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1.
Artigo em Chinês | MEDLINE | ID: mdl-38858116

RESUMO

Objective:To summarize the procedures and efficacy of surgical treatment for Andrew stage Ⅰ-Ⅲ juvenile nasopharyngeal angiofibroma(JNA). Methods:A total of 12 patients with JNA who underwent surgery from 2016 to 2021 were enrolled, including 1 case in stage Ⅰ, 3 cases in stage Ⅱ, and 8 cases in stage Ⅲ. JNA was resected by transnasal endoscopic approach alone, or combined with transoral approach or Caldwell-Luc approach was performed. Results:Eleven cases underwent complete resection without recurrence and 1 case had residual tumor. There were no serious complications. The median intraoperative blood loss was 200 mL, and 1 patient received blood transfusion. The median operative time was 110 minutes. Conclusion:JNA in Andrew stage Ⅰ-Ⅲ can be quickly and completely resected by standardized surgical procedures using endoscopy and coblation technology.


Assuntos
Angiofibroma , Endoscopia , Neoplasias Nasofaríngeas , Humanos , Angiofibroma/cirurgia , Neoplasias Nasofaríngeas/cirurgia , Masculino , Endoscopia/métodos , Adolescente , Resultado do Tratamento , Feminino , Duração da Cirurgia , Adulto Jovem , Perda Sanguínea Cirúrgica , Adulto
2.
J Craniofac Surg ; 35(4): 1272-1275, 2024 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-38710071

RESUMO

The BiZact device, a bipolar electrosurgical scissor designed for tonsillectomy, minimizes thermal tissue damage and seals blood vessels <3 mm in diameter while dividing the soft tissue. This study describes the authors' experience with sinonasal tumor surgery using a BiZact and discusses its clinical utility and advantages. The authors analyzed BiZact-assisted endoscopic sinonasal tumor surgery cases between January 2021 and May 2023. Data were collected on patients' demographics, histopathology, extent of tumor involvement, surgical records, and postoperative medical records. Clinical utility was assessed using the success rate of complete tumor excision, estimated blood loss during surgery, device-related complications, and operation time. A survey of the surgeons' BiZact experience was also conducted. The diagnoses of the 20 patients in this study included squamous cell carcinoma (n = 2), malignant melanoma (n = 1), sarcoma (n = 1), natural killer cell lymphoma (n = 1), inverted papilloma (n = 12), angiofibroma (n = 2), and schwannoma (n = 1). This pilot study demonstrated a shortened operative time, with a median of 0.8 hours and <100 mL of intraoperative blood loss. In addition, no BiZact-related complications were observed. The BiZact device allows efficient sinonasal surgery because it has the unique advantage of one-step sealing and cutting. BiZact-assisted endoscopic sinonasal tumor surgery is a beneficial and safe procedure that reduces blood loss during surgery, shortens the operative time, and minimizes postoperative complications.


Assuntos
Endoscopia , Duração da Cirurgia , Neoplasias dos Seios Paranasais , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Idoso , Neoplasias dos Seios Paranasais/cirurgia , Neoplasias dos Seios Paranasais/patologia , Endoscopia/métodos , Projetos Piloto , Eletrocirurgia/instrumentação , Eletrocirurgia/métodos , Perda Sanguínea Cirúrgica , Carcinoma de Células Escamosas/cirurgia , Carcinoma de Células Escamosas/patologia , Melanoma/cirurgia , Melanoma/patologia , Angiofibroma/cirurgia , Angiofibroma/patologia , Sarcoma/cirurgia , Sarcoma/patologia , Resultado do Tratamento , Papiloma Invertido/cirurgia , Papiloma Invertido/patologia , Idoso de 80 Anos ou mais
3.
J Dermatol ; 51(6): 752-758, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38619178

RESUMO

The Index for Facial Angiofibromas (IFA), a novel scoring system for angiofibromas, has been validated in patients with tuberous sclerosis complex (TSC). The objective of this analysis was to further validate the IFA using data from a clinical trial of topical sirolimus in patients with TSC. This was an analysis of photographs from a Phase III trial conducted in Japan (NCT02635789). Patients (n = 62) were randomized 1:1 to receive sirolimus or placebo gel for 12 weeks. Changes in angiofibromas were independently assessed using the primary composite endpoint, the Facial Angiofibroma Severity Index (FASI), and the IFA. Thresholds for a clinically meaningful change in IFA score were evaluated using receiver operating characteristic (ROC) analysis. The IFA scores had good-to-excellent inter-assessor reliability, very high intra-assessor reliability, and could be used to evaluate the distribution of disease severity at baseline. High correlations were observed between the categorized change from baseline in IFA scores and the primary composite endpoint (Kendall's coefficient of concordance, W = 0.8655, p < 0.0001), and between the change from baseline in IFA and FASI scores (Kendall's coefficient of concordance, W = 0.745, p < 0.0001). By ROC analysis, an optimal IFA cut-off point of 1.667 was determined to distinguish patients with markedly improved or improved angiofibromas from those with slightly improved or unchanged angiofibromas (area under the curve 0.937) as determined by the primary composite endpoint. The IFA score is potentially clinically useful because of its high validity and reliability. A decrease in score from baseline of ≥1.667 may be considered clinically meaningful.


Assuntos
Angiofibroma , Neoplasias Faciais , Géis , Índice de Gravidade de Doença , Sirolimo , Esclerose Tuberosa , Humanos , Esclerose Tuberosa/tratamento farmacológico , Esclerose Tuberosa/diagnóstico , Esclerose Tuberosa/complicações , Angiofibroma/tratamento farmacológico , Angiofibroma/diagnóstico , Sirolimo/administração & dosagem , Sirolimo/uso terapêutico , Masculino , Feminino , Neoplasias Faciais/tratamento farmacológico , Neoplasias Faciais/patologia , Reprodutibilidade dos Testes , Adolescente , Adulto , Adulto Jovem , Resultado do Tratamento , Método Duplo-Cego , Fotografação , Japão , Curva ROC
4.
J Med Case Rep ; 18(1): 170, 2024 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-38600580

RESUMO

INTRODUCTION: Paratesticular cellular angiofibroma is a rare benign mesenchymal tumor. The optimal management is surgical resection due to the difficulty of preoperative accurate diagnosis. CASE PRESENTATION: A 51-year-old Japanese male visited our hospital complaining of asymptomatic left scrotal swelling. Physical examination revealed a nontender elastic paratesticular mass (5.5 cm in diameter). Although testicular germ cell tumor was ruled out clinically, the possibility of malignant potential remained for the tumor. Since the patient consented to complete resection, a transinguinal radical orchiectomy was performed. The pathological diagnosis revealed cellular angiofibroma. The patient recovered without perioperative complications, and no apparent recurrence was observed at 5 years after surgery. CONCLUSION: The pathological findings were compatible for cellular angiofibroma. The tumor was successfully resected, and no apparent recurrence was observed at 5 years after surgery.


Assuntos
Angiofibroma , Neoplasias Embrionárias de Células Germinativas , Neoplasias Testiculares , Humanos , Masculino , Pessoa de Meia-Idade , Angiofibroma/diagnóstico por imagem , Angiofibroma/cirurgia , Neoplasias Testiculares/diagnóstico , Neoplasias Testiculares/cirurgia , Neoplasias Testiculares/patologia , Orquiectomia , Neoplasias Embrionárias de Células Germinativas/cirurgia
6.
BMJ Case Rep ; 17(3)2024 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-38479825

RESUMO

We report the first case of a juvenile nasal angiofibroma (JNA) fed by multiple arteries from the internal carotid artery (ICA), removed without complications by temporarily blocking the ICA with two balloons. An early adolescent with JNA underwent preoperative embolisation of feeding arteries arising from the external carotid artery (ECA) (University of Pittsburgh Medical Centre classification IV). Endoscopic resection was attempted once but discontinued due to massive bleeding (7000 mL). 17 months later, the JNA had grown to fill both nasal cavities. Repeated preoperative embolisation of the feeders from the ECA was performed, followed by surgery combined with endoscopic and external incision. Intraoperatively, two balloons were inserted into the right ICA, which were inflated at the proximal and distal sites of the feeder vessels to cut-off blood flow to the tumour. The tumour was almost completely resected with 6270 mL of blood loss and no postoperative neurological deterioration.


Assuntos
Angiofibroma , Oclusão com Balão , Embolização Terapêutica , Neoplasias de Cabeça e Pescoço , Neoplasias Nasofaríngeas , Adolescente , Humanos , Artéria Carótida Interna/diagnóstico por imagem , Artéria Carótida Interna/cirurgia , Angiofibroma/complicações , Resultado do Tratamento , Neoplasias Nasofaríngeas/complicações , Neoplasias de Cabeça e Pescoço/complicações , Artéria Carótida Externa/cirurgia
7.
Diagn Pathol ; 19(1): 50, 2024 Mar 08.
Artigo em Inglês | MEDLINE | ID: mdl-38459589

RESUMO

BACKGROUND: Tuberous sclerosis complex (TSC) is a rare, complex genetic disorder characterized by hamartomas and neoplastic lesions in various organ systems. With the development of radiology and gene testing, the diagnostic criteria for TSC were updated in 2012 at the International Consensus Conference. Intraoral fibromas have long been associated with TSC. However, the incidence of giant cell angiofibroma (GCA) in TSC patients is extremely rare. Here, we report the first case of GCA in the gingival tissue of a patient with TSC. CASE PRESENTATION: A 41-year-old woman first visited the Department of Oral and Maxillofacial Surgery, Chonnam National University Dental Hospital, complaining of gingival enlargement. Clinical examination revealed several manifestations associated with TSC, including intraoral fibromas, facial angiofibromas, dental enamel pits, ungual fibromas, "confetti" skin lesions, hypomelanotic macules, and a shagreen patch. Intraoral examination revealed a 6.0 × 5.0 cm gingival overgrowth on the left mandible. Surgical excision was performed, and subsequent histopathological examination confirmed the diagnosis of GCA. There was no evidence of recurrence within the 24- months of surgery. CONCLUSIONS: We report the first case of GCA in the gingival tissue of a patient with TSC. This report would contribute to an improved understanding of this rare disease. However, further case reports are necessary to clarify the relationship between GCA and TSC.


Assuntos
Angiofibroma , Fibroma , Esclerose Tuberosa , Feminino , Humanos , Adulto , Esclerose Tuberosa/complicações , Esclerose Tuberosa/diagnóstico , Esclerose Tuberosa/genética , Angiofibroma/diagnóstico , Angiofibroma/patologia , Angiofibroma/cirurgia , Gengiva/patologia , Células Gigantes/patologia
8.
Medicine (Baltimore) ; 103(6): e37135, 2024 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-38335392

RESUMO

RATIONALE: Tuberous sclerosis complex (TSC) is a rare autosomal dominant inherited disorder characterized by the development of nonmalignant tissue growths (hamartomas) in various organ systems, often located in the brain, skin, heart, lung and kidneys. The delayed diagnosis could be attributed to low expectation or exposure of physicians to this rare disease. High index of clinical suspicion is required for early diagnosis of rare diseases to prevent adverse outcomes. PATIENT CONCERNS: The first patient, a 27-year-old man, presented with intermittent left flank pain and hematuria of 5 months duration. On examination of the skin and oral cavity, he had fibrous cephalic plaque, facial angiofibromas, ungual fibromas, confetti skin lesions, and intraoral fibromas. A CT scan of the chest, abdomen, and brain displayed cystic lung parenchymal changes and multifocal micronodular pneumocyte hyperplasia, angiomyolipomas in both kidneys, and multiple calcified subependymal nodules (SEN), respectively. The second patient, a 28-year-old woman, presented with a seizure disorder in the last 1 year, and papular and nodular lesions over her face since childhood. On examination of the skin and oral cavity, she had hypomelanotic macules, facial angiofibromas, shagreen patches, ungual fibromas, intraoral fibromas, and dental enamel pits. DIAGNOSES: Definitive diagnosis of TSC was made in both patients using the "2012 tuberous sclerosis complex diagnostic criteria consensus statement." INTERVENTIONS: The first patient was seen by various medical discipline teams, and suggested close follow-up in the "chronic illness clinic" of the hospital. The second patient was scheduled in dermatology clinic for electrocautery for disfiguring facial nodules. OUTCOME: Both patients were scheduled for close follow-up in the hospital. LESSONS: The patients described had TSC using "clinical diagnostic criteria." Under the clinical diagnostic criteria of TSC, 4 of 11 major criteria and 3 of 7 minor criteria are skin features. Hence, awareness on skin features as clinical markers to suspect TSC should be emphasized in resource-limited countries.


Assuntos
Angiofibroma , Fibroma , Hamartoma , Dermatopatias , Esclerose Tuberosa , Adulto , Feminino , Humanos , Masculino , Angiofibroma/patologia , Fibroma/patologia , Hamartoma/patologia , Hiperplasia/patologia , Pele/patologia , Dermatopatias/diagnóstico , Esclerose Tuberosa/complicações , Esclerose Tuberosa/diagnóstico , Esclerose Tuberosa/patologia
9.
Ann Pharmacother ; 58(4): 428-433, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37386842

RESUMO

OBJECTIVE: This article assesses the efficacy, safety, pharmacology, and clinical applications of topical sirolimus 0.2% gel for the treatment of tuberous sclerosis complex (TSC)-associated facial angiofibromas. DATA SOURCES: A review of the literature was conducted using the Medline (PubMed) and EMBASE databases using the keywords topical sirolimus, rapamycin, Hyftor, and tuberous sclerosis. STUDY SELECTION AND DATA EXTRACTION: Articles written in English and relevant to the topic were included. DATA SYNTHESIS: In the phase 2 trial, the mean improvement factor, a composite measure of improved tumor size and redness, was achieved in all patient groups (P < 0.001) with significant responses among the adult and pediatric subgroups at week 12. There were no serious adverse events recorded. In the phase 3 trial, 60% of participants responded to treatment in the sirolimus group compared with 0% in the placebo group with different response rates between the adult and pediatric subgroups at week 12. Sirolimus gel had no serious adverse events, and dry skin was the most common adverse reaction. Patients who had completed the 12-week trials were then enrolled in a long-term trial; angiofibromas had response rates of 78.2% to 0.2% sirolimus gel. RELEVANCE TO PATIENT CARE AND CLINICAL PRACTICE IN COMPARISON TO EXISTING DRUGS: Topical sirolimus 0.2% is a first-in-class, newly Food and Drug Administration (FDA)-approved, mammalian target of rapamycin (mTOR) inhibitor that is a promising and safe, noninvasive alternative to surgical procedures for TSC-associated angiofibromas. CONCLUSIONS: Topical sirolimus 0.2% gel is a moderately effective treatment for TSC-associated facial angiofibromas with an adequate safety profile.


Assuntos
Angiofibroma , Neoplasias Faciais , Esclerose Tuberosa , Adulto , Humanos , Criança , Esclerose Tuberosa/complicações , Esclerose Tuberosa/tratamento farmacológico , Esclerose Tuberosa/patologia , Angiofibroma/tratamento farmacológico , Angiofibroma/etiologia , Neoplasias Faciais/etiologia , Neoplasias Faciais/induzido quimicamente , Imunossupressores , Sirolimo/efeitos adversos , Géis/uso terapêutico
11.
Histopathology ; 84(2): 381-386, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37855220

RESUMO

AIMS: Angiofibroma of soft tissue (AFST) is a benign, morphologically distinctive tumour type that harbours recurrent AHRR::NCOA2 fusions in 60-70% of cases and shows a non-specific immunophenotype, expressing EMA in roughly half of cases. The AHRR::NCOA2 fusion results in increased expression of cytochrome P450 1A1 (CYP1A1); a recent study demonstrated CYP1A1 immunohistochemistry (IHC) to be moderately sensitive and highly specific for AFST. METHODS AND RESULTS: In this study, we sought to validate these findings in a larger independent cohort of 30 AFST, as well as 215 morphological mimics, including 30 solitary fibrous tumours, 29 myxoid liposarcomas, 28 low-to-intermediate grade myxofibrosarcomas (MFS), 20 atypical spindle cell lipomatous tumours (ASCLT), 20 cellular angiofibromas, 10 cases each of spindle cell lipoma, neurofibroma, malignant peripheral nerve sheath tumour, superficial angiomyxoma, cellular myxoma, soft tissue perineurioma and deep fibrous histiocytoma, and nine cases each of low-grade fibromyxoid sarcoma and mammary-type myofibroblastoma. We found CYP1A1 IHC to be 70% sensitive for AFST, with granular cytoplasmic staining in 21 of 30 tumours, and 98% specific, with staining in only five morphological mimics: two deep fibrous histiocytomas, one MFS, one cellular angiofibroma and one ASCLT. CONCLUSIONS: These findings confirm that CYP1A1 is 70% sensitive, consistent with the prevalence of AHRR::NCOA2 fusions that up-regulate this protein, and that it is highly specific among morphological mimics.


Assuntos
Angiofibroma , Fibrossarcoma , Lipoma , Neoplasias de Tecidos Moles , Adulto , Humanos , Angiofibroma/diagnóstico , Angiofibroma/genética , Angiofibroma/metabolismo , Imuno-Histoquímica , Citocromo P-450 CYP1A1 , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/metabolismo
12.
Laryngoscope ; 134(4): 1897-1900, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37721203

RESUMO

A 30-year-old man presented with minute-long episodes of vertigo and severe autophony. CVEMP showed a decreased threshold when testing the left side, potentially indicating SSCD. A subsequent MRI demonstrated a multi-lobulated, cystic mass in the temporal bone and the radiological diagnosis at that time was ELST. Tumor excision was performed, and microscopic examination of the excised material revealed fibrovascular tissue without signs of papillary or cystic projections. The conclusion of the histological assessment rendered a diagnosis of angiofibroma. We were unable to find a previous report of ENA originating around the endolymphatic sac. Laryngoscope, 134:1897-1900, 2024.


Assuntos
Angiofibroma , Neoplasias Ósseas , Neoplasias da Orelha , Saco Endolinfático , Doenças do Labirinto , Masculino , Humanos , Adulto , Saco Endolinfático/cirurgia , Saco Endolinfático/patologia , Angiofibroma/diagnóstico por imagem , Angiofibroma/cirurgia , Doenças do Labirinto/patologia , Neoplasias da Orelha/diagnóstico por imagem , Neoplasias da Orelha/cirurgia , Vertigem , Neoplasias Ósseas/patologia
13.
J Otolaryngol Head Neck Surg ; 52(1): 85, 2023 Dec 19.
Artigo em Inglês | MEDLINE | ID: mdl-38115030

RESUMO

BACKGROUND: Juvenile Nasopharyngeal Angiofibroma (JNA) is a fibrovascular tumor of the nasopharynx that classically presents in adolescent males. The reported mean age of onset is between 13 and 22 years old [1-6]. Significant androgen stimulation is hypothesized to explain the strong predisposition for JNA to present in young adolescent males. However, considerable variability in age at diagnosis exists with rare involvement of very young patients incongruent with typical male pubertal growth patterns. OBJECTIVE: The purpose of this systematic review is to identify cases of early-onset JNA (EOJNA), (defined as age < 10 years) in the literature and to examine the disease characteristics and treatments used in this patient group. A case of a 7 year old boy with EOJNA at our institution is also described and presented. METHODS: We searched Embase, Cochrane database and MEDLINE from 1996 to February 2021 for studies that reported cases of EOJNA. Relevant clinico-demographic data, disease severity and treatment outcomes were recorded and analyzed using descriptive statistics. We compared our findings with reported means for JNA in all ages. RESULTS: We identified 29 studies containing a total of 34 cases of EOJNA. The vast majority (31/34) of patients were males and the mean age of diagnosis was 8.15 years old. The most common presenting symptoms were nasal obstruction (65.2%) and epistaxis (60.9%). Patients were most commonly Radkowski stage II (39.4%) and III (39.4%). Primary treatment modalities included open surgery (66.7%), endoscopic surgery (24.2%), and radiotherapy (9.1%). Recurrence was evident in 30%. Radkowski stage and type of treatment did not differ significantly within the EOJNA group (p = 0.440 and p = 0.659, respectively). CONCLUSION: This systematic review suggests that rare cases of EOJNA have distinct disease characteristics. Patients in this cohort appeared to have more advanced disease and higher recurrence rates when compared with reported averages. We hope that this review prompts increased clinical awareness of this potentially more aggressive subtype of JNA. As more cases of EOJNA are reported, a more powered statistical analysis of this cohort would be feasible.


Assuntos
Angiofibroma , Obstrução Nasal , Neoplasias Nasofaríngeas , Adolescente , Humanos , Masculino , Adulto Jovem , Adulto , Criança , Feminino , Angiofibroma/diagnóstico , Angiofibroma/cirurgia , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/cirurgia , Epistaxe , Resultado do Tratamento , Obstrução Nasal/etiologia , Estudos Retrospectivos
14.
Indian J Cancer ; 60(4): 572-574, 2023 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-38159202

RESUMO

Cellular angiofibroma (AF)/Angiomyofibroblastoma (AMF)-like tumor is a rare benign mesenchymal neoplasm. It is very challenging to distinguish benign versus malignant mass radiologically. It is of paramount importance to distinguish Cellular Angiofibroma (CAF) microscopically from its differential diagnoses. A 64-year-old man presented with scrotal swelling. Pathological examination showed features of cellular AF/AMF- like tumor, which shows positivity for CD34, with negativity for S-100 Protein, smooth muscle actin and desmin.


Assuntos
Angiofibroma , Neoplasias de Tecidos Moles , Neoplasias Vulvares , Humanos , Masculino , Idoso , Pessoa de Meia-Idade , Feminino , Angiofibroma/diagnóstico , Angiofibroma/metabolismo , Angiofibroma/patologia , Testículo/patologia , Diagnóstico Diferencial , Proteínas S100 , Neoplasias Vulvares/diagnóstico , Neoplasias Vulvares/patologia
16.
Rev Med Inst Mex Seguro Soc ; 61(Suppl 2): S263-S268, 2023 Sep 18.
Artigo em Espanhol | MEDLINE | ID: mdl-38016122

RESUMO

Background: Juvenile nasoangiofibroma (JNA) is a rare, highly vascular, locally aggressive benign tumor which affects male adolescents. It accounts for 0.05-0.5% of head and neck tumors with recurrence rates of 6-50%. The internal maxillary artery is the main source of JNA. Objective: To evaluate the relationship between vascular supply as a factor associated with JNA recurrence. Material and methods: An cohort study was performed in patients diagnosed with NAJ. We collected demographic data, vascular contribution by angiography and tomography results to classify them according to their stage (Radkowski classification), and if they received adjuvant radiotherapy. Post-surgical CT scans were requested to evaluate recurrence and if any of the variables were related to this. Results: A sample of 14 male patients who met the inclusion criteria was collected. The mean age was 14.71 ± 4.08 years. According to Radkowski classification, stage IA, IIA and IIC were reported in 14.3%, IIB and IIB in 7.1% and IIIA in 42.9%. 42.9% had recurrence and out of these, 66.7% had irrigation of the right carotid system and the same percentage of patients received radiotherapy as adjuvant treatment. Conclusions: There is a tendency in tumor recurrence associated with vascular contribution from the right carotid system, as well as with patients who received radiotherapy.


Introducción: el nasoangiofibroma juvenil (NAJ) es un tumor benigno, raro, altamente vascular y localmente agresivo que afecta a adolescentes del sexo masculino. Representa de 0.05 a 0.5% de los tumores de cabeza y cuello con tasas de recurencia del 6-50%. La arteria maxilar interna se considera el principal aporte de los NAJ. Objetivo: evaluar la relación entre el aporte vascular como factor asociado con la recurrencia de NAJ. Material y métodos: se realizó un estudio de cohorte en pacientes con diagnóstico de NAJ. Se recabaron datos demográficos, el aporte vascular por resultados de angiografía y de tomografía para clasificarlos según su estadio (clasificación de Radkowski), y si recibieron radioterapia adyuvante. Se solicitaron tomografías postquirúrgicas para evaluar la recurrencia y si alguna de las variables tiene relación con esta. Resultados: se recolectó una muestra de 14 pacientes del sexo masculino que cumplieron con los criterios de inclusión. La edad promedio fue de 14.71 ± 4.08 años. Según la clasificación de Radkowski, se reportó un estadio IA, IIA y IIC en 14.3%, IIB y IIB en un 7.1% y IIIA en 42.9%. El 42.9% tuvo recurrencia y de estos, el 66.7% tenía irrigación del sistema carotídeo derecho y recibieron radioterapia como tratamiento adyuvante el mismo porcentaje de pacientes. Conclusiones: existe una tendencia en la recurrencia del tumor asociada al aporte vascular proveniente del sistema carotídeo derecho y también a los pacientes que recibieron radioterapia.


Assuntos
Angiofibroma , Neoplasias Nasofaríngeas , Adolescente , Humanos , Masculino , Criança , Estudos de Coortes , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/patologia , Neoplasias Nasofaríngeas/cirurgia , Estadiamento de Neoplasias , Recidiva Local de Neoplasia/diagnóstico , Recidiva Local de Neoplasia/cirurgia , Angiofibroma/diagnóstico , Angiofibroma/patologia , Angiofibroma/cirurgia , Estudos Retrospectivos
19.
Int J Pediatr Otorhinolaryngol ; 174: 111746, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37801829

RESUMO

BACKGROUND: The literature on microvessel density (MVD) signifying neoangiogenesis/tumour-activity in juvenile nasopharyngeal angiofibroma (JNA) is limited. Accordingly, this study evaluates and correlates MVD characteristics with clinical parameters/aggressiveness/recurrence. MATERIAL AND METHODS: Sixty-two paraffin blocks of JNA were studied histopathologically and MVD was assessed following immunohistochemistry using VEGF and CD34 as vascular markers. A clinical correlation of MVD was undertaken in 43 cases. RESULTS: MVD scores of VEGF and CD34 showed strong inter-correlation. The 'age', 'duration of disease' and 'haemoglobin%' were the only clinical parameters that revealed significance with MVD. Significantly higher MVD scores were appreciated in recurrent cases as well as some other clinical differences from upfront cases. CONCLUSION: This is the first study of MVD with CD34 and VEGF simultaneously depicting clinical correlation. The strong correlation, supports a prognostic role of MVD scores in JNA and this can be better established in a larger multicentre study involving comprehensive examination of tumour dimensions.


Assuntos
Angiofibroma , Neoplasias Nasofaríngeas , Humanos , Fator A de Crescimento do Endotélio Vascular/metabolismo , Angiofibroma/patologia , Densidade Microvascular , Fatores de Crescimento do Endotélio Vascular , Neoplasias Nasofaríngeas/patologia , Prognóstico
20.
Ann Dermatol Venereol ; 150(4): 270-273, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37821253

RESUMO

AIMS: Topical rapamycin is used to reduce facial angiofibromas in patients with tuberous sclerosis (TSC). In the absence of a commercially available preparation, numerous formulations have been tested clinically, although only in the short term. METHODS: The pharmacy at Angers University Hospital (France) produced a cream formulation that was administered to people presenting this genetic disease. We conducted a questionnaire-based survey among 79 patients with TSC about their perceptions regarding the short-, medium- and long-term efficacy and safety of a topical rapamycin preparation in relation to facial angiofibromas. RESULTS: This formulation was very well tolerated and its efficacy was sustained over the long term with a mean treatment duration of 33 months (extremes 1-60). Efficacy was rated ≥ 8/10 by 67.1% of patients while safety was rated ≥ 8/10 by 84.8% of patients. CONCLUSION: This survey supports the safety and efficacy of topical rapamycin in the short-, medium- and long-term in the treatment of facial angiofibromas in a cohort of 79 patients with TSC.


Assuntos
Angiofibroma , Neoplasias Faciais , Esclerose Tuberosa , Humanos , Esclerose Tuberosa/complicações , Angiofibroma/tratamento farmacológico , Angiofibroma/complicações , Neoplasias Faciais/tratamento farmacológico , Neoplasias Faciais/etiologia , Imunossupressores/uso terapêutico , Sirolimo/efeitos adversos
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