Paratesticular tumors. A clinicopathological study from a single tertiary hospital in North India.

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.

Juvenile Nasopharyngeal Angiofibroma Outcomes and Cost: Analysis of the Kids' Inpatient Database.

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.

Juvenile nasopharyngeal angiofibroma: a single centre's 11-year experience.

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.

Infratemporal fossa tumors: When to suspect a malignant tumor? A retrospective cohort study of 62 cases.

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.

Variable expression of molecular markers in juvenile nasopharyngeal angiofibroma.

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.

Nasal juvenile angiofibroma: Current perspectives with emphasis on management.

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.

Current molecular profile of juvenile nasopharyngeal angiofibroma: First comprehensive study from India.

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.

Time trends in recurrence of juvenile nasopharyngeal angiofibroma: Experience of the past 4 decades.

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.

Angiofibroma nasofaríngeo juvenil: estudio de 6 casos en el Hospital Carlos van Buren de Chile / Juvenile nasopharyngeal angiofibroma: study of 6 cases in the Carlos van Buren Hospital in Chile

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.

Changing trends in the incidence of juvenile nasopharyngeal angiofibroma: seven decades of experience at King George's Medical University, Lucknow, India.

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.

Molecular pathogenesis of juvenile nasopharyngeal angiofibroma in brazilian patients.

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.

Spectrum of periorbital dermatoses in South Indian population.

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.

[Angifibroma juvenile--evolution of surgical management]. / Ewolucja chirurgicznych metod leczenia wlókniaka mlodzienczego.

INTRODUCTION: Juvenile Angiofibroma has been challenged generations of surgeons. The problem is, not only in specific clinical features of the tumour, but also in difficult surgical approach to anatomical area in which it is located. THE AIM OF THE STUDY: was to analyze development of surgical techniques in treatment of juvenile angiofibroma and potential conditions which influence on therapy results. MATERIAL AND METHOD: The retrospective study that analyzes 47 juvenile angiofibroma cases, which were treated in the Otorhinolaryngology Department of Warsaw Medical University in years 1980­2010. RESULTS: The five surgical approaches were used in the group of patients, filled in this study. The use of midfacial degloving has become increasingly popular, replacing lateral rhinotomy and transpalatal approach, which were commonly used in early nineties of the last century. The infratemporal approach was used in one case of disease with parapharyngeal and infratemporal extension. One of the recent patients was treated, first time in history of the Department, using endoscopic approach. The routine protocol of treatment includes preoperative CT and MRI and angiography with embolization of the main tumour vessels. The gradual, but consequent improvement in therapy results is revealed as reduction of recurrence from 35% to 20%. CONCLUSIONS: In therapy of juvenile angiofibroma become popular surgical techniques, which are not connected with visible scars and which reduce tissue trauma in operated area. Besides mastering surgical skills, the independent condition, which improves therapy results, is using modern diagnostic and therapeutic procedures. They contribute to better assessment of tumour location and extension, and reduction of intraoperative blood loss.

[Evolution in the treatment of juvenile nasopharyngeal angiofibroma]. / Evolución en el tratamiento de los angiofibromas nasofaríngeos juveniles.

INTRODUCTION: Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumour in adolescent males. It may be associated with a significant morbidity because of its anatomical location and its locally destructive growth pattern. Severe haemorrhage constitutes a high risk in JNA and its surgical management could be complex. MATERIAL AND METHOD: We retrospectively analysed the clinical data from 43 patients with JNA surgically treated in our Department from 1993 until 2010. Mean postoperative follow-up time was 85 months. RESULTS: Analysis was performed on 42 males and one female. Mean patient age was 16 years old. The most common presenting symptoms were unilateral epistaxis (56%) and nasal obstruction (56%). Using the Fisch staging scale, tumours were classified as stage I in 2 patients, stage II in 9, stage III-a in 13, stage III-b in 13 and stage IV-a in 6. Preoperative selective embolisation was performed on 32 patients (74%). Thirty-three patients (77%) underwent an open surgical approach and 10 (23%) were treated by endoscopic approach. Complete resection of the lesion was achieved in 35 patients (81%) and tumour recurrence was observed in 2 (5%). All lesions treated via transnasal endoscopic approach were stage I and stage II lesions. CONCLUSION: Surgery is the treatment of choice for JNA. An endoscopic approach is feasible for early-stage lesions (Fisch I and II) and conservative external approaches are still useful in advanced stages (Fisch III and IV). The open approaches proved helpful with respect to exposure, safety, cosmetic outcome and low morbidity. Preoperative embolisation, if possible, is mandatory.

Nasoangiofibroma juvenil: nuestra experiencia en los últimos 10 años y revisión de la literatura / Juvenile nasopharyngeal angiofibroma: our experience in the last 10 years and review of the literature

Introducción: El nasoangiofibroma juvenil es un tumor benigno con comportamiento de agresividad local, dadas las importantes epistaxis que provoca, la frecuente recidiva local y la complejidad terapéutica que se presenta ante esta patología; se presenta exclusivamente en hombres adolescentes y corresponde al 0,5 por ciento-0,05 por ciento de todos los tumores de cabeza y cuello. Objetivo: Determinar el manejo y las características del paciente con el diagnóstico de nasoagiofibroma juvenil que acude a nuestro servicio. Evaluar necesidad de transfusión sanguínea. Exponer las complicaciones encontradas. Material y método: Se realizó un trabajo retrospectivo observacional de corte transversal de pacientes con diagnóstico de nasoangiofibroma juvenil (NAJ), confirmado por estudios histológicos en la Cátedra de Otorrinolaringología de la Facultad de Ciencias Médicas de la Universidad Nacional de Asunción, desde enero de 1998 hasta septiembre de 2008. Resultados: Con un total de 45, de los cuales 44 recibieron tratamiento quirúrgico. El rango de edad estaba comprendido entre 7 a 34 años, con una mediana de 16 años y un promedio de 17 años, todos de sexo masculino. Se presentaron con mayor frecuencia en estadio Fisch II, rango de evolución sintomática de 1 a 60 meses, con una mediana de 7 meses de evolución de epistaxis recurrente, obstrucción nasal y rinorrea. La mayoría de grupo sanguíneo O Rh+, provenientes del Departamento Central, recibiendo como tratamiento la resección endoscópica. Siete pacientes presentaron recidivas, con una mediana de 7 meses desde el tratamiento. En 58,8 por ciento de los procedimientos quirúrgicos fue necesaria la transfusión de derivados sanguíneos. Conclusión: Los pacientes que acuden al servicio con el diagnóstico de nasoangiofibroma juvenil, son sometidos a tratamiento quirúrgico en la totalidad de los casos. En la mayoría de los casos se requirió algún tipo de transfusión sanguínea. No hubo complicaciones por...

Introduction: The juvenile Nasopharyngeal Angiofibroma is a benign tumor with behavior of local aggressiveness, given the important epistaxis that it provokes, the frequent local recidiva and the therapeutic complexity that one presents before this pathology; he appears exclusively in teen men and corresponds to 0,5 percent-0,05 percent of all the tumors of head and neck. Aim: To determine the managing and the characteristics of the patient with juvenile Nasopharyngeal Angiofibroma diagnosis that comes to our service. To evaluate need of blood transfusion. To expose the opposing complications. Material and method: There realized a retrospective work observacional of patients' transverse court (cut) with diagnosis of juvenile Nasopharyngeal Angiofibroma (NAJ), confirmed by histological studies in the Service of Otolaryngology of the Asuncion's National University, from January, 1998 until September, 2008. Results: With a whole of 45, of which 44 received surgical treatment. The range of age was understood (included) between (among) 7 to 34 years, with a median of 16 years and an average of 17 years, all of masculine sex. Appellants presented with major frequency in estadio Fisch II, range of symptomatic evolution from 1 to 60 months, with a median of 7 months of evolution of epistaxis, nasal obstruction and rinorrea. The majority of blood group O Rh +, from the Central department, receiving as treatment the resection endoscopic. 7 patients presented recidivas, with a median of 7 months from the treatment. In 58,8 percent of the surgical procedures there was necessary the transfusion of blood derivatives. Conclusion: The patients who come to the service with juvenile nasopharyngeal angiofibroma diagnosis, are submitted to surgical treatment in the totality of the cases. In most cases there was needed some type of blood transfusion. There were no complications for the surgical procedure.

Efficacy of selective percutaneous embolization for the treatment of intractable posterior epistaxis and juvenile nasopharyngeal angiofibroma (JNA).

CONCLUSION: Percutaneous embolization reduces the reappearance of epistaxis and the mean length of hospital stay for patients with intractable epistaxis or juvenile nasopharyngeal angiofibroma (JNA). OBJECTIVES: To assess whether percutaneous embolization is effective for the treatment of intractable epistaxis and JNA. PATIENTS AND METHODS: Twenty-eight patients with intractable posterior epistaxis treated by embolization (25 males; m = 59.78 years; SD = 14.3) and 28 unembolized patients (24 males; m = 59.21 years; SD = 15.13) were studied retrospectively. Eight patients with JNA (all males; mean = 16.5 years; SD = 2.35; four embolized before surgery and four unembolized) were also analyzed. RESULTS: Embolization was bilateral in 71.4% of subjects, blood transfusion was required in 28.57%, incidence of complications was 53.57%, and seven of the embolized patients with intractable epistaxis suffered from recurrence of the epistaxis. There were no statistically significant differences between the embolized and unembolized groups. The mean hospital stay was longer in unembolized patients (4.46 days; SD = 2.42) than for the embolized patients (3.78 days; SD = 3.9), p=0.394. The most serious complications occurred in patients embolized with idiopathic epistaxis and the mean post-embolization hospital stay was longer in this subgroup (4.14 days; SD = 4.39) than in patients with Rendu-Osler-Weber syndrome (2.40 days; SD = 1.140), p=0.395. Unembolized patients with JNA presented greater hemorrhage (m = 2025 ml; SD = 325) and a longer mean hospital stay (m = 18 days; SD = 3) than the group of embolized patients (m = 360 ml; SD = 185 and m = 9 days; SD = 1, respectively).

Cutaneous lesions associated to multiple endocrine neoplasia syndrome type 1.

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.

Juvenile nasopharyngeal angiofibromas in Denmark 1981-2003: diagnosis, incidence, and treatment.

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.