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(Patient 1): A. Several squamous papillomas at age 5. Fibrovascular cores are surfaced by moderately dysplastic squamous epithelium (basilar expansion focally into the mid portion). Extensive surface viropathic change (H&E; 40×). B. ISH for LR-HPV demonstrating extensive nuclear reactivity (LR-HPV ISH; 200×).
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Introduction:
Recurrent respiratory papillomatosis (RRP) is a human papillomavirus (HPV) related disease in both children and adults, characterized by recurrent benign squamous papillomas of the respiratory mucosa. Malignant transformation is rare. The present report concerns the natural history of RRP in two children.
Materials and methods:
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Citations
... Malignant transformation of RRP is even rarer still, associated exclusively with squamous cell carcinoma (SCC) and occurring in 3%-7% of cases in adults and less than 1% in children. 1,2 The aetiologic role of HPV in RRP is well-established, and most commonly associated with the so-called low-risk virotypes HPV-6 and HPV-11. 1,3 Due to the refractory nature of the disease, its current management focuses on regular resection of disease, typically with CO 2 laser. ...
... 1,2 The aetiologic role of HPV in RRP is well-established, and most commonly associated with the so-called low-risk virotypes HPV-6 and HPV-11. 1,3 Due to the refractory nature of the disease, its current management focuses on regular resection of disease, typically with CO 2 laser. In a minority of cases, spontaneous remission can occur. ...
Recurrent respiratory papillomatosis is a human papillomavirus-mediated condition characterised by the development of benign squamous papillomata of the respiratory tract. Malignant transformation of recurrent respiratory papillomatosis, while rare, carries a poor prognosis and there are limited data surrounding treatment options, particularly in inoperable disease. We present the case of a 64-year-old male who developed malignant airway obstruction secondary to primary tracheal squamous cell carcinoma in the setting of a 5-year history of recurrent laryngotracheal papillomatosis, requiring placement of tracheostomy while on veno-venous extracorporeal membranous oxygenation. He was managed with cisplatin-based definitive chemoradiotherapy and had a complete metabolic response on post-treatment positron emission tomography/computed tomography, and remains free of recurrent squamous cell carcinoma at 16 months following treatment. This case supports the use of combined chemoradiotherapy as a potential therapeutic option for patients with primary tracheal squamous cell carcinoma, and emphasises the challenges associated with the long-term management of recurrent respiratory papillomatosis.
... Los pacientes infectados por VPH-11 presentan un cuadro más agresivo en comparación con los infectados con VPH-6, sin embargo, la recomendación de la genotificación no está clara aún 14 . La transformación maligna es infrecuente y se ha asociado en limitados casos a VPH de bajo riesgo 15 , sin embargo, se sugiere que la transformación a carcinoma laríngeo está asociada principalmente a factores de riesgo como tabaquismo, consumo de alcohol, inmunosupresión y radiación 11 . Por otra parte, varios estudios han sugerido la co-infección con genotipos 16 y 18 de VPH como un importante factor de transformación maligna 16 . ...
... Los pacientes infectados por VPH-11 presentan un cuadro más agresivo en comparación con los infectados con VPH-6, sin embargo, la recomendación de la genotificación no está clara aún 14 . La transformación maligna es infrecuente y se ha asociado en limitados casos a VPH de bajo riesgo 15 , sin embargo, se sugiere que la transformación a carcinoma laríngeo está asociada principalmente a factores de riesgo como tabaquismo, consumo de alcohol, inmunosupresión y radiación 11 . Por otra parte, varios estudios han sugerido la co-infección con genotipos 16 y 18 de VPH como un importante factor de transformación maligna 16 . ...
Human papillomavirus (HPV) is a double stranded circular DNA virus with around 200 genotypes. This virus is related to different tumor lesions that affect the head and neck, including malignant squamous lesions caused by high-risk genotypes such as HPV-16 and HPV-18. The objective of this review is to determine the role of HPV in different pathologies, distinguishing between benign and malignant lesions, and with particular emphasis on those in which a causal association with the virus has been demonstrated, such as laryngeal papillomatosis and oropharyngeal squamous carcinoma. In addition, molecular damage mechanisms, detection and prevention methods such as vaccination against the virus will be analyzed. It is necessary to know the relevance of HPV in our specialty, since its determination may have implications in terms of patient management and prognosis.
... The higher Derkay's anatomic score and number of previous surgeries implies the extensive and recurrent nature of RRP but had not shown any significant association with most of the trace elements except cobalt, gallium, and thallium whose role in pathogenesis of papilloma needs more investigations. Recent literature had extensively studied the role of respiratory papillomatosis as the pre-cancerous lesions of upper aero-digestives malignancies [35,36]. The subgroup analysis had demonstrated that RRP with dysplastic changes were having deficiency of copper and zinc and an excess of arsenic in tissue compared to healthy respiratory mucosa, the findings which were established earlier about the role of these trace elements in the developments of various pre-cancerous lesions. ...
Recurrent respiratory papillomatosis (RRP) is a chronic airway disease characterized by unpredictable recurrences and relapses. Our study aimed to identify the role of trace elements, a proven risk factor for various malignancies, in the development of papilloma and to correlate their serum levels with various disease parameters. 32 RRP patients with 20 age-matched controls were recruited in the study. Analysis of serum trace elements was performed by atomic absorption spectrophotometry in whole blood samples from cases and controls. The serum levels of trace elements were correlated with age of onset, history of previous surgeries, tracheostomy status, Derkay’s score, and presence of dysplasia. Among the 32 cases there were 18 males and 14 females with a mean age of 20.85 years (range: 3–60 years). The mean serum levels of Thallium, Zinc, and Gallium were higher among cases compared to their controls whereas the levels of Arsenic, Copper, Cobalt, Selenium, Cadmium, and Lead were higher in control subjects compared to RRP patients. There was a statistically significant correlation of serum level of Cobalt with more than 3 surgeries per year (p = 0.02), Gallium and Thallium with Derkay’s score of more than 20 (p = 0.04 and 0.05) and Lead, Arsenic and Gallium with presence tissue dysplasia (p = 0.05, 0.04 and 0.04). Our study had shown a variable association of trace elements in RRP patients. The usefulness of these values need to be completely elucidated and our study calls for future investigations to identify the casual association of various trace elements in the pathogenesis of RRP.
... ough most adult-onset laryngeal papillomatosis cases were benign [13], the high-risk HPV types 16, 18, 31, and 33 have been reported in RRP [14], and they may cause potential malignant transformation in less than 1% of cases [14]. e risk factors of malignant transformation in RRP are believed to be (1) radiation; (2) immunosuppression; (3) chemotherapy; (4) multiple recurrences; and (5) smoking and/or alcohol addiction [15]. ...
Recurrent respiratory papillomatosis is a noninvasive benign epithelial tumor caused by human papillomavirus. Clinically, it featured rapid growth, multifocus, and frequent recurrence. Though a number of therapies have been investigated, the recurrence after treatment is always a challenge. In this report, we describe a 27-year-old male patient with recurrent respiratory papillomatosis who was treated with CO 2 laser therapy followed by 5-aminolevulinic acid photodynamic therapy (ALA-PDT). There was no adverse reaction after treatment and no recurrence during the follow-up time.
... 3 T h e c o u r s e o f t h e r e c u r r e n t r e s p i r a t o r y papillomatosis (RRP) is unpredictable, as some patients present a mild form with spontaneous recovery or cure after a single exeresis of the laryngeal papilloma, while others present an aggressive form of the disease with many recurrences requiring multiple interventions and hospitalizations. 4,5 The latter situation is less common, with the incidence rate of 1.8 cases per 100,000 adults and 4.3 cases per 100,000 children. A smaller number of patients present the malignant transformation of the lesion. ...
... 9 The worse prognosis is characterized by repeated polyp excisions, tracheostomy, and ultimately death due to respiratory failure or infections. 4,19 Malignant transformation to squamous cell carcinoma, as found in our case study, is a rare event that, according to Gélinas et al., 10 occurs in 0.55% (9 in 1666 patients with RRP), and may be found in the larynx, the bronchi, or the lungs. Malignant transformation into squamous cell carcinoma is more frequent in older children, and the median time interval between the diagnosis of JORRP and the diagnosis of cancer is 19 years (range 4-45 years). ...
... Therefore, a long-term follow-up of patients with relapsed JORRP is recommended. 4,21 In our case, the malignancy was diagnosed 33 years after the diagnosis of JORRP. ...
Papilloma associated with recurrent respiratory papillomatosis (RRP), caused by human papilloma virus (HPV) infection types 6 and 11, is the most common benign neoplasm of the larynx. The clinical features of RRP vary widely from mild to aggressive forms. RRP in children is known as juvenile-onset recurrent respiratory papillomatosis (JORRP). Its outcome may be poor or even fatal due to the high rate of recurrence and eventual spread to the entire respiratory tract. Pulmonary invasion is reported to occur in 3.3% of patients with RRP, and malignant transformation in 0.5% of patients. We report the case of a 39-year-old female patient with a diagnosis of JORRP from the age of 3 years, with extensive bilateral pulmonary involvement and malignant transformation. Analysis of the papilloma and carcinomatous tissues revealed the presence of HPV type 11, which is associated with rapid and aggressive progression. We discussed the case on the basis of a literature review on pulmonary invasion, malignant transformation, and HPV 11 aggressiveness.
... Recurrent respiratory papillomatosis (RRP) is a disease caused by infection with the human papillomavirus (HPV) characterized by multiple recurring papillomas throughout the respiratory tract (1). Extra laryngeal involvement is rare, with tracheal or proximal bronchial lesions appearing in only 5% of cases and pulmonary involvement in 3% of cases (2,3). The risk of malignant transformation is approximately 3-7% in adults, and is associated mainly with HPV serotypes 6 and 11 (4,5). ...
Recurrent respiratory papillomatosis (RRP) is a disease of the respiratory tract caused by infection with the human papillomavirus (HPV) and is characterized by multiple recurring papillomas throughout the respiratory tract. Although rare, extra laryngeal involvement carries the risk of malignant transformation in 3-7% of adults. We report the case of a patient with unmonitored juvenile onset RRP with pulmonary involvement found to have malignant transformation to squamous cell carcinoma (SCC). Incidentally found on chest radiography for mild chest wall trauma, she was found to have a large left lower lobe mass with pathology consistent with SCC. The patient underwent surgical management with thoracoscopic left lower lobectomy and mediastinal lymph node dissection followed by adjuvant chemoradiation for pathologic stage IIIA SCC. Surveillance imaging at 6 months shows no evidence of disease progression.
Introduction:
Recurrent respiratory papillomatosis (RRP) is a chronic disease of the upper respiratory tract caused by human papillomavirus types 6 and 11. The disease course is characteristically unpredictable, ranging from spontaneous remission to aggressive, recurrent disease. Thus, management is often challenging and requires unique approaches tailored to each individual patient. While recent literature has described risk factors for more aggressive disease, few sources have investigated the impact of smoking on RRP disease course and risk for malignant transformation.
Methods:
A retrospective chart review was conducted for adult RRP patients evaluated at an academic tertiary care center between 2005 and 2020. A total of 188 patients were identified. Demographic and clinical data were collected, including smoking and alcohol history, HPV subtype, history of dysplasia and/or carcinoma, voice handicap index scores, Derkay scores, debulkings (in office and operating room), and days to papilloma recurrence.
Results:
Malignant degeneration in RRP occurred in 16.3% of smokers and 3.6% of nonsmokers. Smokers who developed carcinoma had less debulkings per years of evaluation than those not developing carcinoma (0.21 vs 0.92, P = .004). Additionally, patients that either presented with or developed carcinoma during their course had a higher pack-year smoking history (18.0 vs 12.21, P = .0002). No difference in days to recurrence or inter-surgical interval was demonstrated between smokers and nonsmokers.
Conclusions:
The report demonstrates that smoking can increase the risk of malignant transformation in RRP patients.
Background
The incidence and risk factors for the development of high‐grade dysplasia (HG‐D) and laryngeal squamous cell carcinoma (LSCC) were assessed in patients with laryngeal squamous cell papillomas (LSP).
Methods
Clinical data, human papillomaviruses (HPV) typing, HPV E6/E7 mRNA in situ hybridization, and sequencing of host genes in LSP biopsies of 163 patients were analyzed.
Results
Progression to HG‐D and LSCC was identified in 21.5% and 4.3% of LSP patients, respectively. A more advanced age at LSP onset and lack of HPV infection were detected as risk factors for the development of HG‐D and LSCC (P < .05). The identification of HG‐D was associated with its progression to LSCC (P < .05). Host gene mutations were identified in 3 of 7 patients with LSCC.
Conclusions
The histological monitoring of LSP and HPV typing are necessary for early detection of epithelial changes. Further research is needed to elucidate the role of host gene mutations in LSCC transformation.
This chapter discusses laryngeal anatomy and the range of benign and malignant laryngeal tumors. The most common squamous carcinoma variants in the larynx are covered: spindle cell variant, papillary variant and verrucous carcinoma. The changes in AJCC staging are discussed, as well as the two tiered system of laryngeal precursor lesions. The most common non-squamous laryngeal carcinoma is neuroendocrine carcinoma. The most common true sarcoma is chondrosarcoma, which is usually low-grade.
