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1.
J Cancer Res Ther ; 16(4): 933-934, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32930145

RESUMO

Hemothorax cannot always be treated by thoracic surgeon. Rapidly improved interventional pulmonology broadens the application of medical thoracoscopy. We attempt to share our experiences of medical thoracoscopy for hemothorax and discuss the value of medical thoracoscopy in pleural diseases. We reported a 76-year-old male with hemothorax who was cured by medical thoracoscopy under local anesthesia together with argon plasma coagulation. Moreover, final pathological diagnosis was acquired as pleural sarcomatoid carcinoma. The unusual manifestation under medical thoracoscopy of such a relative rare disease was also described in this paper. The medical thoracoscopy could be used successfully for hemothorax instead of treating with surgeon, especially for those who cannot tolerate procedure of operation or surgical thoracoscopy.


Assuntos
Carcinossarcoma/patologia , Hemotórax/diagnóstico , Hemotórax/terapia , Derrame Pleural/patologia , Toracoscopia/métodos , Idoso , Biópsia , Carcinossarcoma/diagnóstico , Carcinossarcoma/terapia , Hemotórax/patologia , Humanos , Masculino , Doenças Pleurais/diagnóstico , Doenças Pleurais/patologia , Doenças Pleurais/terapia
2.
Am J Surg Pathol ; 44(10): 1331-1339, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32520761

RESUMO

Molecular analysis has reshaped the landscape of high grade sinonasal tumors by defining novel entities and identifying recurrent mutations in established tumor types. However, sinonasal teratocarcinosarcoma (TCS), a rare and aggressive tumor with intermixed teratomatous, carcinomatous, and sarcomatous elements, remains poorly understood. The multiphenotypic differentiation of TCS has engendered persistent controversy about its histogenesis and leads to diagnostic overlap with several other malignancies. In this study, we evaluated the molecular underpinnings of TCS to clarify its pathogenesis and diagnosis. We performed SMARCA4 immunohistochemistry (IHC) on 22 TCS and 153 other sinonasal tumors. We identified loss of SMARCA4 expression in 18 TCS (82%), including 15 (68%) with complete loss and 3 (14%) with partial loss. Although we also identified partial SMARCA4 loss in 1 of 8 SMARCB1-deficient sinonasal carcinomas (13%), SMARCA4 was intact in all other sinonasal carcinomas and neuroendocrine tumors. We then selected 3 TCS with complete SMARCA4 loss by IHC for a targeted next-generation sequencing panel that included 1425 cancer-related genes. We confirmed biallelic somatic inactivation of SMARCA4 without other known oncogenic mutations in these 3 cases. Overall, these findings suggest that SMARCA4 inactivation may be the dominant genetic event in TCS, expanding understanding of this gene's role in sinonasal tumorigenesis. They also raise the possibility that TCS is on a diagnostic spectrum with the newly described SMARCA4-deficient sinonasal carcinoma, blurring the lines between established and emerging sinonasal entities. In addition, SMARCA4 IHC may provide a useful adjunct for confirming a diagnosis of TCS in limited material.


Assuntos
Carcinossarcoma/genética , DNA Helicases/genética , Neoplasias Nasais/genética , Proteínas Nucleares/genética , Teratoma/genética , Fatores de Transcrição/genética , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/análise , Carcinossarcoma/patologia , Feminino , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Nasais/patologia , Teratoma/patologia , Adulto Jovem
3.
Oncology ; 98(10): 699-705, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32526764

RESUMO

INTRODUCTION: Carcinosarcoma is a rare cancer, and its prognosis is poor. There are few reports on the prognostic factors of patients with carcinosarcoma who receive second-line chemotherapy. OBJECTIVE: To investigate the outcome and prognostic factors of patients who received second-line chemotherapy for gynecologic carcinosarcoma. METHODS: We retrospectively investigated patients with ovarian or uterine carcinosarcoma, who were treated at two institutions from July 2006 to March 2018. All patients who had received second-line chemotherapy for advanced or recurrent disease were eligible. The efficacy of second-line chemotherapy and prognostic factors were evaluated. RESULTS: Forty-six patients were eligible. Combination chemotherapy was used in approximately half (52.2%) of the patients. The response rate and disease control rate of second-line chemotherapy were 32.6 and 60.9%, respectively. The median follow-up period was 11.0 (range, 8.8-107.5) months. The median progression-free survival and overall survival were 6.3 (95% CI, 3.2-7.5) months and 12.9 (95% CI, 7.8-16.0) months, respectively. In the multivariate analysis of overall survival, a treatment-free interval >180 days was a significant good prognostic factor. The median overall survival was 7.8 (95% CI, 5.1-10.5) months in the <180 days group and 16.4 (95% CI, 13.1-130.6) months in the >180 days group (p = 0.0052; hazard ratio, 0.26; 95% CI, 0.10-0.66), respectively. CONCLUSION: The outcome of gynecologic carcinosarcoma in the second-line setting is poor, especially in patients with a short treatment-free interval.


Assuntos
Carcinossarcoma/tratamento farmacológico , Neoplasias Ovarianas/tratamento farmacológico , Neoplasias Uterinas/tratamento farmacológico , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carboplatina/administração & dosagem , Carboplatina/uso terapêutico , Carcinossarcoma/patologia , Docetaxel/administração & dosagem , Doxorrubicina/uso terapêutico , Feminino , Humanos , Ifosfamida/administração & dosagem , Pessoa de Meia-Idade , Neoplasias Ovarianas/patologia , Paclitaxel/administração & dosagem , Prognóstico , Intervalo Livre de Progressão , Pirimidinas/uso terapêutico , Estudos Retrospectivos , Sulfonamidas/uso terapêutico , Resultado do Tratamento , Neoplasias Uterinas/patologia
4.
Eur J Cancer ; 133: 104-111, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32454416

RESUMO

BACKGROUND: Patients with International Federation of Gynaecology and Obstetrics (FIGO) stage III endometrial cancer (EC) have a substantial risk of adverse outcomes. After surgery, adjuvant therapy is recommended with external beam radiotherapy (EBRT), chemotherapy (CT) or both EBRT and CT. Recent trials suggest that EBRT + CT is superior to EBRT or CT alone but also results in more toxicity. We have compared the outcome of different adjuvant treatments in a population-based cohort to identify subgroups that benefit most from EBRT + CT. METHODS: All patients diagnosed with FIGO stage III EC and treated with surgery in 2005-2016 were identified from the Netherlands Cancer Registry. The primary outcome was overall survival (OS); associations with adjuvant treatment were analysed using Cox regression analysis. RESULTS: Among 1241 eligible patients, EBRT + CT was associated with a better OS than CT (hazard ratio [HR] = 1.84, 95% confidence interval [CI] = 1.34-2.52) and EBRT alone (HR = 1.37, 95% CI = 1.05-1.79). In stage IIIC, there was a significant benefit of EBRT + CT compared with CT or EBRT alone. In stage IIIA-B, there was no difference between EBRT + CT or EBRT alone. In endometrioid EC (EEC) and carcinosarcomas, EBRT + CT was associated with a better OS than CT or EBRT alone. For uterine serous cancers, there was no survival benefit of EBRT + CT over CT. In all analysis by stage and histology, any adjuvant treatment was superior to no adjuvant therapy. CONCLUSIONS: In this population-based study, adjuvant EBRT + CT was associated with improved OS compared with CT or EBRT alone in FIGO stage IIIC EC, EEC and carcinosarcoma. This suggests that application of EBRT + CT in stage III should be further stratified according to these subgroups.


Assuntos
Carcinoma Endometrioide/mortalidade , Carcinoma Endometrioide/terapia , Carcinossarcoma/mortalidade , Carcinossarcoma/terapia , Neoplasias do Endométrio/mortalidade , Neoplasias do Endométrio/terapia , Idoso , Idoso de 80 Anos ou mais , Braquiterapia/mortalidade , Braquiterapia/estatística & dados numéricos , Carcinoma Endometrioide/patologia , Carcinossarcoma/patologia , Quimiorradioterapia Adjuvante/métodos , Quimiorradioterapia Adjuvante/mortalidade , Quimioterapia Adjuvante/métodos , Quimioterapia Adjuvante/mortalidade , Quimioterapia Adjuvante/estatística & dados numéricos , Estudos de Coortes , Terapia Combinada/métodos , Terapia Combinada/mortalidade , Neoplasias do Endométrio/patologia , Feminino , Humanos , Histerectomia/mortalidade , Histerectomia/estatística & dados numéricos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Países Baixos/epidemiologia , Radioterapia Adjuvante/métodos , Radioterapia Adjuvante/mortalidade , Radioterapia Adjuvante/estatística & dados numéricos , Sistema de Registros , Análise de Sobrevida
5.
Anticancer Res ; 40(4): 2225-2229, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32234918

RESUMO

BACKGROUND: Primary hepatic carcinosarcoma is a rare subtype of liver malignancy, with only a small number of cases described in the English literature. CASE REPORT: We report the case of a 72-year-old man with a history of hepatitis C, who presented with complaints of abdominal pain. The patient's alpha fetoprotein (AFP) level was highly elevated at 7,406 ng/ml. His albumin, total bilirubin, aspartate aminotransferase, alanine aminotransferase, and alkaline phosphatase levels were within normal ranges. Computer tomographic scans discovered a 12×9×8 cm mass in the left lobe of the liver, extending to the anterior gastric wall. A partial hepatectomy of segments 2 and 3 with en bloc distal gastrectomy and omentectomy, a Roux-en-Y gastrojejunostomy, and a cholecystectomy were performed. Pathology revealed the mass to be a hepatic carcinosarcoma composed of collision tumor of four malignant components: hepatocellular carcinoma, cholangiocarcinoma, osteosarcoma and rhabdomyosarcoma. One and half month post-surgery, the patient was found to have a mass confirmed by biopsy as hepatocellular carcinoma in the right lobe, nodules in his lung and bone, and his AFP level elevated to 51,027.6 ng/ml. He died after two months during hospice care. CONCLUSION: To the best of our knowledge, this is the first documented case of primary hepatic carcinosarcoma with collision tumor of four malignant entities (hepatocellular carcinoma, cholangiocarcinoma, osteosarcoma and rhabdomyosarcoma). The pathogenesis, diagnosis, treatment and prognosis of this disease are discussed.


Assuntos
Carcinoma Hepatocelular/parasitologia , Carcinossarcoma/patologia , Colangiocarcinoma/patologia , Neoplasias Hepáticas/patologia , Fígado/patologia , Idoso , Neoplasias dos Ductos Biliares/diagnóstico por imagem , Neoplasias dos Ductos Biliares/patologia , Neoplasias dos Ductos Biliares/cirurgia , Biópsia/métodos , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Carcinoma Hepatocelular/diagnóstico por imagem , Carcinoma Hepatocelular/cirurgia , Carcinossarcoma/diagnóstico por imagem , Carcinossarcoma/cirurgia , Colangiocarcinoma/diagnóstico por imagem , Colangiocarcinoma/cirurgia , Colecistectomia/métodos , Evolução Fatal , Gastrectomia/métodos , Derivação Gástrica/métodos , Hepatectomia/métodos , Humanos , Fígado/diagnóstico por imagem , Fígado/cirurgia , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/cirurgia , Masculino , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/patologia , Osteossarcoma/cirurgia , Prognóstico , Rabdomiossarcoma/diagnóstico por imagem , Rabdomiossarcoma/patologia , Rabdomiossarcoma/cirurgia , Tomografia Computadorizada por Raios X , alfa-Fetoproteínas/metabolismo
6.
Bull Cancer ; 107(3): 385-390, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32115180

RESUMO

The group of rare malignant ovarian tumors includes the group of germ cell tumors, sex cords stromal ovarian tumors, small cell carcinoma, malignant Brenner tumors, rare epithelial tumors such as mucinous carcinoma, clear cell carcinoma, or low-grade serous carcinoma, as well as ovarian carcinosarcoma. Together they comprise about 10% of all ovarian tumors. Due to their low prevalence and their heterogeneity, data and treatment recommendations are limited. Even though all ovarian tumors are staged according to the FIGO staging of epithelial ovarian tumors, treatment differs especially in germ cell tumors and sex cords stromal ovarian tumors. Non-epithelial ovarian tumors can arise from a variety of ovarian precursor cells such as germ cells, granulosa cells, theca cells, or stromal fibroblasts. As can be expected already due to their divergent precursor lesions, these malignancies are substantially different but united by their rarity. This overview article gives a comprehensive summary on the pathology and clinical presentation, as well as therapy recommendations of a selection of those rare ovarian tumors, based on the latest national guidelines and related important publications.


Assuntos
Neoplasias Ovarianas , Doenças Raras , Adenocarcinoma de Células Claras/patologia , Adenocarcinoma de Células Claras/terapia , Adenocarcinoma Mucinoso/patologia , Adenocarcinoma Mucinoso/terapia , Tumor de Brenner/patologia , Tumor de Brenner/terapia , Carcinoma de Células Pequenas/patologia , Carcinoma de Células Pequenas/terapia , Carcinossarcoma/patologia , Carcinossarcoma/terapia , Cistadenocarcinoma Seroso/patologia , Cistadenocarcinoma Seroso/terapia , Feminino , Humanos , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/terapia , Neoplasias Ovarianas/patologia , Neoplasias Ovarianas/terapia , Doenças Raras/patologia , Doenças Raras/terapia , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/terapia
7.
J Urol ; 204(2): 260-266, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32141804

RESUMO

PURPOSE: The American Joint Committee on Cancer recognizes 6 rare histological variants of prostate adenocarcinoma. We describe the contemporary presentation and overall survival of these rare variants. MATERIALS AND METHODS: We examined 1,345,618 patients who were diagnosed with prostate adenocarcinoma between 2004 and 2015 within the National Cancer Database. We focused on the variants mucinous, ductal, signet ring cell, adenosquamous, sarcomatoid and neuroendocrine. Characteristics at presentation for each variant were compared with nonvariant prostate adenocarcinoma. Cox regression was used to study the impact of histological variant on overall mortality. RESULTS: Few (0.38%) patients presented with rare variant prostate adenocarcinoma. All variants had higher clinical tumor stage at presentation than nonvariant (all p <0.001). Metastatic disease was most common with neuroendocrine (62.9%), followed by sarcomatoid (33.3%), adenosquamous (31.1%), signet ring cell (10.3%) and ductal (9.8%), compared to 4.2% in nonvariant (all p <0.001). Metastatic disease in mucinous (3.3%) was similar to nonvariant (p=0.2). Estimated 10-year overall survival was highest in mucinous (78.0%), followed by nonvariant (71.1%), signet ring cell (56.8%), ductal (56.3%), adenosquamous (20.5%), sarcomatoid (14.6%) and neuroendocrine (9.1%). At multivariable analysis, mortality was higher in ductal (HR 1.38, p <0.001), signet ring cell (HR 1.53, p <0.01), neuroendocrine (HR 5.72, p <0.001), sarcomatoid (HR 5.81, p <0.001) and adenosquamous (HR 9.34, p <0.001) as compared to nonvariant. CONCLUSIONS: Neuroendocrine, adenosquamous, sarcomatoid, signet ring cell and ductal variants more commonly present with metastases. All variants present with higher local stage than nonvariant. Neuroendocrine is associated with the worst and mucinous with the best overall survival.


Assuntos
Adenocarcinoma/patologia , Neoplasias da Próstata/patologia , Adenocarcinoma/mortalidade , Adenocarcinoma Mucinoso/mortalidade , Adenocarcinoma Mucinoso/patologia , Carcinoma Adenoescamoso/mortalidade , Carcinoma Adenoescamoso/patologia , Carcinoma Ductal/mortalidade , Carcinoma Ductal/patologia , Carcinoma de Células em Anel de Sinete/mortalidade , Carcinoma de Células em Anel de Sinete/patologia , Carcinossarcoma/mortalidade , Carcinossarcoma/patologia , Bases de Dados Factuais , Humanos , Masculino , Estadiamento de Neoplasias , Neoplasias da Próstata/mortalidade , Taxa de Sobrevida , Estados Unidos
8.
Ann Ital Chir ; 92020 Jan 19.
Artigo em Inglês | MEDLINE | ID: mdl-32129176

RESUMO

BACKGROUND: Uterine sarcomas are mesenchymal tumors; they are rare, representing less than 2-3% of all uterine malignancies. Among them, we can define four types: leiomyosarcoma (LMS), endometrial stromal sarcoma (ESS), Adenosarcoma and Carcinosarcoma. This last type was recently reclassified by FIGO as a Mullerian type of the endometrial adenocarcinoma. Therefore, today only the first three types are histologically considered. METHODS: In this paper, we reported a case of simultaneous presence of three different rare neoplasms in the same surgical specimen, resulting from a hysterectomy of a premenopausal woman. The woman presented to the ED with a six-months history of vaginal bleeding. Given the complexity of the clinical picture, we suggested hospitalization in our Department of Gynecology, to perform appropriate diagnostic tests. Because of the persistent hemorrhage and the absence of required fertility preservation, a laparotomic hysterectomy with bilateral annessiectomy was performed. RESULTS: The postoperative histology of the specimen described the myoma at the fundus as a leiomyosarcoma. The myoma of the uterine anterior wall appeared as an endometrial stromal sarcoma of low-grade. Moreover, an intramural cavernous hemangioma of 3 cm in diameter was reported at the uterine corpus. CONCLUSION: All these described pathologies have no specific clinic characteristics; the most common symptom is abnormal uterine bleeding. To date, hysterectomy and bilateral salpingo-oophorectomy are the standards of care in the management of all early stage uterine sarcomas. To our knowledge, cases of LMS, ESS and cavernous haemangioma coexisting in the same patient have not been reported in literature to date. The pathogenesis of this combination remains to be elucidated. Key words: Cavernous hemangioma, Endometrial stromal sarcoma, Leiomyosarcoma, Uterine sarcomas.


Assuntos
Adenossarcoma/patologia , Carcinossarcoma/patologia , Leiomiossarcoma/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Uterinas/patologia , Adenossarcoma/cirurgia , Carcinossarcoma/cirurgia , Feminino , Humanos , Histerectomia , Leiomiossarcoma/cirurgia , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/cirurgia , Neoplasias Uterinas/cirurgia
9.
BMC Cancer ; 20(1): 248, 2020 Mar 24.
Artigo em Inglês | MEDLINE | ID: mdl-32209061

RESUMO

BACKGROUND: This study was designed to investigate the clinicopathologic features of pulmonary blastomatoid carcinosarcoma and explore the genomic profiles of epithelial and mesenchymal components in this tumor. METHODS: Three cases of pulmonary blastomatoid carcinosarcoma were enrolled in this study. Clinicopathologic information and prognostic data were retrospectively reviewed. Diagnostic immunohistochemistry was performed. The epithelial and mesenchymal components were microdissected to investigate the genomic profiles by performing capture-based targeted next generation sequencing. RESULTS: The epithelial components in patient one consisted of low-grade and high-grade fetal lung adenocarcinoma. Low-grade epithelial cells showed nuclear expression of ß-catenin and missense mutation of CTNNB1. The epithelial components in another two patients consisted of high-grade fetal lung adenocarcinoma/enteric adenocarcinoma. The epithelial cells showed membrane staining of ß-catenin and harbored no mutation of CTNNB1. The mesenchymal components in all three tumors were composed of primitive round/spindle cells without definite differentiation and showed cytoplasmic dot positive of ß-catenin and no corresponding mutation. Within a tumor, both components exhibited relatively comparable molecular profile. In patient one, 4 mutations: RB1, FAT3, PTCH1 and LRP1B were shared by both epithelial and mesenchymal components. Epithelial component had additional mutations in BCOR, CTNNB1, CTCF, FAT1 and DICER1. In patient two, 12 mutations were shared. The epithelial component had BRCA2 mutation and the mesenchymal had mutations in CREBBP, ALK, DNMT3A, ASXL2, MYCN and RICTOR. Patient three had 6 shared mutations. The epithelial component had an additional mutation in KAT6A and the mesenchymal had an additional mutation in APC. Collectively, we observed heterogeneity between epithelial and mesenchymal components of the same tumor. CONCLUSIONS: Blastomatoid carcinosarcoma showed characteristic morphology and immunophenotype. Parallel detection of genetic abnormalities in epithelial and mesenchymal components could provide further evidence for tumor differentiation, molecular targeting and differential diagnosis.


Assuntos
Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Carcinossarcoma/patologia , Neoplasias Pulmonares/patologia , Blastoma Pulmonar/patologia , Análise de Sequência de DNA/métodos , Proteína da Polipose Adenomatosa do Colo/genética , Adulto , Idoso , Proteína BRCA2/genética , Carcinossarcoma/genética , Carcinossarcoma/metabolismo , Núcleo Celular/metabolismo , Feminino , Redes Reguladoras de Genes , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/metabolismo , Masculino , Pessoa de Meia-Idade , Mutação , Gradação de Tumores , Blastoma Pulmonar/genética , Blastoma Pulmonar/metabolismo , Estudos Retrospectivos , beta Catenina/genética , beta Catenina/metabolismo
10.
Diagn Pathol ; 15(1): 12, 2020 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-32035484

RESUMO

BACKGROUND: Squamous cell carcinoma is the most common malignant tumor of the uterine cervix with a well-documented link to infection with human papillomaviruses (HPV). According to a recent classification, there are several morphological variants of cervical squamous carcinoma, without reference to sarcomatoid squamous cell carcinoma, which is well described in other organs. CASE PRESENTATION: In this paper, we describe an extremely rare case of a 77-year-old woman with primary malignant cervical tumor displaying biphasic histomorphology with an epithelioid and sarcomatoid part; the latter was immunohistochemistry positive for cytokeratin and vimentin. The association with a high-grade squamous intraepithelial lesion and molecular proof of HPV33 infection in the tumor tissue supported our diagnosis of carcinoma with partial sarcomatoid differentiation. CONCLUSION: We report a rare case of a primary cervical epithelial tumor with a partial sarcomatoid phenotype, an unequivocal HPV infection, and an associated precancerous lesion in the cervical mucosa. This is the first description of an HPV33 infection underlying a biphasic epithelioid-sarcomatous tumor of the uterine cervix. The terminology overlap between sarcomatoid carcinoma and carcinosarcoma is also discussed.


Assuntos
Carcinoma de Células Escamosas/patologia , Carcinossarcoma/patologia , Colo do Útero/patologia , Infecções por Papillomavirus/virologia , Neoplasias do Colo do Útero/patologia , Idoso , Carcinoma de Células Escamosas/complicações , Carcinoma de Células Escamosas/diagnóstico , Carcinossarcoma/complicações , Carcinossarcoma/diagnóstico , Diferenciação Celular/fisiologia , Colo do Útero/virologia , Feminino , Humanos , Queratinas/metabolismo , Sarcoma/patologia , Sarcoma/virologia , Neoplasias do Colo do Útero/diagnóstico
11.
Diagn Pathol ; 15(1): 7, 2020 Jan 31.
Artigo em Inglês | MEDLINE | ID: mdl-32005258

RESUMO

BACKGROUND: Cutaneous pilomatrical carcinosarcoma (CS) is a very rare biphasic tumor composed of admixed epithelial and mesenchymal malignant cells, with limited information on its pathogenesis. We report a case of pilomatrical CS of the scalp with comparative immunohistochemical and molecular analysis together with a review of the literature. CASE PRESENTATION: A 74-year-old woman presented with a rapidly growing long-standing tumor of the scalp. The tumor was surgically resected. Histologically, the tumor was 25 mm in diameter, and was composed of carcinoma showing a clear pilomatrical differentiation and sarcoma with pleomorphic spindle cells and giant cells. Both epithelial and mesenchymal components shared focal cytoplasmic and/or nuclear accumulation of ß-catenin based on immunohistochemical analysis, although a mutation of exon 3 of the CTNNB1 gene was not detected. Fluorescence in situ hybridization analysis revealed gains of chromosomes 9p21, 3, and 7 in both the epithelial and sarcomatous components. CONCLUSIONS: The current case demonstrated characteristic findings of pilomatricoma and further evidence of partial clonality between the carcinomatous and sarcomatous component, suggesting the possibility of malignant transformation of pilomatricoma. Rapid growth of a pilomatrical tumor should warrant the development of a malignant tumor, including CS.


Assuntos
Carcinossarcoma/diagnóstico , Pilomatrixoma/diagnóstico , Sarcoma/diagnóstico , Idoso , Carcinossarcoma/patologia , Transformação Celular Neoplásica , Feminino , Humanos , Hibridização in Situ Fluorescente , Pilomatrixoma/patologia , Sarcoma/patologia , Pele/patologia
12.
Gynecol Oncol ; 157(1): 67-77, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32029291

RESUMO

OBJECTIVE: To investigate racial disparities in uterine carcinosarcoma (UCS) and ovarian carcinosarcoma (OCS) in Commission on Cancer®-accredited facilities. METHODS: Non-Hispanic Black (NHB) and non-Hispanic White (NHW) women in the National Cancer Database diagnosed with stage I-IV UCS or OCS between 2004 and 2014 were eligible. Differences by disease site or race were compared using Chi-square test and multivariate Cox analysis. RESULTS: There were 2830 NHBs and 7366 NHWs with UCS, and 280 NHBs and 2586 NHWs with OCS. Diagnosis of UCS was more common in NHBs (11.5%) vs. NHWs (3.7%) and increased with age (P < .0001). OCS diagnosis remained <5% in both races and all ages. NHBs with UCS or OCS were more common in the South and more likely to have a comorbidity score ≥ 1, low neighborhood income and Medicaid or no insurance (P < .0001). Diagnosis at stage II-IV was more common in NHBs than NHWs with UCS but not OCS. NHBs with both UCS and OCS were less likely to undergo surgery and to achieve no gross residual disease with surgery (P = .002). Risk of death in NHB vs. NHW patients with UCS was 1.38 after adjustment for demographic factors and dropped after sequential adjustment for comorbidity score, neighborhood income, insurance status, stage and treatment by 4%, 16%, 7%, 19% and 10%, respectively, leaving 43.5% of the racial disparity in survival unexplained. In contrast, risk of death in NHBs vs. NHWs with OCS was 1.19 after adjustment for demographic factors and became insignificant after adjustment for comorbidity. Race was an independent prognostic factor in UCS but not in OCS. CONCLUSIONS: Racial disparities exist in characteristics, treatment and survival in UCS and OCS with distinctions that merit additional research.


Assuntos
Grupo com Ancestrais do Continente Africano/estatística & dados numéricos , Carcinossarcoma/etnologia , Grupo com Ancestrais do Continente Europeu/estatística & dados numéricos , Disparidades nos Níveis de Saúde , Disparidades em Assistência à Saúde/estatística & dados numéricos , Neoplasias Ovarianas/etnologia , Neoplasias Uterinas/etnologia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Carcinossarcoma/mortalidade , Carcinossarcoma/patologia , Comorbidade , Feminino , Humanos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias Ovarianas/mortalidade , Neoplasias Ovarianas/patologia , Prognóstico , Neoplasias Uterinas/mortalidade , Neoplasias Uterinas/patologia
16.
Interact Cardiovasc Thorac Surg ; 30(1): 4-10, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31518405

RESUMO

OBJECTIVES: Pulmonary carcinosarcoma (PCS) is a rare neoplasm. This study explored the clinicopathological characteristics and survival outcomes of PCS. METHODS: The Surveillance, Epidemiology and End Results (SEER) database (1988-2014) was queried for PCS. Overall survival (OS) was evaluated by multivariable Cox regression and nomograms were constructed to predict 3-year OS for PCS. Prognostic performance was evaluated using concordance index and area under the curve analysis. In M0 surgically treated patients, interaction assessments were performed using likelihood ratio tests. Subgroup analysis was performed according to patient age. The clinical features of PCSs were further compared to other non-small-cell lung cancers (NSCLCs). RESULTS: Multivariable analysis identified age [hazard ratio (HR) 1.03, 95% confidence interval (CI) 1.01-1.04], surgery (HR 0.53, 95% CI 0.36-0.77) and chemotherapy (HR 0.51, 95% CI 0.36-0.73) as significantly associated with OS. The nomogram had a concordance index of 0.747 and an area under the curve of 0.803. The association between age and OS was stronger in those receiving pneumonectomy (P = 0.04 for interactions) compared to those that did not (HR 5.14, 95% CI 1.64-16.07), and was associated with a poorer outcome compared to lobectomy amongst the elderly (age ≥ 70 years). Patients with PCS were more likely to receive surgical treatment and had lower lymphatic metastasis compared to adenocarcinoma, squamous cell carcinoma and large cell carcinoma (all P < 0.05). CONCLUSIONS: PCS had unique clinical features compared to common types of NSCLCs in terms of lymphatic invasion and surgical treatment. Pneumonectomy was associated with poorer survival in elderly patients.


Assuntos
Carcinossarcoma/mortalidade , Neoplasias Pulmonares/mortalidade , Idoso , Carcinossarcoma/patologia , Carcinossarcoma/cirurgia , Feminino , Humanos , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/cirurgia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Nomogramas , Pneumonectomia/mortalidade , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Programa de SEER
17.
Clin J Gastroenterol ; 13(1): 110-115, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31264080

RESUMO

We present an extremely rare case of carcinosarcoma with 4 different tumor components in an 88-year-old female. After a diagnosis of acute cholecystitis, we performed percutaneous transhepatic gallbladder drainage in the patient without success, followed by a cholecystectomy and choledocholithotomy. The mass was a 60 × 25 mm polypoid lesion of the gallbladder identified histologically as a carcinosarcoma with adenocarcinoma, neuroendocrine carcinoma, undifferentiated carcinoma and chondrosarcoma components. The biliary-type adenocarcinoma portion exhibited acinar growth patterns with columnar cells having large and markedly hyperchromatic nuclei. These tumor cells were immunohistochemically positive for MUC1 and CDX2. The neuroendocrine carcinoma, small cell type, cells were densely packed and small, with scant cytoplasm, finely granular nuclear chromatin and absence of nucleoli. The mitotic index was high. These tumor cells were immunohistochemically positive for synaptophysin, Ki-67 (index 40%), MUC1, CDX2 and c-Kit. The undifferentiated carcinoma consisted exclusively of spindle cells containing large, markedly hyperchromatic nuclei with a high mitotic index. These tumor cells were immunohistochemically positive for AE1/AE3. The chondrosarcoma was composed of blue-gray chondroid matrix and atypical chondrocytes containing large, hyperchromatic nuclei. These tumor cells were immunohistochemically positive for S100. Its attributes might be suggestive of a greater malignant potential and pathogenesis of carcinosarcoma.


Assuntos
Adenocarcinoma/patologia , Carcinoma Neuroendócrino/patologia , Carcinossarcoma/patologia , Condrossarcoma/patologia , Neoplasias da Vesícula Biliar/patologia , Tumor Misto Maligno/patologia , Adenocarcinoma/complicações , Adenocarcinoma/diagnóstico por imagem , Adenocarcinoma/metabolismo , Idoso , Fator de Transcrição CDX2/metabolismo , Carcinoma/complicações , Carcinoma/diagnóstico por imagem , Carcinoma/metabolismo , Carcinoma/patologia , Carcinoma Neuroendócrino/complicações , Carcinoma Neuroendócrino/diagnóstico por imagem , Carcinoma Neuroendócrino/metabolismo , Carcinossarcoma/complicações , Carcinossarcoma/diagnóstico por imagem , Carcinossarcoma/metabolismo , Colecistectomia , Colecistite Aguda/complicações , Colecistite Aguda/diagnóstico por imagem , Colecistite Aguda/cirurgia , Colecistolitíase/complicações , Colecistolitíase/diagnóstico por imagem , Colecistolitíase/cirurgia , Coledocolitíase/complicações , Coledocolitíase/diagnóstico por imagem , Coledocolitíase/cirurgia , Condrossarcoma/complicações , Condrossarcoma/diagnóstico por imagem , Condrossarcoma/metabolismo , Feminino , Neoplasias da Vesícula Biliar/complicações , Neoplasias da Vesícula Biliar/diagnóstico por imagem , Neoplasias da Vesícula Biliar/metabolismo , Humanos , Antígeno Ki-67/metabolismo , Tumor Misto Maligno/complicações , Tumor Misto Maligno/diagnóstico por imagem , Tumor Misto Maligno/metabolismo , Mucina-1/metabolismo , Proteínas Proto-Oncogênicas c-kit/metabolismo , Proteínas S100/metabolismo , Tomografia Computadorizada por Raios X
18.
Am J Dermatopathol ; 42(3): 208-210, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31633597

RESUMO

We present a case report of the exceptionally rare pilomatrical carcinosarcoma in an even rarer pediatric age group, a 9-year-old female patient. The tumor showed biphasic pilomatrical carcinoma and malignant sarcomatous transformation. To date, the patient is healing well without signs of recurrence. Although limited clinical follow-up is available due to the recent diagnosis, this case may provide a rare look at the clinical outcome of this very rare tumor.


Assuntos
Carcinossarcoma/patologia , Doenças do Cabelo/patologia , Pilomatrixoma/patologia , Neoplasias Cutâneas/patologia , Criança , Feminino , Humanos
19.
Breast ; 49: 157-164, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31812891

RESUMO

OBJECTIVES: Carcinosarcoma of the breast is a rare disease. Its clinicopathological features and prognosis are not well defined. The aim of this study was to compare the clinicopathological features and clinical outcome between breast carcinosarcoma and breast invasive ductal carcinoma (IDC). MATERIALS AND METHODS: Patients with breast carcinosarcoma and breast IDC were identified through the Surveillance, Epidemiology, and End Results (SEER) database from 2010 to 2015. Then a comparison was conducted between these two groups. Propensity score matching (PSM) was performed to balance the effects of baseline clinicopathological differences. The Cox proportional hazard model was used to identify potential prognostic factors of breast carcinosarcoma. RESULTS: In total, we identified 63 patients with breast carcinosarcoma and 200,596 cases with breast IDC. Comparing with IDC, breast carcinosarcoma was significantly correlated with higher grading, higher staging, larger tumor size, lower lymph node involvement, and a higher proportion of triple negative breast cancer (TNBC), suggesting a significantly worse clinical outcome. After adjusting for the uneven clinicopathological variables with PSM, significant differences were still observed between these two histology types. Subgroup analysis further showed that carcinosarcoma-TNBC has an inferior clinical outcome compared with IDC-TNBC. Finally, we identified independent prognostic factors, namely, stage, tumor size, and distant metastasis. CONCLUSION: It is concluded that breast carcinosarcoma has distinct clinicopathological features and a significantly worse clinical outcome than common IDC.


Assuntos
Neoplasias da Mama/mortalidade , Carcinoma Ductal de Mama/mortalidade , Carcinossarcoma/mortalidade , Adulto , Idoso , Neoplasias da Mama/patologia , Carcinoma Ductal de Mama/patologia , Carcinossarcoma/patologia , Feminino , Humanos , Estimativa de Kaplan-Meier , Metástase Linfática , Pessoa de Meia-Idade , Gradação de Tumores , Metástase Neoplásica , Estadiamento de Neoplasias , Prognóstico , Pontuação de Propensão , Modelos de Riscos Proporcionais , Programa de SEER , Neoplasias de Mama Triplo Negativas/mortalidade , Neoplasias de Mama Triplo Negativas/patologia , Carga Tumoral
20.
Lab Invest ; 100(5): 682-695, 2020 05.
Artigo em Inglês | MEDLINE | ID: mdl-31857700

RESUMO

Uterine carcinosarcoma (UCS) represents a true example of cancer associated with epithelial-mesenchymal transition (EMT), which exhibits cancer stem cell (CSC)-like traits. Although S100A4 is an inducer of EMT, little is known about its involvement in UCS tumorigenesis. Herein, we focused on the functional role of S100A4 during development of UCS. Expression of S100A4 and molecules associated with its function were also examined in 35 UCS cases. In endometrial carcinoma cell lines, S100A4 promoter activity and mRNA levels were significantly increased by the transfection of NF-κB/p65, independent of a putative κB-binding site in the promoter. Cells stably overexpressing S100A4 showed enhancement of CSC properties, along with decreased cell proliferation and acceleration of cell migration. These phenotypes were abrogated in S100A4-knockdown cells. A combination of S100A4 antibody-mediated co-immunoprecipitation and shotgun proteomics analysis revealed that S100A4 strongly interacted with non-muscle myosin II (NMII) heavy chains, including myosin 9 and myosin 14. Specific inhibition of NMII by blebbistatin phenocopied S100A4 overexpression and induced a fibroblast-like morphology. In clinical samples, S100A4 score was significantly higher in sarcomatous as compared with carcinomatous components of UCS, and was positively correlated with ALDH1, Slug, and vimentin scores, and inversely with Ki-67 labeling indices. These findings suggest that an S100A4/NMII-related signaling cascade may contribute to the establishment and maintenance of EMT/CSC properties, along with changes in cell proliferation and migration capability. These events may be initiated in carcinomatous components in UCS and lead to divergent sarcomatous differentiation.


Assuntos
Carcinossarcoma/patologia , Transição Epitelial-Mesenquimal/fisiologia , Proteína A4 de Ligação a Cálcio da Família S100 , Transdução de Sinais/fisiologia , Neoplasias Uterinas/patologia , Carcinossarcoma/química , Linhagem Celular Tumoral , Feminino , Técnicas de Silenciamento de Genes , Humanos , Proteína A4 de Ligação a Cálcio da Família S100/genética , Proteína A4 de Ligação a Cálcio da Família S100/metabolismo , Neoplasias Uterinas/química , Útero/química , Útero/patologia
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