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1.
Asian J Neurosurg ; 16(1): 200-203, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34211895

RESUMEN

Intravascular papillary endothelial hyperplasia (IPEH) also known as Masson's tumor, is a benign, slow growing, vascular lesion which is seen very rarely and only a few cases have been reported intracranially in the literature. It has been reported at many sites, but the posterior fossa involvement is very rare. The preoperative diagnosis is very difficult, as there is no enough cases to achieve a clear understanding about the details of its radiological findings. Differential diagnosis have to be made especially from angiosarcoma and meningioma. It is curable by total surgical removal. In this article we presented the characteristic clinical, radiological, perioperative and pathological findings in a case of IPEH in an unusual location, origin and behavior. To best of our knowledge, we presented the first case of IPEH originating from tentorium.

2.
Histopathology ; 79(1): 23-33, 2021 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-33406290

RESUMEN

AIMS: Hepatocellular adenoma (HCA) is an uncommon liver neoplasm, and studies of HCA subtypes have been primarily limited to France, the USA, and Japan. The aim of this study was to describe the clinicopathological features of HCA subtypes in Turkey. METHODS AND RESULTS: The resection specimens of 59 cases diagnosed as 'hepatocellular adenoma' collected from 15 institutions were reviewed to confirm the diagnosis and to classify them according to the current World Health Organization 2019 classification. Immunostaining for glutamine synthetase, liver fatty acid-binding protein, C-reactive protein, ß-catenin and reticulin was performed. Of the 59 cases, 48 (81%) were diagnosed as HCA. We identified 24 (50%) hepatocyte nuclear factor 1α (HNF1α)-inactivated HCAs, five (10%) inflammatory HCAs, 15 (32%) ß-catenin-activated HCAs, three (6%) ß-catenin-activated inflammatory HCAs, and one (2%) unclassified HCA. HCA patients were predominantly female (female/male ratio of 5:1); they had a median age of 34 years and a median tumour diameter of 60 mm. In the ß-catenin-activated HCA group, nine cases (19%) showed cytoarchitectural atypia, and were also referred to as atypical hepatocellular neoplasms. In the ß-catenin-activated HCA group, three cases (6%) showed focal areas supportive of transition to HCA. The original diagnosis of HCA was changed to well-differentiated hepatocellular carcinoma in nine cases and to focal nodular hyperplasia in two cases. CONCLUSION: In our series, the major HCA subtype was HNF1α-inactivated HCA. We found a low incidence of inflammatory-type HCA. Our data also showed that ß-catenin-activated hepatocellular neoplasms, including cases with atypical histology, constituted a relatively high proportion of the cases. These findings are in contrast to those of most other studies of HCA subtypes.


Asunto(s)
Adenoma de Células Hepáticas/clasificación , Adenoma de Células Hepáticas/patología , Neoplasias Hepáticas/clasificación , Neoplasias Hepáticas/patología , Adolescente , Adulto , Anciano , Biomarcadores de Tumor/análisis , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Turquía , Organización Mundial de la Salud , Adulto Joven
3.
Hepatol Forum ; 2(1): 12-19, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-35782888

RESUMEN

Background and Aim: This study was designed to predict the fibrosis stage with a clinical scoring system that may reduce the need for liver biopsy. Materials and Methods: The study cohort included the treatment of 430 chronic hepatitis B (CHB) and 170 chronic hepatitis C (CHC) of naive patients. The patients were divided into two groups as mild to moderate and severe fibrosis. After an index obtained in the study cohort, the index was tested in a validation cohort and compared with the FIB-4 Index. Results: The AUC of CHC index was found of 0.89 the sensitivity of 0.91 the specificity of 0.74, the positive predictive value (PPV) of 0.54 and the negative predictive value (NPV) of 0.96. The FIB-4 Index was applied to the CHC study cohort and the ATA Index Hepatitis C was found to be superior in terms of AUC (0.89-0.82), sensitivity (0.91-0.76) and NPV (0.96-0.86). The AUC of CHB Index was determined of 0.92, the sensitivity of 0.90, the specificity of 0.84, the PPV of 0.53 and the NPV of 0.98. Compared to the FIB-4 Index in CHB study cohort, the ATA Index Hepatitis B was predominant in terms of AUC (0.92-0.88), sensitivity (0.90-0.75), NPV (0.98-0.94) and PPV (0.53-0.49). Conclusion: ATA Indexes can predict the non-existence of severe fibrosis with an accuracy similar to FIB-4 Index and may reduce the need for liver biopsy.

4.
J Plast Surg Hand Surg ; 53(6): 335-340, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31240978

RESUMEN

The stromal vascular fraction (SVF) obtained from inguinal adipose tissue was injected into the sciatic nerve region in diabetic rats. The effects of the SVF on the sciatic nerve and functional, electrophysiological and histopathological changes were examined in this study. Rats were divided into five groups; a non-diabetic control group, and four diabetic groups. In the first diabetic rat group, the SVF was obtained from inguinal adipose tissue. The remaining diabetic groups included a sham control group, a phosphate-buffered saline (PBS) injection group and an SVF injection group. Injections were made into the sciatic nerve region. Electromyography and walking track analyses were conducted on all groups at the beginning of the experiment. Diabetes was induced via a single dose of streptozotocin. Walking track analysis and electromyography measurements were repeated in week 8. SVF or PBS was injected into the right sciatic nerve region on week 8 of experiment group rats. Walking track analysis and electromyography were repeated in week 12 and all sciatic nerves were examined histopathologically. In the diabetic SVF group, the sciatic functional index calculated from walking track analysis in week 12 was better than week 8. Additionally, the myelin sheaths of the right sciatic nerve were thicker and more uniform and the nerve fibers were thicker than those of the left, untreated sciatic nerve. No statistical differences were detected in electromyographic measurements. The adipose-derived SVF may be beneficial for nerve regeneration in diabetic neuropathy.


Asunto(s)
Tejido Adiposo/citología , Neuropatías Diabéticas/terapia , Regeneración Nerviosa , Trasplante de Células Madre , Células del Estroma/trasplante , Animales , Diabetes Mellitus Experimental , Electromiografía , Análisis de la Marcha , Microscopía , Vaina de Mielina/patología , Fibras Nerviosas/patología , Ratas Wistar , Nervio Ciático/fisiología
5.
Endocr Pathol ; 30(1): 49-55, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-30610566

RESUMEN

The fourth edition of the World Health Organization (WHO) classification of pituitary tumors recommended evaluation of tumor proliferation and invasion to identify aggressiveness. We aimed to assess the prognostic roles of the Ki-67 proliferation index, mitotic index, P53 expression, and cavernous sinus invasion in pituitary adenomas (PAs). Among the 601 patients who underwent transnasal/transsphenoidal adenomectomy from 2001 to 2016, 101 patients (16.8%) who had tumors with a high (≥ 3%) Ki-67 index (group A) and a control group consisting of 43 patients with a low (< 3%) Ki 67 index who were matched for age, gender, and tumor type were included. Mitotic index and P53 expressions were evaluated. Patient characteristics, histopathology reports, pre/postoperative magnetic resonance imaging (MRI), and follow-up data were assessed retrospectively. The frequency of macroadenomas and mean tumor size were greater in group A when compared to group B (67.4 vs. 94.1%, p< 0.01 and 25 ± 10.6 vs. 18 ± 11 mm, p< 0.01, respectively). Invasion to cavernous sinus was found in 53 (36.8%) patients and was more frequent in group A (p<0.01). The mean number of surgery was higher in group A than group B (p< 0.05). The mean follow-up period was 46.6 ± 34 months. The postoperative MRIs and follow-up data for at least 24 months were available in 117 patients. Recurrence risk was higher in group A than group B (p = 0.03). Tumors with high Ki-67 proliferation index were grouped as 3-5, 6-10, 11-15, and > 15%. The risk of recurrence was not different between groups of high Ki-67 index. The optimal cutoff point of the Ki-67 proliferation index that predicted recurrence was 2.5% with 84.6% sensitivity and 47.4% specificity. The cavernous sinus invasion on MRI was associated with recurrence (p = 0.03). Tumor size and recurrence risk were not associated with P53 expression. High P53 expression was related with cavernous sinus invasion (p = 0.03). The mitotic index was not associated with recurrence risk and tumor invasion. Recurrence risk was higher in tumors with ≥ 2 histopathological atypia criteria (p = 0.01). High Ki-67 index with a 2.5% cutoff point and cavernous sinus invasion on MRI are reliable markers for predicting recurrence in PAs. Recurrence risk is also higher in tumors with two histopathological aggressiveness criteria. Strict follow-up and more aggressive treatment approaches may be necessary for invasive-proliferative PAs.


Asunto(s)
Adenoma/patología , Proliferación Celular/fisiología , Antígeno Ki-67/metabolismo , Neoplasias Hipofisarias/patología , Proteína p53 Supresora de Tumor/metabolismo , Adenoma/metabolismo , Adulto , Biomarcadores de Tumor/metabolismo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Índice Mitótico , Neoplasias Hipofisarias/metabolismo , Pronóstico , Estudios Retrospectivos
6.
Int J Ophthalmol ; 11(8): 1296-1302, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30140632

RESUMEN

AIM: To evaluate the clinical, radiological, and treatment features of lacrimal gland tumors. METHODS: Retrospective review of 99 eyes of 92 patients with lacrimal gland tumors diagnosed and managed in a single institution between January 1999 and March 2017. Clinical and radiological features, histopathology, treatment methods, and prognosis were evaluated. RESULTS: The mean patient age was 40.3 (range: 7-80)y. The diagnosis was made histopathologically in 91 (91.9%) tumors and on a clinical and radiological basis in 8 (8.1%) tumors. Final diagnoses included idiopathic orbital inflammation (pseudotumor) in 46 (46.5%) lesions, pleomorphic adenoma in 14 (14.1%), adenoid cystic carcinoma in 12 (12.1%), granulomatous inflammation in 10 (10.1%), lymphoma in 5 (5.0%), benign reactive lymphoid hyperplasia in 3 (3.0%), dacryops in 3 (3.0%), carcinoma ex pleomorphic adenoma in 2 (2.0%), adenocarcinoma in 1 (1.0%), dermoid cyst in 1 (1.0%), cavernous hemangioma in 1 (1.0%), and leukemic infiltration in 1 (1.0%). Non-epithelial tumors comprised 64.6% (n=64) of all lacrimal gland tumors, epithelial tumors 32.3% (n=32), dermoid cyst 1% (n=1), cavernous hemangioma 1% (n=1), and leukemic infiltration 1% (n=1). There were in total 78 (78.8%) benign and 21 (21.2%) malignant tumors. CONCLUSION: Overall, 65% of lacrimal gland tumors were of non-epithelial origin and 32% of epithelial origin. By histopathology and clinical evaluation, 79% of lacrimal gland tumors were benign. The most common lacrimal gland tumors include idiopathic orbital inflammation (46.5%), epithelial (32.3%), and lymphoproliferative (8.1%) lesions.

7.
J Neurosurg Pediatr ; 22(2): 151-157, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29726794

RESUMEN

In August 2016, an 11-year-old boy presented to the authors' institution with a right orbital tumor that was located superotemporally (superolaterally) and adherent to the sclera. The patient's past medical history revealed that he had undergone 2 previous craniotomies elsewhere in June 2008 and July 2010 for a superomedially located orbital lesion that had been histopathologically diagnosed as a neurothekeoma. After the second craniotomy, the patient underwent adjuvant intensity modulated radiotherapy (IMRT) to the right medial orbit. At the authors' institution, total excision of the orbital tumor was performed via an anterior conjunctival orbitotomy. Histopathological examination revealed a rhabdoid meningioma. Review of the histopathology obtained at the time of previous tumor excisions showed that the lesion was misdiagnosed as neurothekeoma and instead represented a meningioma from the beginning. The patient was started on a regimen of oral sunitinib and remained free of recurrence at 1.5 years of follow-up. Ectopic meningioma of the orbit is a rare entity. Rhabdoid meningioma is a rarely seen subtype of meningioma, accounting for 1%-3% of all intracranial meningiomas. To the best of the authors' knowledge, this is the first case of an ectopic orbital rhabdoid meningioma reported in the literature. They suspect that tumor seeding during the previous surgeries might have played a role in the occurrence of the tumor in an orbital location not targeted by IMRT.


Asunto(s)
Craneotomía/métodos , Neoplasias Meníngeas/terapia , Meningioma/terapia , Radioterapia Adyuvante/métodos , Tumor Rabdoide/terapia , Niño , Humanos , Imagen por Resonancia Magnética , Masculino , Neoplasias Meníngeas/complicaciones , Neoplasias Meníngeas/diagnóstico por imagen , Meningioma/complicaciones , Meningioma/diagnóstico por imagen , Mucina-1/metabolismo , Neurotecoma/complicaciones , Neurotecoma/terapia , Tumor Rabdoide/complicaciones , Tumor Rabdoide/diagnóstico por imagen , Proteína SMARCB1/metabolismo , Resultado del Tratamiento
8.
Childs Nerv Syst ; 34(8): 1583-1587, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29557987

RESUMEN

INTRODUCTION: Dermoid cysts (DCs) are unusual benign congenital intracranial tumors that typicallyarise in the midline and form as a result of abnormal sequestration of ectodermal cells during neural tubeformation. In all age groups, endoscopic approaches are preferable for the removal of sellar lesions. A 6-year-old girl with recurrent meningitis underwent endoscopic endonasal surgery forsellar DC. CONCLUSION: To the best of our knowledge, we present the first case of a suprasellar DC in a pediatric patient that was removed endoscopically.


Asunto(s)
Neoplasias Encefálicas/cirugía , Quistes del Sistema Nervioso Central/cirugía , Quiste Dermoide/cirugía , Neuroendoscopía/métodos , Neoplasias Encefálicas/diagnóstico por imagen , Quistes del Sistema Nervioso Central/diagnóstico por imagen , Niño , Quiste Dermoide/diagnóstico por imagen , Femenino , Humanos , Cavidad Nasal/diagnóstico por imagen , Cavidad Nasal/cirugía
9.
Surv Ophthalmol ; 63(1): 114-118, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-28254446

RESUMEN

Orbital cellulitis associated with retinoblastoma is uncommon and is characterized by noninfectious inflammation of the periorbital structures. The underlying mechanism is thought to be necrosis of the intraocular tumor, leading to intraocular and periorbital inflammation. We report 2 retinoblastoma patients who presented with an orbital cellulitis-like picture and discuss clinical characteristics, histopathologic features, and treatment.


Asunto(s)
Celulitis Orbitaria/patología , Neoplasias de la Retina/patología , Retinoblastoma/patología , Preescolar , Femenino , Humanos , Masculino , Necrosis
10.
Turk Patoloji Derg ; 34(1): 41-48, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-28984336

RESUMEN

OBJECTIVE: As there is continuing disagreement among the observers on the differential diagnosis between the epithelial changes/lesions and neoplasms of the gallbladder, this multicentre study was planned in order to assess the rate of the epithelial gallbladder lesions in Turkey and to propose microscopy and macroscopy protocols. MATERIAL AND METHOD: With the participation of 22 institutions around Turkey that were included in the Hepato-Pancreato-Biliary Study Group, 89,324 cholecystectomy specimens sampled from 2003 to 2016 were retrospectively evaluated. The numbers of adenocarcinomas, dysplasias, intracholecystic neoplasms/adenomas, intestinal metaplasias and reactive atypia were identified with the review of pathology reports and the regional and countrywide incidence rates were presented in percentages. RESULTS: Epithelial changes/lesions were reported in 6% of cholecystectomy materials. Of these epithelial lesions, 7% were reported as adenocarcinoma, 0.9% as high-grade dysplasia, 4% as low-grade dysplasia, 7.8% as reactive/regenerative atypia, 1.7% as neoplastic polyp, and 15.6% as intestinal metaplasia. The remaining lesions (63%) primarily included non-neoplastic polypoids/hyperplastic lesions and antral/pyloric metaplasia. There were also differences between pathology laboratories. CONCLUSION: The major causes of the difference in reporting these epithelial changes/lesions and neoplasms include the differences related to the institute's oncological surgery frequency, sampling protocols, geographical dissimilarities, and differences in the diagnoses/interpretations of the pathologists. It seems that the diagnosis may change if new sections are taken from the specimen when any epithelial abnormality is seen during microscopic examination of the cholecystectomy materials.


Asunto(s)
Enfermedades de la Vesícula Biliar/diagnóstico , Enfermedades de la Vesícula Biliar/patología , Neoplasias de la Vesícula Biliar/diagnóstico , Neoplasias de la Vesícula Biliar/patología , Patología Quirúrgica/normas , Humanos , Patología Quirúrgica/métodos , Estudios Retrospectivos , Turquía
11.
Turk Patoloji Derg ; 34(2): 127-133, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-28984346

RESUMEN

OBJECTIVE: Pancreatic ductal adenocarcinoma is an aggressive tumor with short survival. In this study we aimed to investigate the effect of well-known prognostic parameters on survival in these tumors. MATERIAL AND METHOD: A total of 56 pancreatic ductal adenocarcinoma cases diagnosed between 2005 and 2014 were included in the study. Survival data were obtained and histopathological parameters were re-evaluated in each patient. RESULTS: Tumor size (p=0.029), mitotic count (p=0.030), lymph node metastasis (p=0.003), metastatic lymph node ratio (p < 0.001) and ampullary invasion (p=0.044) had a statistically significant relationship with survival. However, there was no relationship between survival and tumor grade, lymphovascular and perineural invasion, and peripancreatic soft tissue invasion. CONCLUSION: Our results showed that existent 2010 WHO pancreatic ductal adenocarcinoma grading parameters excluding mitotic count are subjective and not applicable. Considering that almost all of the tumors in our series were larger than 2 cm, we think that the 2 cm cut-off in tumor size is insufficient to make the tumor stage pT2. Peripancreatic soft tissue invasion, which is a common finding in pancreatic ductal adenocarcinoma, should also not be assessed like adjacent tissue invasion and make the tumor reach pT3 stage independent of tumor size. It is clear that the existent WHO tumor grading and pT staging parameters need to be revised and the mitotic count, which correlates with survival, should be presented in pathology reports.


Asunto(s)
Carcinoma Ductal Pancreático/mortalidad , Carcinoma Ductal Pancreático/patología , Neoplasias Pancreáticas/mortalidad , Neoplasias Pancreáticas/patología , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Clasificación del Tumor/métodos , Clasificación del Tumor/normas , Estadificación de Neoplasias/métodos , Estadificación de Neoplasias/normas , Pronóstico , Organización Mundial de la Salud , Neoplasias Pancreáticas
12.
Horm Res Paediatr ; 85(6): 421-5, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-27173951

RESUMEN

BACKGROUND: Isolated hyperinsulinaemic hypoglycaemia (HH) commonly results from recessively inherited mutations in the ABCC8 and KCNJ11 genes that are located on chromosome 11p15.1. More rarely, HH can feature in patients with Beckwith-Wiedemann syndrome (BWS), a congenital overgrowth disorder, resulting from defects at a differentially methylated region telomeric to the K-ATP channel genes at chromosome 11p15.5. SUBJECT: We undertook genetic testing in a patient with diazoxide-unresponsive HH diagnosed at birth. Physical examination later revealed hemihypertrophy of the right arm, a feature of BWS. RESULTS: We identified a novel mosaic, paternally-inherited KCNJ11 mutation(s) in the patient. Further analysis confirmed uniparental disomy (UPD) of chromosome 11, which extended across the KCNJ11 gene at 11p15.1 and the BWS locus at 11p15.5. CONCLUSION: These results highlight the importance of considering UPD as a mechanism of disease in patients with HH and a paternally inherited K-ATP channel mutation, especially when additional syndromic features are present.


Asunto(s)
Síndrome de Beckwith-Wiedemann/genética , Cromosomas Humanos Par 11/genética , Hiperinsulinismo Congénito/genética , Mosaicismo , Mutación , Canales de Potasio de Rectificación Interna/genética , Disomía Uniparental/genética , Síndrome de Beckwith-Wiedemann/patología , Hiperinsulinismo Congénito/patología , Femenino , Sitios Genéticos , Humanos , Masculino , Disomía Uniparental/patología
13.
Surv Ophthalmol ; 59(6): 643-8, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25444364

RESUMEN

Ectopic orbital meningioma is a rare tumor usually affecting the medial orbit. We present two cases that occurred in a 56-year-old woman and a 28-year-old man. The tumors in both patients were subtotally excised via orbitotomy surgery and were located in the superior quadrant in one of our patients and in the temporal quadrant in the other. Following histopathologic diagnosis, external beam radiotherapy (EBRT) was administered to one patient and intensity modulated radiotherapy to the other. We identified 12 other well-documented cases of ectopic orbital meningioma previously reported. Ectopic meningioma should be considered in the differential diagnosis of medial as well as lateral and superior orbital tumors. The tumor is usually well circumscribed but can be ill defined in imaging studies. There are intralesional calcifications and sclerosis of adjacent bone in some cases. Ectopic orbital meningioma can recur after incomplete excision. Based on the efficacy of EBRT in optic nerve sheath meningioma, we used this treatment to decrease the risk of recurrence in our two patients and found no tumor recurrence at follow-ups of 24 and 74 months, but one patient had severe vision loss from radiation retinopathy.


Asunto(s)
Coristoma/patología , Neoplasias Meníngeas , Meningioma/patología , Neoplasias Orbitales/patología , Adulto , Coristoma/radioterapia , Coristoma/cirugía , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Meningioma/radioterapia , Meningioma/cirugía , Persona de Mediana Edad , Procedimientos Quirúrgicos Oftalmológicos , Neoplasias Orbitales/radioterapia , Neoplasias Orbitales/cirugía , Terapia de Protones , Radioterapia de Intensidad Modulada , Tomografía Computarizada por Rayos X , Agudeza Visual
14.
Clinics (Sao Paulo) ; 69(8): 515-23, 2014 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-25141109

RESUMEN

OBJECTIVES: Breast hamartoma is an uncommon breast tumor that accounts for approximately 4.8% of all benign breast masses. The pathogenesis is still poorly understood and breast hamartoma is not a well-known disorder, so its diagnosis is underestimated by clinicians and pathologists. This study was designed to present our experience with breast hamartoma, along with a literature review. METHOD: We reviewed the demographic data, pathologic analyses and imaging and results of patients diagnosed with breast hamartoma between December 2003 and September 2013. RESULTS: In total, 27 cases of breast hamartoma operated in the Ankara University Medicine Faculty's Department of General Surgery were included in the study. All patients were female and the mean age was 41.8±10.8 years. The mean tumor size was 3.9±2.7 cm. Breast ultrasound was performed on all patients before surgery. The most common additional lesion was epithelial hyperplasia (22.2%). Furthermore, lobular carcinoma in situ was identified in one case and invasive ductal carcinoma was observed in another case. Immunohistochemical staining revealed myoid hamartoma in one case (3.7%). CONCLUSION: Breast hamartomas are rare benign lesions that may be underdiagnosed because of the categorization of hamartomas as fibroadenomas by pathologists. Pathologic examinations can show variability from one case to another. Thus, the true incidence may be higher than the literature indicates.


Asunto(s)
Angiomatosis/patología , Enfermedades de la Mama/patología , Hamartoma/patología , Hiperplasia/patología , Adulto , Angiomatosis/diagnóstico por imagen , Enfermedades de la Mama/diagnóstico por imagen , Femenino , Hamartoma/diagnóstico por imagen , Humanos , Hiperplasia/diagnóstico por imagen , Mamografía , Persona de Mediana Edad , Turquía , Ultrasonografía , Adulto Joven
15.
Clinics ; Clinics;69(8): 515-523, 8/2014. tab, graf
Artículo en Inglés | LILACS | ID: lil-718193

RESUMEN

OBJECTIVES: Breast hamartoma is an uncommon breast tumor that accounts for approximately 4.8% of all benign breast masses. The pathogenesis is still poorly understood and breast hamartoma is not a well-known disorder, so its diagnosis is underestimated by clinicians and pathologists. This study was designed to present our experience with breast hamartoma, along with a literature review. METHOD: We reviewed the demographic data, pathologic analyses and imaging and results of patients diagnosed with breast hamartoma between December 2003 and September 2013. RESULTS: In total, 27 cases of breast hamartoma operated in the Ankara University Medicine Faculty's Department of General Surgery were included in the study. All patients were female and the mean age was 41.8±10.8 years. The mean tumor size was 3.9±2.7 cm. Breast ultrasound was performed on all patients before surgery. The most common additional lesion was epithelial hyperplasia (22.2%). Furthermore, lobular carcinoma in situ was identified in one case and invasive ductal carcinoma was observed in another case. Immunohistochemical staining revealed myoid hamartoma in one case (3.7%). CONCLUSION: Breast hamartomas are rare benign lesions that may be underdiagnosed because of the categorization of hamartomas as fibroadenomas by pathologists. Pathologic examinations can show variability from one case to another. Thus, the true incidence may be higher than the literature indicates. .


Asunto(s)
Adulto , Femenino , Humanos , Persona de Mediana Edad , Adulto Joven , Angiomatosis/patología , Enfermedades de la Mama/patología , Hamartoma/patología , Hiperplasia/patología , Angiomatosis , Enfermedades de la Mama , Hamartoma , Hiperplasia , Mamografía , Turquía
16.
Turk J Gastroenterol ; 24(4): 359-62, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-24254270

RESUMEN

In this paper, we report the case of a 19-year-old male patient who presented with lymphoblastic phase of chronic myeloid leukemia and received an allogeneic bone marrow transplant from his cousin. The patient experienced severe, steroid-refractory acute graft versus-host disease of skin, gastrointestinal tract and liver that required further immunosuppression. However, hepatic graft-versus-host disease was complicated with vanishing bile duct syndrome, characterized by progressive destruction of small intrahepatic bile ducts, which was refractory to all available therapies and eventually led to end-stage liver disease. The pathogenesis and treatment of graft-versus-host disease after allogeneic hematopoietic cell transplantation is discussed with an emphasis on liver transplantation for intractable hepatic graft-versus-host disease.


Asunto(s)
Enfermedades de los Conductos Biliares/etiología , Enfermedades de los Conductos Biliares/patología , Conductos Biliares Intrahepáticos/patología , Enfermedad Injerto contra Huésped/patología , Leucemia Mielógena Crónica BCR-ABL Positiva/terapia , Trasplante de Médula Ósea/efectos adversos , Enfermedad Hepática en Estado Terminal/etiología , Enfermedad Hepática en Estado Terminal/patología , Resultado Fatal , Enfermedad Injerto contra Huésped/tratamiento farmacológico , Enfermedad Injerto contra Huésped/etiología , Humanos , Inmunosupresores/uso terapéutico , Masculino , Adulto Joven
17.
J Pediatr Ophthalmol Strabismus ; 50(5): 296-303, 2013.
Artículo en Inglés | MEDLINE | ID: mdl-23937863

RESUMEN

PURPOSE: To evaluate the presentation patterns and results of management of retinoblastoma at a tertiary care center in Ankara, Turkey, with special emphasis on globe conservation rate in unilateral and bilateral intraocular retinoblastoma. METHODS: Patients were grouped according to the International Classification of Retinoblastoma. For intraocular retinoblastoma, group E and some group D eyes underwent primary enucleation. Secondary enucleation was performed after failure of chemoreduction, focal treatments, external beam radiotherapy (EBRT), and intra-arterial chemotherapy used in various combinations. For extraocular retinoblastoma cases, treatment consisted of enucleation/exenteration or orbital biopsy, high-dose chemotherapy, and EBRT to the orbit and metastatic sites. RESULTS: During the study period from October 1998 to May 2010, 165 of 192 (85.9%) patients had intraocular disease and 27 (14.1%) patients had extraocular disease. In total, primary or secondary enucleation was performed in 70 of 94 eyes with unilateral retinoblastoma (74.5%) and in 34 of 142 eyes with bilateral retinoblastoma (23.9%). The overall globe conservation rate was 69.6%. Only one patient in the intraocular retinoblastoma group died of metastatic retinoblastoma to the central nervous system. Twenty of 27 patients (74.1%) with extraocular retinoblastoma were found to have metastasis to the central nervous system, bone, bone marrow, and/or lymph nodes. At a mean follow-up of 28.0 months (median: 12 months; range: 1 to 120 months), survival was 33.3% despite intensive treatment. CONCLUSIONS: The overall risk of enucleation was 75% in eyes with unilateral retinoblastoma and 24% in eyes with bilateral retinoblastoma. Extraocular retinoblastoma carries a 75% risk of systemic metastasis and 67% risk of death.


Asunto(s)
Neoplasias de la Retina/epidemiología , Retinoblastoma/epidemiología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Enucleación del Ojo , Evisceración del Ojo , Femenino , Humanos , Lactante , Masculino , Radioterapia Adyuvante , Neoplasias de la Retina/diagnóstico , Neoplasias de la Retina/terapia , Retinoblastoma/diagnóstico , Retinoblastoma/terapia , Centros de Atención Terciaria , Resultado del Tratamiento , Turquía/epidemiología
18.
Turk J Haematol ; 30(4): 416-7, 2013 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24385835
19.
Exp Clin Transplant ; 9(5): 349-52, 2011 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-21967264

RESUMEN

X-linked Hyper IgM is characterized by an absence of the CD40 ligand on activated T lymphocytes resulting in defects of both cellular and humoral immunity. Patients usually present with recurrent bacterial and opportunistic infections. Chronic liver disease is seen in about 75% of patients as a complication. Here, we report a 3.5-year-old boy with X-linked Hyper IgM referred to our clinic for bone marrow transplant. He was transplanted from an HLA-identical sibling donor using a new conditioning agent, treosulphan, together with cyclophosphamide. Since 6 months of age, he has had recurrent respiratory infections, and his XHIGM was diagnosed when he was 1.5 years old. The diagnosis was confirmed by sequence analysis of the CD40L gene. On physical examination, growth failure, bilateral fine crackles in both lungs, and hepatosplenomegaly were detected. The results of his liver function tests were abnormal, and a liver biopsy showed grade III fibrosis and compensated cirrhosis. After conditioning with treosulphan (12 g/m(2)/d x 3 d) and cyclophosphamide (50 mg/kg/d x 4 d), bone marrow from his HLA-identical sister was infused. CD40L expression on activated lymphocytes of the patient was 84% on day +21. His posttransplant period was uneventful. He is now at posttransplant 2 years, with full donor chimerism, and mild, chronic, graft-versus-host disease on his tongue. In conclusion, treosulphan is a new agent for conditioning regimen with less toxicity in patients with severe liver disease.


Asunto(s)
Trasplante de Médula Ósea , Busulfano/análogos & derivados , Ligando de CD40/deficiencia , Síndrome de Inmunodeficiencia con Hiper-IgM Tipo 1/cirugía , Cirrosis Hepática/cirugía , Agonistas Mieloablativos/uso terapéutico , Acondicionamiento Pretrasplante/métodos , Biopsia , Trasplante de Médula Ósea/efectos adversos , Busulfano/efectos adversos , Busulfano/uso terapéutico , Preescolar , Ciclofosfamida/uso terapéutico , Prueba de Histocompatibilidad , Humanos , Síndrome de Inmunodeficiencia con Hiper-IgM Tipo 1/inmunología , Síndrome de Inmunodeficiencia con Hiper-IgM Tipo 1/patología , Cirrosis Hepática/inmunología , Cirrosis Hepática/patología , Masculino , Agonistas Mieloablativos/efectos adversos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Acondicionamiento Pretrasplante/efectos adversos , Resultado del Tratamiento
20.
Turk J Gastroenterol ; 22(3): 305-14, 2011 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-21805422

RESUMEN

BACKGROUND/AIMS: Liver biopsy to assess fibrosis is invasive and prone to sampling error. While algorithms of serum markers to predict fibrosis stage have been described for chronic hepatitis C, these cannot be applied equally well to hepatitis B. METHODS: We therefore determined 9 serum fibrosis markers, liver biochemical tests and ultrasound parameters in 109 consecutive adult patients with chronic hepatitis B and D. All patients had compensated liver disease. Using the METAVIR score, advanced disease was defined as fibrosis stage ≥F2, and active inflammation as grade ≥A2. A gold standard was created considering splenomegaly and/or platelets <150,000 as indicators of advanced fibrosis irrespective of histology. Area under receiver operating characteristics curves was used for assessment of single markers and odds ratio for their combinations. RESULTS: Patients with advanced disease were older, had lower albumin, higher gamma glutamyl transferase and lower platelet. Levels of 6 of the 9 fibrosis markers, tissue inhibitor of metalloproteinases-1, procollagen type III aminoterminal propeptide, matrix metalloproteinase-2, laminin, hyaluronan and collagen IV correlated with advanced fibrosis. Markers useful for fibrosis prediction also predicted marked inflammation. Using the gold standard, age, prothrombin time, gamma glutamyl transferase and albumin were independent predictors of fibrosis with odds ratio's of 3.11, 4.18, 3.35 and 5.25, respectively. Their combined use predicted fibrosis with an odds ratio of 228.8. Tissue inhibitor of metalloproteinases-1 and hyaluronan were powerful predictors of fibrosis (Odds ratio's of 8.65 and 8.38). Their combined use revealed an odds ratio of 28.6, when compared with the gold standard. CONCLUSION: In conclusion, advanced liver fibrosis in chronic hepatitis B and D may be predicted with use of these two fibrosis markers.


Asunto(s)
Hepatitis B Crónica/sangre , Hepatitis D Crónica/sangre , Ácido Hialurónico/sangre , Cirrosis Hepática/sangre , Inhibidor Tisular de Metaloproteinasa-1/sangre , Adulto , Análisis de Varianza , Biomarcadores/sangre , Biopsia , Femenino , Hepatitis B Crónica/diagnóstico por imagen , Hepatitis D Crónica/diagnóstico por imagen , Humanos , Cirrosis Hepática/diagnóstico por imagen , Pruebas de Función Hepática , Modelos Logísticos , Masculino , Valor Predictivo de las Pruebas , Curva ROC , Ultrasonografía
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