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/cirurgiaRESUMO
In human and veterinary medicine, mixed Müllerian tumors (MMTs) are rarely diagnosed neoplasms of the tubular female genital tract. Although there are case reports of malignant MMTs in various species, benign MMTs have only been described once in a macaque. Here we present a case of benign MMT in a 12-y-old goat, and review the literature on uterine, cervical, and vaginal neoplasia in goats. The doe was presented with vaginal discharge and was euthanized because of the high suspicion of intraabdominal neoplasia. On gross examination, an ulcerated vaginal mass was identified. Histologically, 2 distinct cell populations were present: smooth muscle cells that were well differentiated and positive for alpha-smooth muscle actin, and ciliated columnar epithelial cells that lined ductal structures and had no signs of malignancy. These findings led to the diagnosis of neoplasia of Müllerian origin. Benign MMT should be considered as a differential diagnosis for uterine and vaginal neoplasms in goats.
Assuntos
Doenças das Cabras , Tumor Mulleriano Misto , Neoplasias Vaginais , Animais , Diagnóstico Diferencial , Feminino , Doenças das Cabras/diagnóstico , Doenças das Cabras/patologia , Cabras , Humanos , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/patologia , Tumor Mulleriano Misto/veterinária , Vagina , Neoplasias Vaginais/diagnóstico , Neoplasias Vaginais/patologia , Neoplasias Vaginais/veterináriaRESUMO
INTRODUCTION: Abdominopelvic washing cytology is a common specimen evaluated for ovarian, fallopian tubal, and peritoneal cancer staging or other nongynecologic malignancies presented as metastases. We reviewed our experience in diagnosing abdominopelvic washing specimens and assessing the primary tumor types and origins of the positive abdominopelvic washings. MATERIALS AND METHODS: A pathology archive database search was performed for abdominopelvic washing specimens from 2007 to 2018. The corresponding cytologic diagnoses, results of ancillary studies, clinical histories, and surgical follow-up were reviewed. The primary sites were determined based on the synoptic reports, when available. RESULTS: A total of 5.8% (350 of 6023) of cases were positive for malignancy or neoplasm. Additionally, 1.3% (78 of 6023) were diagnosed as atypical cells. Of the 350 positive cases, 93.4% were müllerian tumors. The frequency of primary sites for müllerian tumors in descending order were: ovary, uterus, fallopian tube, peritoneum, and uncertain müllerian sites. The common ovarian tumors identified in pelvic washing in descending order were: high-grade serous carcinoma, serous borderline tumor, clear cell carcinoma, low-grade serous carcinoma, and endometrioid carcinoma. Gastrointestinal, breast, bladder, and lymphoma primaries were the 23 nongynecologic tumors identified in pelvic washings. CONCLUSIONS: Positive findings in abdominopelvic washing cytology is rare. The majority of the positive cases were from müllerian origins, with ovary and uterus as the most common sites. Endometrial adenocarcinoma, endometrioid type and ovarian high-grade serous carcinoma were the most common tumor types. Knowing prior history of malignancy, morphologic comparison with concurrent surgical cases, and performing ancillary studies are keys to improve diagnostic accuracy of abdominopelvic washings.
Assuntos
Adenocarcinoma de Células Claras/diagnóstico , Carcinoma Endometrioide/diagnóstico , Citodiagnóstico/métodos , Neoplasias das Tubas Uterinas/diagnóstico , Tumor Mulleriano Misto/diagnóstico , Neoplasias Ovarianas/diagnóstico , Neoplasias Peritoneais/diagnóstico , Adenocarcinoma de Células Claras/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Endometrioide/patologia , Neoplasias das Tubas Uterinas/patologia , Tubas Uterinas/patologia , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Tumor Mulleriano Misto/patologia , Estadiamento de Neoplasias , Neoplasias Ovarianas/patologia , Ovário/patologia , Pelve/patologia , Neoplasias Peritoneais/patologia , Peritônio/patologia , Adulto JovemRESUMO
Background. Ovarian carcinosarcomas are rare aggressive biphasic tumors. Evidence suggests that these tumors are monoclonal and that the sarcoma component is derived from a stem cell undergoing divergent differentiation. Currently, there remains a paucity of data regarding its origin, with few reports suggesting an association with serous tubal intraepithelial carcinoma (STIC) by immunohistochemistry and genetics. Objective. We sought to determine the relationship of carcinosarcoma to high-grade serous carcinoma and STIC by investigating for similar mutation signatures through next-generation sequencing. Methodology. A case of carcinosarcoma with associated high-grade serous carcinoma and STIC was macrodissected, and next-generation sequencing was performed on each component separately. Results. The STIC, high-grade serous carcinoma component, and chondrosarcoma component were all diffusely positive for p53 and p16 by immunohistochemistry. Next-generation sequencing demonstrated an identical TP53 gene c.376-1G>A 5' splice site pathogenic mutation in all 3 components. Conclusions. Our findings suggest that carcinosarcomas may also originate from the fallopian tube.
Assuntos
Carcinossarcoma/genética , Cistadenocarcinoma Seroso/genética , Neoplasias das Tubas Uterinas/patologia , Tumor Mulleriano Misto/genética , Neoplasias Ovarianas/genética , Idoso , Carcinossarcoma/diagnóstico , Carcinossarcoma/secundário , Inibidor p16 de Quinase Dependente de Ciclina/genética , Cistadenocarcinoma Seroso/diagnóstico , Cistadenocarcinoma Seroso/secundário , Análise Mutacional de DNA , Neoplasias das Tubas Uterinas/diagnóstico , Neoplasias das Tubas Uterinas/genética , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/secundário , Mutação , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/secundário , Proteína Supressora de Tumor p53/genéticaRESUMO
Immunohistochemistry (IHC) is often critical for distinction between metastatic carcinomas of Mullerian organ and breast origin. Paired box family protein 8 (PAX8) has been described as a transcription factor highly specific to neoplasms derived from Mullerian organs, thyroid, and kidney. PAX8 IHC with polyclonal and monoclonal antibody reagents was performed on 27 primary and 22 metastatic breast carcinomas. Eight of 27 primary breast carcinomas (30%) were positive for PAX8 with the monoclonal antibody reagent only; 0 of 22 were polyclonal anti-PAX8 immunoreactive. Substantial numbers of metastases had positive immunoreactivity for polyclonal anti-PAX8 (23%). Each of these metastases and additional cases (45% total) also had positive immunoreactivity for monoclonal anti-PAX8, including 5 of 7 brain metastases. IHC with monoclonal anti-PAX8 was positive on 6 of 7 primary breast carcinomas corresponding to PAX8-positive metastases. Together, these results indicate a significant fraction of breast carcinoma metastases and corresponding primary neoplasms have immunoreactivity for PAX8, and positivity rates depend on the antibody used. Diagnoses of metastatic breast carcinoma were achieved with the aid of clinical history and additional IHC in cases of PAX8 immunoreactivity. Contextual interpretation is imperative for PAX8 IHC, particularly when the differential diagnosis includes metastatic breast carcinoma with limited diagnostic material available.
Assuntos
Neoplasias da Mama/diagnóstico , Imuno-Histoquímica/métodos , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/metabolismo , Fator de Transcrição PAX8/metabolismo , Anticorpos Monoclonais , Biomarcadores Tumorais/metabolismo , Neoplasias da Mama/metabolismo , Neoplasias da Mama/patologia , Diagnóstico Diferencial , Feminino , Humanos , Tumor Mulleriano Misto/patologia , Metástase Neoplásica , Estadiamento de Neoplasias , Fator de Transcrição PAX8/imunologiaRESUMO
Uterine carcinosarcomas are rare tumors that account for less than 5% of all uterine malignancies. These tumors (previously called malignant mixed Müllerian tumors) are dedifferentiated carcinomas that comprise carcinomatous and sarcomatous elements and arise from a single malignant clone. They are considered a high-risk variant of endometrial adenocarcinoma because carcinosarcomas share more similarities in epidemiology, risk factors, and clinical behavior with endometrial carcinoma than with uterine sarcomas. The clinical features, diagnosis, staging, and treatment of uterine carcinosarcoma will be discussed in this review.
Assuntos
Carcinossarcoma/diagnóstico , Tumor Mulleriano Misto/diagnóstico , Recidiva Local de Neoplasia/diagnóstico , Neoplasias Uterinas/diagnóstico , Carcinossarcoma/epidemiologia , Carcinossarcoma/patologia , Carcinossarcoma/terapia , Quimioterapia Adjuvante/métodos , Endométrio/diagnóstico por imagem , Endométrio/patologia , Feminino , Procedimentos Cirúrgicos em Ginecologia/métodos , Humanos , Incidência , Imageamento por Ressonância Magnética , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Tumor Mulleriano Misto/epidemiologia , Tumor Mulleriano Misto/patologia , Tumor Mulleriano Misto/terapia , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Seleção de Pacientes , Prognóstico , Radioterapia Adjuvante/métodos , Taxa de Sobrevida , Neoplasias Uterinas/epidemiologia , Neoplasias Uterinas/patologia , Neoplasias Uterinas/terapiaRESUMO
Carcinosarcomas are biphasic malignant tumors composed of 2 distinct neoplastic cell populations, epithelial cells and mesenchymal cells. A 13-y-old, female, mixed-breed goat was presented with a 1-wk history of anuria and lethargy. Transabdominal ultrasonography showed an irregular and heterogeneous structure in the region of the bladder and uterus and changes in the echogenicity of both kidneys. Given the poor prognosis, euthanasia was elected. Autopsy revealed a large mass within the uterine cervix and confirmed the changes in the urinary tract. Histopathology and immunohistochemistry revealed a mixed, anti-cytokeratin AE1/AE3-positive epithelial, and vimentin-positive mesenchymal neoplasm consistent with a homologous carcinosarcoma, also called malignant mixed Müllerian tumor, with areas of double-labeling. We highlight the complexity of the diagnosis of uterine neoplasms in domestic animals and in goats in particular.
Assuntos
Doenças das Cabras/diagnóstico , Tumor Mulleriano Misto/veterinária , Neoplasias Uterinas/veterinária , Animais , Diagnóstico Diferencial , Feminino , Doenças das Cabras/patologia , Doenças das Cabras/cirurgia , Cabras , Imuno-Histoquímica/veterinária , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/patologia , Neoplasias Uterinas/diagnóstico , Neoplasias Uterinas/patologiaRESUMO
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/patologiaRESUMO
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/patologiaRESUMO
Uterine adenosarcomas are uncommon tumors. It is a biphasic tumor with both epithelial and mesenchymal component. The epithelial component is benign in nature, and the mesenchymal component is malignant. Metastasis is rare in adenosarcoma. We report a case of adenosarcoma with lymph nodal metastasis. A 20-year-old female presented with history of per vaginal bleeding for 1 month. Per vaginal examination revealed a fungating mass protruding through the cervical os. Ultrasonography and magnetic resonance imaging showed a large intrauterine mass. Biopsy of the mass done at an outside hospital was reported as rhabdomyosarcoma. Hence, she was given one cycle of neoadjuvant chemotherapy. Following this, she had profuse bleeding. Emergency hysterectomy with pelvic lymph nodal dissection was performed. The final histopathology was reported as adenosarcoma. One pelvic lymph node showed metastatic deposit of rhabdomyosarcomatous element. In young females presenting with polypoidal mass, uterine adenosarcoma can be considered in the differential diagnosis.
Assuntos
Adenossarcoma/diagnóstico , Linfonodos/patologia , Tumor Mulleriano Misto/diagnóstico , Rabdomiossarcoma/diagnóstico , Neoplasias do Colo do Útero/diagnóstico , Neoplasias Uterinas/diagnóstico , Adenossarcoma/patologia , Adenossarcoma/secundário , Antineoplásicos/administração & dosagem , Antineoplásicos/uso terapêutico , Biópsia , Diagnóstico Diferencial , Feminino , Humanos , Histerectomia , Excisão de Linfonodo , Imageamento por Ressonância Magnética , Tumor Mulleriano Misto/tratamento farmacológico , Tumor Mulleriano Misto/patologia , Metástase Neoplásica , Rabdomiossarcoma/tratamento farmacológico , Rabdomiossarcoma/patologia , Ultrassonografia , Neoplasias do Colo do Útero/tratamento farmacológico , Neoplasias do Colo do Útero/patologia , Neoplasias do Colo do Útero/secundário , Hemorragia Uterina/etiologia , Neoplasias Uterinas/patologia , Neoplasias Uterinas/secundário , Útero/diagnóstico por imagem , Útero/patologia , Útero/cirurgia , Adulto JovemRESUMO
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/cirurgiaRESUMO
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/diagnósticoRESUMO
BACKGROUND: Carcinosarcomas of the gynecologic tract, also known as malignant mixed Müllerian tumors, are aggressive 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. In fact, there are currently no consensus guidelines for the treatment of these neoplasms and the search for targetable biomarkers has not been successful so far. Programmed death-ligand 1 (PD-L1) has emerged as a potential target for therapeutics in a number of malignant tumors, including melanoma, lung, and colorectal cancer. In normal conditions, PD-L1 is thought to promote immune homeostasis via a number of pathways, but mainly through downregulation of cytotoxic T cells. In some human neoplasms, however, overexpression of PD-L1 by tumor cells has been observed, which can modulate the immune system to allow cancer cells to evade host response. As this marker could potentially be a therapeutic target for these tumors, the immunohistochemical expression of PD-L1 in a group of carcinosarcomas was evaluated in the present study. MATERIAL AND METHODS: Twenty-nine cases of gynecologic carcinosarcomas were analyzed, corresponding to tumors originating from the uterus (25), ovary (2), fallopian tube (1), and pelvic epithelium (1). Immunohistochemistry for PD-L1 was performed on paraffin sections and the staining results were assessed semiquantitatively in both epithelial and mesenchymal components of each tumor. RESULTS: Positive membranous staining for PD-L1 was detected in 25/29 tumors (86%). The epithelial components were strongly positive in 19/29 (65%) and weakly positive in 6/29 tumors (21%). The mesenchymal elements were strongly positive in 8/29 (27%) and weakly positive in 3/29 tumors (10%). With exception of 1, all tumors with positive sarcomatous components had staining of the carcinomatous element. Four tumors were negative for PD-L1 in both components. CONCLUSIONS: This study shows that PD-L1 is expressed by the majority of carcinosarcomas, predominantly in the epithelial components. This is particularly important as most locoregional recurrences and distant metastases are of epithelial origin. This finding may serve as a basis for possible therapeutic approaches using antibodies that have already shown significant value in a number of other malignant tumors.
Assuntos
Antígeno B7-H1/metabolismo , Biomarcadores Tumorais/metabolismo , Carcinossarcoma/metabolismo , Imunoterapia/métodos , Tumor Mulleriano Misto/metabolismo , Neoplasias Uterinas/metabolismo , Idoso , Carcinossarcoma/diagnóstico , Carcinossarcoma/terapia , Feminino , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/terapia , Prognóstico , Neoplasias Uterinas/diagnóstico , Neoplasias Uterinas/terapiaRESUMO
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/patologiaRESUMO
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/patologiaAssuntos
Carcinossarcoma/diagnóstico , Neoplasias Peritoneais/diagnóstico , Dor Abdominal , Idoso , Carcinossarcoma/complicações , Carcinossarcoma/patologia , Colonoscopia , Evolução Fatal , Feminino , Gastroscopia , Humanos , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/patologia , Neoplasias Peritoneais/complicações , Neoplasias Peritoneais/patologia , Embolia Pulmonar/complicações , Tomografia Computadorizada por Raios X , Trombose Venosa/complicaçõesAssuntos
Adenossarcoma/diagnóstico , Neoplasias do Endométrio/patologia , Pólipos/patologia , Neoplasias Uterinas/diagnóstico , Adenossarcoma/patologia , Adenossarcoma/cirurgia , Adenossarcoma/ultraestrutura , Adulto , Diagnóstico Diferencial , Neoplasias do Endométrio/diagnóstico , Neoplasias do Endométrio/cirurgia , Neoplasias do Endométrio/ultraestrutura , Endométrio/patologia , Endométrio/ultraestrutura , Feminino , Humanos , Tumor Mulleriano Misto/diagnóstico , Tumor Mulleriano Misto/ultraestrutura , Pólipos/diagnóstico , Pólipos/cirurgia , Pólipos/ultraestrutura , Células Estromais/patologia , Tomografia Computadorizada por Raios X , Neoplasias Uterinas/patologia , Neoplasias Uterinas/cirurgia , Neoplasias Uterinas/ultraestruturaRESUMO
Chemo-radiation represents an effective therapy for carcinoma of the uterine cervix. The endometrium may however receive a consistent dose of mutagenic radiations and patients may have an increased risk of secondary malignancies. Endometrial mixed malignant mullerian tumor (MMMT) is a rare, highly aggressive disease, and neuroendocrine features are even rarer. A 68 years old woman underwent radio-chemotherapy for a squamous cell carcinoma of the cervix. Follow up was uneventful until, eight years after radio-chemotherapy, imaging exams detected a diffuse enlargement of the uterine body. Radical hysterectomy revealed a multiphasic lesion with both sarcomatous and mixed carcinomatous components. The carcinomatous, component presented neuroendocrine histologic and ultrastuctural features and an intense expression of neuroendocrine immunohistochemistry markers. No residual cervical carcinoma was documented (pR0). The patient died of disease after 9 months. Reported cases further demonstrate how the irradiation of the uterus for cervical cancer carries a not negligible risk of developing a second endometrial cancer. The second cancer may develop years after initial therapy and may have aggressive histologic and clinical features. This case underlines the importance for a long follow-up in women having received radio-chemotherapy alone.
Assuntos
Carcinoma de Células Escamosas/diagnóstico , Tumor Mulleriano Misto/diagnóstico , Neoplasias do Colo do Útero/diagnóstico , Neoplasias Uterinas/diagnóstico , Idoso , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/patologia , Carcinoma de Células Escamosas/cirurgia , Feminino , Humanos , Histerectomia , Imuno-Histoquímica , Tumor Mulleriano Misto/tratamento farmacológico , Tumor Mulleriano Misto/patologia , Tumor Mulleriano Misto/cirurgia , Neoplasias do Colo do Útero/tratamento farmacológico , Neoplasias do Colo do Útero/patologia , Neoplasias do Colo do Útero/cirurgia , Neoplasias Uterinas/tratamento farmacológico , Neoplasias Uterinas/patologia , Neoplasias Uterinas/cirurgia , Útero/patologiaRESUMO
Primary malignant mixed Müllerian tumour (MMMT) of the vagina is a rare entity. We report a case of a 62-year-old woman who presented with a fixed and hard anterior vaginal wall mass with contact bleeding. She proceeded to have an anterior infralevator pelvic exenteration with urethrectomy and anterior vaginectomy, creation of an ileal conduit and bilateral lymph node dissection. Histopathological examination and immunohistochemistry confirmed the diagnosis of primary MMMT of the vagina. The patient was stage IVA at diagnosis. Despite chemotherapy and radiotherapy, she had progressive disease and eventually passed away at the age of 65â years.
Assuntos
Tumor Mulleriano Misto/cirurgia , Exenteração Pélvica , Vagina/patologia , Neoplasias Vaginais/patologia , Biópsia , Evolução Fatal , Feminino , Humanos , Pessoa de Meia-Idade , Tumor Mulleriano Misto/diagnóstico , Estadiamento de Neoplasias , Vagina/cirurgia , Neoplasias Vaginais/cirurgiaRESUMO
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.