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Objective To explore the clinicopathological features and prognosis of the patients newly diagnosed with lung adenocarcinoma with both EGFR mutation and C-MET amplification.Methods The pathological sections were reviewed.EGFR mutation was detected by amplification refractory mutation system-quantitative real-time polymerase chain reaction,and C-MET amplification by fluorescence in situ hybridization.The clinicopathological features and survival data of the patients newly diagnosed with lung adenocarcinoma with both EGFR mutation and C-MET amplification were analyzed retrospectively.Results In 11 cases of EGFR mutation combined with C-MET amplification,complex glands and solid high-grade components were observed under a microscope in 10 cases except for one case with a cell block,the tissue structure of which was difficult to be evaluated.The incidence of lung adenocarcinoma in the patients with EGFR mutation combined with C-MET amplification at clinical stage â £ was higher than that in the EGFR mutation or C-MET amplification group (all P<0.001),whereas the difference was not statistically significant between the EGFR mutation group and C-MET amplification group at each clinical stage (all P>0.05).There was no significant difference in the trend of survival rate between EGFR gene group and C-MET amplification group (χ2=0.042,P=0.838),while the survival of the patients with EGFR mutation combined with C-MET amplification was worse than that of the patients with EGFR mutation (χ2=246.72,P<0.001) or C-MET amplification (χ2=236.41,P<0.001).Conclusions The patients newly diagnosed with lung adenocarcinoma with EGFR mutation plus C-MET amplification demonstrate poor histological differentiation,rapid progress,and poor prognosis.The patients are often in the advanced stage when being diagnosed with cancer.Attention should be paid to this concurrent adverse driving molecular event in clinical work.With increasing availability,the inhibitors targeting C-MET may serve as an option to benefit these patients in the near future.
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Adenocarcinoma de Pulmão , Neoplasias Pulmonares , Humanos , Hibridização in Situ Fluorescente , Estudos Retrospectivos , Prognóstico , Adenocarcinoma de Pulmão/genética , Mutação , Neoplasias Pulmonares/genética , Receptores ErbB/genéticaRESUMO
Objective To analyze the clinicopathological characteristics of lymphadenitis caused by Talaromyces marneffei (TM).Method s The clinical data,pathological features,pathogen examination,and treatment of 15 cases of TM-caused lymphadenitis were analyzed retrospectively.Results The 15 cases included 14 males and 1 females,who were aged 26-67 years,with an average age of (49.1±11.87) years.The 15 cases,including 13 cases of acquired immunodeficiency syndrome and 2 cases of diabetes mellitus,were accompanied by superficial lymph node enlargement in the neck and supraclavicular,axillary,and inguinal regions.The structure of cord-like lymph node tissue punctured by thick needle was completely or partially replaced by inflammatory lesions. Under microscope,8 cases showed mainly diffuse infiltration of phagocytes with pathogens;5 cases presented mainly extensive coagulation necrosis with a small amount of pathogens and nuclear debris;2 cases were characterized by small nodular hyperplasia of fibroblasts,formation of granulomatous structure,and scattered distribution of a few multinucleated giant cells.The pathogens were relatively consistent in size and shape,which were round,oval or sausage-shaped and clustered like mulberry.Diastase periodic acid-Schiff staining and hexamine silver staining highlighted the bacterial structure with transverse septum.TM growth was detected in the blood,alveolar lavage fluid,sputum or lymph node extract fungal culture of the 15 patients.Owing to the adequate antifungal treatment in time,these 15 patients were discharged after their conditions were improved.Conclusion Lymphadenitis is one of the major manifestations of the systemic invasion of TM at the late stage,which is tended to be misdiagnosed.Through core needle biopsy of lymph node,it can be diagnosed as soon as possible to avoid delayed treatment and improve the cure rate.
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Linfadenite , Micoses , Talaromyces , Adulto , Idoso , Biópsia com Agulha de Grande Calibre , Feminino , Humanos , Linfadenite/diagnóstico , Linfadenite/microbiologia , Masculino , Pessoa de Meia-Idade , Micoses/complicações , Estudos RetrospectivosRESUMO
Epithelioid angiomyolipoma (EAML) is a rare variant of angiomyolipoma (AML). Previous studies have demonstrated that epithelial (E-)cadherin is expressed in two subtypes of AML, EAML and triphasic AML; however, the expression pattern of E-cadherin remains unclear. In the present study, a preliminary case-control study was conducted to determine the expression pattern of E-cadherin between EAML and triphasic AML, the control, focusing on the subcellular localization and expression category of E-cadherin. No significant difference was identified in the age, sex, history of tuberous sclerosis, smoking and alcohol consumption between the two groups (P>0.05). In EAML, 9 patients were categorized as exhibiting a low risk of malignant behavior and the other two were categorized as exhibiting an intermediate or high risk of malignant behavior. The proportion of cases expressing E-cadherin, human melanoma black-45 (HMB45), melanoma antigen recognized by T cells 1 (Mart1/Melan A), smooth muscle actin and progesterone receptor were 95.5 (21/22), 95.5 (21/22), 86.4 (19/22), 77.3 (17/22) and 86.4% (19/22), respectively. E-cadherin was identified to be localized, using staining techniques, in the cell membrane and/or cytoplasm. The subcellular localization of E-cadherin was significantly different between EAML and triphasic AML; the majority of EAML cases revealed membranous and cytoplasmic staining, whereas triphasic AML cases demonstrated cytoplasmic staining (P=0.0093). The expression of E-cadherin may be positively associated with HMB45 (P=0.0044) and Mart1/Melan A (P=0.0049). The results of the present study identified that the subcellular localization of E-cadherin may be different between EAML and the control group of triphasic AML. Additionally, E-cadherin and melanocytic markers may be co-expressed in distinct subtypes of AML. A follow-up study with a large sample size to validate the results of the present study, followed by a mechanistic study based on cell lines to determine any significance, are warranted.
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Localization of burn was variable: head and face in 76 patients (29%), trunk in 58 (49%), upper limb in 37 (52%), lower limbs in 44 (41%), hands in 16 (15%), perinea area in 26 (5.5%) and whole body except perinea area in 10 (9%) patients. Inhalation syndrome was present in 56 (44%) patients. Ninety patients (82%) had indwelling venous catheters, 83 (75.5%) patients' arterial catheter and 86 (78%) patients' urinary catheters. By multivariate analysis: age ≤4 years, Garcés 4, colistin use in documented multiresistant infections, and mechanical ventilation were independent variables related with mortality and graft requirement was a protective factor for mortality. Despite advances in care, gram negative bacterial infections and infection with Pseudomonas aeruginosa remain the most common cause of bacteria related mortality early in the hospital course. Viral infections are also associated with mortality and numbers have remained stable when compared to data from prior years.
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BACKGROUND: Vascular endothelial growth factor-targeted agents are standard treatments in advanced clear-cell renal cell carcinoma (ccRCC), but biomarkers of activity are lacking. The aim of this study was to investigate the association of Von Hippel-Lindau (VHL) gene status, vascular endothelial growth factor receptor (VEGFR) or stem cell factor receptor (KIT) expression, and their relationships with characteristics and clinical outcome of advanced ccRCC. METHODS: A total of 59 patients who received targeted treatment with sunitinib or pazopanib were evaluated for determination at Cancer Hospital and Institute, Chinese Academy of Medical Sciences between January 2010 and November 2012. Parafï¬n-embedded tumor samples were collected and status of the VHL gene and expression of VEGFR and KIT were determined by VHL sequence analysis and immunohistochemistry. Clinical-pathological features were collected and efficacy such as response rate and Median progression-free survival (PFS) and overall survival (OS) were calculated and then compared based on expression status. The Chi-square test, the Kaplan-Meier method, and the Lon-rank test were used for statistical analyses. RESULTS: Of 59 patients, objective responses were observed in 28 patients (47.5%). The median PFS was 13.8 months and median OS was 39.9 months. There was an improved PFS in patients with the following clinical features: Male gender, number of metastatic sites 2 or less, VEGFR-2 positive or KIT positive. Eleven patients (18.6%) had evidence of VHL mutation, with an objective response rate of 45.5%, which showed no difference with patients with no VHL mutation (47.9%). VHL mutation status did not correlate with either overall response rate (P = 0.938) or PFS (P = 0.277). The PFS was 17.6 months and 22.2 months in VEGFR-2 positive patients and KIT positive patients, respectively, which was significantly longer than that of VEGFR-2 or KIT negative patients (P = 0.026 and P = 0.043). CONCLUSION: VHL mutation status could not predict the efficacy of sunitinib or pazopanib. Further investigation of VHL/VEGFR pathway components is needed.
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Carcinoma de Células Renais/genética , Neoplasias Renais/genética , Adulto , Idoso , Antineoplásicos/uso terapêutico , Carcinoma de Células Renais/mortalidade , Carcinoma de Células Renais/patologia , Intervalo Livre de Doença , Feminino , Humanos , Imuno-Histoquímica , Indazóis , Indóis/uso terapêutico , Neoplasias Renais/mortalidade , Neoplasias Renais/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Pirimidinas/uso terapêutico , Pirróis/uso terapêutico , Sulfonamidas/uso terapêutico , Sunitinibe , Fator A de Crescimento do Endotélio Vascular/genética , Proteína Supressora de Tumor Von Hippel-Lindau/genética , Adulto JovemRESUMO
OBJECTIVE: Since the principles of treatment of primary esophageal small cell carcinoma (PESCC) remain still in controversy, the aim of this study was to investigate the clinical characteristics, treatment modalities and prognostic factors of this malignancy. METHODS: The clinical data of 109 patients treated by surgery in our hospital between October 1989 and April 2009 were retrospectively reviewed and analyzed. According to the most recently published TNM staging system for esophageal cancer (AJCC 2009), there were 17 patients in stage Ib, 31 patients in stage II, 59 patients in stage III, and 2 patients in stage IV. All the data were analyzed using SPSS 15.0 software. The median survival time (MST) and overall survival rate (OS) were calculated and compared by the Kaplan-Meier method and log-rank test. The prognostic factors were calculated by Cox hazard regression model. RESULTS: Among all the 109 patients included, 93 patients were treated by radical esophagectomy, and 11 patients by palliative resection, while 5 patients by exploration. The median survival time (MST) of the whole group was 14.4 months and the 1-, 3- and 5-year overall survival rates (OS) were 56.9%, 17.6%, and 12.0%, respectively. The median survival time (MST) and 5-year overall survival rates (OS) were 18.5 months and 21.4% for pathological N0 cases, 23.5 months and 24.0% for N1 cases, 8.5 months and 0% for N2 cases, and 10.5 months and 0% for N3 cases, respectively (P < 0.001). The MST and 1-, 3- and 5-year OS of patients treated with postoperative chemotherapy were 17.0 months, 60.7%, 19.8%, and 13.0%, respectively, statistically significantly longer than the 7.0 months, 28.5%, 8.9% and 8.9%, respectively, of the patients without chemotherapy (P = 0.005). The pathological N stage and postoperative chemotherapy were independent prognostic factors by Cox multivariate analysis. CONCLUSIONS: Primary esophageal small cell carcinoma is an aggressive systemic disease, characterized by early and wide dissemination of lymph nodes and poor prognosis while treated with surgery or chemotherapy alone. Multimodality treatment based on radical esophagectomy should be recommended for patients in pathological stage I and II.
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Carcinoma de Células Pequenas/cirurgia , Neoplasias Esofágicas/cirurgia , Esofagectomia/métodos , Adulto , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma de Células Pequenas/tratamento farmacológico , Carcinoma de Células Pequenas/patologia , Terapia Combinada , Neoplasias Esofágicas/tratamento farmacológico , Neoplasias Esofágicas/patologia , Feminino , Seguimentos , Humanos , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
OBJECTIVE: To investigate the clinical characteristics and prognostic factors of esophageal carcinosarcoma. METHODS: The clinical data of patients treated by surgery and pathologically diagnosed as esophageal carcinosarcoma between January 1967 and December 2008 were retrospectively reviewed. There were 28 male and 4 female patients aged from 39 to 76 years with a median age of 58 years. All the data were analyzed using SPSS 15.0 software. The overall survival rates were calculated and compared with the Kaplan-Meier method and the Log-rank test. The prognostic factors were identified by Cox hazard regression model. RESULTS: Among all the 32 patients included, 29 patients were polypoid type, 2 patients were fungoid type and 1 patient were medullary type. With regard to the depth of tumor infiltration, 17 patients involved the mucosa or submucosa (pT1), 13 patients involved the muscularis propria (pT2), 2 patients involved the adventitia (pT3). The involvement of local lymph nodes was present in 10 patients, with an incidence of 31.3%, including metastatic carcinoma alone in 7 patients and both carcinomatous and sarcomatous components in 3 patients. According to the most recently published international TNM staging system for esophageal carcinoma (AJCC 2009), 15 patients were in stage I, 13 patients in stage II, 4 patients in stage III. The 1-, 3- and 5-year overall survival rates of the whole group were 90.0%, 72.1% and 57.0%, respectively. Both in single-factor prognostic analysis and in Cox multivariate analysis, pathological N stage was the only prognostic factor (RR = 2.531, 95%CI: 1.055 - 6.070). CONCLUSIONS: Esophageal carcinosarcoma is consisted of both sarcomatous component and carcinomatous component, while the latter one appears more frequently in local lymph node metastasis. In multivariate prognostic analysis, pathological N stage is the only independent prognostic factor. Curative resection of this tumor may achieve good prognosis because of its' lower incidence of lymph node metastasis and less invasive tendency.
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Carcinossarcoma/cirurgia , Neoplasias Esofágicas/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Modelos de Riscos Proporcionais , Estudos RetrospectivosRESUMO
OBJECTIVE: To evaluate the imaging features of different subtypes of renal cell carcinoma (RCC) by double-phase helical computed tomography (CT) and to enhance its pre-operative diagnosis level. METHODS: A total of 460 RCC were reviewed retrospectively. Tumor size, enhancement degree, enhancement drop between corticomedullary (CMP), nephrographic (NP), enhancement pattern, and tumor density (homogeneity, degeneration or necrosis, calcification) were observed respectively. The clear cell, chromophobe and papillary types were analyzed for statistical analysis. RESULTS: They had difference in enhancement pattern and tumor density of clear cell, chromophobe (P < 0.05) and papillary types (P < 0.05). There were differences in enhancement degree, enhancement drop and combine degeneration or necrosis in all subtypes of RCC (P < 0.05). The clear cell type was of hypervascular tumor and showed a stronger enhancement (80.9%) than other types. The enhancement drop was also higher than other types and 57.9% of it was ≥ 30 HU. As for ≥ 30 HU, the clear cell type was diagnosed with a 57.9% sensitivity and a 91.9% specificity. Clear cell type tended to appear as peripheral and heterogeneous enhancement (66.3%, 94.6%); it was likely heterogeneous in density (91.6%) with frequent degeneration or necrosis (60.5%). The chromophobe type was of moderate vascular tumor and it exhibited middle-level enhancement (77.1%). The enhancement drop was low (< 30 HU, 80.0%). As for 0-30 HU, the chromophobe type was diagnosed with an 80.0% sensitivity and a 66.1% specificity. Its pattern ended to appear as homogeneous enhancement (65.7%); Homogeneous density, degeneration and necrosis were characteristic signs of chromophobe type. The papillary type was of hypovascular tumor and it exhibited slight or no enhancement (70.4%). The enhancement drop was low (< 30 HU, 92.6%) and the gradual enhancement (63.0%) was characteristic of this type. As for 0 Hu, the papillary type was diagnosed with a 63.0% sensitivity and a 91.8% specificity. It tended to appear as homogeneous density (63.0%). The unclassified type tended to appear as peripheral (9/12 cases) and stronger enhancement (7/12 cases) and heterogeneous density with degeneration or necrosis (9/12 cases). The multilocular cystic RCC appeared as complex cyst. And cystic wall enhancement was an important diagnostic point of cystic RCC. CONCLUSION: Double-phase helical CT plays an important role in the pre-operative differentiation of subtypes of RCC. Each type of RCC has its own features. A clinician may reach a correct pre-operative diagnosis.
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Carcinoma de Células Renais/diagnóstico por imagem , Neoplasias Renais/diagnóstico por imagem , Tomografia Computadorizada Espiral , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma de Células Renais/classificação , Carcinoma de Células Renais/patologia , Feminino , Humanos , Neoplasias Renais/classificação , Neoplasias Renais/patologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND AND OBJECTIVE: Diffuse large B-cell lymphoma (DLBCL), the most common type of non-Hodgkin's lymphoma (NHL), is heterogeneous on molecular and clinical levels, therefore, its prognosis is difficult to predict. This study aimed to evaluate the value of Blimp-1 protein and Hans classification in predicting the prognosis of DLBCL and their interrelation. METHODS: The clinical records of 136 patients with DLBCL were reviewed. The patients were followed up for 5-80 months (median, 39 months). Immunohistochemical staining for CD10, MUM1, Bcl-6, and Blimp-1 were performed on paraffin-embedded tumor tissues from the 136 patients. The correlations of Blimp-1 protein and Hans classification in prognosis of DLBCL and their interrelation were analyzed. RESULTS: Blimp-1 was detected in 38 (30.0%) patients, and was associated with a significantly shorter overall survival (OS) (P = 0.030). Using the Hans classification based upon the expression of CD10, Bcl-6, and MUM1, 54 patients had germinal center B-cell (GCB) phenotype and 82 had non-GCB phenotype. The 5-year OS rate was 75% in the GCB group and 52% in the non-GCB group (P = 0.020). The positive rate of Blimp-1 was 22.2% in the GCB group and 31.7% in the non-GCB group (P = 0.329). The Cox regression multivariate analysis showed that international prognosis index (IPI) and Hans classification had independent prognostic significance, whereas Blimp-1 was not an independent prognostic factor. CONCLUSIONS: The patients with GCB subtype of DLBCL had better prognosis than the non-GCB subtype. High level of Blimp-1 expression in the patients with DLBCL implies a shorter survival, but it is not associated with Hans classification.
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Linfoma Difuso de Grandes Células B/classificação , Linfoma Difuso de Grandes Células B/metabolismo , Proteínas Repressoras/metabolismo , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Proteínas de Ligação a DNA/metabolismo , Feminino , Seguimentos , Humanos , Imunofenotipagem , Fatores Reguladores de Interferon/metabolismo , Masculino , Pessoa de Meia-Idade , Neprilisina/metabolismo , Fator 1 de Ligação ao Domínio I Regulador Positivo , Prognóstico , Modelos de Riscos Proporcionais , Proteínas Proto-Oncogênicas c-bcl-6 , Taxa de Sobrevida , Adulto JovemRESUMO
OBJECTIVES: The aim of this article was to evaluate the clinical and pathologic characteristics, therapy, and prognostic factors of vulvar sweat gland carcinoma. MATERIALS: Clinical and pathologic data for 12 patients with vulvar sweat gland carcinoma treated at our institution from January 1958 to April 2009 were retrospectively analyzed. Of the 12 cases, 7 cases were vulvar sweat gland carcinoma, 3 cases were vulvar Paget disease with underlying sweat gland adenocarcinoma, 1 case was vulvar apocrine adenocarcinoma, and 1 case was adenoid cystic carcinoma of the vulvar sweat gland. Two patients were treated with simple vulvar tumor excision at other medical institutions without adjuvant therapy. Among the other 10 patients, 6 underwent radical vulvectomy; 3, wide local excision of the vulva; and 1, a simple vulvectomy. For 5 of the 12 patients, bilateral or unilateral inguinal lymph nodes excision and biopsy were performed. For 1 patient with bulky inguinal lymph nodes, only a biopsy was performed, and the patient received radiotherapy after vulvar surgery. RESULTS: A follow-up for 11 patients was conducted until death or April 1, 2009. Five of the 11 patients had recurrences after primary treatment. For 2 of these patients, recurrence was local 6 and 48 months after treatment. For 3 patients, distant metastasis was found 18, 5, and 31 months after surgery at our institution. Five of 11 patients died, 1 of whom died of irrelevant disease and 4 of tumor progression. The total survival periods of the 4 patients who died of tumor progression were 24, 36, 44, and 203 months. The other 6 patients have survived for more than 5 years without local failure. In total, there are 7 patients who have survived for 5 years or more. CONCLUSIONS: Vulvar sweat gland carcinoma is a very rare entity. Surgery is the primary treatment modality, and the function of radiotherapy and chemotherapy is uncertain. The vulvar tumor size and inguinal lymph nodes metastasis will influence the prognosis, with pathologic differentiation and surgical margin status being the probable prognostic factors.
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Neoplasias das Glândulas Sudoríparas/diagnóstico , Neoplasias Vulvares/diagnóstico , Adulto , Idoso , Feminino , Humanos , Metástase Linfática , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Neoplasias das Glândulas Sudoríparas/patologia , Neoplasias das Glândulas Sudoríparas/terapia , Vulva , Neoplasias Vulvares/patologia , Neoplasias Vulvares/terapiaRESUMO
OBJECTIVE: To evaluate the clinicopathological characteristics and treatment of adenoid cystic carcinoma of the Bartholin gland. METHODS: The clinicopathological data of six patients with adenoid cystic carcinoma of the Bartholin gland were retrospectively analyzed. The median age was 40.8 years (range 30 to 54 years). Surgery was the primary treatment. Simple vulvar tumor resection was performed in 1 patient. Four cases underwent radical vulvectomy with bilateral inguinal lymph node dissection and 1 case underwent wide local excision of the vulva with bilateral inguinal lymph node biopsy. Two cases with high risk factors received postoperative radiotherapy. RESULTS: All patients had definite pathological diagnosis. Cribriform arrangement of tubules and gland-like elements and infiltration of perineural spaces were two main microscopic features of this type of tumor. The pathological examination after surgery revealed that two patients had positive surgical margins, one had negative margin, 1 adjacent to the tumor and 1 unknown; 5 cases had negative inguinal lymph nodes and 1 unknown. All the 6 patients were followed-up. Recurrence developed in 4 cases including 3 with both local recurrence and lung metastasis, and one had lung metastasis only. One patient died of lung metastasis and her total survival period was 135 months. The other 3 recurrent patients survived with tumor and the total survival period was 241, 128 and 103 months, respectively. Two cases without recurrence survived 8 and 121 months, respectively. CONCLUSION: Adenoid cystic carcinoma of the Bartholin gland is a slow growing but locally very aggressive neoplasm with a high capacity for local recurrence and lung metastasis. Surgery is the most common and useful treatment. Radiation is a choice of treatment for patients with high risk factors after surgery such as positive surgical margin, deep local invasion and infiltration of perineural spaces or for recurrent patients without opportunity of excision.
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Glândulas Vestibulares Maiores/patologia , Carcinoma Adenoide Cístico/cirurgia , Vulva/cirurgia , Neoplasias Vulvares/cirurgia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Glândulas Vestibulares Maiores/cirurgia , Carcinoma Adenoide Cístico/patologia , Carcinoma Adenoide Cístico/radioterapia , Carcinoma Adenoide Cístico/secundário , Terapia Combinada , Feminino , Seguimentos , Humanos , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/cirurgia , Excisão de Linfonodo , Metástase Linfática , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/radioterapia , Recidiva Local de Neoplasia/cirurgia , Radioterapia de Alta Energia , Estudos Retrospectivos , Taxa de Sobrevida , Neoplasias Vulvares/patologia , Neoplasias Vulvares/radioterapiaRESUMO
OBJECTIVE: Vulvar epithelioid sarcoma is a rare, undifferentiated soft-tissue sarcoma, with a high rate of local relapse, regional nodal spread and distant metastases. The aim of this study was to investigate the clinical features, diagnosis, treatment and prognosis of this malignancy. METHODS: We studied the clinicopathologic features of 20 cases of vulvar epithelioid sarcoma, of which 4 cases were admitted to our hospital from 1999 to 2009. All of the patients received radical local excision with inguinofemoral lymphadenectomy. Seven patients were treated without adjuvant therapy. Seven patients received postoperative radiotherapy only and three underwent chemotherapy. Chemotherapy plus radiotherapy were given postoperatively in three. RESULTS: The patients ranged in age from 23 to 80 years (median: 36 y). The tumors ranged from 1 to 10 cm in their greatest diameter (median: 5.1 cm). All cases showed immunoreactivity for both vimentin and cytokeratin. Follow-up information on all 20 patients was available, and covered periods ranging from 3 to 104 months.11 patients were alive with no evidence of disease. 2 patients developed lymph node metastases but alive. 7 patients had died of the disease. Survival of the early stage (I-II) patients was significantly longer than those in the advanced stage (III-IV) (median, 21 vs. 6 months, P < 0.01). There was no significant difference between survival of patients with or without inguinofemoral lymphadenectomy (median, 11.5 vs. 6 months, P = 0.086). CONCLUSIONS: Because of the relatively frequent misdiagnosis, a differential diagnosis combined with immunohistochemistry is needed to determine an early and accurate diagnosis. The tumor markers exhibiting immunoreactivity includ vimentin, epithelial membrane antigen (EMA) and cytokeratin (CK). Radical local excision with adequate margin (at least 2 cm) and bilateral inguinofemoral lymphadenectomy is effective for the treatment of vulvar epithelioid sarcoma. The role of adjuvant therapy, chemotherapy and radiation remains unclear but merits consideration.
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Sarcoma/patologia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias Vulvares/patologia , Neoplasias Vulvares/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Quimioterapia Adjuvante , Feminino , Seguimentos , Humanos , Queratinas/metabolismo , Excisão de Linfonodo , Metástase Linfática , Pessoa de Meia-Idade , Mucina-1/metabolismo , Estadiamento de Neoplasias , Radioterapia Adjuvante , Estudos Retrospectivos , Sarcoma/tratamento farmacológico , Sarcoma/metabolismo , Sarcoma/radioterapia , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/metabolismo , Neoplasias de Tecidos Moles/radioterapia , Taxa de Sobrevida , Vimentina/metabolismo , Vulva/cirurgia , Neoplasias Vulvares/tratamento farmacológico , Neoplasias Vulvares/metabolismo , Neoplasias Vulvares/radioterapia , Adulto JovemRESUMO
AIM: To identify clonality and genetic alterations in focal nodular hyperplasia (FNH) and the nodules derived from it. METHODS: Twelve FNH lesions were examined. Twelve hepatocellular adenomas (HCAs) and 22 hepatocellular carcinomas (HCCs) were used as references. Nodules of different types were identified and isolated from FNH by microdissection. An X-chromosome inactivation assay was employed to describe their clonality status. Loss of heterozygosity (LOH) was detected, using 57 markers, for genetic alterations. RESULTS: Nodules of altered hepatocytes (NAH), the putative precursors of HCA and HCC, were found in all the FNH lesions. Polyclonality was revealed in 10 FNH lesions from female patients, and LOH was not detected in any of the six FNH lesions examined, the results apparently showing their polyclonal nature. In contrast, monoclonality was demonstrated in all the eight HCAs and in four of the HCCs from females, and allelic imbalances were found in the HCAs (9/9) and HCCs (15/18), with chromosomal arms 11p, 13q and 17p affected in the former, and 6q, 8p, 11p, 16q and 17p affected in the latter lesions in high frequencies (> or = 30%). Monoclonality was revealed in 21 (40%) of the 52 microdissected NAH, but was not found in any of the five ordinary nodules. LOH was found in all of the 13 NAH tested, being highly frequent at six loci on 8p, 11p, 13q and 17p. CONCLUSION: FNH, as a whole, is polyclonal, but some of the NAH lesions derived from it are already neoplastic and harbor similar allelic imbalances as HCAs.
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Adenoma de Células Hepáticas/genética , Carcinoma Hepatocelular/genética , Hiperplasia Nodular Focal do Fígado/diagnóstico , Hiperplasia Nodular Focal do Fígado/genética , Neoplasias Hepáticas/genética , Adenoma de Células Hepáticas/patologia , Adulto , Alelos , Carcinoma Hepatocelular/patologia , Células Clonais , Feminino , Hiperplasia Nodular Focal do Fígado/patologia , Humanos , Neoplasias Hepáticas/patologia , Perda de Heterozigosidade , Masculino , Pessoa de Meia-Idade , Inativação do Cromossomo X , Adulto JovemRESUMO
OBJECTIVE: To study the neoplasm with perivascular epithelioid cell differentiation (PEComa) with respect to their morphologic, immunohistochemical and clinical phenotypes. METHODS: Three PEComas were included in this study, one located at the left uterine horn, and two presented as a mass in the uterine corpus. The tumors were examined by histopathology and immunohistochemistry. RESULTS: The lesions were composed of spindle, blunt epithelioid cells, with foci of, or scattered, cells showing adipose differentiation in two cases. The myomelanocytic differentiation was demonstrated, proving the diagnosis as PEComa. Mild nuclear atypia and focal necrosis was observed in one lesion, and the rest two showed malignant morphologic phenotypes including moderate nuclear atypia and coagulative necrosis. The mitotic and Ki67-labelling indices ranged from 0.5/10 HPF to 14/10 HPF and 0.6% to 7.0%, respectively. All of the three patients remain alive. Malignant nature of the two lesions with worrisome morphology was confirmed by occurrence of metastases after hysterectomy. CONCLUSION: PEComa is a rare tumor, occurring preferentially in the uterus. It is regarded as a tumor with uncertain malignant potential, but a minority of them shows malignant clinical behaviors. Some pathologic parameters including large tumor size, sheet-like necrosis, marked nuclear atypia, elevated mitotic index (> or = 10/10 HPF), aberrant mitotic figure and vascular invasion may help to establish a diagnosis of malignant PEComa.
Assuntos
Neoplasias de Células Epitelioides Perivasculares/patologia , Neoplasias de Células Epitelioides Perivasculares/cirurgia , Neoplasias Uterinas/patologia , Neoplasias Uterinas/cirurgia , Adulto , Antígenos de Neoplasias/metabolismo , Biomarcadores Tumorais , Desmina/metabolismo , Células Epitelioides/patologia , Feminino , Seguimentos , Humanos , Histerectomia/métodos , Imuno-Histoquímica , Antígeno Ki-67/metabolismo , Neoplasias Pulmonares/secundário , Antígenos Específicos de Melanoma , Pessoa de Meia-Idade , Índice Mitótico , Proteínas de Neoplasias/metabolismo , Neoplasias de Células Epitelioides Perivasculares/metabolismo , Neoplasias de Células Epitelioides Perivasculares/secundário , Neoplasias Uterinas/metabolismo , Adulto JovemRESUMO
OBJECTIVE: To explore the clinical features, prognosis, and optimal treatment strategy of clear cell sarcoma (CCS). METHODS: Nineteen patients, 12 males and 7 females, aged 30. 9, with histologically confirmed CCS, 3 with tumors > or =5 cm and 16 with tumors <5 cm, 5 tumors being located in the upper extremity, 2 in the hand, 6 in the lower extremity, 2 in the foot, 3 in the trunk, and 1 in the head, were hospitalized from March 1973 to March 2007. The primary tumors of all the cases were marginally excised before admission and 10 cases had local relapse at admission. Two presented local lymph node metastasis and 2 presented distant metastasis. Ten patients with tumor relapse underwent re-operation. Eight patients received chemotherapy, 8 radiotherapy and 1 combined chemotherapy and radiotherapy. The patients were followed up for 51.4 months (3-144 months). RESULTS: Tumor recurrence occurred in 1 of the 10 patients who received re-operation. 3 patients developed lymph node metastasis and 2 developed pulmonary metastasis. Of the 9 patients who did not undergo re-operation 7 developed lymph node metastasis, 6 developed pulmonary metastasis and 6 died. The overall 5-year survival rate was 75.2% and the 10-year survival rate was 37.5%. CONCLUSION: CCS is a rare and high grade soft tissue sarcoma with high incidence rates of local recurrence and metastasis, and poor prognosis. The role of chemotherapy and radiotherapy for CCS should be investigated further. The best choice after local recurrence is re-operation.
Assuntos
Sarcoma de Células Claras/terapia , Sarcoma/terapia , Adolescente , Adulto , Idoso , Criança , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Prognóstico , Estudos Retrospectivos , Sarcoma/patologia , Sarcoma de Células Claras/patologia , Análise de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
OBJECTIVE: To evaluate and compare localization by ductoscopy-guided wire with localization by conventional methods in the terminal duct excision for women with pathological nipple discharge. METHODS: Breast terminal duct excision were performed in 174 consecutive patients with intraductal lesions diagnosed by mammary ductoscopy. Sixty-eight of those underwent ductoscopy-guided wire localization for more accurate ductal excision. The patients received mammary ductoscopy and a hooked wire was anchored at the intraductal lesions under endoscopic surveillance just before the operation. Then a biopsy resection of wire-guided terminal duct and frozen section were done. Tbe other 106 patients received terminal duct excision under localization with conventional methods without ductoscopy either by puncturing a needle or injection of blue dye through the duct with pathological discharge. RESULTS: Of the 68 patients with ductoscopy-guided duct excision, 64 had intraductal papillomas and 4 duct carcinoma in situ proved by pathology. All the lesions in these 68 patients were completely resected during biopsy without extra extended resection, and the concordance rate of the pathological result with ductoscopic diagnosis was 100.0%. None of them developed a postoperative breast distortion. In the conventional method localization group, there were 96 intraductal papilloma, 6 duct carcinoma in situ and 4 adenosis. Only 77.4% of the lesions were excised in the primary biopsy, and 22.6% needed extended resection. The concordance rate of the pathological diagnosis with ductoscopic diagnosis was 96.2%. Twenty-six patients had a deformed breast postoperatively. CONCLUSION: Ductoscopy-guided wire localization is superior to the conventional localization method in the surgical terminal duct excision for women with spontaneous nipple discharge. It is not only helpful for more accurate localization and resection as well as pathologic sampling, but also is minimally invasive. Further studies are still required and this method may deserve to be popularized.
Assuntos
Doenças Mamárias/patologia , Neoplasias da Mama/patologia , Carcinoma Intraductal não Infiltrante/patologia , Endoscopia/métodos , Papiloma Intraductal/patologia , Adulto , Idoso , Doenças Mamárias/etiologia , Doenças Mamárias/cirurgia , Neoplasias da Mama/complicações , Neoplasias da Mama/cirurgia , Carcinoma Intraductal não Infiltrante/complicações , Carcinoma Intraductal não Infiltrante/cirurgia , Exsudatos e Transudatos/metabolismo , Feminino , Humanos , Microcirurgia/métodos , Pessoa de Meia-Idade , Mamilos/metabolismo , Mamilos/patologia , Papiloma Intraductal/complicações , Papiloma Intraductal/cirurgia , Adulto JovemRESUMO
OBJECTIVE: To review the survival outcomes in patients with endometrial stromal sarcoma (ESS) in Cancer Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, and to discuss prognostic factors and the role of post-operative adjuvant radiotherapy and chemotherapy. METHODS: Hospital records and pathology reports for 97 patients with ESS were reviewed. Among 97 patients, 69 had low-grade ESS (LGESS), 16 had high-grade ESS (HGESS) and 12 had unclear grade. The median age at diagnosis was 44.0 years. The median follow-up time was 62 months (5 - 277 months). Atypical vaginal bleeding (43%) and prolonged and increased menses (36%) were the main symptoms. RESULTS: Totally 2-year and 5-year cumulative survival rates were 93% and 84%, respectively. Cumulative survival curves were significantly different between LGESS and HGESS, and so did cumulative survival curves between stage I - II and stage III - IV (P < 0.05). Totally, 34 patients (37%) had local or distant recurrence. The median time-to-recurrence (TTR) was 27 months. The recurrence rates of the patients with or without preserve of ovary were 89% and 24%, respectively (P = 0.000). The local-control-rates of the patients who received or did not receive post-operative radiotherapy were 81% and 43%, respectively (P = 0.011). CONCLUSIONS: The prognosis of HGESS is obviously worse than that of LGESS. The risk of recurrence of patients with preserve of ovary was remarkably higher than that of patients without preserve of ovary. Postoperative radiotherapy could increase the local-control-rates.
Assuntos
Neoplasias do Endométrio/patologia , Neoplasias do Endométrio/terapia , Sarcoma do Estroma Endometrial/patologia , Sarcoma do Estroma Endometrial/terapia , Adolescente , Adulto , Idoso , Quimioterapia Adjuvante , Neoplasias do Endométrio/mortalidade , Feminino , Seguimentos , Humanos , Histerectomia , Excisão de Linfonodo , Pessoa de Meia-Idade , Metástase Neoplásica , Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Ovariectomia , Prognóstico , Radioterapia Adjuvante , Estudos Retrospectivos , Sarcoma do Estroma Endometrial/mortalidade , Taxa de Sobrevida , Adulto JovemRESUMO
OBJECTIVE: To study the clinicopathological features of spindle cell rhabdomyosarcoma (SCRMS) in order to differentiate it from other myosarcomas. METHODS: The clinical features, morphologic and immunohistochemical phenotypes of 8 SCRMSs were analyzed. RESULTS: SCRMS cells were found to be arranged in a fascicular or storiform pattern, in which a number of enlarged plump or polygonal shaped rhabdomyoblasts containing abundant eosinophilic cytoplasm with eccentrically placed enlarged hyperchromatic nuclei were mixed. Immunohistochemical staining results showed that vimentin, MyoD1, desmin, actin, myoglobin were positive in tumor cells, but S-100, plap, AE1/AE3, CK, CD117 negative. The follow-up data showed that four cases had died of the recurrent disease, one still alive and the remain three patients lost follow-up. CONCLUSION: Spindle cell rhabdomyosarcoma is a rare embryonal rhabdomyosarcoma which occurs in the childhood or adulthood with a poor prognosis, and is frequently presented as a painless mass most frequently involveing the head and neck or cervical area or para-testis site. A combination of MyoD1, desmin and myoglobin immunohistochemical staining is helpful in differential diagnosis.
Assuntos
Rabdomiossarcoma Embrionário/patologia , Rabdomiossarcoma Embrionário/cirurgia , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Adolescente , Adulto , Criança , Terapia Combinada , Desmina/metabolismo , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Proteína MyoD/metabolismo , Mioglobina/metabolismo , Recidiva Local de Neoplasia , Estudos Retrospectivos , Rabdomiossarcoma Embrionário/metabolismo , Neoplasias de Tecidos Moles/metabolismo , Adulto JovemRESUMO
OBJECTIVE: To investigate the impact of squamous cell carcinoma antigen (SCCAg) in patients with recurrent squamous cell carcinoma of the uterine cervix. METHODS: Totally 72 patients with recurrent squamous cell carcinoma of the uterine cervix treated at the Cancer Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, between 1999 and 2005 were retrospectively analyzed to investigate the impact of SCCAg on diagnosis and prognosis by univariate and multivariate analysis. RESULTS: This study included 30 patients with recurrent disease after primary radical surgery and 42 patients with recurrent cervical cancer after radio-chemotherapy. Sixty one patients (85%) had serum SCCAg elevated (>or=1.5 pg/L), and 20 of these (28%) had an increase of SCCAg before clinical manifestation of relapse. The median leading time was 3 months (range: 1-13 months). Forty five patients had no symptoms with only SCCAg elevation, and 15 patients experienced leg edema and (or) sciatic pain, 7 patients suffered from irregular bleeding and 5 patients had symptoms resulting from distant metastasis. Thirty three patients were diagnosed by histology biopsy and (or) cytology, 39 patients were diagnosed with SCCAg elevation and clinical and radiological examinations, 29 of these patients were diagnosed only by SCCAg elevation and CT or MRI. Fourteen patients recurred limited to the cervix or to the cervix and adjacent tissues (central recurrence), 31 cases recurred at pelvis, and 20 patients with distant metastasis and 7 patients suffered from pelvic recurrence and distant metastasis. Twenty three cases received salvage therapy including surgery for patients recurring after definitive radiotherapy and radiotherapy and or conform radiotherapy for patients after primary radical surgery, 46 patients were given palliative chemotherapy and or radiotherapy, and 3 patients refused any treatment. The median and mean survival time were 11 months and 23 months respectively (2-62 months). The 3-year, 5-year overall survival rate were 25% and 19% respectively. Univariate analysis showed SCCAg elevation before primary treatment, grade, recurrent site, treatment method, SCCAg>or=10 pg/L, SCCAg elevation during treatment, and SCCAg not within normal after treatment were correlated with 3-year survival rate. Twenty patients had an increase of SCCAg before clinical manifestation of relapse compared with other patients who did not, and the 3-year survival rate was not significantly different (22% vs 27%). Multivariate analysis revealed that only grade and treatment methods were independent risk factors. CONCLUSION: The impact of the SCCAg in recurrent squamous cell carcinoma of the uterine cervix needs further study.