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1.
Acta Radiol ; : 2841851241241527, 2024 Apr 13.
Artículo en Inglés | MEDLINE | ID: mdl-38613353

RESUMEN

BACKGROUND: Differential diagnosis of hypothalamic-optic chiasmatic gliomas (HOCGs) and craniopharyngiomas on magnetic resonance imaging (MRI) can be quite challenging. PURPOSE: To compare the MRI features of HOCGs and cranipharyngiomas. MATERIAL AND METHODS: Patients diagnosed with HOCG or craniopharyngioma in histopathological evaluation between 2012 and 2022 and who underwent preoperative contrast-enhanced brain MRI were included. Various MRI features were retrospectively evaluated for each lesion: T2-weighted imaging and fluid attenuation inversion recovery hyperintensity, calcification, cystic change, T1-weighted (T1W) imaging hyperintensity of the cystic component, hemorrhage, involvement of sellar, suprasellar or other adjacent structures, lobulated appearance, presence of hydrocephalus, and contrast enhancement pattern. Apparent diffusion coefficient (ADC) values were also evaluated and compared. RESULTS: Among 38 patients included, 13 (34%) had HOCG and 25 (66%) had craniopharyngioma. Craniopharyngiomas had a significantly higher rate of cystic changes, calcification, and T1W imaging hyperintensity of the cystic component than HOCGs (P <0.05). Of HOCGs, 92% had chiasm involvement, 23% had optic nerve involvement, and 31% had brain stem involvement. On the other hand, chiasm involvement was observed in 8% of craniopharyngiomas, but none had optic nerve and/or brain stem involvement (P <0.05). While 62% (8/13) of HOCGs had diffuse homogeneous enhancement, 80% (20/25) of craniopharyngiomas had a diffuse heterogeneous enhancement pattern. Mean ADC values were significantly higher in craniopharyngiomas compared to HOCGs (2.1 vs. 1.6 ×10-3mm2/s, P <0.05). CONCLUSION: Although some neuroimaging findings may overlap, features such as presence of cyst and calcification, brain stem and optic pathway involvement, different enhancement patterns, and ADC values may be helpful in the differential diagnosis of HOCGs and craniopharyngiomas.

2.
Int J Surg Pathol ; : 10668969241228294, 2024 Feb 06.
Artículo en Inglés | MEDLINE | ID: mdl-38321863

RESUMEN

Certain undifferentiated round cell sarcomas displaying EWSR1::NFATC2 fusion have recently been reported, mostly in the bones. This report presents clinicopathological features of 3 additional EWSR1::NFATC2 fusion sarcomas of bone and soft tissues. We present 2 soft tissue and 1 bone tumors: A 62-year-old man with pain and a slowly growing, 8-cm-sized soft tissue mass in the anterolateral compartment of his right calf, along with multiple pulmonary metastatic lesions; a 63-year-old man with a 5-cm sized axillary mass of 4 months duration and a cystic renal mass; and a 53-year-old man with a complaint of leg pain was found to have a 2-cm diameter, intramedullary, lytic mass in the diaphysis of his left femur. Microscopic examination of the tumors in all patients revealed round to epithelioid cells arranged in cords and trabeculae in a myxohyaline stroma. Immunohistochemically, the tumor cells were positive for MIC2/CD99 (3/3), EMA (3/3), NKX3.1 (3/3), NKX2.2 (2/2), CD10 (2/2), and aggrecan (1/1), while negative for S100P and GFAP. Various keratins were also negative except focal AE1/AE3 positivity in the third tumor. By fluorescence in-situ hybridization, 2 tumors (#1 and #3) revealed EWSR1 gene rearrangement and amplification. Furthermore, 2 tumors (#1 and #2) displayed EWSR1ex8::NFATC2ex3 fusion with next-generation sequencing (NGS). The first patient was offered chemotherapy. However, he died of pulmonary metastasis. This report highlights the value of combining histopathological features and immunostains such as NXK3.1, NKX2.2, CD10, and aggrecan, along with EWSR1 testing for triaging these tumors for rare gene fusions by NGS that has prognostic implications.

3.
Fetal Pediatr Pathol ; 43(2): 140-150, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38268442

RESUMEN

INTRODUCTION: We examined the relationship between proinflammatory cytokines that occur in the inflammatory reaction in the intestine in Hirschsprung disease (HD) and Hirschsprung-associated enterocolitis (HAEC). METHODS: Thirty cases (M:27, F:3) operated on due to HD. The cases were divided into three groups: group 1 with pre and post operative EC, group 2 with post-operative, and group 3 with pre-operative EC. The intestinal segments were evaluated by immunohistochemistry for interleukin 1 beta (IL-1ß), tumor necrosis factor-alpha (TNF-α), and interleukin 6 (IL-6). RESULTS: IL-1ß staining was significantly higher in the ganglionic zone of groups with enterocolitis compared to the control group (p = 0.012). TNF-α staining in the transitional zone of Group 3 and IL-1ß staining in the ganglionic zone of Group 1 was significantly higher than the control group (p = 0.030, p = 0.020). CONCLUSION: In our study, older age at diagnosis and more than 20% IL-1ß staining in the ganglionic segment were found to be risk factors for HAEC. It is noteworthy that the increase in IL-1ß can be associated with HAEC.


Asunto(s)
Enterocolitis , Enfermedad de Hirschsprung , Humanos , Lactante , Enfermedad de Hirschsprung/complicaciones , Enfermedad de Hirschsprung/cirugía , Enfermedad de Hirschsprung/patología , Factor de Necrosis Tumoral alfa , Enterocolitis/etiología , Enterocolitis/patología , Enterocolitis/cirugía , Inflamación , Factores de Riesgo
4.
Am J Med Genet A ; 194(6): e63533, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38234231

RESUMEN

Morbidity and mortality rates in patients with autosomal recessive, congenital generalized lipodystrophy type 4 (CGL4), an ultra-rare disorder, remain unclear. We report on 30 females and 16 males from 10 countries with biallelic null variants in CAVIN1 gene (mean age, 12 years; range, 2 months to 41 years). Hypertriglyceridemia was seen in 79% (34/43), hepatic steatosis in 82% (27/33) but diabetes mellitus in only 21% (8/44). Myopathy with elevated serum creatine kinase levels (346-3325 IU/L) affected all of them (38/38). 39% had scoliosis (10/26) and 57% had atlantoaxial instability (8/14). Cardiac arrhythmias were detected in 57% (20/35) and 46% had ventricular tachycardia (16/35). Congenital pyloric stenosis was diagnosed in 39% (18/46), 9 had esophageal dysmotility and 19 had intestinal dysmotility. Four patients suffered from intestinal perforations. Seven patients died at mean age of 17 years (range: 2 months to 39 years). The cause of death in four patients was cardiac arrhythmia and sudden death, while others died of prematurity, gastrointestinal perforation, and infected foot ulcers leading to sepsis. Our study highlights high prevalence of myopathy, metabolic abnormalities, cardiac, and gastrointestinal problems in patients with CGL4. CGL4 patients are at high risk of early death mainly caused by cardiac arrhythmias.


Asunto(s)
Lipodistrofia Generalizada Congénita , Proteínas de Unión al ARN , Humanos , Masculino , Femenino , Lipodistrofia Generalizada Congénita/genética , Lipodistrofia Generalizada Congénita/complicaciones , Lipodistrofia Generalizada Congénita/patología , Adolescente , Niño , Lactante , Preescolar , Adulto , Adulto Joven , Arritmias Cardíacas/genética , Arritmias Cardíacas/patología , Hipertrigliceridemia/genética , Hipertrigliceridemia/complicaciones , Hipertrigliceridemia/patología
5.
Br J Radiol ; 96(1152): 20220598, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37660368

RESUMEN

OBJECTIVE: The aim of this study is to present the clinical and imaging findings of 16 patients with intraventricular pilocytic astrocytomas (PAs). METHODS: 16 patients with histopathological diagnosis of intraventricular PA between February 2016 and January 2022 were evaluated retrospectively. Imaging and clinical findings of the patients, as well as apparent diffusion coefficient (ADC) measurements were analyzed. RESULTS: Of 16 patients, 8 (%50) were male and 8 (%50) were female. The mean age of the patients was 20.8 years (2-44 years range). The most common symptoms in the patients were headache and ataxia. The mean long-axis size of lesions was found to be 48.19 ± 21.59 (range, 15-92 mm). 9 out of 16 lesions (56.2%) were located in the fourth ventricle. The majority of the lesions were iso-hypointense in T1W and hyperintense in T2W images. The mean ADC value of PAs was 1.57 × 10-3 ± 0.2 mm2/s, while the mean thalamic ADC and white matter ADC values were found to be 0.78 × 10-3 ± 0.04 and 0.76 × 10-3 ± 0.06 mm2/s, respectively. There was a statistically significant difference between the ADC values obtained from the solid components of the lesions and the thalami/white matter (p < 0.001). CONCLUSION: PAs often originate from midline structures, however, they can also be located intraventricularly. Although intraventricular PAs are frequently seen in pediatric population, it should be kept in mind that they can also be seen in adults, albeit rarely. ADVANCES IN KNOWLEDGE: PA should be considered in the differential diagnosis of intraventricular neoplasms in case of high ADC values.


Asunto(s)
Astrocitoma , Imagen de Difusión por Resonancia Magnética , Adulto , Humanos , Niño , Masculino , Femenino , Adulto Joven , Estudios Retrospectivos , Imagen de Difusión por Resonancia Magnética/métodos , Astrocitoma/diagnóstico por imagen , Astrocitoma/patología , Diagnóstico Diferencial , Tálamo
7.
Mod Rheumatol Case Rep ; 7(2): 340-346, 2023 06 19.
Artículo en Inglés | MEDLINE | ID: mdl-36718604

RESUMEN

Rheumatoid arthritis (RA) is an autoimmune disease involving synovial joints, and it is known that extra-articular manifestations that may affect the central and peripheral nervous systems may develop during its course. Rheumatoid meningitis is very rare among all neurological involvements. In this study, cases diagnosed as rheumatoid meningitis with clinical, imaging, laboratory, and histopathological features are presented, and the aim of the study is to present current approaches in the diagnosis and treatment of rheumatoid meningitis in the light of case studies and current literature. The data of the patients who were followed up with the diagnosis of rheumatoid meningitis in neurology clinic between 2017 and 2021 were reviewed retrospectively. Three cases diagnosed with rheumatoid meningitis are presented in detail. In the first case, the diagnosis was reached by clinical, imaging, and laboratory findings as well as treatment response, while the diagnosis was made by histopathological verification in the second case. The third case shows that spontaneous remission can be observed in the course of rheumatoid meningitis. Rheumatoid meningitis, which is one of the rarest involvements in the course of RA, may present with headaches, focal neurological deficits, seizures, and altered consciousness. A meningeal biopsy is recommended when the differential diagnosis cannot be ruled out with imaging and laboratory findings. In the differential diagnosis Mucobacterium tuberculosis, syphilis, neuro-sarcoidosis, immunoglobulin G4-related disease, lymphoproliferative diseases, and systemic metastasis should be kept in mind. Aggressive RA management is recommended for treatment.


Asunto(s)
Artritis Reumatoide , Meningitis , Humanos , Estudios Retrospectivos , Imagen por Resonancia Magnética , Meningitis/diagnóstico , Meningitis/etiología , Meningitis/tratamiento farmacológico , Artritis Reumatoide/complicaciones , Artritis Reumatoide/diagnóstico , Artritis Reumatoide/tratamiento farmacológico , Diagnóstico Diferencial
8.
Fetal Pediatr Pathol ; 42(1): 156-160, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-35535964

RESUMEN

INTRODUCTION: A tumor with EWSR1/FLI fusion displaying extensive well differentiated neuroblastomatous differentiation is presented. CASE REPORT: A nine-year-old female patient had a thoracic vertebra 8 paraspinal mass. The lesion was resected incompletely. Histopathologically, a small round cell tumor with gangliomatous differentiation was seen. This was initially diagnosed as an intermixed ganglioneuroblastoma. In the completion surgery biopsy material, the small round cell component was more prominent. Immunohistochemistry for both samples showed membrane positivity for CD99 and nuclear positivity for NKX2.2 in the small round cell component of the tumor. Molecular analysis revealed EWSR1/FLI fusion. The diagnosis then considered a "Ewing Sarcoma Displaying Extensive Well Differentiated Neuroblastomatous Differentiation". CONCLUSION: Tumors with the EWSR1/FLI fusion may show neuroblastomatous differentiation. We chose to treat this as an Ewing Sarcoma (ES). Recognition of this phenomenon in ES cases may prevent a possible misinterpretation and a failure in oncologic treatment.


Asunto(s)
Sarcoma de Ewing , Sarcoma , Femenino , Humanos , Niño , Sarcoma de Ewing/diagnóstico , Proteína EWS de Unión a ARN/genética , Diferenciación Celular , Biopsia , Biomarcadores de Tumor/análisis
9.
Turk J Pediatr ; 64(2): 385-388, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35611429

RESUMEN

BACKGROUND: Curarino syndrome is a rare and complex anomaly with the triad of anorectal malformation, presacral mass and sacral bone deformation. The most common cause of the presacral mass is meningioma, but teratoma is the diagnosis in about one-third of the cases. Malignant transformation of teratoma in the form of carcinoma, rhabdomyosarcoma and leukemia have previously been reported on rare occasions. CASE: A 19 month-old-girl was referred with a presacral mass of 29mm x 23mm x 24mm. She was diagnosed as Currarino syndrome. The presacral mass was surgically resected and pathological examination revealed a foci of primitive neurectodermal tumor. CONCLUSIONS: This is the first case of Currarino syndrome with a primitive neuroectodermal tumor (PNET) foci in the presacral mass. Considering the risk of malignant transformation, the accurate pathological examination is important for complete systemic evaluation and treatment plan in these children.


Asunto(s)
Anomalías del Sistema Digestivo , Tumores Neuroectodérmicos Primitivos , Teratoma , Canal Anal/anomalías , Canal Anal/patología , Canal Anal/cirugía , Niño , Anomalías del Sistema Digestivo/diagnóstico , Anomalías del Sistema Digestivo/cirugía , Femenino , Humanos , Lactante , Tumores Neuroectodérmicos Primitivos/diagnóstico , Tumores Neuroectodérmicos Primitivos/cirugía , Recto/anomalías , Recto/patología , Sacro/anomalías , Siringomielia , Teratoma/diagnóstico
10.
Pituitary ; 25(3): 486-495, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35435565

RESUMEN

OBJECTIVE: To develop machine learning (ML) models that predict postoperative remission, remission at last visit, and resistance to somatostatin receptor ligands (SRL) in patients with acromegaly and to determine the clinical features associated with the prognosis. METHODS: We studied outcomes using the area under the receiver operating characteristics (AUROC) values, which were reported as the performance metric. To determine the importance of each feature and easy interpretation, Shapley Additive explanations (SHAP) values, which help explain the outputs of ML models, are used. RESULTS: One-hundred fifty-two patients with acromegaly were included in the final analysis. The mean AUROC values resulting from 100 independent replications were 0.728 for postoperative 3 months remission status classification, 0.879 for remission at last visit classification, and 0.753 for SRL resistance status classification. Extreme gradient boosting model demonstrated that preoperative growth hormone (GH) level, age at operation, and preoperative tumor size were the most important predictors for early remission; resistance to SRL and preoperative tumor size represented the most important predictors of remission at last visit, and postoperative 3-month insulin-like growth factor 1 (IGF1) and GH levels (random and nadir) together with the sparsely granulated somatotroph adenoma subtype served as the most important predictors of SRL resistance. CONCLUSIONS: ML models may serve as valuable tools in the prediction of remission and SRL resistance.


Asunto(s)
Acromegalia , Adenoma , Sistemas de Apoyo a Decisiones Clínicas , Adenoma Hipofisario Secretor de Hormona del Crecimiento , Hormona de Crecimiento Humana , Acromegalia/metabolismo , Acromegalia/cirugía , Adenoma/metabolismo , Adenoma/cirugía , Adenoma Hipofisario Secretor de Hormona del Crecimiento/metabolismo , Adenoma Hipofisario Secretor de Hormona del Crecimiento/cirugía , Humanos , Factor I del Crecimiento Similar a la Insulina/metabolismo , Aprendizaje Automático , Estudios Retrospectivos , Resultado del Tratamiento
11.
Pituitary ; 25(3): 474-479, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35334029

RESUMEN

PURPOSE: Hypophysitis is a heterogeneous condition that includes inflammation of the pituitary gland and infundibulum, and it can cause symptoms related to mass effects and hormonal deficiencies. We aimed to evaluate the potential role of machine learning methods in differentiating hypophysitis from non-functioning pituitary adenomas. METHODS: The radiomic parameters obtained from T1A-C images were used. Among the radiomic parameters, parameters capable of distinguishing between hypophysitis and non-functioning pituitary adenomas were selected. In order to avoid the effects of confounding factors and to improve the performance of the classifiers, parameters with high correlation with each other were eliminated. Machine learning algorithms were performed with the combination of gray-level run-length matrix-low gray level run emphasis, gray-level co-occurrence matrix-correlation, and gray-level co-occurrence entropy. RESULTS: A total of 34 patients were included, 17 of whom had hypophysitis and 17 had non-functioning pituitary adenomas. Among the 38 radiomics parameters obtained from post-contrast T1-weighted images, 10 tissue features that could differentiate the lesions were selected. Machine learning algorithms were performed using three selected parameters; gray level run length matrix-low gray level run emphasis, gray-level co-occurrence matrix-correlation, and gray level co-occurrence entropy. Error matrices were calculated by using the machine learning algorithm and it was seen that support vector machines showed the best performance in distinguishing the two lesion types. CONCLUSIONS: Our analysis reported that support vector machines showed the best performance in distinguishing hypophysitis from non-functioning pituitary adenomas, emphasizing the importance of machine learning in differentiating the two lesions.


Asunto(s)
Hipofisitis , Neoplasias Hipofisarias , Humanos , Aprendizaje Automático , Imagen por Resonancia Magnética/métodos , Neoplasias Hipofisarias/diagnóstico por imagen , Neoplasias Hipofisarias/patología , Estudios Retrospectivos
12.
Turk Patoloji Derg ; 38(2): 83-89, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35147974

RESUMEN

OBJECTIVE: Pediatric renal tumors overlap histomorphologically and may cause misdiagnosis. We aimed to determine the role of immunohistochemical staining of Cyclin D1, PTEN, beta-catenin and PDGFR-alpha on pediatric renal tumors. MATERIAL AND METHOD: Thirty-six cases of 8 different tumors were included in the study. Four blocks of paraffin tissue microarray were constructed. Cyclin D1, PTEN, beta-catenin and PDGFR-alpha were used in all cases. Staining intensity and extent were graded. RESULTS: All cases of clear cell sarcoma (CCS) and epithelial components of Wilms tumor (WT) showed immunopositivity for Cyclin D1 but blastemal and stromal components of WT were negative. All cases of CCS and most cases of WT consisting of blastemal and stromal components demonstrated loss of expression with PTEN. CONCLUSION: Cyclin D1 is not a specific immunohistochemical marker due to its strong and diffuse positivity in CCS cases. It may be useful to differentiate CCS from blastemal and stromal components of WT. Other markers except cyclin D1 do not have a role in the differential diagnosis.


Asunto(s)
Neoplasias Renales , Sarcoma de Células Claras , Tumor de Wilms , Biomarcadores de Tumor/metabolismo , Niño , Ciclina D1/metabolismo , Diagnóstico Diferencial , Femenino , Humanos , Inmunohistoquímica , Neoplasias Renales/patología , Masculino , Fosfohidrolasa PTEN , Receptor alfa de Factor de Crecimiento Derivado de Plaquetas , Sarcoma de Células Claras/patología , Tumor de Wilms/diagnóstico , beta Catenina/metabolismo
13.
Front Neurol ; 13: 1086591, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36588881

RESUMEN

Background: Symptomatic spinal metastases of oligodendroglioma are rare. Moreover, none of the previously published cases demonstrated the typical IDH mutation and 1p/19q-codeletion for this glial tumor. This case presents an IDH mutant, 1p/19q-codeleted oligodendroglioma with multiple spinal drop metastases. Case description: We report a case of a 55-year-old woman with left frontal grade 3 oligodendroglioma diagnosed 3 years ago. No tumor recurrence was observed in post-operative follow-up MRI examinations. However, she was admitted to our institution again with severe low back pain. Gadolinium enhanced MRI of the spine revealed an intradural, extramedullary metastatic lesion between T11-L1 levels and multiple enhancing metastatic tumor deposits around cauda equine roots between L4-S1. T11-T12 midline laminectomy was performed and gross total resection of metastatic lesions was achieved. Final histological diagnosis of the spinal lesions was WHO Grade 3 Oligodendroglioma, IDH-mutant, 1p/19q-codeleted. Conclusion: This case is the first molecularly-defined spinal metastatic oligodendroglioma. The possibility of drop metastasis should be kept in mind in oligodendroglioma patients with spinal cord-related symptoms. There is no standard approach for the diagnosis and treatment of spinal metastases of this type of glial tumor.

14.
Exp Clin Endocrinol Diabetes ; 130(2): 125-133, 2022 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-34255320

RESUMEN

PURPOSE: To investigate the relationship of Fibroblast Growth Factor Receptor-4 (FGFR-4) expression with radiologic, pathologic, and clinical parameters in pituitary adenomas. METHODS: Among 307 patients who underwent pituitary surgery for a pituitary adenoma between 2000 and 2015, we included 161 patients (53 gonadotroph, 26 corticotroph, 25 null cell, 22 lactotroph, 13 somatotroph, 8 adenomas with unusual combination, 7 Pit-1 positive adenomas, and 7 lactosomatotroph) based on availability of pathology specimens. Patients' radiologic, pathologic, and clinical parameters were determined. FGFR-4 immunostaining was evaluated using a semi-quantitative histologic score (H-score). RESULTS: The mean follow-up period was 61 (IQR=32-84) months. The median H-scores for FGFR-4 were higher in patients without remission, those with residual lesion, and T2-hyperintense adenoma (p<0.05). Ki-67 level was higher in patients without remission compared to those in remission (p<0.05). The mean Ki-67 levels did not differ between patients with and without residual lesion or T2-hyperintense tumor (p>0.05). There was no significant difference (p>0.05) when the H-score and Ki-67 levels were assessed in terms of sex, sellar-dural invasion, Knosp and a grading system for superior, inferior, parasellar, anterior and posterior tumor extension Classification, tumor function or presence of poor subtype. Adenomas with Ki-67 expression ≥3% had higher FGFR4 expression levels than those with <3% expression (p=0.002). There was a weak positive correlation between H-score and Ki-67 (p=0.011; r=0.201). CONCLUSIONS: Higher levels of FGFR-4 in pituitary adenomas could be use a marker for more aggressive tumor behavior.


Asunto(s)
Adenoma/metabolismo , Adenoma/patología , Biomarcadores de Tumor/metabolismo , Neoplasias Hipofisarias/metabolismo , Neoplasias Hipofisarias/patología , Receptor Tipo 4 de Factor de Crecimiento de Fibroblastos/metabolismo , Adulto , Femenino , Estudios de Seguimiento , Humanos , Antígeno Ki-67/metabolismo , Masculino , Persona de Mediana Edad
15.
J Clin Neurosci ; 90: 105-111, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34275532

RESUMEN

BACKGROUND: Any preoperative diagnostic assessment that can predict the success of the operation in acromegaly will provide a positive impact on overall remission rates. The aim of this study is to reveal whether the signal intensity in T2-weighted Magnetic Resonance Imaging can predict postoperative results in acromegaly patients with macroadenoma. METHODS: We analyzed our surgical results in regard to T2-weighted images in newly diagnosed consecutive 124 patients with acromegaly, operated between 2014 and 2019. The T2-intensity of the pure somatotroph macroadenomas was correlated with the clinical, radiological, surgical and histopathological characteristics of the acromegaly patients. RESULTS: We found a predominance of T2-hyperintensity in our series (45%) and the T2-hypointense pure somatotroph adenomas were detected in only 34% of our patients. Total resection was performed in 72% of newly diagnosed acromegaly patients in this series. Accordingly, total resection was achieved in 69% of the T2-hyperintense group, 77% of the T2-hypointense group and 69% of the T2-isointense group. The surgical remission rates for the T2-hyper-, hypo- and isointense groups were 54.5%, 80.7%, and 68.7%, respectively. The surgical remission rate in the T2-hyperintense group was significantly lower than those of hypo- and isointense groups in newly diagnosed acromegaly patients. CONCLUSIONS: This study demonstrates a close relationship between the T2 signal intensity and the surgical remission rates in acromegaly patients with macroadenoma. Preoperative T2-intensity images may predict the probability of post-surgical remission in patients with newly diagnosed acromegaly. Further support fort this idea comes from recent guidelines for acromegaly management in which the potential utility of using T2 intensity to optimize patient management has been emphasized.


Asunto(s)
Acromegalia/diagnóstico por imagen , Adenoma/diagnóstico por imagen , Neoplasias Hipofisarias/diagnóstico por imagen , Acromegalia/etiología , Acromegalia/cirugía , Adenoma/complicaciones , Adenoma/cirugía , Adulto , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Neoplasias Hipofisarias/complicaciones , Neoplasias Hipofisarias/cirugía , Pronóstico , Inducción de Remisión , Estudios Retrospectivos , Resultado del Tratamiento , Adulto Joven
16.
J Craniofac Surg ; 32(7): e618-e620, 2021 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-33654042

RESUMEN

ABSTRACT: Odontogenic myxofibroma is a benign odontogenic tumor of mesenchymal tissue that generally originates from the mandible. It is an extremely rare tumor accounting for approximately 2% of all odontogenic tumors. In this report, the authors presented a giant right mandibular mass that extends to the angle of the mandible and displacing the tongue laterally caused significant malocclusion, pain, and impaired oral intake. The tumoral involvement of the mandibula required a partial mandibulectomy with the resection of right mandibular ramus, body, and bilateral parasymphysis. Right mandibular condyle was preserved. The mandibular defect was reconstructed with a fibula free flap. Three-dimensional printed maxillofacial bone model of the patient was used as contour modeling and guide. Histopathologic examination confirmed the diagnosis as odontogenic myxofibroma.The purpose of the report is to present a very rare case of odontogenic myxofibroma and its general characteristics, radiological features, differential diagnosis, surgical management, and the use of three-dimensional printing technology in the field of mandibular reconstruction.


Asunto(s)
Fibroma , Colgajos Tisulares Libres , Neoplasias Mandibulares , Reconstrucción Mandibular , Tumores Odontogénicos , Peroné , Humanos , Mandíbula , Neoplasias Mandibulares/diagnóstico por imagen , Neoplasias Mandibulares/cirugía , Tumores Odontogénicos/diagnóstico por imagen , Tumores Odontogénicos/cirugía , Tecnología
17.
Cartilage ; 13(2_suppl): 684S-691S, 2021 12.
Artículo en Inglés | MEDLINE | ID: mdl-32613847

RESUMEN

OBJECTIVE: The aim of this study is to evaluate the effects of intraarticular insulin on the treatment of chondral defects. DESIGN: Twenty-four mature New Zealand rabbits were randomly divided into 3 groups as control (Group 1), microfracture (Group 2), and microfracture and insulin (Group 3). Four-millimeter full-thickness cartilage defects were created to the weight-bearing surface on the medial femoral condyles of each rabbit. In the first group, any additional interventions were not performed. Microfracture was performed on defects in groups 2 and 3. Additionally, 10 IU of insulin glargine was administrated into the knee joints of the third group. Three months after surgery, the knee joints were harvested and cartilage quality was assessed according to Wakitani and ICRS (International Cartilage Repair Society) scores histopathologically. Insulin injections were performed into the knees of 2 additional rabbits without creating a cartilage defect to evaluate the potential adverse effects of insulin on healthy cartilage (Group 4). RESULTS: The total ICRS and Wakitani scores of the insulin group were found to be significantly lower than the microfracture group but similar to the control group. No negative effects of insulin on healthy cartilage were detected. Intraarticular insulin after surgery has led to a statistically significant decrease in systemic blood sugar levels whereas the decrease observed after administration to intact tissues was not statistically significant. CONCLUSIONS: Insulin had a negative influence on the quality of cartilage regeneration and had no effect on healthy cartilage. Intraarticular insulin administration does not cause significant systemic effects in intact tissue.


Asunto(s)
Enfermedades de los Cartílagos , Cartílago Articular , Fracturas del Cartílago , Animales , Conejos , Enfermedades de los Cartílagos/tratamiento farmacológico , Enfermedades de los Cartílagos/cirugía , Cartílago Articular/lesiones , Cartílago Articular/cirugía , Insulina/uso terapéutico , Articulación de la Rodilla/cirugía
18.
Exp Clin Endocrinol Diabetes ; 129(9): 634-643, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-33091936

RESUMEN

PURPOSE: To evaluate the expression of G-protein coupled estrogen receptor (GPER1), aromatase, estrogen receptor α (ERα), estrogen receptor ß (ERß), pituitary tumor transforming gene (PTTG), and fibroblast growth factor 2 (FGF2) in GH-secreting and non-functioning adenomas (NFA). METHODS: Thirty patients with acromegaly and 27 patients with NFA were included. Gene expression was determined via quantitative reverse transcription polymerase chain reaction (QRT-PCR). Protein expression was determined via immunohistochemistry. RESULTS: There was no difference, in terms of gene expression of aromatase, ERα, PTTG, and FGF2 between the two groups (p>0.05 for all). ERß gene expression was higher and GPER1 gene expression was lower in GH-secreting adenomas than NFAs (p<0.05 for all). Aromatase and ERß protein expression was higher in GH-secreting adenomas than NFAs (p=0.01). None of the tumors expressed ERα. GPER1 expression was detected in 62.2% of the GH-secreting adenomas and 45% of NFAs. There was no difference in terms of GPER1, PTTG, FGF2 H scores between the two groups (p>0.05 for all). GPER1 gene expression was positively correlated to ERα, ERß, PTTG, and FGF2 gene expression (p<0.05 for all). There was a positive correlation between aromatase and GPER1 protein expression (r=0.31; p=0.04). CONCLUSIONS: GPER1 is expressed at both gene and protein level in a substantial portion of GH-secreting adenomas and NFAs. The finding of a positive correlation between GPER1 and ERα, ERß, PTTG, and FGF2 gene expression and aromatase and GPER1 protein expression suggests GPER1 along with aromatase and classical ERs might mediate the effects of estrogen through upregulation of PTTG and FGF2.


Asunto(s)
Acromegalia/metabolismo , Adenoma/metabolismo , Hormona de Crecimiento Humana/metabolismo , Neoplasias Hipofisarias/metabolismo , Receptores de Estrógenos/sangre , Receptores Acoplados a Proteínas G/sangre , Acromegalia/sangre , Adenoma/sangre , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Hipofisarias/sangre
19.
Clin Pathol ; 13: 2632010X20964846, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33241207

RESUMEN

AIM: We aimed to show the immunohistochemical expression of programmed death ligand 1 (PD-L1) in laryngeal squamous cell carcinomas (SCCs). MATERIALS AND METHODS: The study includes 52 laryngeal SCC cases that underwent surgical resection. Immunohistochemical staining of PD-L1 (Clone 22C3) was applied to the sections obtained from paraffin blocks. Combined Positive Score (CPS) was evaluated as described in manuals. Tumor Proportion Score (TPS) was assessed by the percentage of positive tumor cells which were designated as positive if ⩾1% of the tumor cells showed membranous staining. RESULTS: There were 35 cases (67.3%) having CPS < 1 and 17 cases (32.7%) having CPS ⩾ 1. There was no relationship between CPS, TPS, and the clinicopathological data. CONCLUSION: Further studies with a large number of advanced-stage cases are needed.

20.
JAAPA ; 33(11): 29-31, 2020 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-33109980

RESUMEN

Chondrosarcoma, a malignant bone tumor, is rarely encountered in the cervical spine. This article describes a patient whose neck pain and dysphagia were caused by an expansive, destructive lesion with calcification that was located in the body of the axis (C2 vertebra), the first time a chondrosarcoma has been reported in this location.


Asunto(s)
Vértebra Cervical Axis/cirugía , Condrosarcoma/cirugía , Endoscopía/métodos , Neoplasias de la Columna Vertebral/cirugía , Adulto , Vértebra Cervical Axis/diagnóstico por imagen , Vértebra Cervical Axis/patología , Vértebras Cervicales , Condrosarcoma/diagnóstico por imagen , Condrosarcoma/patología , Imagen de Difusión por Resonancia Magnética , Femenino , Humanos , Neoplasias de la Columna Vertebral/diagnóstico por imagen , Neoplasias de la Columna Vertebral/patología , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
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