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1.
Int J Gynecol Pathol ; 39(1): 97-102, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31815894

RESUMO

Angiosarcomas of the female genital tract are rare and primary angiosarcoma of the cervix is extremely rare with only one prior case report. We report a case of a primary cervical angiosarcoma in a 43-yr-old woman who presented with heavy vaginal bleeding. Cervical biopsy and subsequent radical hysterectomy showed a malignant vascular tumor which was composed of spindled and epithelioid cells and formed abortive vascular channels. Immunohistochemically, the tumor cells were diffusely positive for CD31, CD34, ERG, and cyclin D1 and focally positive for D2-40. A reverse transcription polymerase chain reaction test for YWHAE-NUTM2 genetic fusion was negative excluding a YWHAE-translocated high-grade endometrial stromal sarcoma. The tumor formed a 5 cm mass within the cervix with microscopic involvement of the endometrium, superficial myometrium, and vagina. Metastatic microscopic tumor deposits were present in both ovaries, left fallopian tube, one paracervical lymph node, and one pelvic lymph node. In reporting this unusual case we discuss the differential diagnosis.


Assuntos
Hemangiossarcoma/diagnóstico , Hemangiossarcoma/patologia , Neoplasias do Colo do Útero/diagnóstico , Neoplasias do Colo do Útero/patologia , Adulto , Diagnóstico Diferencial , Neoplasias do Endométrio/diagnóstico , Neoplasias do Endométrio/patologia , Feminino , Humanos , Imuno-Histoquímica , Metástase Neoplásica , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/patologia
2.
BMJ Case Rep ; 12(12)2019 Dec 08.
Artigo em Inglês | MEDLINE | ID: mdl-31818885

RESUMO

Endometrial stromal sarcoma (ESS) is an uncommon and challenging condition comprising 10% of all uterine sarcomas and found in women 42-58 years of age. ESS is difficult to diagnose in young women as it masquerades as a leiomyoma. We report this tumour in a 20-year-old woman presenting with heavy and prolonged menses and urinary retention. She was not sexually active and did not give consent for pelvic examination. A preoperative diagnosis of a submucous leiomyoma with an adnexal mass was made. At laparotomy, the leiomyoma was found to be wedged between the cervix and the vagina, and was removed vaginally. A 5-6 cm retroperitoneal mass was adherent to the right pelvic wall, which was also removed. Histopathology of both specimens revealed ESS. The final diagnosis according to the International Federation of Gynaecology and Obstetrics classification was stage IV ESS. After oncology consult, she was referred for chemotherapy. She is now on follow-up.


Assuntos
Neoplasias do Endométrio/diagnóstico , Sarcoma do Estroma Endometrial/diagnóstico , Diagnóstico Diferencial , Dismenorreia/etiologia , Neoplasias do Endométrio/complicações , Neoplasias do Endométrio/tratamento farmacológico , Neoplasias do Endométrio/cirurgia , Feminino , Humanos , Laparotomia , Leiomioma/diagnóstico , Menorragia/etiologia , Sarcoma do Estroma Endometrial/complicações , Sarcoma do Estroma Endometrial/tratamento farmacológico , Sarcoma do Estroma Endometrial/cirurgia , Resultado do Tratamento , Retenção Urinária/etiologia , Adulto Jovem
3.
Diagn Pathol ; 14(1): 110, 2019 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-31615558

RESUMO

BACKGROUND: Low-grade endometrial stromal sarcoma (ESS) is rare mesenchymal neoplasm, recently specified as harboring JAZF1-SUZ12 rearrangement. Typical JAZF1-SUZ12 ESS is slow growing, in which high uptake of fluorodeoxyglucose (FDG) on positron emission tomography (PET) and subserosal masses are quite unusual. CASE PRESENTATION: A 69-year-old Japanese woman complained of urinary incontinence. Pelvic magnetic resonance imaging showed uterine lesions composed of (1) a 9 × 8 × 7-cm mass protruding from the right-anterior wall, (2) a 4.5-cm mass attached to the right-posterior wall, and (3) a 6.5-cm intramural mass in the fundus. FDG-PET demonstrated maximum standardized uptake value of 13.28 confined to the two subserosal masses (1 & 2) in contrast to no uptake of the intramural mass (3). She was diagnosed with a high-grade uterine sarcoma concomitant with leiomyomas and underwent total hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy. The removed uterus had three tumors-two in the right-anterior and right-posterior subserosa, respectively, and the remaining in the fundal myometrium. Microscopically, the three tumors shared morphologic features characterized by neoplastic cells similar to proliferative-phase endometrial stromal cells, in which neither round-cell component, pleomorphism, nor high mitotic activity was recognized. Nuclear cyclin D1 immunostaining was identified 50% of neoplastic cells in the two subserosal tumors (1 &2) whereas < 1% positive cells in the intramural component (3). Reverse transcriptase-polymerase chain reaction showed the same-sized electrophoretic bands indicating JAZF1-SUZ12 gene fusion shared by the three uterine tumors and a focal tumor extension into the extrauterine vein. The patient is alive without evidence of recurrence at 14 months after surgery. CONCLUSIONS: Pathologists and clinicians should not exclude the possibility of JAZF1-SUZ12 ESS even when uterine subserosal masses demonstrate extraordinary FDG uptake on PET. Molecular analysis is helpful for diagnostic confirmation of JAZF1-SUZ12 ESS with a complex growth pattern.


Assuntos
Proteínas Correpressoras/genética , Proteínas de Ligação a DNA/genética , Recidiva Local de Neoplasia/genética , Complexo Repressor Polycomb 2/genética , Sarcoma do Estroma Endometrial/genética , Idoso , Neoplasias do Endométrio/genética , Neoplasias do Endométrio/patologia , Feminino , Humanos , Hibridização in Situ Fluorescente/métodos , Proteínas de Neoplasias/genética , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/patologia
4.
Indian J Cancer ; 56(4): 335-340, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31607703

RESUMO

Background: Endometrial stromal sarcoma (ESS) is a common uterine mesenchymal malignancy. According to World Health Organisation (WHO) 2014 classification, ESSs are further subdivided into low-grade ESS (LGESS) and high-grade ESS (HGESS). HGESS is defined by the presence of YWHAE gene rearrangement and has a poorer prognosis compared to LGESS. METHODS: Twenty-four cases comprising of 16 endometrial stromal sarcoma and 8 lesions mimicking ESS were retrieved from the archives of the Department of Pathology and subjected to fluorescent in situ hybridization (FISH) analysis for YWHAE gene rearrangement. Immunohistochemistry for CD10, ER, PR, Cyclin D1, SMA, H-Caldesmon, Desmin, Ki-67, and Pan Cytokeratin was performed. RESULTS: Two cases with histological features similar to HGESS were positive for YWHAE gene rearrangement while 1 was indeterminate. No cases of LGESS and histological mimics of ESS were positive for this rearrangement. CONCLUSIONS: HGESSs are defined by the presence of YWHAE rearrangement. These tumors present at higher stage and have poorer prognosis. They may not respond to hormonal therapy and may be treated with chemotherapy. Cyclin D1 though not specific remains a sensitive tool to triage endometrial stromal sarcomas for this FISH study.


Assuntos
Proteínas 14-3-3/genética , Ciclina D1/metabolismo , Células-Tronco Mesenquimais/patologia , Sarcoma do Estroma Endometrial/diagnóstico , Neoplasias Uterinas/diagnóstico , Adulto , Idoso , Biomarcadores Tumorais , Ciclina D1/genética , Feminino , Rearranjo Gênico , Humanos , Hibridização in Situ Fluorescente , Índia , Pessoa de Meia-Idade , Gradação de Tumores , Estadiamento de Neoplasias , Prognóstico , Sarcoma do Estroma Endometrial/genética , Centros de Atenção Terciária , Neoplasias Uterinas/genética
6.
Surg Pathol Clin ; 12(2): 363-396, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31097109

RESUMO

The spectrum of mesenchymal neoplasia in the uterus has expanded in recent years. First, the identification of prevalent, recurrent molecular alterations has led to a more biologically and clinically congruent classification of endometrial stromal tumors. Likewise, the diagnostic criteria of several rare and miscellaneous tumor types have been refined in recent case series (Perivascular Epithelioid Cell tumor, inflammatory myofibroblastic tumor). Pure mesenchymal tumors are still broadly classified based on morphology according to the tumor cell phenotype. Smooth muscle tumors predominate in frequency, followed by tumors of endometrial stromal derivation; the latter are covered in depth in this article with an emphasis on defining molecular alterations and their morphologic and clinical correlates. The remaining entities comprise a miscellaneous group in which cell derivation does not have a normal counterpart in the uterus (eg, rhabdomyosarcoma) or is obscure (eg, undifferentiated uterine sarcoma). This article discusses their clinical relevance, recent insights into their molecular biology, and the most important differential diagnoses. Regarding the latter, immunohistochemistry and (increasingly) molecular diagnostics play a role in the diagnostic workup. We conclude with a few considerations on intraoperative consultation and macroscopic examination, as well as pathologic staging and grading of uterine sarcomas as per the most recent American Joint Cancer Commission and the Fédération Internationale de Gynécologie et d'Obstétrique staging systems.


Assuntos
Neoplasias Uterinas/diagnóstico , Adenossarcoma/diagnóstico , Adenossarcoma/genética , Adenossarcoma/patologia , Biomarcadores Tumorais/metabolismo , Diagnóstico Diferencial , Feminino , Predisposição Genética para Doença , Humanos , Mutação , Estadiamento de Neoplasias , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/genética , Sarcoma do Estroma Endometrial/patologia , Neoplasias Uterinas/genética , Neoplasias Uterinas/patologia
7.
Diagn Pathol ; 14(1): 30, 2019 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-31010432

RESUMO

BACKGROUND: Metastatic tumors to the pancreas are uncommon, accounting for approximately 2% of pancreatic malignancies. The most common primary tumors to give rise to pancreatic metastases are carcinomas. CASE PRESENTATION: A 50-year old female patient was investigated for a cause of abdominal discomfort. She had a 2-year history of menorrhagia and dysmenorrhea which was ascribed to a fibroid uterus. On imaging, she was found to have a large solid and cystic mass in the tail of the pancreas. Imaging also confirmed a fibroid uterus. A distal pancreatectomy and splenectomy showed a 9 cm circumscribed mass within, and grossly confined to, the parenchyma of the pancreatic tail. Microscopically, the pancreatic lesion was lobulated, and well-circumscribed, but focally infiltrative. It comprised sheets of uniform spindled to epithelioid cells with round to oval nuclei, coarse to vesicular chromatin, visible nucleoli, nuclear grooves and clear to eosinophilic cytoplasm. Prominent arterioles were identified. The stroma was collagenized in areas. Occasional hemosiderin-laden macrophages were seen, and focal cystic change was present. There was no evidence of nuclear pleomorphism, mitotic activity or necrosis, and there was no evidence of endometriosis despite multiple sections being taken. Immunohistochemistry showed that the tumor cells were positive for CD10, estrogen receptor (ER), progesterone receptor (PR), Wilms tumor-1 (WT-1) and smooth muscle actin (SMA). RNA sequencing detected a PHF1 rearrangement. The morphological, immunohistochemical and molecular features were of a low-grade endometrial stromal sarcoma (LG-ESS). Subsequent total hysterectomy and bilateral salpingo-oophorectomy 3 months later, showed uterine fibroids and a 5 cm low-grade endometrial stromal sarcoma confined to the uterus, with lymphatic invasion. CONCLUSIONS: To the best of our knowledge, this is the first documented case of metastatic endometrial stromal sarcoma of uterus presenting as a primary pancreatic neoplasm. An unexpected extra-uterine location and unusual presentation of ESS may make the diagnosis challenging, despite classic histological features. Morphological, immunohistochemical and molecular findings must be combined to render the correct diagnosis.


Assuntos
Biomarcadores Tumorais/análise , Neoplasias do Endométrio/diagnóstico , Leiomioma/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Sarcoma do Estroma Endometrial/diagnóstico , Neoplasias do Endométrio/secundário , Neoplasias do Endométrio/cirurgia , Células Epitelioides/patologia , Feminino , Humanos , Histerectomia , Imuno-Histoquímica , Leiomioma/patologia , Leiomioma/cirurgia , Pessoa de Meia-Idade , Pâncreas/patologia , Neoplasias Pancreáticas/patologia , Sarcoma do Estroma Endometrial/secundário , Sarcoma do Estroma Endometrial/cirurgia , Útero/patologia , Útero/cirurgia
8.
Korean J Radiol ; 20(1): 18-33, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30627019

RESUMO

In this study, we summarize the clinical role of magnetic resonance imaging (MRI) in the diagnosis of patients with malignant uterine neoplasms, including leiomyosarcoma, endometrial stromal sarcoma, adenosarcoma, uterine carcinosarcoma, and endometrial cancer, with emphasis on the challenges and disadvantages. MRI plays an essential role in patients with uterine malignancy, for the purpose of tumor detection, primary staging, and treatment planning. MRI has advanced in scope beyond the visualization of the many aspects of anatomical structures, including diffusion-weighted imaging, dynamic contrast enhancement-MRI, and magnetic resonance spectroscopy. Emerging technologies coupled with the use of artificial intelligence in MRI are expected to lead to progressive improvement in case management of malignant uterine neoplasms.


Assuntos
Imagem por Ressonância Magnética/métodos , Neoplasias Uterinas/diagnóstico por imagem , Neoplasias Uterinas/diagnóstico , Útero/diagnóstico por imagem , Adenossarcoma/diagnóstico , Adenossarcoma/diagnóstico por imagem , Adenossarcoma/patologia , Carcinossarcoma/diagnóstico , Carcinossarcoma/diagnóstico por imagem , Carcinossarcoma/patologia , Feminino , Humanos , Leiomiossarcoma/diagnóstico , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/patologia , Compostos Radiofarmacêuticos/uso terapêutico , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/diagnóstico por imagem , Sarcoma do Estroma Endometrial/patologia , Neoplasias Uterinas/patologia , Útero/patologia
9.
Ann Diagn Pathol ; 39: 25-29, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30611894

RESUMO

Low grade endometrial stromal sarcoma (LGESS) is a rare neoplasm that typically arises in the uterine corpus and accounts for less than 1% of uterine sarcomas. Infrequently, extra-uterine LGESS can occur. Histologically, LGESS is characterized by a monotonous population of cells that resemble the proliferative phase of endometrial stroma and in their classic form they exhibit tongue-like growth pattern of infiltration and/or lymphovascular invasion. Infrequently LGESS can demonstrate various morphologic differentiation patterns, including endometrioid-type glands. We report the first fine needle aspiration (FNA) case of a periduodenal mass that was incidentally discovered on Computed Tomography (CT) scan of a 60-year-old female. The cytomorphologic and histologic findings and the immunohistochemical staining were consistent with a LGESS with endometrioid glandular differentiation. We are presenting the correlation between the cytologic, radiologic and pathologic features.


Assuntos
Neoplasias do Endométrio/diagnóstico , Tumores do Estroma Endometrial/diagnóstico , Neoplasias Retroperitoneais/diagnóstico por imagem , Sarcoma do Estroma Endometrial/diagnóstico , Biomarcadores Tumorais/metabolismo , Biópsia por Agulha Fina , Neoplasias do Endométrio/metabolismo , Neoplasias do Endométrio/patologia , Tumores do Estroma Endometrial/metabolismo , Tumores do Estroma Endometrial/patologia , Feminino , Humanos , Imuno-Histoquímica , Achados Incidentais , Pessoa de Meia-Idade , Neoplasias Retroperitoneais/etiologia , Neoplasias Retroperitoneais/patologia , Sarcoma do Estroma Endometrial/metabolismo , Sarcoma do Estroma Endometrial/patologia , Tomografia Computadorizada por Raios X
11.
Gen Thorac Cardiovasc Surg ; 67(3): 324-327, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29488074

RESUMO

Entometrioid stromal sarcomas are seen in extra-uterine as well as extra-gonadal sites and have a strong association with endometriosis. Although having better prognosis than other sarcomas, yet these tumors may relapse (whether local or distant) in up to 56% of cases, even as late as 20 years after surgery. We report a case of a 30-year-old female patient with a mass in the inferior vena cava and right atrium which was surgically removed using cardiopulmonary bypass and deep hypothermic circulatory arrest and turned to be an entometrioid stromal sarcoma. The patient gave a history of endometriosis followed by the appearance of a low-grade ovarian endometrioid stromal sarcoma 4 years before the development of the mass in the IVC and right atrium.


Assuntos
Átrios do Coração/cirurgia , Neoplasias Cardíacas/diagnóstico , Neoplasias Ovarianas/diagnóstico , Sarcoma do Estroma Endometrial/diagnóstico , Veia Cava Inferior/cirurgia , Adulto , Ponte Cardiopulmonar , Parada Circulatória Induzida por Hipotermia Profunda , Diagnóstico Diferencial , Feminino , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/secundário , Neoplasias Cardíacas/cirurgia , Humanos , Neoplasias Ovarianas/diagnóstico por imagem , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/cirurgia , Sarcoma do Estroma Endometrial/diagnóstico por imagem , Sarcoma do Estroma Endometrial/secundário , Sarcoma do Estroma Endometrial/cirurgia
12.
Rom J Morphol Embryol ; 59(3): 933-938, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30534836

RESUMO

Endometrial stromal tumors are very rare, representing approximately 0.2% of uterine malignancies, having an incidence of one to two from a million of women. The diagnosis cannot be established by imaging, it is histopathological only, often necessitate supplementary immunohistochemistry tests. We report the case of a 27-year-old woman who had an initial diagnosis, in another hospital, of uterine adenomyoma, established by dilatation and uterine curettage and then by subsequently histopathological exam. This diagnosis led to an initial non-oncological surgery, with interannexial total hysterectomy. The establishment of the final histopathological diagnosis of stromal endometrial sarcoma has led to a serious reassessment of the case. Making a review of the literature, we found very few cases of endometrial stromal sarcoma in young women less than 30 years old and we have not identified any clear strategy of treatment. However, from precautionary and considering that may be at risk, even with very few cases reported, the distance metastases can be present, sometimes at large intervals of time, we decided, for oncological safety, reintervention after one month. At the second surgery, it was practiced bilateral salpingo-ovarectomy, cardinal ligaments excision, partial omentectomy, bilateral pelvic lymphadenectomy extended lumbo-aortic and interaortico-cava, sampling biopsy from the inguinal femoral adenopathy and re-excision of the vaginal vault. The evolution was favorable, the patient being follow-up together with the oncologist specialist.


Assuntos
Sarcoma do Estroma Endometrial/diagnóstico , Adulto , Feminino , Humanos , Sarcoma do Estroma Endometrial/patologia
13.
BMC Cancer ; 18(1): 1247, 2018 Dec 13.
Artigo em Inglês | MEDLINE | ID: mdl-30541504

RESUMO

BACKGROUND: Uterine sarcomas consist a heterogeneous group of mesenchymal gynecological malignancies with unclear therapeutic recommendations and unspecific but poor prognosis, since they usually metastasize and tend to recur very often, even in early stages. METHODS: We retrospectively analyzed all female patients with uterine sarcomas treated in our institution over the last 17 years. Clinico-pathological data, treatments and outcomes were recorded. Kaplan-Meier curves were plotted and time-to-event analyses were estimated using Cox regression. RESULTS: Data were retrieved from 61 women with a median age of 53 (range: 27-78) years, at diagnosis. Fifty-one patients were diagnosed with leiomyosarcoma (LMS), 3 with high grade endometrial stromal sarcoma (ESS), 5 with undifferentiated uterine sarcoma (UUS), 1 with Ewing sarcoma (ES) and 1 with Rhabdomyosarcoma (RS). 24 cases had stage I, 7 stage II, 14 stage III and 16 stage IV disease. Median disease-free survival (DFS) in adjuvant approach was 18.83 months, and median overall survival (OS) 31.07 months. High mitotic count (> 15 mitoses) was significantly associated with worse OS (P < 0.001) and worse DFS (P = 0.028). CONCLUSIONS: Mitotic count appears to be independent prognostic factor while further insights are needed to improve adjuvant and palliative treatment of uterine sarcomas.


Assuntos
Gerenciamento Clínico , Rabdomiossarcoma/diagnóstico , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma de Ewing/diagnóstico , Adulto , Idoso , Feminino , Grécia/epidemiologia , Humanos , Pessoa de Meia-Idade , Índice Mitótico/métodos , Índice Mitótico/tendências , Prognóstico , Estudos Retrospectivos , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/terapia , Sarcoma do Estroma Endometrial/epidemiologia , Sarcoma do Estroma Endometrial/terapia , Sarcoma de Ewing/epidemiologia , Sarcoma de Ewing/terapia
14.
Oncol Res Treat ; 41(11): 687-692, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30317238

RESUMO

Like other uterine sarcomas, low-grade endometrial stromal sarcomas (LG-ESS) are a very rare tumor entity. In the past, research studies therefore discussed the various different types of the disease in combination. In addition, the classification of endometrial stromal tumors presented difficulties for quite some time so that in earlier studies it was not always possible to precisely distinguish between LG-ESS, high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. For LG-ESS, surgery with hysterectomy and adnexectomy is the first-line treatment. The benefits of lymphadenectomy and tumor debulking are unclear. Endocrine therapy with gestagens and aromatase inhibitors is under discussion to provide adjuvant treatment for patients with advanced stages of the disease. As radiotherapy only provides locoregional control, and in view of the usually good prognosis of patients with LG-ESS, its benefits need to be weighed against its side effects. In the case of recurrence, repeat surgery is the first choice. Further research studies viewing LG-ESS as a distinct entity are needed in order to improve treatment options for patients with LG-ESS.


Assuntos
Neoplasias do Endométrio/terapia , Endométrio/patologia , Recidiva Local de Neoplasia/terapia , Sarcoma do Estroma Endometrial/terapia , Antineoplásicos Hormonais/uso terapêutico , Quimioterapia Adjuvante/métodos , Neoplasias do Endométrio/diagnóstico , Neoplasias do Endométrio/patologia , Endométrio/cirurgia , Feminino , Humanos , Histerectomia/métodos , Excisão de Linfonodo/métodos , Gradação de Tumores , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/patologia , Radioterapia Adjuvante/métodos , Ensaios Clínicos Controlados Aleatórios como Assunto , Reoperação/métodos , Salpingo-Ooforectomia/métodos , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/patologia
15.
Clín. investig. ginecol. obstet. (Ed. impr.) ; 45(3): 127-130, jul.-sept. 2018. ilus
Artigo em Espanhol | IBECS | ID: ibc-180043

RESUMO

La variante mixoide del sarcoma del estroma endometrial es una neoplasia uterina extremadamente rara caracterizada por la presencia de predominante material mixoide y células fusiformes atípicas que muestran características histopatológicas e inmunohistoquímicas de sarcoma del estroma endometrial. Presentamos 2 casos clinicopatológicos de mujeres de 54 y 76 años de edad, ambas con metrorragia posmenopáusica, útero aumentado de tamaño e imagen de tumoración dependiente de la pared miometrial, sometidas a histerectomía total con doble anexectomía con diagnóstico anatomopatológico de sarcoma del estroma endometrial variante mixoide


Myxoid endometrial stromal sarcoma is an extremely rare uterine neoplasm characterised by the presence of atypical predominantly myxoid material fusiform cells showing histopathological and immunohistochemical characteristics of endometrial stromal sarcoma. We present two clinicopathological cases of a 54-year-old woman and 76-year-old woman, both with postmenopausal vaginal bleeding, enlarged uterus and myometrial wall-dependent tumour imaging, undergoing total hysterectomy with double oophorectomy with pathological diagnosis of myxoid endometrial stromal sarcoma


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Idoso , Sarcoma do Estroma Endometrial/diagnóstico , Biópsia , Leiomioma/diagnóstico , Leiomioma/cirurgia , Sarcoma do Estroma Endometrial/patologia , Sarcoma do Estroma Endometrial/cirurgia , Leiomiossarcoma/patologia , Imuno-Histoquímica , Histerectomia/métodos
16.
Ann Diagn Pathol ; 36: 38-43, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30055523

RESUMO

Low-grade endometrial stromal sarcoma (LGESS) is the second most common malignant mesenchymal tumor of the uterus. The most common location is the uterine corpus, but it can also primarily arise in a variety of extrauterine locations such as pelvis, ovary, abdominal cavity, vagina, and vulva. We are reporting a case of a 47-year-old female with no significant medical history who presented with multiple pulmonary nodules. Fine needle aspiration (FNA) specimen revealed spindle cell neoplasm consistent with the diagnosis of LGESS. The differential diagnosis included neuroendocrine tumor, synovial sarcoma, solitary fibrous tumor, smooth muscle tumors, and peripheral nerve sheath tumors. The clinical, cytological, and histopathologic details of this case, as well as a discussion of the potential pitfalls and differential diagnosis of spindle cell lesions of the lung are described.


Assuntos
Tumores do Estroma Endometrial/patologia , Nódulos Pulmonares Múltiplos/patologia , Sarcoma do Estroma Endometrial/patologia , Sarcoma Sinovial/patologia , Biópsia por Agulha Fina/métodos , Diagnóstico Diferencial , Neoplasias do Endométrio/diagnóstico , Neoplasias do Endométrio/patologia , Tumores do Estroma Endometrial/diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade , Nódulos Pulmonares Múltiplos/diagnóstico , Gradação de Tumores/métodos , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma Sinovial/diagnóstico , Tumor de Músculo Liso/diagnóstico , Tumor de Músculo Liso/patologia
17.
Virchows Arch ; 473(1): 61-69, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29869299

RESUMO

To evaluate the immunohistochemical (IHC) expression of hormone receptors and analyze the prognostic implication of these receptors in patients with endometrial stromal sarcoma (ESS). Fifty-one patients with ESS whose paraffin blocks and pathologic slides, which were obtained after hysterectomy, were available and included in this study. Clinicopathologic data were gathered from patients' medical records, and IHC staining of hormone receptors was performed using tissue microarrays. Estrogen receptor (ER)-alpha was expressed in 37 patients (72.5%), and strong immunoreactivity was observed in 27 patients (52.9%). However, ER-beta expression was observed in only two patients (3.9%). Progesterone receptor (PR) expression was identified in 36 patients (70.6%), and strong immunoreactivity was found in 26 patients (51%). Androgen receptor (AR) expression was observed in 30 patients (58.8%), and strong immunoreactivity was noted in 14 patients (27.5%). Gonadotropin-releasing hormone receptor (GnRH-R) expression was observed in 49 patients (96.1%), but no patient exhibited strong immunoreactivity. All patients expressed CYP19A1, and 43 patients (84.3%) had strong immunoreactivity. ER-alpha, PR, and AR positivity was associated with significantly better overall survival (OS). No patient with AR positivity died of ESS. When the patients were categorized according to ER-alpha, PR, and AR immunoreactivity, triple-positive ESS had the best OS, and triple-negative ESS was linked to the worst OS. Expression of hormone receptors was associated with favorable survival outcome in ESS. Hormone receptors that showed strong expression deserve further evaluation to clarify their importance as a therapeutic target and predictor of treatment response.


Assuntos
Biomarcadores Tumorais/metabolismo , Neoplasias do Endométrio/metabolismo , Receptores de Progesterona/metabolismo , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/metabolismo , Adulto , Idoso , Neoplasias do Endométrio/diagnóstico , Neoplasias do Endométrio/patologia , Feminino , Humanos , Imuno-Histoquímica/métodos , Masculino , Pessoa de Meia-Idade , Prognóstico , Receptores Androgênicos/metabolismo , Receptores Estrogênicos/metabolismo , Sarcoma do Estroma Endometrial/patologia , Adulto Jovem
19.
Curr Opin Oncol ; 30(4): 246-251, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29846243

RESUMO

PURPOSE OF REVIEW: In this article, we focus on recent published data (2017) on the management of gynecologic sarcomas. RECENT FINDINGS: The most significant data published in 2017 develop definition of a new molecular subtype of high grade endometrial stromal sarcoma (ESS) using molecular technics added to histological analysis. The identification of a new translocation on presumed uterine leiomyosarcoma (LMS) points to refinement of nosological classification, with fragmentation of even rare tumors into distinct molecular entities: gynecologic sarcomas are now distinguished into distinct entities from a heterogeneous group of tumors. Other articles have discussed the real incidence of unsuspected sarcomas after fibroid mini-invasive surgery and evaluate the risk of relapse and dissemination after morcellation. Among several criteria, preoperative imagery could become a useful tool. For systemic treatment, no clinical trials changing practices were published, only one positive nonrandomized phase II with carboplatin and pegylated liposomal doxorubicin (PLD) in the treatment of uterine sarcomas after the conventional first line, especially in LMSs and ESSs. SUMMARY: Many articles were published on this confidential domain in oncology demonstrating interests on rare sarcomas. All specialties were represented in the literature, even though we are still waiting for urgent improvements in early diagnosis and therapeutic strategies to transform the poor prognostic of these tumors.


Assuntos
Neoplasias dos Genitais Femininos/diagnóstico , Neoplasias dos Genitais Femininos/terapia , Sarcoma/diagnóstico , Sarcoma/terapia , Ensaios Clínicos Fase II como Assunto , Feminino , Neoplasias dos Genitais Femininos/patologia , Humanos , Publicações , Ensaios Clínicos Controlados Aleatórios como Assunto , Sarcoma/patologia , Sarcoma do Estroma Endometrial/diagnóstico , Sarcoma do Estroma Endometrial/patologia , Sarcoma do Estroma Endometrial/terapia
20.
Cancer Sci ; 109(6): 1743-1752, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29660202

RESUMO

Uterine leiomyosarcoma (u-LMS) and endometrial stromal sarcoma (ESS) are among the most frequent soft tissue sarcomas, which, in adults, lead to fatal lung metastases and patients have an extremely poor prognosis. Due to their rarity and heterogeneity, there are no suitable biomarkers for diagnosis and prognosis, although some biomarker candidates have appeared. In 2017, The Cancer Genome Atlas (TCGA) Research Network's work on u-LMS has confirmed mutations and deletions in RB1, TP53 and PTEN. In addition, whole-exome sequencing of u-LMS has confirmed and demonstrated frequent alterations in TP53, RB1, α-thalassemia/mental retardation syndrome X-linked (ATRX) and mediator complex subunit 12 (MED12). MED12 is a useful biomarker to diagnose uterine-derived LMS and tumors arising from (LM) with a relatively favorable prognosis. TP53 and ATRX mutations can be important mechanisms in the pathogenesis of u-LMS and are correlated with a poor prognosis. In an update based on the 2014 WHO classification, low-grade ESS is often associated with gene rearrangement bringing about the JAZF 1-SUZ12 (formerly JAZF1-JJAZ1) fusion gene, whereas high-grade ESS is associated with the YWHAE-NUTM fusion gene. Low-grade ESS with JAZF1 rearrangement may correlate with metastasis. However, high-grade ESS with metastasis with YWHAE rearrangement shows a relatively favorable prognosis. The genetic/molecular genetic aberrations in u-LMS and ESS are reviewed, focusing on molecular biomarkers for these primary and metastatic tumors.


Assuntos
Biomarcadores Tumorais/genética , Leiomiossarcoma/genética , Sarcoma do Estroma Endometrial/genética , Neoplasias Uterinas/genética , Feminino , Humanos , Leiomiossarcoma/diagnóstico , Proteínas de Fusão Oncogênica/genética , PTEN Fosfo-Hidrolase/genética , Proteínas de Ligação a Retinoblastoma/genética , Sarcoma do Estroma Endometrial/diagnóstico , Proteína Supressora de Tumor p53/genética , Ubiquitina-Proteína Ligases/genética , Neoplasias Uterinas/diagnóstico
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