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1.
Int J Gynecol Pathol ; 37(6): 564-574, 2018 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-30059455

RESUMEN

Undifferentiated endometrial carcinoma (UEC) is a rare and poorly recognized entity, associated with a poor outcome. The clinical, pathologic, and immunohistochemical features of 17 cases diagnosed at our center are described. The median age was 60 yr. Postmenopausal bleeding was the most common presenting symptom (76.9%). Most patients presented with advanced stage (64.7%). Total hysterectomy with bilateral salpingo-oophorectomy was the commonly offered surgical treatment (80.0%). Nine (52.9%) patients received adjuvant treatment. The median overall survival was 11 mo. Pure UEC was seen in 8 cases (47.0%), while dedifferentiated carcinoma in 5 cases (29.4%). The epithelial component was part of carcinosarcoma or was mixed with serous carcinoma in 2 cases (11.8%) each. Positivity for one or more of the cytokeratin cocktails, mostly as strong focal staining, was evident in 16 cases (94.1%). PAX-8 was negative in 13 cases (86.7%). BRG-1/SMARCA4 was lost in 3 cases (20.0%). Eleven cases (64.7%) were MLH1/PMS2 deficient. Ten cases (66.7%) were positive for programmed death ligand 1, with positivity in 10%, 20%, and 100% of tumor cells detected in a single case each. Only 2 of 11 (18.2%) referral cases were correctly diagnosed as UEC. UEC is a rare tumor that is frequently misdiagnosed. A panel of immunostains is necessary to make the correct diagnosis. The range of positivity for programmed death ligand 1 testing suggests that immunotherapy might be considered in the adjuvant setting, especially with the poor response of this tumor to traditional therapies.


Asunto(s)
Antígeno B7-H1/metabolismo , Carcinoma/patología , Neoplasias Endometriales/patología , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor/metabolismo , Carcinoma/metabolismo , Carcinoma/mortalidad , Carcinoma/cirugía , Neoplasias Endometriales/metabolismo , Neoplasias Endometriales/mortalidad , Neoplasias Endometriales/cirugía , Femenino , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Tasa de Supervivencia
2.
Mol Genet Genomic Med ; 11(4): e2125, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36537080

RESUMEN

BACKGROUND: Ovarian cancer is one of the most common gynecological malignancies. Due to the absence of effective screening methods, ovarian cancer is usually diagnosed at late stages. Patients with pathogenic and likely-pathogenic germline variants (PGVs) in BRCA1 or BRCA2 harbor elevated risk of developing both ovarian and breast cancers. Identifying PGVs may help in both cancer prevention and active disease treatment. Worldwide prevalence of PGVs varies and the matter is poorly addressed among Arab patients. METHODS: Patients with epithelial ovarian, fallopian tube or primary peritoneal cancers were offered the universal 20 or 84-multi-gene panel testing as per standard guidelines. Cascade family screening was also offered to all first and second-degree relatives of PGV positive patients. Genetic testing was done at a referral lab using a next generation sequencing (NGS)-based platform. RESULTS: During the study period, 152 patients, median age (range): 50 (18-79) years old, were tested. The majority (n = 100, 65.8%) had high-grade serous carcinoma, and 106 patients (69.7%) had metastatic disease at presentation. In total, 38 (25.0%) had PGVs, while 47 (30.9%) others had variants of uncertain significance (VUS). PGVs were mostly in BRCA1 (n = 21, 13.8%) and in BRCA2 (n = 12, 7.9%), while 6 (3.9%) others had PGVs in non-BRCA1/2 genes. PGV rates were significantly higher among 15 patients with a positive family history of ovarian cancer (60.0%, p = .022) and among 52 patients with a positive family history of breast cancer (40.4%, p = .017). CONCLUSIONS: PGVs are common among Jordanian women with ovarian cancer, and mostly occur in BRCA1/2. Given its clinical impact on disease prevention and precision therapy, universal testing should be routinely offered.


Asunto(s)
Neoplasias de la Mama , Neoplasias Ováricas , Humanos , Femenino , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Mutación de Línea Germinal , Prevalencia , Jordania/epidemiología , Predisposición Genética a la Enfermedad , Neoplasias Ováricas/epidemiología , Neoplasias Ováricas/genética , Neoplasias Ováricas/patología , Neoplasias de la Mama/genética
3.
Cancers (Basel) ; 15(16)2023 Aug 15.
Artículo en Inglés | MEDLINE | ID: mdl-37627130

RESUMEN

DTC accounts for the majority of endocrine tumors. While the incidence of thyroid cancer has been increasing globally over the past few decades, papillary thyroid carcinoma (PTC) generally shows an excellent prognosis, except in cases with aggressive clinicopathological features. This study aimed to assess the 5- and 10-year overall survival (OS) and progression-free survival (PFS) of 528 Arabic patients diagnosed with primary DTC from 1998 to 2021. Additionally, the study aimed to analyze the impact of various factors on both OS and PFS. An univariable survival analysis was conducted using Kaplan-Meier curves. The 5- and 10-year OS for patients with DTC have exceeded 95%. Additionally, PFS showed very good rates (ranging between 96.5 and 85% at 5 and 10 years, respectively). Age, male gender, risk of recurrence, and distant metastasis were identified as the main negative prognostic factors for both OS and PFS, while RAI treatment was found to be a significant factor in improving OS. Moreover, adherence to the King Hussein Cancer Center's (KHCC) CPG demonstrated significant improvement in PFS. These findings highlight common prognostic factors and favorable outcomes in Arabic patients with DTC treated at a tertiary cancer center using standard of care approaches.

4.
Med Oncol ; 31(6): 958, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24770952

RESUMEN

Synovial sarcoma is a rare type of sarcoma with poor prognosis. Data on relevant prognostic factors are inconsistent. The objective of this study was to determine the independent prognostic factors that govern local recurrence, distant metastasis and overall survival. A retrospective analysis of 51 patients treated for localized synovial sarcoma at a single institution by a multidisciplinary/multimodality approach over a period of 12 years. Patients, tumor and treatment characteristics were collected including age, sex, tumor site, location, depth, size, status of margins, histology subtype and involvement of bone or lymph nodes. Type of surgery, adjuvant chemotherapy and radiotherapy were also examined. The endpoints analyzed were local recurrence-free survival (LRFS), metastasis-free survival (MFS) and overall survival (OS). Median age of patients was 26 years (range 3-64 years) with 73 % above the age of 20 year. All patients received surgery, 57 % received adjuvant radiotherapy and 45 % adjuvant chemotherapy. The median survival was 111 months, and 5-year OS was 73 %. Deep seatedness of the tumor was linked to OS as the only independent risk factor. Twelve patients had local recurrence, and the 5-year LRFS was 61 %. Multivariate analysis determined negative margins and adjuvant radiotherapy as independent predicting factors for LRFS. Five-year MFS was 48 %; multivariate analysis identified monophasic subtype and site other than lower extremity as the only independent factors associated with inferior MFS. The most important factors that govern LRFS and MFS are negative margins and adjuvant radiotherapy for LRFS and tumor histology and site for MFS, while deep seatedness of the tumor is the sole independent factor that governs OS.


Asunto(s)
Sarcoma Sinovial/mortalidad , Sarcoma Sinovial/patología , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Quimioterapia Adyuvante , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Recurrencia Local de Neoplasia/patología , Pronóstico , Radioterapia Adyuvante , Estudios Retrospectivos , Sarcoma Sinovial/terapia , Resultado del Tratamiento , Adulto Joven
5.
Med Oncol ; 30(3): 639, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-23780657

RESUMEN

Metastatic synovial sarcoma (SS) is associated with poor prognosis. Nevertheless, data addressing prognostic factors for patients with metastatic disease are very limited. We identified patients with SS who presented with or developed metastases at our institution from January 2000 to October 2012. Potential demographic and disease-related factors were analyzed for possible influence on survival. A second analysis for patients who received chemotherapy was undertaken to assess efficacy of first-line regimens. Thirty-three patients with metastatic SS were included in this analysis. The following factors were associated with inferior overall survival (OS); age >30 years, presence of extra-pulmonary metastases, lymph node (LN) involvement, presence of uncontrolled primary site, and treatment not including pulmonary metastasectomy. Multivariate analysis identified LN metastases (HR 6.06, 95% CI 1.18-31) and extra-pulmonary metastases (HR 4.06, 95% CI 1.22-13.57) as the only independent factors associated with inferior OS. Assessment of efficacy of first-line chemotherapy showed superiority in progression-free survival (PFS) for ifosfamide-containing regimens versus non-ifosfamide-containing regimens (median PFS of 8.3 and 2.5 months, respectively, p = 0.002). No such significant difference in PFS was detected for comparison between doxorubicin- and non-doxorubicin-containing regimens (p = 0.45). The current study highlights that the pattern of metastases at first detection of metastatic disease is an important determinant of survival. Future studies evaluating therapeutic strategies for metastatic SS should address the comparability of those factors among study arms. In addition, our results suggest that high-dose ifosfamide should be an integral component of first-line chemotherapy regimen.


Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Metástasis de la Neoplasia/tratamiento farmacológico , Sarcoma Sinovial/tratamiento farmacológico , Sarcoma Sinovial/mortalidad , Adolescente , Adulto , Niño , Supervivencia sin Enfermedad , Doxorrubicina/administración & dosificación , Femenino , Humanos , Ifosfamida/administración & dosificación , Masculino , Persona de Mediana Edad , Metástasis de la Neoplasia/patología , Pronóstico , Sarcoma Sinovial/patología , Adulto Joven
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