, were cytogenetically investigated in our institution. Karyotypes were successfully obtained in 14/21 (67%) cases and 9 (45%) had cytogenetic aberrations. Two (1 MA with SO and 1 metastatic MA) were markedly complex, displaying extreme aneuploidy with numerous rearrangements. Seven (2 MA without SO, 3 MA with SO, and 2 metastatic MA) demonstrated noncomplex clonal aberrations, of which 5 (71%) included an abnormality involving chromosome 8. Two tumors had rearrangements at 8q13 and another 3 tumors had extra copies of chromosome 8. In 5 cases, a normal karyotype (46,XX) was obtained (2 MA without SO, 2 MA with SO, and 1 metastatic MA). Further study is warranted to explore the genetic mechanism by which chromosome abnormalities, particularly those at 8q13, contribute to MA tumorigenesis.


Assuntos
Adenossarcoma/genética , Aberrações Cromossômicas , Cromossomos Humanos Par 8/genética , Tumor Mulleriano Misto/genética , Adenossarcoma/diagnóstico , Adenossarcoma/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Pessoa de Meia-Idade , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/patologia
12.
Int J Gynecol Pathol ; 36(5): 412-419, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28700424

RESUMO

Carcinosarcomas (malignant mixed Müllerian tumors or MMMT) are rare malignant tumors in the female genital tract composed of both malignant epithelial and malignant mesenchymal components. They comprise <5% of all neoplasms in the gynecologic tract and have an aggressive clinical course. The purpose of this study is to evaluate the immunophenotype and possible histogenesis of carcinosarcomas of the uterus. Sixty-two cases of uterine carcinosarcomas diagnosed between 1995 and 2011 were retrieved from the gynecologic pathology files at Columbia University Medical Center. Representative tissue blocks containing both epithelial and mesenchymal components were selected from each case for histologic and immunohistochemical studies. Clinical data from each case were retrieved. The epithelial component was poorly differentiated adenocarcinoma in the majority (80.7%) of cases; in 17.7%, the carcinoma was moderately differentiated, and in only 1.6% the carcinoma was well differentiated. 53% of the tumors had homologous stromal elements and 47% displayed heterologous stromal elements. Immunohistochemical study revealed almost equal staining in both epithelial and mesenchymal components of carcinosarcomas for p16 and p53. PAX8 positivity was noted in 73% of epithelial components, but only 13% of stromal components, and PAX8 stromal positivity was never seen in the absence of PAX8 epithelial positivity. Expression of p16, p53, and PAX8 in both malignant components lends support to the monoclonal theory of uterine carcinosarcoma tumorigenesis. The roles of these tumor markers in the diagnosis and pathogenesis of this tumor and associations between clinical characteristics, tumor pathologic features, and prognosis are discussed.


Assuntos
Biomarcadores Tumorais/metabolismo , Carcinossarcoma/metabolismo , Tumor Mulleriano Misto/metabolismo , Neoplasias Uterinas/metabolismo , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinossarcoma/diagnóstico , Carcinossarcoma/patologia , Inibidor p16 de Quinase Dependente de Ciclina/metabolismo , Feminino , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/patologia , Fator de Transcrição PAX8/metabolismo , Prognóstico , Proteína Supressora de Tumor p53/metabolismo , Neoplasias Uterinas/diagnóstico , Neoplasias Uterinas/patologia
13.
Int J Gynecol Pathol ; 36(4): 386-392, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28221217

RESUMO

Distinguishing hyalinized stroma from osteoid production by a heterologous osteosarcomatous component can be challenging in gynecologic tract carcinosarcomas. As heterologous components in a carcinosarcoma may have prognostic and therapeutic implications, it is important that these are recognized. This study examines interobserver reproducibility among gynecologic pathologists in the diagnosis of osteosarcomatous components, and its correlation with expression of the novel antibody SATB2 (marker of osteoblastic differentiation) in these osteosarcomatous foci. Digital H&E images from 20 gynecologic tract carcinosarcomas were reviewed by 22 gynecologic pathologists with a request to determine the presence or absence of an osteosarcomatous component. The 20 preselected cases included areas of classic heterologous osteosarcoma (malignant cells producing osteoid; n=10) and osteosarcoma mimics (malignant cells with admixed nonosteoid matrix; n=10). Interobserver agreement was evaluated and SATB2 scored on all 20 cases and compared with the original diagnoses. Moderate agreement (Fleiss' κ=0.483) was identified for the 22 raters scoring the 20 cases with a median sensitivity of 7/10 and a median specificity of 9/10 for the diagnosis of osteosarcoma. SATB2 showed 100% sensitivity (10/10) and 60% (6/10) specificity in discriminating classic osteosarcoma from osteosarcoma mimics. Utilizing negative SATB2 as a surrogate marker to exclude osteosarcoma, 73% (16/22) of the reviewers would have downgraded at least 1 case to not contain an osteosarcomatous component (range, 1-6 cases, median 1 case). Gynecologic pathologists demonstrate only a moderate level of agreement in the diagnosis of heterologous osteosarcoma based on morphologic grounds. In such instances, a negative SATB2 staining may assist in increasing accuracy in the diagnosis of an osteosarcomatous component.


Assuntos
Carcinossarcoma/patologia , Neoplasias dos Genitais Femininos/patologia , Variações Dependentes do Observador , Osteossarcoma/patologia , Patologistas , Biomarcadores Tumorais/análise , Feminino , Neoplasias dos Genitais Femininos/terapia , Humanos , Proteínas de Ligação à Região de Interação com a Matriz/análise , Tumor Mulleriano Misto/patologia , Osteossarcoma/química , Prognóstico , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Fatores de Transcrição/análise
14.
Int J Mol Sci ; 18(5)2017 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-28531111

RESUMO

Carcinosarcomas (CS) in gynecology are very infrequent and represent only 2-5% of uterine cancers. Despite surgical cytoreduction and subsequent chemotherapy being the primary treatment for uterine CS, the overall five-year survival rate is 30 ± 9% and recurrence is extremely common (50-80%). Due to the poor prognosis of CS, new strategies have been developed in the last few decades, targeting known dysfunctional molecular pathways for immunotherapy. In this paper, we aimed to gather the available evidence on the latest therapies for the treatment of CS. We performed a systematic review using the terms "uterine carcinosarcoma", "uterine Malignant Mixed Müllerian Tumors", "target therapies", "angiogenesis therapy", "cancer stem cell therapy", "prognostic biomarker", and "novel antibody-drug". Based on our results, the differential expression and accessibility of epithelial cell adhesion molecule-1 on metastatic/chemotherapy-resistant CS cells in comparison to normal tissues and Human Epidermal Growth Factor Receptor 2 (HER2) open up new possibilities in the field of target therapy. Nevertheless, future investigations are needed to clarify the impact of these new therapies on survival rate and medium-/long-term outcomes.


Assuntos
Carcinossarcoma/tratamento farmacológico , Tumor Mulleriano Misto/tratamento farmacológico , Terapia de Alvo Molecular/métodos , Neoplasias Uterinas/tratamento farmacológico , Anticorpos Biespecíficos/uso terapêutico , Biomarcadores Tumorais/antagonistas & inibidores , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Carcinossarcoma/patologia , Epigênese Genética , Molécula de Adesão da Célula Epitelial/antagonistas & inibidores , Molécula de Adesão da Célula Epitelial/genética , Molécula de Adesão da Célula Epitelial/metabolismo , Receptores ErbB/antagonistas & inibidores , Receptores ErbB/genética , Receptores ErbB/metabolismo , Feminino , Humanos , Imunoterapia , Tumor Mulleriano Misto/patologia , Neovascularização Patológica , Prognóstico , Taxa de Sobrevida , Neoplasias Uterinas/patologia
15.
Ceska Gynekol ; 82(5): 390-395, 2017.
Artigo em Tcheco | MEDLINE | ID: mdl-29020787

RESUMO

OBJECTIVE: The case report presents a case of 60-year old woman with a rare malignant mixed Müllerian tumor with melanomatous differentiation diagnosed from a histology after cervical polyp ablation and curettage. DESIGN: Case report. SETTING: Department of gynecology and obstetrics, University Hospital in Pilsen. CONCLUSION: Carcinosarcoma, previously malignant mixed Müllerian tumor, is a very rare aggressive endometrial carcinoma with low incidence, which typically occurs among older women and commonly affects the uterine body and cervix. Clinically, the carcinosarcoma is impossible to be distinguished from endometrial carcinoma or uterine sarcoma and the definitive diagnosis can only be made based on histological examination.


Assuntos
Carcinossarcoma/patologia , Neoplasias do Endométrio/patologia , Tumor Mulleriano Misto/patologia , Neoplasias Uterinas/patologia , Endométrio , Feminino , Humanos , Pessoa de Meia-Idade
16.
Int J Gynecol Pathol ; 35(1): 78-81, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26598986

RESUMO

The recent 2014 World Health Organization (WHO) Classification of Tumours of the Female Reproductive Organs introduced a new category of ovarian neoplasm designated "seromucinous tumours". The recognition of this distinctive group of tumors is an important addition to the classification but the term "seromucinous" has serious flaws that obscures the nature of these neoplasms. Morphologically, seromucinous tumors in addition to serous and endocervical-type mucinous epithelium, contain endometrioid, indifferent and squamous type epithelium. Their immunoprofile is characterized by frequent expression of ER, PR, infrequent expression of WT1 and lack of expression of CK20 and CDX2, an immunostaining pattern consistent with a "müllerian" immunophenotype. Unlike serous and intestinal type mucinous tumors, seromucinous tumors are frequently associated with endometriosis making them more analogous to endometrioid and clear cell neoplasms. Indeed, recent studies have shown that a high proportion of seromucinous tumors lost expression of ARID1A, a tumor suppressor gene, that is mutated in approximately 50% of endometrioid and clear cell tumors, in sharp contrast to serous and intestinal-type mucinous tumors which do not contain ARID1A mutations or lose its expression. Therefore, based on their clinicopathologic, immunohistochemical and molecular genetic features we believe a more appropriate designation for this group of tumors is "mixed müllerian tumors" which can be subcategorized as "mixed müllerian cystadenomas", "mixed müllerian atypical proliferative (borderline) tumors" and "mixed müllerian carcinomas".


Assuntos
Biomarcadores Tumorais/metabolismo , Endometriose/classificação , Tumor Mulleriano Misto/classificação , Neoplasias Ovarianas/classificação , Biomarcadores Tumorais/genética , Proteínas de Ligação a DNA , Endometriose/genética , Endometriose/patologia , Feminino , Humanos , Tumor Mulleriano Misto/genética , Tumor Mulleriano Misto/patologia , Mutação , Proteínas Nucleares/genética , Proteínas Nucleares/metabolismo , Neoplasias Ovarianas/genética , Neoplasias Ovarianas/patologia , Fatores de Transcrição/genética , Fatores de Transcrição/metabolismo
17.
Int J Gynecol Pathol ; 35(2): 142-6, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26535981

RESUMO

Malignant mixed Müllerian tumors (MMMTs) are aggressive malignant neoplasms with a high recurrence rate and poor prognosis. Despite advances in adjuvant therapies in recent years, the prognosis of these tumors has not improved. Growth hormone-releasing hormone (GHRH) is produced by a variety of malignant tumors and acts as a growth factor in an autocrine/paracrine manner. Its function requires the presence of its receptors to exert its effects on neoplastic cells. In this study, we evaluated the expression of GHRH receptors (GHRH-R) in a group of MMMTs. Thirty-one examples of MMMTs from endometrium, ovary, uterine tube, and pelvic peritoneum were retrieved from the files of Department of Pathology at the University of Miami, Jackson Memorial Hospital. Immunohistochemistry for GHRH-R was performed on paraffin sections and the staining results were evaluated separately in both epithelial and mesenchymal components of each tumor. The presence of pituitary type growth hormone-releasing hormone receptor mRNA and that of its biologically active splice variant were also evaluated by RT-PCR in 6 of the tumors. Positive immunohistochemical reaction for GHRH-R was detected in 30 tumors (96%). The epithelial and sarcomatous components were positive in 30 (96%), whereas one endometrial tumor was negative in both components. The mRNA for GHRH-R and its splice variant was found in all 6 tested tumors. This study shows that GHRH-R is expressed by the majority of MMMTs in both epithelial and mesenchymal components. This finding could potentially serve as a basis for therapeutic approaches using synthetic peptide antagonists of GHRH-R that have shown significant efficacy with minimal side effects in experimental models.


Assuntos
Biomarcadores Tumorais/análise , Tumor Mulleriano Misto/metabolismo , Tumor Mulleriano Misto/patologia , Receptores de Neuropeptídeos/biossíntese , Receptores de Hormônios Reguladores de Hormônio Hipofisário/biossíntese , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Receptores de Neuropeptídeos/análise , Receptores de Hormônios Reguladores de Hormônio Hipofisário/análise
18.
Arch Gynecol Obstet ; 294(2): 343-51, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-26711836

RESUMO

PURPOSE: There is low evidence regarding the optimal treatment in patients with uterine sarcomas and malignant mixed Mullerian tumors (MMMTs). This study provides an overview of experience at our center with patients diagnosed with uterine sarcoma and MMMT, in relation to the clinical management and outcome. METHODS: The medical records for 143 patients with low-grade endometrial stromal sarcoma (ESS), leiomyosarcoma (LMS), and high-grade (undifferentiated) endometrial sarcoma (UES) and MMMT were reviewed. All available clinical and pathological data were collected and analyzed. Putative prognostic factors were entered into a multivariate analysis using a Cox proportional hazards ratio model, and survival data were calculated. RESULTS: The 5-year overall survival rates were significantly different between patients with ESS, LMS, and UES and MMMT (86 vs. 40 vs. 57 vs. 45 %; P < 0.001). The multivariate analysis showed that the patients' age, higher FIGO stage (III-IV), a history of smoking, prior pelvic radiation, diabetes, and residual tumor after surgery were associated with a poorer overall survival. Histological subtypes of LMS (HR 4.68; 95 % CI 1.35-16.17), UES (HR 1.21; 95 % CI 0.26-5.77) and MMMT (HR 1.63; 95 % CI 0.42-6.43) were also associated with a poorer overall survival than ESS (P = 0.008). Adjuvant therapies showed no associations with overall survival. CONCLUSIONS: Adjuvant therapy has so far not shown any overall survival benefit, and the focus is therefore on primary surgery. In future studies, the entities should be investigated separately in relation to prognostic factors and effective therapeutic management.


Assuntos
Tumores do Estroma Endometrial/patologia , Leiomiossarcoma/patologia , Tumor Mulleriano Misto/patologia , Sarcoma do Estroma Endometrial/patologia , Neoplasias Uterinas/patologia , Adulto , Idoso , Terapia Combinada , Intervalo Livre de Doença , Neoplasias do Endométrio/mortalidade , Neoplasias do Endométrio/patologia , Neoplasias do Endométrio/terapia , Tumores do Estroma Endometrial/mortalidade , Tumores do Estroma Endometrial/terapia , Feminino , Humanos , Estimativa de Kaplan-Meier , Leiomiossarcoma/mortalidade , Leiomiossarcoma/terapia , Pessoa de Meia-Idade , Tumor Mulleriano Misto/mortalidade , Tumor Mulleriano Misto/terapia , Análise Multivariada , Prognóstico , Modelos de Riscos Proporcionais , Sarcoma/patologia , Sarcoma do Estroma Endometrial/mortalidade , Sarcoma do Estroma Endometrial/terapia , Taxa de Sobrevida , Neoplasias Uterinas/mortalidade , Neoplasias Uterinas/cirurgia
19.
Rozhl Chir ; 95(1): 45-7, 2016 Jan.
Artigo em Tcheco | MEDLINE | ID: mdl-26982192

RESUMO

UNLABELLED: The authors present the case of a 57-year-old woman with a very rare extragenital malignant retroperitoneal Müllerian carcinosarcoma invading the inferior vena cava. Tumor resection with partial resection of the vena cava wall and resection of metastases in the pelvic area is described. The authors further discuss diagnostic options of metastases of this tumour and the recommended adjuvant chemotherapy. KEY WORDS: extragenital Müllerian carcinosarcoma malignant mixed Müllerian tumour - diagnosis therapy.


Assuntos
Carcinossarcoma/patologia , Tumor Mulleriano Misto/patologia , Neoplasias Retroperitoneais/patologia , Neoplasias Vasculares/patologia , Veia Cava Inferior/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Invasividade Neoplásica , Neoplasias Vasculares/cirurgia , Veia Cava Inferior/cirurgia
20.
J Comput Assist Tomogr ; 39(2): 276-80, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25474142

RESUMO

We illustrate the magnetic resonance imaging features of 3 cases of rare ovarian Mullerian mixed epithelial borderline tumor (MEBT) and identify important diagnostic clues based on their detailed histologic, morphologic, and clinical features. Mullerian mixed epithelial borderline tumor has good prognosis, and adequate management is essential. In order to avoid unnecessary aggressive treatment, radiologists should become familiar with the imaging findings of MEBT. To the best of our knowledge, no articles have described the detailed images of MEBT.


Assuntos
Imageamento por Ressonância Magnética , Tumor Mulleriano Misto/patologia , Neoplasias Ovarianas/patologia , Idoso , Feminino , Humanos , Pessoa de Meia-Idade
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