Acute Airway Obstruction: An Unusual Presentation of Posttransplant Lymphoproliferative Disorder in a Renal Transplant Recipient.

Posttransplant lymphoproliferative disorder is a life-threatening complication after solid-organ transplants. In adults, recipients of heart transplants have the highest risk, whereas renal transplant recipients have the lowest risk among all solid-organ transplants. The most common site for posttransplant lymphoproliferative disorders are gastrointestinal tract followed by the graft itself. Airway involvement in posttransplant lymphoproliferative disorder is rarely encountered. We report a case of a 26-year-old renal allograft recipient who presented to the emergency room with airway obstruction necessitating an emergency tracheostomy. Imaging revealed a left tonsillar mass extending into the nasopharynx and retropharyngeal space causing complete oropharyngeal occlusion. Endoscopic biopsy from nasopharyngeal mass showed a diffuse large B-cell lymphoma and was Ebstein-Barr virus positive. Reduction in immunosuppression and treatment with posttransplant lymphoproliferative disorder-1 risk-stratified approach resulted in complete remission.

Efficacy of salvage surgery versus re-irradiation for isolated regional lymph node recurrence in patients with nasopharyngeal carcinoma.

BACKGROUND: To compare the clinical characteristics and prognoses of patients with isolated regional lymph node recurrent nasopharyngeal carcinoma (irrNPC) who underwent surgery or re-irradiation treatment. METHODS: We retrospectively reviewed 124 irrNPC patients who underwent initial radiotherapy between January 2010 and December 2020. The staging of regional lymph node recurrence was as follows: 75.8% for rN1, 14.5% for rN2, and 9.7% for rN3. Fifty-five patients underwent regional lymph node surgery (Surgery group), and sixty-nine patients received salvage radiotherapy with or without chemotherapy (Re-irradiation group). The survival rate was compared using Kaplan‒Meier analysis and evaluated by the log-rank test. Cox proportional hazard models were used to analyze prognostic factors. RESULTS: The median follow-up time was 70 months, the 5-year overall survival (OS) was 74%, and the median survival time was 60.8 months. There were no significant differences in 5-year OS (75.6% vs. 72.4%, P = 0.973), regional recurrence-free survival (RRFS, 62.7% vs. 71.1%, P = 0.330) or distant metastasis-free survival (DMFS, 4.2% vs.78.7%, P = 0.677) between the Surgery group and Re-irradiation group. Multivariate analysis revealed age at recurrence, radiologic extra-nodal extension (rENE) status, and recurrent lymph node (rN) classification as independent prognostic factors for OS. The rENE status was an independent prognostic factor for DMFS. Subgroup analysis of the Surgery group revealed that the rN3 classification was an adverse prognostic factor for OS. Age at recurrence ≥ 50 years, GTV-N dose, and induction chemotherapy were found to be independent prognostic factors for OS, RRFS, and DMFS, respectively, in the Re-irradiation group. CONCLUSIONS: For NPC patients with isolated regional lymph node recurrence after initial radiotherapy, those who underwent surgery had survival prognosis similar to those who underwent re-radiotherapy with or without chemotherapy. A prospective study is needed to validate these findings.

Salvage neck surgery in recurrent nodal NPC: Do all patients require a comprehensive neck dissection in the modern MRI era?

OBJECTIVE: The standard treatment for regional failure in nasopharyngeal carcinoma (NPC) is the radical neck dissection (RND). Our study sought to determine if magnetic resonance imaging (MRI) may accurately predict nodal involvement to allow selected levels of neck dissection to be preserved. STUDY DESIGN AND SETTING: We analysed retrospectively all NPC patients in our centre undergoing neck dissections as salvage therapy for nodal recurrence. Nodal involvement based on the preoperative MRI was assessed and compared with postoperative histopathology. METHODS: This is a retrospective study conducted on patients in our centre with recurrent NPC from February 2002 to February 2017. Patients were identified from the database of the otolaryngology oncology division at our institution. Of these, 28 patients met all our inclusion and exclusion criteria. We calculated sensitivity and specificity as well as average number of nodes per patient. RESULTS: In our study, we calculated the false negative and false positive rates of preoperative MRI neck by levels. Overall sensitivity of MRI picking up disease by level was 76% and specificity was 86%. CONCLUSION: Based on our study, we will be missing a total of 10 (7.1%) diseased neck levels in eight (28.5%) patients. MRI alone, therefore, does not provide enough information to allow safe selective preservation of neck levels in surgical salvage of neck recurrences in NPC.

Anatomical studies and early results on endoscopic transoral medial pterygomandibular fold approach to salvage retropharyngeal lymphadenectomy in nasopharyngeal carcinoma.

OBJECTIVE: Retropharyngeal lymphadenectomy is challenging. This study investigated a minimally invasive approach to salvage retropharyngeal lymphadenectomy in patients with nasopharyngeal carcinoma. METHODS: An anatomical study of four fresh cadaveric heads was conducted to demonstrate the relevant details of retropharyngeal lymphadenectomy using the endoscopic transoral medial pterygomandibular fold approach. Six patients with nasopharyngeal cancer with retropharyngeal lymph node recurrence, who underwent retropharyngeal lymphadenectomy with the endoscopic transoral medial pterygomandibular fold technique at the Eye and ENT Hospital of Fudan University from July to December 2021, were included in this study. RESULTS: The anatomical study demonstrated that the endoscopic transoral medial pterygomandibular fold approach offers a short path and minimally invasive approach to the retropharyngeal space. The surgical procedure was well tolerated by all patients, with no significant post-operative complications. CONCLUSION: The endoscopic transoral medial pterygomandibular fold approach is safe and efficient for retropharyngeal lymphadenectomy.

Endoscopic endonasal combined transoral medial approach to the nasopharynx, parapharyngeal space, and jugular foramen.

OBJECTIVE: This study aimed to validate the feasibility of an endoscopic endonasal combined transoral medial approach for treating lesions in the nasopharynx, parapharyngeal space (PPS), and jugular foramen. METHODS: Anatomical and imaging information of six patients who underwent surgery via this approach were reviewed and analyzed. RESULTS: The feasibility and advantages of the endoscopic endonasal combined transoral medial approach, which uses an inside-to-outside medial surgical corridor, were identified. Total resection was achieved in 3 cases with benign tumors. Safe resection margins were obtained in 2 cases with recurrent nasopharyngeal carcinoma (NPC). Pathological biopsy of NPC lesion between the Eustachian tube and arterial sheath was achieved. The internal carotid artery (ICA) was accurately located and protected in all cases and no complications occurred. CONCLUSION: Lesions in the nasopharynx, PPS, and jugular foramen can be directly assessed via this approach. The ICA can be well identified during the surgery.

Surgical treatment of nasopharyngeal cancer - a consensus recommendation from two Chinese associations.

BACKGROUND: Surgical treatment is playing an increasingly important role in the management of nasopharyngeal carcinoma (NPC). This consensus focuses on the indications for optimal surgery, and surgical methods in the whole process of treatment for NPC to provide a useful reference to assist these difficult clinical decisions. METHODOLOGY: A thorough review of available literature on NPC and surgery was conducted by the Association for the prevention and treatment of nasopharyngeal carcinoma in China, international exchange and promotion Association for medicine and healthcare, and the Committee on nasopharyngeal cancer of Guangdong provincial anticancer association. A set of questions and a preliminary draft guideline was circulated to a panel of 1096 experienced specialists on this disease for voting on controversial areas and comments. A refined second proposal, based on a summary of the initial voting and different opinions expressed, was recirculated to the experts in two authoritative medical science and technology academic groups in the prevention and treatment of NPC in China for review and reconsideration. RESULTS: The initial round of questions showed variations in clinical practice even among similar specialists, reflecting the lack of high-quality supporting data and resulting difficulties in formulating clinical decisions. Through exchange of comments and iterative revisions, recommendations with high-to-moderate agreement were formulated on general treatment strategies and details of surgery, including indications and surgical approaches. CONCLUSION: By standardizing the surgical indications and practice, we hope not only to improve the surgical outcomes, but also to highlight the key directions of future clinical research in the surgical management of NPC.

Surgical treatment and prognosis of recurrent and radiotherapy insensitive nasopharyngeal carcinoma.

OBJECTIVE: To explore the effect of surgical treatment and related prognostic factors for recurrent Nasopharyngeal Carcinoma (NPC) after radiotherapy and the pathological types of nasopharyngeal carcinoma insensitive to radiotherapy. METHODS: A total of 70 NPC patients who underwent surgery at the Department of Otolaryngology, head and neck surgery, from January 2005 to December 2020 were retrospectively included: 41 males and 29 females, aged 21-75 years, 47 patients were pathologically classified as NPC (nonkeratinizing, undifferentiated type), 10 patients as adenoid cystic carcinoma, 13 patients as other types, 45 patients had received radiotherapy preoperatively, and 25 patients had not received radiotherapy preoperatively. All patients underwent surgical treatment under general anesthesia. Fifty-six patients underwent nasoendoscopic NPC resection, seven patients underwent open surgery, and seven patients underwent combined nasoendoscopic and open surgery. The median follow-up was 39 months. Tumor volume, extent of involvement, lymph node metastasis, imaging characteristics, surgical approach and efficacy, postoperative complications, and 2-, 3-, and 5-year postoperative survival rates were calculated for all patients. Statistical analysis was performed using spss22 Kaplan Meier survival analysis and Cox regression analysis were performed. RESULTS: Among the 70 patients, the overall 2-year survival rate was 93.4%, the 3-year survival rate was 90.8%, and the 5-year survival rate was 80.3%. Multivariate analysis showed that TNM stage and age at onset were independent prognostic factors for NPC outcome. CONCLUSION: Depending on the size and location of the tumor, endoscopic surgery, open surgery, and combined open surgery with nasoendoscopy may be considered for recurrent and radiotherapy insensitive NPC. LEVEL OF EVIDENCE: Level 4.

A Case of Nasopharyngeal Pleomorphic Lipoma.

We describe the presentation and treatment of the first reported case of a nasopharyngeal pleomorphic lipoma. The mass was successfully treated with a combined endoscopic trans-oral surgical excision approach by using low temperature-controlled plasma technology, resulting in optimal patient outcomes. Laryngoscope, 134:2710-2712, 2024.

Preliminary evidence for endoscopic surgery combined with postoperative anti-PD-1 immunotherapy in advanced recurrent nasopharyngeal carcinoma.

BACKGROUD: Endoscopic surgery can be used as the main treatment for advanced recurrent nasopharyngeal carcinoma (rNPC). However, there is a huge clinical controversy about the need for consolidated immunotherapy after surgery. METHODS: We performed a retrospective propensity score-matched analysis (1:2) of patients with locally advanced rNPC who underwent endoscopic nasopharyngectomy (ENPG) combined with anti-programmed cell death protein-1 (PD-1) monotherapy or ENPG alone. The survival rate was analyzed by Kaplan-Meier method. The primary endpoint was progression-free survival (PFS). The secondary endpoints included overall survival (OS), objective response rate (ORR) and disease control rate (DCR). Potential surgical-related complications and immune-related adverse events (AEs) were also assessed. RESULTS: We recruited 10 patients receiving ENPG plus anti-PD-1 monotherapy and 20 receiving ENPG alone. During the mean follow-up of 23.8 months, a significant improvement in the 2-year PFS was detected in the consolidation immunotherapy group compared to the ENPG alone group (80.0% vs. 40.0%; HR = 0.258; 95% CI: 0.09-0.72; p = 0.04), while the 2-year OS in the consolidation immunotherapy group was not significantly longer than that in the ENPG alone group (90.0% vs. 75.0%; HR = 0.482; 95% CI: 0.08-3.00; p = 0.50). The incidence of surgical-related complications in the consolidation immunotherapy group and ENPG alone group was 70.0 and 60.0%, respectively. Immune-related AEs were similar between the toripalimab arm (75.0%) and the camrelizumab arm (66.7%). Surgical-related complications depend on symptomatic treatments. Immune-related AEs were mild and tolerable. CONCLUSIONS: Consolidation immunotherapy regimen for patients with advanced rNPC after ENPG compared to ENPG alone provides a superior PFS rate with a manageable safety profile.

Early-onset juvenile nasopharyngeal angiofibroma (JNA): a systematic review.

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

[Association between vascularization and its impact for recurrence of juvenile nasoangiofibroma]. / Asociación entre vascularización y su impacto en la recurrencia de nasoangiofibroma juvenil.

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

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

[Clinical study of endoscopic surgery for recurrent nasopharyngeal carcinoma].

Objective:To compare the clinical effects and complications of surgery + chemotherapy and radiotherapy + chemotherapy in patients with nasopharyngeal carcinoma recurrence, so as to compare the safety and efficacy of two different therapeutic methods. Methods:A retrospective analysis was performed on 40 patients with recurrent nasopharyngeal carcinoma after radiotherapy and chemotherapy admitted to our hospital from January 2016 to June 2020. Among them, 26 patients were treated with surgery. The recurrent tumor was removed under nasal endoscope, and the frozen resection margin was negative during the operation. Chemotherapy was continued for stage Ⅲ and Ⅳ patients from 3 to 5 weeks after surgery. Fourteen patients received secondary radiotherapy and chemotherapy. Postoperative complications and survival rate were observed. Results:There were 14 patients in the secondary chemoradiotherapy group（control group） and 26 patients in the nasal endoscopic surgery group（observation group）. Among the 26 patients, 19 patients underwent nasal septal mucosal repair, 5 patients underwent temporal muscle flap repair, 2 patients underwent submental flap repair, 2 patients had nasal septal mucosal flap necrosis and cerebrospinal fluid leakage, and the temporal muscle flap was used for secondary repair in the second stage operation, and 8 patients needed cervical lymph node dissection. The patients recovered well after surgery, and the patients in stage Ⅲ and Ⅳ were treated with chemotherapy after 3 weeks to 5 weeks according to the patient's wound condition. There were significant differences in the incidence of complications and 1-, 2-, and 3-year survival rates between the two groups（P<0.05）. Conclusion:Patients with recurrent nasopharyngeal carcinoma can be treated by nasal endoscopic surgery to remove the tumor, and the use of pedicled nasal septal mucosal flap or temporal muscle flap for skull base reconstruction, The operation can effectively prevent major complications such as internal carotid artery rupture and hemorrhage, and improve the survival rate and quality of life of patients. It provides a safe and effective treatment for patients with recurrent nasopharyngeal carcinoma.

Extended Endonasal Endoscopic (EEE) Surgery with Almost No Use of Adjuvant Radiotherapy for Juvenile Nasopharyngeal Angiofibroma (JNA).

Background and Objectives: Juvenile nasopharyngeal angiofibroma (JNA) is an angiomatous hamartoma of the nasal cavity. It is a benign but locally aggressive vascular tumor of the nasopharynx affecting adolescent males. Many surgical procedures are in practice, but the extended endonasal endoscopic (EEE) approach for JNAs is a suitable and effective technique. Materials and Methods: Fifteen adolescent patients having JNA who underwent extended endonasal endoscopic (EEE) surgery from January 2010 to January 2022 were studied retrospectively. Patients having residual and recurrent JNAs and those who underwent surgery other than EEE were excluded. Results: The average age of the patients was 18.3 years of age. A total of six patients (40%) each had stage V and IV while three patients (20%) had stage III JNAs. Gross total removal was achieved in eight (53.3%) patients and seven (43.7%) had partial removal. There was no per or postoperative mortality. All the patients had at least 3 years of postoperative follow-up and during follow-ups, seven patients were found to have residual tumors, and two had recurrences. Discussion: During the last decades, the endoscopic approach for the resection of JNAs has gained increasing popularity due to its obvious advantages over transfacial approaches. The magnified and angled field of view "behind the corner" helping in a more complete inspection for the resection and shorter hospitalization time makes it a better choice than the other approaches. Conclusions: Endoscopy is an excellent approach for primary JNA. It allows well visualization and precise removal of the angiofibroma. An endoscopic multiangle, multicorridor skull base approach including Denker's anteromedial maxillotomy is suitable and preferable for the resection of extensive JNAs.

Combined endoscopic endonasal and sublabial transmaxillary approaches for resection of intracranially extended juvenile nasopharyngeal angiofibroma.

BACKGROUND: Juvenile nasopharyngeal angiofibromas (JNAs) are rare, benign, and locally invasive nasopharyngeal tumors. Endoscopic endonasal resection is effective, non-invasive, with low complication rates. Until recently, endoscopic resection was not suitable for intracranially invasive tumors. PURPOSE/METHOD: We describe resection steps of an intracranially extending JNA with a combined endoscopic endonasal and endoscopic-assisted sublabial transmaxillary approaches. Indications, advantages, and approach-specific complications are also discussed. The main surgical steps are shown in an operative video. CONCLUSION: Surgical excision of JNAs by a combined endoscopic endonasal and sublabial transmaxillary approaches represents a safe and effective treatment for selected intracranially invasive JNA.

Anatomic Considerations Guiding Single Versus Multiportal Endoscopic Approaches for Resection of Juvenile Nasopharyngeal Angiofibroma: Cases Series With Graded Multicorridor Resections.

BACKGROUND: Juvenile nasopharyngeal angiofibromas (JNAs) are characterized by expansive and destructive growth, often invading the midline/paranasal sinuses, pterygopalatine fossa, and infratemporal fossa and can extend into the orbit, cavernous sinus, or intracranially. OBJECTIVE: To evaluete the major benefits of the extended endoscopic endonasal approach (EEA) for JNA resection as compared with more traditional and invasive transpalatal and transfacial approaches. When JNAs extend into lateral anatomic compartments, the optimal operative trajectory often requires additional approach strategies or surgical staging. METHODS: We retrospectively reviewed 8 cases of large JNAs arising in symptomatic adolescent boys (University of Pittsburgh Medical Center Stages II, III, and V) and discuss anatomic and tumor considerations guiding the decision of a pure EEA vs combined EEA and sublabial transmaxillary approach (Caldwell-Luc). RESULTS: A pure extended EEA was used in 6 JNA cases (UPMC Stages II-III); a multiportal EEA + Caldwell-Luc maxillotomy was used in 2 cases. One of the 2 patients (UPMC Stage V) previously treated with multiportal EEA + Caldwell-Luc maxillotomy underwent staged left temporal/transzygomatic craniotomy, obtaining gross total resection. Seven patients ultimately underwent complete removal without recurrence. One patient with a small residual JNA (UPMC II) underwent stereotactic radiosurgery without progression to date. CONCLUSION: JNAs with lateral extension into the infratemporal fossa often benefited from additional lateral exposure using a Caldwell-Luc maxillotomy. Cases with significant skull base and/or dural involvement may undergo staged surgical treatment; temporalis + transzygomatic craniotomy is often useful for second-stage approaches for residual tumor in these lateral infratemporal or intracranial regions. SRS should be considered for residual tumor if additional surgery is not warranted.

Deconstructive versus reconstructive approach to internal carotid artery blowout in radiated nasopharyngeal carcinoma: A tertiary center experience and systematic review.

The objective of this study was to compare the outcomes of parent artery occlusion (PAO) versus stent-assisted reconstruction in radiated nasopharyngeal carcinoma (NPC) patients with internal carotid artery (ICA) blowouts. A retrospective review from our institution (2011-2021) and systematic review of Pubmed and Embase (1995-2022) was performed. Twenty-eight eligible studies were identified. Eighty-six PAOs and 37 stent-assisted reconstructions were analyzed, including 11 PAOs and 5 stents from our institution. Stents were associated with significantly higher incidence of overall re-bleeding (16.2% [95% CI 7.4-31.9] vs. 4.6% [95% CI 1.3-13.5], p = 0.047), delayed stroke (5.4% [95% CI 1.3-19.4] vs. 0%, p = 0.034) and reduced median survival (7.1 [95% CI 3.8-14.0] months vs. 29.0 [95% CI 9.4-63.4] months, p = 0.017) compared to PAO. There were no significant differences in terms of overall stroke, infection, extruded/migrated foreign body, and peri-procedure death. PAO is preferred over reconstructive treatment in patients with adequate collateral circulation.