Juvenile Papillomatosis Research Articles

Juvenile Laryngeal Papillomatosis is More Aggressive Than the Adult Variant

Juvenile Laryngeal Papillomatosis is More Aggressive Than the Adult Variant

Molecular insights into paediatric breast fibroepithelial tumours.

This study aims to examine the molecular genetics of paediatric breast fibroepithelial tumours through the targeted sequencing of 50 genes. Formalin-fixed paraffin-embedded tissues of fibroepithelial tumours diagnosed in a cohort of patients aged 18 years and below were subjected to next generation sequencing using the Haloplex Target Enrichment System. Twenty-five conventional and 17 juvenile fibroadenomas were studied, with MED12 mutations found in 53.8 and 35% of the tumours, respectively. There was also one benign fibroepithelial neoplasm with hybrid features of juvenile papillomatosis and infarcted benign phyllodes tumour-like areas. Most tumours did not have mutations in well-known cancer driver genes, none harboured TERT promoter mutations, while 25.6% (11 of 43) showed no mutations. Metachronous and synchronous tumours were found to have mutational heterogeneity with some containing mutations in MED12; other genes or no mutations were detected at all. Four of eight giant fibroadenomas (size 5 cm or larger) had no mutations detected, suggesting that there are other molecular mechanisms driving their growth. Tumours with MED12 mutations incidentally had a significantly higher stromal mitotic count compared with those without. While paediatric fibroepithelial lesions can have cellular stroma potentially raising concern for phyllodes tumour, their lack of TERT promoter and cancer driver mutations is reassuring. The absence of mutations in a significant proportion of tumours, especially the giant fibroadenomas, warrants investigation of pathogenetic mechanisms beyond those involving the 50 genes.

Laryngeal papillomatosis: A rare cause of dysphonia in the child. Case series

Juvenile laryngeal papillomatosis is an uncommon disease caused by human papilloma virus, mainly types 6 and 11. It is the most common benign laryngeal tumor in children. This condition should be suspected in any children with persistent and progressive dysphonia with or without stridor and respiratory distress. Flexible laryngoscopy under local anesthesia allows to visualize the typical wart-like lesions. The diagnosis is confirmed by pathological anatomy. Existing treatment is palliative and consists mainly of the surgical excision of the papillomas to maintain the airway without obstruction and to improve the quality of the voice, but it has a high rate of relapse. We present 20 patients with juvenile laryngeal papillomatosis. We describe the clinical manifestations, the diagnostic methods and the treatment of this pathology.

Genital Wart and Human Papillomavirus Prevalence in Men in the United States From Penile Swabs: Results From National Health and Nutrition Examination Surveys.

Human papillomavirus (HPV) is a common sexually transmitted infection in the United States that can lead to both malignant (high-risk) and benign (low-risk) findings. These low-risk findings include both genital condyloma, anal warts, and adult or juvenile papillomatosis, which are directly attributable to subtypes HPV 6 and HPV 11. We aim to estimate the prevalence of genital wart infections in men in the US population using a nationwide sample. The NHANES database was queried for all men aged 18 to 59 years during the years 2013 to 2014. During these years, the survey included penile swabs that were tested for HPV infection from 37 subtypes using PCR. Information was also obtained regarding patient reported history of having a genital wart infection previously. A total of 1757 men had information regarding HPV DNA. Fifty-three men tested positive for HPV 6 or HPV 11 DNA. This corresponds to an estimated prevalence of 2.9% (95% confidence interval, 2.2-3.8) of men aged 18-59 years. In addition, 2.2% (95% confidence interval, 1.5-3.3) of men reported a history of genital wart infection. There was no significant association with genital HPV DNA detection with age. Increasing number of sexual partners was associated with higher rates of both genital warts and HPV 6 and HPV 11 DNA. The estimated prevalence of genital HPV DNA in the US male population is 2.9%. This burden of disease could potentially be lowered with increased usage of quadrivalent and nonavalent vaccinations.

Juvenile recurrent respiratory papillomatosis in pregnancy: a case report

Juvenile recurrent respiratory papillomatosis in pregnancy: a case report

Clinical and sociodemographic characteristics associated with disease severity in juvenile recurrent respiratory papillomatosis: A study of 104 patients in a tertiary care pediatric hospital

BackgroundJuvenile recurrent respiratory papillomatosis (JRRP) is generally aggressive and with a high recurrence rate. Currently, there is no definite curative treatment for JRRP. Therefore, a greater understanding of the aspects that influence the severity and prognosis of the disease is required. ObjectiveThe aim of this study was to establish the clinical and socioeconomic characteristics of pediatric patients with JRRP and its relationship with the severity of the disease in a tertiary care pediatric hospital. ResultsA strong relationship was observed between the severity of the disease and the age at the time of diagnosis, and having a tracheostomy. A moderate association was found between the severity of the disease and the age at the time of the study, the area of origin and the recurrence rate. None of the socioeconomic statuses had a correlation with the severity of the JRRP. ConclusionsJRRP is associated with multiple surgeries due to the recurrence and aggressiveness of the disease. The socioeconomic status does not seem to influence the severity of the disease, whereas younger patients and users of tracheostomy should receive a more strict follow-up given the increased risk of severe disease.

A retrospective analysis of CO(2) laser and microdebrider for the treatment of severe juvenile on-set recurrent respiratory papillomatosis

Objective: Juvenile on-set recurrent respiratory papillomatosis (JORRP) can be an aggressive and potentially life-threatening disease. To emphasize the importance of complication prevention in local excision surgery, the single-center experience of RRP treatment in a group of patients with relatively short recurrence-free-interval (>4 times/yrs) was reported. Methods: Data of 49 patients underwent papilloma resection during 2002-2013 were retrospectively analyzed. Combined techniques of laser microsurgery and microdebriders were used to remove papillomas, prevent the formation of scar tissue and improve respiratory distress. Forty-nine cases were followed up for 99.0[74.5; 121.0]months, ranging from 39 to 185 months. The age of disease onset was 2[1; 3] years old. Most of the patients had difficulty in breathing(42 of 49 cases). Seven patients had hoarseness.53.0% patients had subglottic or trachea papillomatosis spreading. The rate of complications, synechia formation, tracheal stenosis, needing for tracheostomy, mortality was recorded. Patients were followed up for at least three years. The characteristics of the patients, improvement in symptoms, recurrence-free interval, and the rate of tracheal extubation were evaluated. Wilcoxon signed ranks test and chi-square test were used for data comparation. Results: Long-term relieve in disease(≤2 surgeries were needed/year) were achieved in 29(59.1%) subjects, in which 8(16.3%) of the subjects did not need surgical treatment for at least 3 years. Long-term relieve had been achieved in 25 of subjects who did not accept tracheostomy. Seven subjects did not need surgical treatment for at least 3 years. The rate of successful tracheal extubation was 53.8% in 13 patients who had undergone tracheostomy. Three cases dead of intrapulmonary spread. Conclusions: Combined techniques of laser microsurgery and microdebriders are effective in improvement in both respiratory distress and voice quality. The combined technique are helpful to avoid inevitable long-term stenotic complications, ultimately affecting the quality of life.

Sonographic and Magnetic Resonance Imaging Characteristics of Juvenile Papillomatosis: Three Cases With Different Manifestations.

Juvenile papillomatosis (JP) is an infrequently seen benign proliferative lesion in women younger than 30 years. Herein, we present different clinical manifestations of histopathologically proven JP of the breast, in addition to magnetic resonance and sonographic imaging features of these cases. Patient 1 exhibited nipple discharge and papillary carcinoma accompanied after the operation. Patient 2 presented with a giant mass with cystic and numerous solid nodular components that filled the entire right breast. Patient 3 exhibited cystic areas in a well-circumscribed hypoechoic solid mass besides ahypoechoic mass with indistinct borders, which was evaluated as multifocal JP.

PD-L1 expression and CD8+ infiltration shows heterogeneity in juvenile recurrent respiratory papillomatosis

IntroductionTumor immunotherapy have broadened therapeutic options for tumor treatment. The role of immune function in juvenile recurrent respiratory papillomatosis (JRRP) has not been investigated. Applying immunoblockade inhibitors as a novel disease treatment is unclear. Our study, for the first time, evaluates immune infiltration and immuno-suppressive molecule expression in JRRP. Our study provides insights in possibly treating this disease with tumor immunotherapies. We aimed to determine expression of programmed death-ligand 1 (PD-L1), a cancer escape protein, and presence of CD8+ T cell infiltration in tumor microenvironment. Material and methodsSeven patients with JRRP (mean age: 7.43; age range 3–17) in this study routinely have their tumors surgical debulked at Massachusetts Eye and Ear Infirmary. Following surgery, samples were de-identified and sent to pathology where they were stained and analyzed. ResultsSix out of seven patients expressed PD-L1 on tumor cells to various extents. Three patients showed concurrent PD-L1 expression on tumor cells and abundant CD8+ tumor infiltrating lymphocytes as well as PD-L1+ stromal lymphocytes, while PD-L1 expression on tumor cells were not associated with CD8+ tumor infiltrating T cells nor PD-L1+ stromal lymphocytes in the other three patients. HPV 6/11 and p16 was detected in all the patients. There appeared to be no correlation between either PD-L1 expression and CD8+ infiltration and clinical severity as measured by both the number of surgeries per year or Derkay score. ConclusionsDespite a small cohort, the expression of p16 and HPV 6/11 in all of the patients confirms the tissues were HPV tumor cells. PD-L1 expression was detected in the vast majority of tumor samples, while inflammatory cell compartments showed a higher degree of variation. Expression of PD-L1 on tumor cells but not inflammatory cells raises the possibility of a tumor cell intrinsic manner of PD-L1 expression. In contrast, a group of patients showed PD-L1 positivity in both tumor and inflammatory cells along with abundant CD8+ tumor infiltrating lymphocytes, suggesting adoptive immune resistance in these tumors and potential benefits from tumor immunotherapy.

The present-day approach to the treatment of recurrent juvenile laryngeal papillomatosis

40 children aged 1 through 16 years, suffering with juvenile recurrent laryngeal papillomatosis were treated in ENT Clinic of TMA in 2015–2016. All the patients underwent clinical, endoscopic, laboratory, immunological and virological examinations. The present-day approach to the complex treatment included surgical interference and conservative therapy. Larynx papillomas excision was performed by means of cryosurgery using microforceps under endoscopic control. The complex treatment included endolaryngeal injection of prospidin in the interoperational period and interferon – in the postoperational period, and celandine solution, prospidin and interferon inhalation as local therapy. The therapy duration exceeded two months. A substantial extension of interrecurrent periods (more than twofold) and the decrease of prevalence of papillomatosis foci have been achieved.

Photodynamic therapy by topical drup for the treatment of juvenile onset laryngeal papillomatosis

Objective:To observe the therapeutic effect of photodynamic therapy(PDT) on the treatment of juvenile onset laryngeal papillomatosis. Method:Twenty-eight cases of children with laryngeal papilloma were treated,only 2 cases for the first time, and the rest were repeatedly treated outside our hospital, the average hospital surgery were more than 4 times. Under self retaining laryngoscope and microscope and endoscope assisted by semiconductor laser and plasma and cold instrument method，visible tumor resection and local affixed deposited 20% 5-aminolevulinic acid(photosensitizer) 3 hours later, with 635 nm semiconductor laser photodynamic,200-280 mW and can volume density of 80 to 120 J/cm², 20 min irradiation. PDT should be repeated after 25 days until no visible tumor.Then,2 times PDT must be done. Result:In 28 cases, 24 cases were followed up for more than 1 years(12 cases were followed up for 3 years),19 had no recurrence, the cure rate was 79.2%(19/24)；5 cases recurrence, and the recurrence rate was 20.8%(5/24)，among them,2 cases were abandoned because of the relapse，the other 3 cases were cure after 3 times of PDT.The main complications were adhesion of larynx. Conclusion:The preliminary effect of PDT by topical drug for the treatment of juvenile onset laryngeal papillomatosis is encouraging. The principle of PDT and the principle of the recurrence of laryngeal papilloma in children were also introduced in this paper.

Juvenile recurrent respiratory papillomatosis in pregnancy: a case report

Juvenile recurrent respiratory papillomatosis in pregnancy: a case report

Clinical features and surgical treatment for Chinese juvenile onset current respiratory papillomatosis (JORRP).

Juvenile onset current respiratory papillomatosis (JORRP), which is the commonest benign laryngeal disease in children, has not been well investigated. This study aimed at further evaluating the clinical features and surgery for such children with JORRP. Patients diagnosed as JORRP in our department from January 2011 to June 2014 were enrolled, and the demographic and clinical data were collected. All the patients were followed up after surgery, and the recurrences were recorded. A total of 124 patients were included and categorized into low recurrence (<4/year, n=97) and high recurrence (≥4/year, n=27) groups based on the recurrence at diagnosis, respectively. The demographic characteristics were comparable in two groups. There were more patients in high recurrence group who had dyspnea II-III, abnormal chest X-ray findings and severe illness, and needed electrocardiscope monitoring (P<0.05). Patients who had dyspnea II (P=0.008) and severe illness (P=0.002) needed electrocardiscope monitoring (P=0.014) in high recurrence group were more prone to have recurrence after surgery. Multivariate logistic regression analysis proved that high recurrence at diagnosis was an independent risk factor for recurrence after surgery in JORRP [95%CI OR 17.342 (1.266, 237.608), P=0.033]. The recurrence at diagnosis could be used as a prognostic factor for JORRP after surgery, which could help to improve the therapeutic efficacy in such children.

Juvenile Papillomatosis of Breast (“Swiss Cheese Disease”) Occurs More Commonly in Adults than in “Juveniles”

Juvenile Papillomatosis of Breast (“Swiss Cheese Disease”) Occurs More Commonly in Adults than in “Juveniles”

Immunological aspects of the juvenile respiratory papillomatosis of the larynx

Immunological aspects of the juvenile respiratory papillomatosis of the larynx

Pulmonary Spread of Juvenile Laryngeal Papillomatosis: A Case Report

Introduction: Recurrent respiratory papillomatosis (RRP) is usually a relatively benign disease but can have an aggressive clinical course. The primary treatment for laryngeal papillomatosis is endoscopic resection. However, there is no effective treatment for pulmonary spread of laryngeal papillomatosis. Case Presentation: We describe a rare case of a 19-year-old man with pulmonary spread of laryngeal papillomatosis. The treatment regimen was surgical resection under general anesthesia using suspension laryngoscopy and endoscopy. The patient had been diagnosed with laryngeal papillomatosis and had undergone a tracheotomy when he was 18 months old. He underwent 17 endoscopic resections for laryngeal papillomatosis at intervals of 3 months to 1 year. At follow-up, the patient continued to have tracheal intubation and was not extubated. CT scans revealed multiple nodular and cavitary lesions in both lungs. Laryngoscopy performed when the patient was 18 years showed diffuse papillomata in the glottis and left ventricular fold. Conclusions: This article presents a rare case of juvenile laryngeal papillomatosis associated with the human papilloma virus (HPV) HPV-11 and pulmonary spread. Intravenous cidofovir has oncogenic potential, but it has been used successfully to treat five patients with pulmonary spread of RRP. Intravenous cidofovir should be considered as salvage therapy.

Pubertal Development

Pubertal Development

HPV type determination and clinical characteristics in juvenile onset recurrent respiratory papillomatosis

Objective:To investigate the relationship between different subtypes of human papillomavirus(HPV) infection and clinical features in juvenile onset recurrent respiratory papillomatosis(JORRP) patients. Method:Two hundred and forty cases with JORRP from 2010 to 2014 were retrospectively analyzed. HPV tpye were determined using tumor specimens taken from surgery, and all patients were divided into different groups based on HPV type. Clinical characteristics of patients from each groups were analyzed. Result:Except for HPV11 single infection or HPV6 single infection, some HPV11 and 6 co-infections were found in children with JORRP. Compared with patients with HPV6 single infection, HPV11 positive patients present an earlier age of onset, more times of operation and higher derkay score during surgery.Conclusion:JORRP in patients infected with HPV11 is more severe than that in patients with HPV6 infection in children.

Juvenile recurrent respiratory papillomatosis: 10-year audit and Australian prevalence estimates.

To estimate the prevalence of juvenile onset recurrent respiratory papillomatosis (RRP) in Australia, describe its epidemiological profile, and assess the positive predictive value of International Classification of Disease, 10th revision (ICD-10) code D14.1 (benign neoplasm of larynx) in children for hospitalization due to RRP. Retrospective case series. Retrospective case review undertaken at the three tertiary pediatric hospitals in New South Wales (Australia's largest state), by reviewing medical records of patients aged 0 to 16 years admitted during 2000-2009 containing the ICD-10 Australian modification code D14.1 or other possible disease (D14.2-4, D14.3, D14.4) and RRP-related procedure codes. For RRP diagnoses, we recorded treatment dates, length of stay, extent of disease, and surgical and adjuvant treatments. The positive predictive value (PPV) of code D14.1 and median number of hospitalizations per year were applied to national hospital separations data from 2000/2001 to 2012/2013 to estimate disease prevalence. We identified 30 cases of RRP using code D14.1, which had a PPV of 98.1%, with no further cases identified using other codes. Fifty-seven percent of cases were female, median age of onset was 36 months, and median treatment duration was 36 months (mean = 40 months, range = 1-118). There was one patient death. Between 2000 and 2013, the estimated national prevalence rate was 0.81 per 100,000 aged < 15 years, peaking at age 5 to 9 years (1.1 per 100,000). RRP prevalence can be monitored after human papillomavirus vaccination programs using routine hospital data. 4 Laryngoscope, 126:2827-2832, 2016.

Social­hygienic characteristic of larynx papillomatosis in children

Based on the analysis of 94 medical records of children with juvenile laryngeal papillomatosis aged 2–14 years old, who underwent in-patient treatment in the State Institution Scientific and Medical Center at the period of 2009 through 2015, the authors assessed the problem of laryngeal papillomatosis (LP) in children having investigated the morbidity structure and approximate cause and reason factors of the disease development in the country. The authors revealed the increased incidence, the prevalence of LP in the regions of the Republic and made a conclusion concerning the necessity of preventive measures among the population, aimed at avoidance of occurrence and recurrence of the disease.