RESUMO
OBJECTIVE: Paratesticular tumors (PTT) are rare and form a heterogenous group, ranging from benign to malignant high grade sarcomas. This study was undertaken to describe the clinicopathological spectrum of PTTs received over a 20-year period. METHODS: All primary and secondary PTTs diagnosed from 2000 to 2019 in the pathology department of a tertiary care hospital in North India were retrospectively reviewed. Gross, histopathological features and immunohistochemistry (IHC) findings were correlated with clinical details. RESULTS: A total of 169 intra-scrotal tumors were diagnosed during the study period, out of which there were 30 PTTs (in 27 patients) comprising 17.75%. Age range was 4 to 85 years (median 58 years). Benign PTTs were the commonest (n = 21, 70%), followed by metastasis to the paratesticular region (n = 6, 20%) and then primary malignant PTTs (n = 3, 10%). The commonest benign PTT was lipoma (n = 16, 76.19%), followed by adenomatoid tumor (n = 3, 14.28%) with one case each (4.76%) of cellular angiofibroma and hemangioma. Among primary malignant PTT, there were two cases of rhabdomyosarcoma, and one case of biphasic malignant mesothelioma. Metastatic tumors included four cases of prostatic adenocarcinoma, and one case each of pancreatic signet ring cell carcinoma and clear cell renal cell carcinoma. CONCLUSION: PTTs show a wide clinicopathological spectrum. Benign PTTs are commoner than malignant PTTs. Meticulous grossing and histopathological examination supplemented by IHC is essential for an accurate diagnosis of this heterogenous class of tumors, which influences the role of adjuvant therapy and patient prognosis.
Assuntos
Neoplasias dos Genitais Masculinos/patologia , Imuno-Histoquímica/métodos , Gradação de Tumores/métodos , Sarcoma/diagnóstico , Escroto/patologia , Tumor Adenomatoide/diagnóstico , Tumor Adenomatoide/epidemiologia , Tumor Adenomatoide/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Angiofibroma/diagnóstico , Angiofibroma/epidemiologia , Angiofibroma/patologia , Criança , Pré-Escolar , Hemangioma/diagnóstico , Hemangioma/epidemiologia , Hemangioma/patologia , Humanos , Índia/epidemiologia , Lipoma/diagnóstico , Lipoma/epidemiologia , Lipoma/patologia , Masculino , Mesotelioma Maligno/diagnóstico , Mesotelioma Maligno/epidemiologia , Mesotelioma Maligno/patologia , Pessoa de Meia-Idade , Metástase Neoplásica/diagnóstico , Metástase Neoplásica/patologia , Prognóstico , Estudos Retrospectivos , Rabdomiossarcoma/diagnóstico , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/patologia , Sarcoma/epidemiologia , Sarcoma/patologia , Adulto JovemRESUMO
BACKGROUND: An analysis of time distribution of juvenile nasopharyngeal angiofibroma (JNA) from the last 4 decades is presented. METHODS: Sixty recurrences were analyzed as per actuarial survival. SPSS software was used to generate Kaplan-Meier (KM) curves and time distributions were compared by Log-rank, Breslow and Tarone-Ware test. RESULTS: The overall recurrence rate was 17.59%. Majority underwent open transpalatal approach(es) without embolization. The probability of detecting a recurrence was 95% in first 24months and comparison of KM curves of 4 different time periods was not significant. CONCLUSION: This is the first and largest series to address the time-distribution. The required follow up period is 2years. Our recurrence is just half of the largest series (reported so far) suggesting the superiority of transpalatal techniques. The similarity of curves suggests less likelihood for recent technical advances to influence the recurrence that as per our hypothesis is more likely to reflect tumor biology per se.
Assuntos
Angiofibroma/epidemiologia , Neoplasias Nasofaríngeas/epidemiologia , Recidiva Local de Neoplasia/epidemiologia , Angiofibroma/patologia , Angiofibroma/terapia , Criança , Humanos , Neoplasias Nasofaríngeas/patologia , Neoplasias Nasofaríngeas/terapia , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Estudos Retrospectivos , Fatores de TempoRESUMO
Juvenile nasopharyngeal angiofibroma (JNA) is a vascular tumor of the nasopharynx that accounts for 0.5% of all cancers of the head and neck. It primarily affects males aged 14-25 years. Of the many genes that mediate the development of JNA, GSTM1 has been most frequently associated with this vascular tumor. The loss of expression of GSTM1 (null genotype) is linked to the development of these tumors. The aim of this cross-sectional case study was to examine the prevalence of the GSTM1-null genotype in Brazilian patients with JNA. DNA was extracted from the leukocytes of blood samples from 10 patients. GSTM1 genotypes were analyzed using a PCR-based assay that was designed to identify the wild-type allele of GSTM1. All 10 patients (100%) were males, with a mean age of 17.8 years. The null genotype for GSTM1 was noted in 4 patients (40%)-1 (10%) at Fisch stage I, 1 (10%) at stage III, and 2 (20%) at stage II. No patient with this genotype had stage IV disease. There was no correlation between Fisch classification and GSTM1 genotype (P = .5695). The correlation between age at diagnosis and GSTM1 genotype was not significant (P = .728). The present findings indicate that there is evidence of an association between the GSTM1-null genotype and JNA in this studied Brazilian population.
Assuntos
Alelos , Angiofibroma/genética , Genótipo , Glutationa Transferase/genética , Neoplasias Nasofaríngeas/genética , Adolescente , Adulto , Angiofibroma/epidemiologia , Angiofibroma/patologia , Brasil/epidemiologia , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Neoplasias Nasofaríngeas/epidemiologia , Neoplasias Nasofaríngeas/patologia , Estadiamento de NeoplasiasRESUMO
OBJECTIVE: To report trends in Juvenile Nasopharyngeal Angiofibroma (JNA) hospitalizations and identify key factors affecting treatment outcomes and cost of care in JNA patients. METHODS: The Healthcare Cost and Utilization Project (HCUP) Kids' Inpatient Database was queried for all cases of JNA between the years of 1997 and 2016. Key factors extracted were patient demographics, geographic region, hospital size, teaching status, elective admissions, and number of diagnoses and procedures performed during the hospitalization. These elements were correlated to length of stay (LOS) and cost-per-day (CPD) using a multiple linear regression (MLR). Regional variation in JNA diagnosis and changes in LOS and CPD trends over time were also analyzed. RESULTS: A total of 614 JNA patients were hospitalized in this time period, with a majority of patients identifying as male (98%) and Caucasian/White (55%). The average LOS has decreased by 0.14 day per year since 1997 (P = .0034) whereas the CPD has steadily increased by $2 380 per year (P < .001). MLR analysis revealed that while holding all other factors constant, patients who stayed at teaching hospitals had an increased LOS of 1.7 days (P = .026), but paid $11 961 less per day (P = .05). Regional variation in CPD was found in the Northeast region, where hospitalizations were more expensive by $9 801 per day compared to the South (P = .017). CONCLUSION: These results indicate hospital characteristics, such as teaching status and geographic region, may predict differences in JNA outcomes and cost. Healthcare providers should be cognizant of these variations to ensure optimal patient outcomes and expenditures.
Assuntos
Angiofibroma/terapia , Efeitos Psicossociais da Doença , Gerenciamento Clínico , Hospitalização/economia , Pacientes Internados/estatística & dados numéricos , Neoplasias Nasofaríngeas/terapia , Adolescente , Angiofibroma/economia , Angiofibroma/epidemiologia , Custos e Análise de Custo , Bases de Dados Factuais , Feminino , Seguimentos , Humanos , Incidência , Masculino , Neoplasias Nasofaríngeas/economia , Neoplasias Nasofaríngeas/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento , Estados Unidos/epidemiologiaRESUMO
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a genetic disease that predisposes to endocrine tumour development. Some cutaneous lesions (angiofibromas, collagenomas, melanosis guttaca, lipomas, melanomas, 'cafe au lait macules') have been associated to this syndrome. We compare the prevalence of cutaneous lesion in affected patients with their non-carrier relatives. PATIENTS AND METHOD: We studied 9 patients with MEN1 and 20 non-carrier, first-degree relatives. Genetic screening was realized in all of them. Patients were examined by dermatologist, and biopsy was performed when necessary. RESULTS: Patients with MEN1 presented hyperparathyroidism (100%), neuroendocrine tumours of pancreas (66%) and pituitary adenomas (44%); their relatives were free of endocrine features of MEN1. The studied cutaneous lesions were more prevalent in affected patients than in non-carriers (55.5% vs. 25%; P = 0.029). Odds ratio of developing cutaneous lesions in MEN1 patients was 6.6 (95% confidence interval, 1.09-40.43). The frequency of angiofibromas was lower (22.2%) than the reported in other studies (43-88%), and we did not find any collagenoma. CONCLUSIONS: MEN1 is associated to some cutaneous lesions and could be useful for detecting MEN1 carriers in an affected family. Cutaneous lesions should be assessed in MEN1 patients.
Assuntos
Angiofibroma/epidemiologia , Lipoma/epidemiologia , Neoplasia Endócrina Múltipla Tipo 1/epidemiologia , Proteínas Proto-Oncogênicas/genética , Neoplasias Cutâneas/epidemiologia , Adenoma/epidemiologia , Adenoma/genética , Adulto , Angiofibroma/genética , Feminino , Predisposição Genética para Doença/epidemiologia , Testes Genéticos , Humanos , Hiperparatireoidismo/epidemiologia , Hiperparatireoidismo/genética , Lipoma/genética , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/genética , Neoplasias Pancreáticas/epidemiologia , Neoplasias Pancreáticas/genética , Neoplasias Hipofisárias/epidemiologia , Neoplasias Hipofisárias/genética , Prevalência , Neoplasias Cutâneas/genéticaRESUMO
OBJECTIVE: This study aimed to present the clinical features and surgical outcomes of juvenile nasopharyngeal angiofibroma patients who were surgically treated. METHODS: The medical records of 48 male patients histologically confirmed as having juvenile nasopharyngeal angiofibroma, who underwent transnasal endoscopic surgery between 2005 and 2016, were retrospectively reviewed. RESULTS: The overall recurrence rate was 20.8 per cent; however, the recurrence rate differed significantly between patients diagnosed aged less than 14 years (34.7 per cent) and more than 14 years (8 per cent) (p < 0.05). Advanced-stage tumours (Radkowski stage of IIC or more, and Önerci stage of III or more) were more aggressive than earlier stage tumours (p < 0.05 and p < 0.01, respectively). Pre-operative embolisation significantly prolonged mean hospitalisation duration, but had no effect on intra-operative blood loss in patients with advanced-stage tumours (p < 0.001 and p = 0.09, respectively). CONCLUSION: The findings show that transnasal endoscopic surgery could be considered the treatment of choice for juvenile nasopharyngeal angiofibroma. Patients diagnosed when aged less than 14 years and those with advanced-stage tumours are at risk of recurrence, and should be monitored with extreme care.
Assuntos
Angiofibroma/terapia , Embolização Terapêutica/métodos , Neoplasias Nasofaríngeas/terapia , Recidiva Local de Neoplasia/terapia , Adolescente , Fatores Etários , Angiofibroma/epidemiologia , Angiofibroma/patologia , Criança , Endoscopia , Humanos , Masculino , Neoplasias Nasofaríngeas/epidemiologia , Neoplasias Nasofaríngeas/patologia , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Cuidados Pré-Operatórios , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVES: Infratemporal fossa (ITF) tumors are rare and little is known about their general epidemiology, making it sometimes difficult for clinicians, who seldom encounter them, to distinguish between benign and malignant forms on the basis of the initial clinical and radiological work-up alone. The objectives of this retrospective study were: (i) to determine the respective prevalences of the various histologic types of ITF tumor, and (ii) to assess associations between certain clinical and radiological features and malignancy. METHODS: A single-center observational study in a university hospital included all new consecutive cases of ITF tumor treated from January 2000 to December 2016. Histologic type, demographics, clinical presentation and imaging findings were analyzed. RESULTS: In total, 62 patients were included. 74% of tumors were benign (n=46) and 26% malignant. Juvenile nasopharyngeal angiofibroma, adenoid cystic carcinoma and schwannoma were the most frequent histologic types, accounting for 47%, 16% and 10% of cases, respectively. The only clinical or imaging signs significantly associated with malignancy were trismus, facial pain, facial hypoesthesia and neural invasion on magnetic resonance imaging (all P-values<0.05). CONCLUSION: This study provides general epidemiological data on ITF tumors, and identified several clinical and radiologic signs to help clinicians suspect malignancy.
Assuntos
Neoplasias Cranianas/epidemiologia , Neoplasias Cranianas/patologia , Adolescente , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Angiofibroma/epidemiologia , Angiofibroma/patologia , Carcinoma Adenoide Cístico/epidemiologia , Carcinoma Adenoide Cístico/patologia , Criança , Displasia Fibrosa Craniofacial/epidemiologia , Displasia Fibrosa Craniofacial/patologia , Dor Facial/etiologia , Feminino , França/epidemiologia , Humanos , Hipestesia/etiologia , Masculino , Pessoa de Meia-Idade , Neurilemoma/epidemiologia , Neurilemoma/patologia , Neurofibroma/epidemiologia , Neurofibroma/patologia , Estudos Retrospectivos , Distribuição por Sexo , Trismo/etiologia , Adulto JovemRESUMO
CONCLUSIONS: Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor in young males, with a non-negligible potential for recurrence. Preoperative embolization is a safe procedure that diminishes the peroperative blood loss and the need for blood transfusion. The endoscopic approach was used with good results in JNA stage I and II (Chandler). OBJECTIVES: To estimate the incidence rate of JNA in the Danish population and to describe symptoms and treatment. PATIENTS AND METHODS: This was a national retrospective cohort study. All cases of JNA diagnosed in Denmark from 1981 to 2003 were identified. Data were extracted from medical records. RESULTS: Forty-five male (no female) JNA cases were identified. In 43 cases, clinical data were recovered. Median age was 15 years. The incidence rate in Denmark was 0.4 cases per million inhabitants per year and 3.7 cases per million males (aged 10-24) per year. All patients underwent surgery, and the endoscopic approach was increasingly being used. The embolization procedure proved to be safe and decreased the intraoperative blood loss statistically to 650 ml in the embolized group from an average of 1200 ml in the non-embolized group (p<0.05). Similarly, the need for peroperative blood transfusion was reduced (p<0.005). The primary recurrence rate was 23% and no patients died.
Assuntos
Angiofibroma/diagnóstico , Neoplasias Nasofaríngeas/diagnóstico , Adolescente , Adulto , Angiofibroma/epidemiologia , Angiofibroma/patologia , Angiofibroma/cirurgia , Perda Sanguínea Cirúrgica/prevenção & controle , Criança , Estudos de Coortes , Terapia Combinada , Estudos Transversais , Dinamarca/epidemiologia , Embolização Terapêutica , Endoscopia , Humanos , Incidência , Masculino , Neoplasias Nasofaríngeas/epidemiologia , Neoplasias Nasofaríngeas/patologia , Neoplasias Nasofaríngeas/cirurgia , Terapia Neoadjuvante , Estadiamento de Neoplasias , Estudos RetrospectivosRESUMO
Juvenile angiofibroma is an uncommon, benign, locally aggressive vascular tumor. It is found almost exclusively in young men. Common presenting symptoms include nasal obstruction and epistaxis. More advanced tumors may present with facial swelling and visual or neurological disturbances. The evaluation of patients with juvenile angiofibroma relies on diagnostic imaging. Preoperative biopsy is not recommended. The mainstay of treatment is resection combined with preoperative embolization. Endoscopic surgery is the approach of choice in early stages, whereas, in advanced stages, open or endoscopic approaches are feasible in expert hands. Postoperative radiotherapy (RT) or stereotactic radiosurgery seem valuable in long-term control of juvenile angiofibroma, particularly those that extend to anatomically critical areas unsuitable for complete resection. Chemotherapy and hormone therapy are ineffective. The purpose of the present review was to update current aspects of knowledge related to this rare and challenging disease. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1033-1045, 2017.
Assuntos
Angiofibroma/diagnóstico , Angiofibroma/terapia , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/terapia , Angiofibroma/epidemiologia , Criança , Humanos , Neoplasias Nasofaríngeas/epidemiologiaRESUMO
BACKGROUND: Molecular categorisation may explain the wide variation in the clinical characteristics of juvenile nasopharyngeal angiofibroma. METHODS: Variations in molecular markers in juvenile nasopharyngeal angiofibroma in an Indian population were investigated and compared with global reports. RESULTS: Variable molecular marker expression was demonstrated at the regional and global levels. A wide variation in molecular characteristics is evident. Molecular data have been reported for only 11 countries, indicating a clear geographical bias. Only 58 markers have been studied, and most are yet to be validated. CONCLUSION: Research into the molecular epidemiology of juvenile nasopharyngeal angiofibroma is still in its infancy. Although the molecular variation is not well understood, data obtained so far have prompted important research questions. Hence, multicentre collaborative molecular studies are needed to establish the aetiopathogenesis and establish molecular surrogates for clinical characteristics.
Assuntos
Angiofibroma/genética , Marcadores Genéticos/genética , Neoplasias Nasofaríngeas/genética , Adolescente , Angiofibroma/epidemiologia , Regulação Neoplásica da Expressão Gênica , Predisposição Genética para Doença , Mapeamento Geográfico , Humanos , Índia , Epidemiologia Molecular , Neoplasias Nasofaríngeas/epidemiologia , Adulto JovemRESUMO
OBJECTIVE: An attempt is made to analyze the molecular behavior of juvenile nasopharyngeal angiofibroma (JNA). STUDY DESIGN: Case Series METHODS: Quantification of mRNAs expression was undertaken through real-time polymerase chain reaction in JNA (9-24) samples for VEGF-A, basic fibroblast growth factor (b-FGF), platelet-derived growth factor PDGF-A, KIT proto-oncogene receptor tyrosine kinase (c-Kit), Avian myelomatosis viral oncogene homolog (c-Myc), Harvey rat sarcoma viral oncogene homolog (H-Ras), tumor suppressor gene TP53, and androgen receptor and interleukin 6 (IL-6). The ß-catenin expression was evaluated by western blot in 16 samples. Nasal polyp was taken as control. RESULTS: A significantly increased (P < 0.01) expression of c-myc, VEGFA, bFGF, PDGFA, c-kit, and TP53 was seen, along with enhanced expression of ß-catenin. A massive enhancement of H-Ras expression was seen for the first time. Androgen receptor expression was no different, whereas IL-6 despite showing upregulation trend was not significant. CONCLUSION: The enhanced expressions of various markers suggest their potential role in JNA. Although the biological significance of c-kit, c-myc, and one of the novel markers H-Ras has yet to be defined, their significant expression may have a therapeutic importance. LEVEL OF EVIDENCE: NA. Laryngoscope, 127:E100-E106, 2017.
Assuntos
Angiofibroma/genética , Angiofibroma/patologia , Neoplasias Nasofaríngeas/genética , Neoplasias Nasofaríngeas/patologia , Adolescente , Angiofibroma/epidemiologia , Biópsia por Agulha , Criança , Estudos de Coortes , Feminino , Fator 2 de Crescimento de Fibroblastos/genética , Fator 2 de Crescimento de Fibroblastos/metabolismo , Marcadores Genéticos , Estudo de Associação Genômica Ampla , Humanos , Imuno-Histoquímica , Índia/epidemiologia , Masculino , Neoplasias Nasofaríngeas/epidemiologia , Fator de Crescimento Derivado de Plaquetas/genética , Fator de Crescimento Derivado de Plaquetas/metabolismo , Proto-Oncogene Mas , Reação em Cadeia da Polimerase em Tempo Real/métodos , Receptores Androgênicos/genética , Receptores Androgênicos/metabolismo , Sensibilidade e Especificidade , Adulto JovemRESUMO
BACKGROUND: The occurrence of juvenile nasopharyngeal angiofibroma is reportedly higher in India than in some other parts of the world, and our centre has seen a four-fold increase in its occurrence across seven decades. METHODS: This paper reports a retrospective archival analysis of 701 juvenile nasopharyngeal angiofibroma cases from 1958 to 2013, and considers probable environmental factors in an Indian context that may affect its biology and the global distribution, as reported in the literature. RESULTS: A continuously progressive increase in occurrence was evident, but the rapid rise observed in the current decade was alarming. The world map of juvenile nasopharyngeal angiofibroma incidence does not reflect true global distribution given the paucity of reporting. Our centre has dealt with approximately 400 cases in the last 24 years. CONCLUSION: With the alarming increase in juvenile nasopharyngeal angiofibroma incidence, there is a need for a registry to define its epidemiology. The world literature needs to reflect the status of juvenile nasopharyngeal angiofibroma incidence in the third world as well. Environmental factors known for hormone disruptive actions may influence its occurrence. Such aspects need to be considered to plan specific prevention policies.
Assuntos
Angiofibroma/epidemiologia , Neoplasias Nasofaríngeas/epidemiologia , Adolescente , Angiofibroma/etiologia , Criança , Meio Ambiente , Mapeamento Geográfico , Humanos , Incidência , Índia/epidemiologia , Neoplasias Nasofaríngeas/etiologia , Estudos RetrospectivosRESUMO
BACKGROUND: Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor of adolescent males and there is a paucity of Indian studies on this subject. AIMS: To present the experience of management of JNA at a single institution. SETTING AND DESIGN: This is a retrospective observational study of patients with JNA who presented at the Tata Memorial Hospital between May 1988 and August 2001. MATERIALS AND METHODS: Thirty-two patients with JNA were treated in the study period. Since the time period was prolonged and diagnostic and therapeutic protocols had undergone many changes, the patients were divided into two groups, namely 1988-1996 and 1997-2001. The age distribution, disease patterns, management approaches and treatment outcomes of patients in the two groups were recorded. Statistical analyses were done using students 't' test and test for proportion. RESULTS: The mean age at presentation was 16 years and more than 90% of the patients had Stage III or IV disease. Preoperative embolization was carried out in 19 patients. The surgical approaches used were median maxillectomy, infratemporal fossa, transpalatal, maxillary swing and craniofacial approach. The recurrence rate, complete resection rate and cure rates were 12.5%, 41% and 63% respectively. CONCLUSION: Surgery is the mainstay of treatment of JNA. Preoperative embolization and newer surgical approaches result in less blood loss and complete resection. Aggressive re-resection should be done for resectable recurrences reserving radiotherapy for unresectable, recurrent/ residual disease.
Assuntos
Angiofibroma/epidemiologia , Neoplasias Nasofaríngeas/epidemiologia , Recidiva Local de Neoplasia/epidemiologia , Adolescente , Adulto , Angiofibroma/etiologia , Angiofibroma/patologia , Angiofibroma/terapia , Terapia Combinada , Embolização Terapêutica/estatística & dados numéricos , Feminino , Humanos , Índia/epidemiologia , Masculino , Prontuários Médicos , Neoplasias Nasofaríngeas/etiologia , Neoplasias Nasofaríngeas/patologia , Neoplasias Nasofaríngeas/terapia , Recidiva Local de Neoplasia/etiologia , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Estadiamento de Neoplasias , Procedimentos Cirúrgicos Otorrinolaringológicos/estatística & dados numéricos , Complicações Pós-Operatórias , Estudos RetrospectivosRESUMO
A treatment and follow-up study of 32 patients with juvenile nasopharyngeal angiofibroma (JNA) was performed at our clinic between 1974 and 1998. The majority had undergone surgery either via an antral approach or with a lateral rhinotomy. In the 1970s, surgery was combined with ligature of the external carotid artery and, since 1981, it has been combined with preoperative embolization. Two patients received radiotherapy (45 Gy) as primary treatment and the 3 cases of multiple recurrence received radiotherapy (30-45 Gy) as secondary treatment. No recurrence was found in patients treated with radiotherapy. The overall recurrence rate was 25%; the recurrence rate in non-embolized patients was 8% and among embolized patients it was 41%. We found no statistically verified differences in recurrence rate between embolized and non-embolized patients. No statistically significant difference was found in either recurrence or peroperative bleeding when comparing preoperatively embolized patients with non-embolized patients. Regression analyses showed that the only factor affecting recurrence was age, i.e. the younger the patient was at diagnosis the greater the risk of developing recurrence. The development of imaging and embolization techniques will hopefully contribute in the future towards reducing the recurrence rate.
Assuntos
Angiofibroma/epidemiologia , Embolização Terapêutica , Neoplasias Nasofaríngeas/epidemiologia , Adolescente , Angiofibroma/terapia , Seguimentos , Humanos , Masculino , Neoplasias Nasofaríngeas/terapia , Recidiva Local de Neoplasia , Cuidados Pré-Operatórios , Análise de Regressão , Fatores de TempoRESUMO
OBJECTIVE: Angiofibromas in the head and neck area usually arise in the nasopharynx (NA) in adolescent males. They may also occur outside the nasopharynx (ENA) and can therefore be misdiagnosed. An ill-advised biopsy may result in brisk bleeding. This study was undertaken to evaluate the incidence and clinical features of ENA. MATERIAL AND METHODS: A review of the international literature was performed. RESULTS: A total of 65 patients with ENAs from 16 different countries were reported in the literature. Two patients had a congenital lesion, the oldest being 78 years old. A total of 48 patients were male (73%). The maxilla was the most commonly affected site (24.6%), with the ethmoid, nasal cavity or septum and other sites being involved less frequently. Symptoms arose in 40 patients within 6 months. Among a wide variety of symptoms, epistaxis with or without nasal obstruction was reported for 18 patients. Brisk bleeding resulting from a total of 23 biopsies occurred in 13 patients, and required blood transfusion in 11. Death was reported for two patients as a result of acute respiratory compromise and endocranial extension. CONCLUSION: ENAs are extremely rare compared to NAs and have to be acknowledged as a different entity. In comparison to patients with NAs, as female adults are affected, the lesion is diagnosed earlier and is less vascularized and the patients are older. Surgical resection is sufficient treatment due to a tendency for local and less aggressive growth.
Assuntos
Angiofibroma , Neoplasias de Cabeça e Pescoço , Adolescente , Adulto , Distribuição por Idade , Idoso , Angiofibroma/diagnóstico , Angiofibroma/epidemiologia , Angiofibroma/etiologia , Angiofibroma/terapia , Biópsia , Quimioterapia Adjuvante , Criança , Pré-Escolar , Epistaxe/etiologia , Feminino , Neoplasias de Cabeça e Pescoço/diagnóstico , Neoplasias de Cabeça e Pescoço/epidemiologia , Neoplasias de Cabeça e Pescoço/etiologia , Neoplasias de Cabeça e Pescoço/terapia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Obstrução Nasal/etiologia , Neoplasias Nasofaríngeas/diagnóstico , Neoplasias Nasofaríngeas/epidemiologia , Neoplasias Nasofaríngeas/etiologia , Neoplasias Nasofaríngeas/terapia , Procedimentos Cirúrgicos Otorrinolaringológicos , Radioterapia Adjuvante , Tomografia Computadorizada por Raios XRESUMO
In the present study we review ENT tumor pathology in childhood. Only the most salient aspects are emphasized and the variety of entities reviewed was restricted. Molecular biology techniques reveal infection by human papilloma virus (types 6 and 11) in 50 % of papillomas, while immunohistochemical techniques are less effective in papilloma virus detection. The myofibroblastic nature of nasal angiofibroma has been demonstrated and its incidence is 25 times more frequent in patients with familial polyposis of the colon. Overexpression of p53 occurs in the initial stages of nasopharyngeal carcinoma, while overexpression of c-myc is correlated with an unfavorable prognosis. Recently, olfactory neuroblastoma has been shown not to express the protein product of the MIC-2 gene (antibody 12E7), thus the hypothesis that it could be a member of the Ewing tumor family (neuroectodermal peripheral tumors) has not been confirmed, although it is a primitive neural tumor. The head and neck rhabdomyosarcoma with the best prognosis is that located in the orbit, and cytogenetic studies have shown chromosomic translocation t(2;13) in 50 % of these childhood tumors when they are of the alveolar-type, while trisomy of chromosome 2 or 20 is more characteristic of the embryonic-type. Currently, any classifying features of ENT lymphomas must be based on the Revised European-American Classification of Lymphoid Neoplasms (REAL). Papillary and medullary carcinomas are the most common histological types of thyroid carcinoma in childhood. Alterations in ret/PTC play a significant role in the pathogenesis of both.
Assuntos
Carcinoma/patologia , Neoplasias Laríngeas/patologia , Neoplasias Bucais/patologia , Neoplasias Nasofaríngeas/patologia , Papiloma/patologia , Neoplasias Faríngeas/patologia , Neoplasias da Glândula Tireoide/patologia , Adolescente , Angiofibroma/epidemiologia , Angiofibroma/patologia , Carcinoma/epidemiologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Estesioneuroblastoma Olfatório/epidemiologia , Estesioneuroblastoma Olfatório/patologia , Humanos , Neoplasias Laríngeas/epidemiologia , Neoplasias Laríngeas/virologia , Linfoma/epidemiologia , Linfoma/patologia , Neoplasias Bucais/epidemiologia , Neoplasias Nasofaríngeas/epidemiologia , Papiloma/epidemiologia , Papiloma/virologia , Papillomaviridae/isolamento & purificação , Neoplasias Faríngeas/epidemiologia , Neoplasias Faríngeas/virologia , Rabdomiossarcoma/epidemiologia , Rabdomiossarcoma/patologia , Neoplasias da Glândula Tireoide/epidemiologiaRESUMO
30 patients suffering from angiofibroma juvenile were treated in the Department of Otolaryngology in Poznan in the years 1977-2000. The patients aged from 9-36 years. All the patients were treated surgically and the method of choice was Denker's surgery. 3 patients were reoperated because of local recurrence. On the base of own material good results of full recovery of patients with angiofibroma treated surgically are presented.
Assuntos
Angiofibroma/cirurgia , Neoplasias Nasofaríngeas/cirurgia , Recidiva Local de Neoplasia/cirurgia , Adolescente , Adulto , Angiofibroma/epidemiologia , Angiofibroma/patologia , Criança , Feminino , Humanos , Masculino , Neoplasias Nasofaríngeas/epidemiologia , Neoplasias Nasofaríngeas/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias Otorrinolaringológicas/cirurgia , Polônia/epidemiologia , Reoperação , Estudos Retrospectivos , Fatores Sexuais , Fatores de Tempo , Resultado do TratamentoRESUMO
BACKGROUND: Periorbital dermatoses are very common dermatological disorders which pose a diagnostic and therapeutic challenge for the treating dermatologist due to the similarity of symptoms in this area. AIMS: To study the spectrum of periorbital dermatoses and to assess the association of systemic diseases with periorbital dermatoses. METHODS: This was a hospital based descriptive study done on 250 consecutive patients irrespective of their age and sex who attended the Dermatology out patient department (OPD), with dermatological conditions pertaining to the periorbital area over a period of 2 years from October 2010 to September 2012. RESULTS: The most common dermatological conditions seen in the periorbital region were benign and malignant skin tumors observed in 63 (25.2%) cases, followed by the disorders of pigmentation in 51 (20.4%) and eczema in 44 cases (17.6%), infections in 33 (13.2%) cases and nevoid conditions in 26 (10.4%) cases. Certain periorbital dermatoses were significantly more in females than in males in the present study like skin tags, connective tissue diseases, and periorbital melanosis (POM). Some periorbital dermatoses were significantly more common in older age group such as seborrheic keratoses, skin tags and airborne contact dermatitis whereas infections, syringomas, allergic contact dermatitis and atopic dermatitis were more prevalent in younger age group. Syringomas and POM were more common in middle-aged women. CONCLUSIONS: The most common dermatological condition seen in the periorbital region in this study are benign skin tumors (keratosis and skin tags), followed by the disorders of pigmentation and eczema, infections and nevoid conditions.
Assuntos
Angiofibroma/epidemiologia , Carcinoma Basocelular/epidemiologia , Eczema/epidemiologia , Dermatoses Faciais/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adulto , Feminino , Humanos , Hiperpigmentação/epidemiologia , Hipopigmentação/epidemiologia , Índia/epidemiologia , Ceratose/epidemiologia , Hanseníase/epidemiologia , Masculino , Órbita , PrevalênciaAssuntos
Doenças da Unha/epidemiologia , Neoplasias Cutâneas/epidemiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Angiofibroma/epidemiologia , Carcinoma de Células Escamosas/epidemiologia , Criança , Pré-Escolar , Cistos/epidemiologia , Cisto Epidérmico/epidemiologia , Feminino , Fibroma/epidemiologia , Tumores de Células Gigantes/epidemiologia , Tumor Glômico/epidemiologia , Hemangioma/epidemiologia , Humanos , Masculino , Melanoma/epidemiologia , México/epidemiologia , Pessoa de Meia-Idade , Neoplasias/epidemiologia , Neurofibroma/epidemiologia , Estudos Retrospectivos , Fatores SexuaisRESUMO
Introducción: El angiofibroma nasofaríngeo juvenil es una neoplasia vascular benigna y localmente agresiva, que se desarrolla casi exclusivamente en adolescentes de sexo masculino. Sus manifestaciones clínicas habituales son epistaxis y obstrucción nasal. Objetivo: Dar a conocer la experiencia en el Servicio de Otorrinolaringología del Hospital Carlos van Buren de pacientes con angiofibroma operados por vía endoscópica y abierta entre los años 2008 y 2015. Material y método: Estudio descriptivo retrospectivo de pacientes con diagnóstico de angiofibroma nasofaríngeo juvenil que ingresaron al Servicio de Otorrinolaringología del Hospital Carlos van Buren entre los años 2008 y 2015. Resultados: Hubo un total de 6 casos. La edad de los pacientes fluctuó entre los 12 y los 29 años, el 100% fueron pacientes masculinos. Los síntomas de presentación más frecuente fueron epistaxis recurrente y obstrucción nasal, presentes en 5/6 de los pacientes. La totalidad de los casos fueron estudiados con TC, RM y angiografía. El manejo en todos los casos fue con embolización endovascular 48 horas previo a la resección. Conclusión: Los resultados obtenidos se correlacionan con la literatura. El abordaje endoscópico sigue siendo de elección. Este tiene como ventajas menores pérdidas sanguíneas intraoperatorias, una disminución del número días de hospitalización y las tasas de recurrencia.
Introduction: Juvenile nasopharyngeal angiofibroma is a benign vascular neoplasm, locally aggressive that develops almost exclusively in adolescent males. Its usual clinical manifestations are epistaxis and nasal obstruction. Aim: To show the experience in the Department of Otolaryngology Hospital Carlos van Buren of angiofibromas operated by endoscopic and open surgery between the years 2008 and 2015, and review of the literature. Material and Method: Retrospective descriptive study of patients diagnosed with juvenile nasopharyngeal angiofibroma admitted in the Department of Otolaryngology Hospital Carlos van Buren, Valparaiso between 2008 and 2015. Results: A total of 6 cases were identified. The age of patients ranged from 12 to 29 years. The most common presenting symptoms were recurrent epistaxis and nasal obstruction, both present in 5/6 of patients. All the cases were studied with CT, MRI and angiography. All cases had pre-surgical endovascular embolisation48 hours prior to excision. Conclusions: The results correlate with those seen in the literature. The endoscopic approach is the better option, because of its lower intraoperative blood loss, days of hospitalization and recurrence.