Efficacy of endoscopic surveillance for pharyngeal mucosa during endoscopic resection for pharyngeal carcinoma: a multicenter prospective study.

INTRODUCTION: Since patients with pharyngeal squamous cell carcinoma (SCC) often have multiple pharyngeal lesions, evaluation of pharyngeal lesions before endoscopic resection (ER) is important. However, detailed endoscopic observation of the entire pharyngeal mucosa under conscious sedation is difficult. We examined the usefulness of endoscopic surveillance with narrow band imaging (NBI) and lugol staining for detection of pharyngeal sublesions during ER for pharyngeal SCC under general anesthesia (endoscopic surveillance during treatment; ESDT). METHODS: From January 2021 through June 2022, we examined 78 patients who were diagnosed with superficial pharyngeal SCC and underwent ER. They underwent the ESDT and for patients who were diagnosed with new lesions of pharyngeal SCC or high-grade dysplasia (HGD) that were not detected in the endoscopic examination before treatment, ER were performed simultaneously for new lesions and the main lesions. The primary endpoint of this study was the detection rate of new lesions of pharyngeal SCC or HGD in the ESDT. RESULTS: Fifteen of the 78 patients were diagnosed as having undetected new pharyngeal lesions in the ESDT and 10 (12.8%) (95% CI 6.9-22.2%) were histopathologically confirmed to have new lesions of pharyngeal SCC or HGD. Among the 13 lesions of SCC or HGD, 8 were found by NBI observation; however, 5 were undetectable using NBI but detectable by lugol staining. All of the 13 lesions had endoscopic findings of pink color sign on lugol staining. CONCLUSIONS: Endoscopic surveillance for pharyngeal sublesions during ER for pharyngeal SCC is feasible and useful.

Transoral Approach to Parapharyngeal Space Tumours: Preliminary Reports from a Single-Centre Retrospective Analysis.

PURPOSE: The aim of this study is to identify certain parapharyngeal space tumours with specific characteristics that can be treated successfully through an endoscopically assisted transoral approach (EATA). METHODS: Nine patients with PPS tumours underwent surgery through an EATA between 2003 and 2021. All patients underwent clinical examination and fibrolaryngoscopy. Preoperative CT and/or MRI was performed on all patients. RESULTS: All the patients were successfully treated through an endoscopically assisted transoral approach. Histological examination revealed five pleomorphic adenomas, two schwannomas, one ectopic thyroid gland and one lipoma. The only long-term sequelae observed was Horner syndrome in the two schwannomas arising from the carotid space. The mean hospitalisation time was 2.6 days, while the mean follow-up time was of 9.7 years. CONCLUSIONS: An endoscopically assisted transoral approach (EATA) is a valid technique for treating benign capsulated tumours of the true PPS and some benign capsulated tumours of the superomedial aspect of the carotid space.

Determining Invasion Depth in Superficial Pharyngeal Carcinoma by Transoral Ultrasonography.

OBJECTIVES: To examine the clinical usefulness of transoral ultrasonography (US) in determining the invasion depth of superficial pharyngeal carcinoma (SPC). Determining the invasion depth of SPC is crucial for transoral surgery including determining treatment strategy. This study aimed to examine the usefulness of transoral US in determining the invasion depth of SPC. METHODS: Forty-six patients with 51 lesions who underwent both magnifying endoscopy with narrow-band imaging (ME-NBI) and transoral US were included. The primary outcomes were the sensitivity, specificity, positive (PPV), and negative predictive values (NPV) of ME-NBI and transoral US findings for pathological tumor depth in SPCs. RESULTS: The accuracy (82.4%), sensitivity (85.2%), PPV (82.1%), and NPV (82.6%) rates of US for subepithelial propria (SEP) were higher than those of ME-NBI and macroscopic classification, indicating that transoral US is superior to ME-NBI in determining the invasion depth. All cases where the SEP was clearly invaded (SEP deep) could be diagnosed as SEP by transoral US. CONCLUSIONS: Transoral US may be useful in determining the invasion depth of SPCs. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:2192-2197, 2023.

Detection Ability of 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography for Clinical T Classification of Synchronous Esophageal Cancer in Pharyngeal Cancer.

BACKGROUND/AIM: The utility of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) for clinical T classification of synchronous esophageal cancer (SEC) in pharyngeal cancer has not been fully elucidated. We investigated the association between 18F-FDG-PET/CT and clinical T classification for SEC. PATIENTS AND METHODS: We retrospectively enrolled 90 patients with pharyngeal cancer having both Lugol chromoendoscopy and 18F-FDG-PET/CT at pretreatment. The association between 18F-FDG-PET/CT and pathological examination was compared by the Fisher's exact test. RESULTS: Pathologically, 12 patients with SEC were diagnosed. The number of locations for SEC with cT1a, cT1b, and cT3 was 20, 1, and 1, respectively. Sensitivity for cT1a-cT3, cT1b-cT3, and cT3 was 9.1%, 100%, and 100%, respectively. Locations of SEC with cT1b-cT3 were significantly detected. The locations of SEC with cT1b-cT3 were more frequently detected than those without cT1b-cT3 by 18F-FDG-PET/CT (p<0.01). CONCLUSION: Detection of SEC with clinical T1b-T3 in comparison to clinical T1a on pretreatment 18F-FDG-PET/CT for patients with pharyngeal cancer, was achieved with high sensitivity.

Surgical Treatment of Parapharyngeal Space Salivary Gland Tumor.

INTRODUCTION: Parapharyngeal space salivary gland tumors are very rare. The authors sought to examine the clinical features, treatment methods, and treatment results of parapharyngeal space salivary gland tumors in our hospital. METHODS: The authors retrospectively enrolled 15 patients who were finally diagnosed as having parapharyngeal space salivary gland tumors from January 2010 to January 2021. RESULTS: All parapharyngeal space salivary gland tumors arose from the prestyloid compartment. This study included 3 males and 12 females. The main symptoms were incidental diagnosis during imaging tests, followed by neck discomfort, oral mass, neck mass, and headache. Surgical methods for parapharyngeal space salivary gland tumors were performed in the following order: transcervical approach (n = 10), transcervical-parotid approach (n = 3), transoral approach (n = 1), and transparotid approach (n = 1). Pleomorphic adenoma was the most common tumor among parapharyngeal space salivary gland tumors. CONCLUSIONS: In the surgery of parapharyngeal space salivary gland tumors, a transcervical or transcervical-parotid approach was mainly used by predicting the origin site through radiologic examinations.

Trans-Oral Robotic Surgery: 14 Cases of Pleomorphic Adenoma of the Parapharyngeal Space.

PURPOSE: The aim of this study has been to describe our experience with pleomorphic adenomas of the parapharyngeal space (PPS) treated with trans-oral robotic surgery (TORS). Tumors arising from the PPS comprise less than 0.5% of all head and neck tumors. Salivary gland tumors account for 40% to 50% of PPS lesions with pleomorphic adenomas representing the most common salivary tumors (80%-90%). Parapharyngeal space tumors cause nonspecific symptoms and may be difficult to diagnose. METHODS: In our study a preoperative diagnosis was conducted by fine needle aspiration biopsy and magnetic resonance imaging and the results were used to plan the correct surgical approach. RESULTS: In all cases we were able to employ TORS, a minimally invasive procedure that allows us to operate in narrow and anatomically complex spaces that we can only reach thanks to the use of well-articulated hand pieces. CONCLUSIONS: This report indicates that TORS is a safe surgical procedure for the excision of benign tumors of the PPS in selected cases.

Management of parapharyngeal space cavernous sinus haemangioma using transoral robotic surgery.

Cavernous haemangiomas are a very rare occurrence in the parapharyngeal space (PPS). Here, we present a case of a 58-year-old woman with an incidentally identified left PPS mass thought to be a pleomorphic adenoma that underwent excision by transoral robotic surgery (TORS). Intraoperative findings demonstrated no solid mass present and histological assessment of resected tissue confirmed a cavernous haemangioma. We discuss our experience in the management of a radiological and surgical mismatch of a PPS mass in what we believe to be the first cavernous haemangioma to be excised by TORS in this region.

Validation of a classification and scoring system for the diagnosis of laryngeal and pharyngeal squamous cell carcinomas by confocal laser endomicroscopy.

INTRODUCTION: Confocal laser endomicroscopy is an optical imaging technique that allows in vivo, real-time, microscope-like images of the upper aerodigestive tract's mucosa. The assessment of morphological tissue characteristics for the correct differentiation between healthy and malignant suspected mucosa requires strict evaluation criteria. OBJECTIVE: This study aims to validate an eight-point score for the correct assessment of malignancy. METHODS: We performed confocal laser endomicroscopy between March and October 2020 in 13 patients. 197 sequences (11.820 images) originated from the marginal area of pharyngeal and laryngeal carcinomas. Specimens were taken at corresponding locations and analyzed in H&E staining as a standard of reference. A total of six examiners evaluated the sequences based on a scoring system; they were blinded to the histopathological examination. The primary endpoints are sensitivity, specificity, and accuracy. Secondary endpoints are interrater reliability and receiver operator characteristics. RESULTS: Healthy mucosa showed epithelium with uniform size and shape with distinct cytoplasmic membranes and regular vessel architecture. Confocal laser endomicroscopy of malignant cells demonstrated a disorganized arrangement of variable cellular morphology. We calculated an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 83.2%, 81.3%, 85.5%, 86.7%, and 79.7%, respectively, with a κ-value of 0.64, and an area under the curve of 0.86. CONCLUSION: The results confirm that this scoring system is applicable in the laryngeal and pharyngeal mucosa to classify benign and malignant tissue. A scoring system based on defined and reproducible characteristics can help translate this experimental method to broad clinical practice in head and neck diagnosis.

Efficacy of endoscopic ultrasonography in evaluating tumor thickness in patients with superficial pharyngeal carcinoma: A pilot study.

OBJECTIVES: A histopathological tumor thickness of ≥1000 µm has been reported as one of many risk factors for recurrent lymph node metastasis in superficial pharyngeal cancer (SPC). However, methods for assessing this risk factor preoperatively have not yet been established. Hence, the current study aimed to evaluate the efficacy of endoscopic ultrasonography (EUS) in measuring tumor thickness preoperatively in patients with SPC. METHODS: This single-center retrospective study included 44 consecutive patients with 47 lesions who underwent endoscopic submucosal dissection (ESD). Prior to surgery, EUS examination was performed while under general anesthesia. Further, microvascular irregularity in the target lesion was evaluated using the Japan Esophageal Society (JES) magnification endoscopic classification system. RESULTS: A significant correlation was noted between histopathological and EUS tumor thickness (Spearman's correlation r == 0.879, p < 0.001). In tumors ≥1000 µm thick on histopathology, the cutoff value for EUS tumor thickness was 2.6 mm, and the following values were obtained: sensitivity, 100%; specificity, 81.8%; positive predictive value (PPV), 70%; negative predictive value (NPV), 100%; and accuracy, 87.2%. In B2 lesions ≥1000-µm thick, the following values were obtained: sensitivity, 85.7%; specificity, 90.9%; PPV, 80%; NPV, 93.8%; and accuracy, 89.4%. The diagnostic accuracy rate of combined EUS and the JES magnifying endoscopic classification system was 95.7%. CONCLUSIONS: Tumor thickness assessed using EUS was effective in diagnosing histopathological tumor thickness of ≥1000 µm. The combined use of EUS and the JES magnifying endoscopic classification system may be useful for assessing preoperative risk factors for lymph node metastasis in SPC.

Primary pharyngeal synovial sarcoma in a pediatric patient: A case report.

RATIONALE: Synovial sarcoma is a rare malignant tumor that typically originates from the soft tissue of the extremities. The occurrence of primary pharyngeal synovial sarcoma is even rarer, and few studies have reported its radiological features. Here, we report a case of pediatric primary pharyngeal synovial sarcoma and describe the conventional and advanced magnetic resonance imaging (MRI) findings with pathologic correlation. PATIENT CONCERNS: An 11-year-old girl presented to the otolaryngologic clinic with dysphagia. DIAGNOSIS: Laryngoscopy revealed a large mass in the oropharynx. MRI revealed a well-defined soft tissue mass with a maximal diameter of approximately 5 cm originating from the submucosal space of the oropharynx. The mass was primarily solid and showed homogeneous contrast-enhancement. The mass was hypointense on T1-weighted images and hyperintense on T2-weighted images. The mass showed a homogeneously low apparent diffusion coefficient value on diffusion-weighted imaging, which indicated high tumor cellularity. Dynamic contrast-enhanced MRI revealed a hypovascular tumor with low values of the volume transfer constant between the extracellular extravascular space and blood plasma and blood plasma volume per unit tissue volume. Amide proton transfer-weighted MRI revealed a relatively high amide proton transfer signal in the tumor, indicating a high protein/peptide component. The patient underwent partial surgical resection of the tumor, and the diagnosis of biphasic synovial sarcoma was confirmed on postoperative pathological examination. INTERVENTION: The patient was started on chemotherapy with vincristine, ifosfamide, doxorubicin, and etoposide. OUTCOMES: The tumor did not respond to the 3 cycles of the chemotherapy. Thus, the patient underwent second surgery and subsequent radiation therapy. The patient is now under ifosfamide/carboplatin/etoposide chemotherapy. LESSON: Synovial sarcoma should be considered in the differential diagnosis of pediatric oropharyngeal submucosal tumors. Multimodal MRI may aid diagnosis, although the final diagnosis should be based on the postoperative pathological examination findings.

Diagnostic Value of Narrow Band Imaging in Visualization of Pathological Lesions in Larynx and Hypopharynx.

INTRODUCTION: Narrow Band Imaging (NBI) is an endoscopic optical imaging enhancement technology that improves the contrast of mucosal surface texture and enhances visualization of mucosal and submucosal vasculature. Due to its properties, it can visualize suspected malignant or precancerous lesions earlier than conventional white light endoscopy. The aim of this study was to analyze the benefit of NBI in visualization of precancerous and malignant lesions in preoperative and intraoperative diagnostics and correlation with histopathologic results. METHODS: A total of 589 patients with suspicious laryngeal or hypopharyngeal lesion were investigated using conventional white light endoscopy (WLE) and NBI endoscopy with high-definition TV (HDTV NBI) from 10/2013 to 12/2019. Patients were divided into two groups based on pre-operative NBI examination (group A, 345 patients) and intraoperative NBI examination (group B, 244 patients). All suspicious lesions were graded to 5 types of Ni classification and correlated with histopathologic results. The SPSS version 8.0.4 statistical software package was used for statistical analysis. In diagnosing premalignant and malignant lesions sensitivity, specificity, positive predictive value, and negative predictive value were calculated. RESULTS: The agreement between NBI endoscopy and histopathological analysis in group A was statistically significant (Қ = 0.76, p < 0.001), with a sensitivity of 86.2% (95% IS: 65.4-95.2) and specificity of 90.9% (95% IS: 70.6-94.1). Moreover, in group B was proven almost perfect agreement between NBI and histopathological analysis (Қ = 0.8461, p < 0.001), with a sensitivity of 84.0% (95% IS: 60.2-92.4) and specificity of 96.0% (95% IS: 87.0-99.2). CONCLUSIONS: Based on our results, NBI using the Ni classification has great potential in improving diagnosis of precancerous and malignant lesions and correlates strongly with histopathologic results. It serves as a useful adjunct to white light endoscopy in the diagnosis of laryngeal and hypopharyngeal lesions, especially using HDTV NBI.

Evaluating Laryngopharyngeal Tumor Extension Using Narrow Band Imaging Versus Conventional White Light Imaging.

OBJECTIVE/HYPOTHESIS: Comparing detection and extension of malignant tumors by flexible laryngoscopy in the outpatient setting with laryngoscopy under general anesthesia using both White Light Imaging (WLI) and Narrow Band Imaging (NBI). STUDY DESIGN: Prospective study. METHODS: Two hundred and thirty-three patients with laryngeal and pharyngeal lesions underwent flexible and rigid laryngoscopy, with both WLI and NBI. Extension of malignant lesions (n = 132) was compared between both techniques in detail. RESULTS: Sensitivity of NBI during flexible endoscopy (92%), was comparable with that of WLI during rigid endoscopy (91%). The correlation of tumor extension between flexible and rigid laryngoscopy was high (rs  = 0.852-0.893). The observed tumor extension was significantly larger when using NBI in both settings. The use of NBI during flexible laryngoscopy leads to upstaging (12%) and downstaging (2%) of the T classification. CONCLUSIONS: NBI during flexible laryngoscopy could be an alternative to WLI rigid endoscopy. NBI improves visualization of tumor extension and accuracy of T staging. LEVEL OF EVIDENCE: 3 Laryngoscope, 131:E2222-E2231, 2021.

Diagnosis of pharyngeal cancer on endoscopic video images by Mask region-based convolutional neural network.

OBJECTIVES: We aimed to develop an artificial intelligence (AI) system for the real-time diagnosis of pharyngeal cancers. METHODS: Endoscopic video images and still images of pharyngeal cancer treated in our facility were collected. A total of 4559 images of pathologically proven pharyngeal cancer (1243 using white light imaging and 3316 using narrow-band imaging/blue laser imaging) from 276 patients were used as a training dataset. The AI system used a convolutional neural network (CNN) model typical of the type used to analyze visual imagery. Supervised learning was used to train the CNN. The AI system was evaluated using an independent validation dataset of 25 video images of pharyngeal cancer and 36 video images of normal pharynx taken at our hospital. RESULTS: The AI system diagnosed 23/25 (92%) pharyngeal cancers as cancers and 17/36 (47%) non-cancers as non-cancers. The transaction speed of the AI system was 0.03 s per image, which meets the required speed for real-time diagnosis. The sensitivity, specificity, and accuracy for the detection of cancer were 92%, 47%, and 66% respectively. CONCLUSIONS: Our single-institution study showed that our AI system for diagnosing cancers of the pharyngeal region had promising performance with high sensitivity and acceptable specificity. Further training and improvement of the system are required with a larger dataset including multiple centers.

External validation of nodal failure prediction models including radiomics in head and neck cancer.

PURPOSE: To externally validate the previously published pre-treatment prediction models for lymph nodes failure after definitive radiotherapy in head and neck squamous cell carcinoma (HNSCC) patients. MATERIALS AND METHODS: This external validation cohort consisted of 143 node positive HNSCC patients treated between July 2007 and June 2016 by curative radiotherapy with or without either cisplatin or cetuximab. Imaging and pathology reports during follow-up were analyzed to indicate persisting or recurring nodes. The previously established clinical, radiomic and combined models were validated on this cohort by assessing the concordance index (c-index) and model calibration. RESULTS: Overall 113 patients with 374 pLNs were suitable for final analysis. There were 20 (5.3%) nodal failures from 15 patients after a median follow-up of 36.1 months. Baseline characteristics and radiomic features were comparable to the training cohort. Both the radiomic model (Least-axis-length of lymph node (LALLN) and correlation of gray level co-occurrence matrix (Corre-GLCM)) and the combined model (T stage, gender, WHO performance score, LALLN and Corre-GLCM) showed good agreement between predicted and observed nodal control probabilities. The radiomic (c-index: 0.71; 95% confidence interval (CI): 0.59-0.84) and combined (c-index: 0.71; 95% CI: 0.59-0.82) models performed better than the clinical model (c-index: 0.57; 95% CI: 0.47-0.68) on this cohort, with a significant difference between the combined and clinical models (z-score test: p = 0.005). CONCLUSION: The combined model including clinical and radiomic features was externally validated and proved useful to predict nodal failures and could be helpful to guide treatment choices before and after curative radiation treatment for node positive HNSCC patients.

Analysis of acute-phase toxicities of intensity-modulated proton therapy using a model-based approach in pharyngeal cancer patients.

Pharyngeal cancer patients treated with intensity-modulated proton therapy (IMPT) using a model-based approach were retrospectively reviewed, and acute toxicities were analyzed. From June 2016 to March 2019, 15 pharyngeal (7 naso-, 5 oro- and 3 hypo-pharyngeal) cancer patients received IMPT with robust optimization. Simulation plans for IMPT and intensity-modulated X-ray therapy (IMXT) were generated before treatment. We also reviewed 127 pharyngeal cancer patients with IMXT in the same treatment period. In the simulation planning comparison, all of the normal-tissue complication probability values for dysphagia, dysgeusia, tube-feeding dependence and xerostomia were lower for IMPT than for IMXT in the 15 patients. After completing IMPT, 13 patients completed the evaluation, and 12 of these patients had a complete response. The proportions of patients who experienced grade 2 or worse acute toxicities in the IMPT and IMXT cohorts were 21.4 and 56.5% for dysphagia (P < 0.05), 46.7 and 76.3% for dysgeusia (P < 0.05), 73.3 and 62.8% for xerostomia (P = 0.43), 73.3 and 90.6% for mucositis (P = 0.08) and 66.7 and 76.4% for dermatitis (P = 0.42), respectively. Multivariate analysis revealed that IMPT was independently associated with a lower rate of grade 2 or worse dysphagia and dysgeusia. After propensity score matching, 12 pairs of IMPT and IMXT patients were selected. Dysphagia was also statistically lower in IMPT than in IMXT (P < 0.05). IMPT using a model-based approach may have clinical benefits for acute dysphagia.

Three unusual parapharyngeal space masses resected via the endoscopy-assisted transoral approach: case series and literature review.

Tumors of the parapharyngeal space (PPS) are rare, most originate from salivary and neurogenic tissues, and most are benign. However, there are some rarer masses in the PPS, with just a few published reports in the literature worldwide, and we may not consider them in the differential diagnosis of PPS neoplasms. We report three cases of rare masses in the PPS: Warthin's tumor, branchial cleft cyst, and carcinoma ex pleomorphic adenoma. The three patients were admitted to our department with complaints of painless swelling in the lower side of the right face or a long history of snoring; diagnoses were confirmed histopathologically. An endoscopy-assisted transoral approach was used that allowed wide visibility for safe resection and resulted in a short hospitalization time and good functional and cosmetic outcomes. All patients have been followed to the current time, and there have been no recurrences. The transoral endoscopy-assisted approach appears to be safe, effective, and less invasive for excision of masses in the PPS.

Adenoma pleomórfico del espacio parafaríngeo: una entidad escondida / Pleomorphic adenoma of the parapharyngeal space: a hidden entity

Resumen Los tumores del espacio parafaríngeo son poco frecuentes. Dentro de ellos, las neoplasias benignas son las más frecuentes, destacando principalmente los adenomas pleomorfos. Estos tumores suelen permanecer asintomáticos hasta alcanzar el tamaño suficiente para producir alteraciones debido al efecto de masa. A pesar de su naturaleza benigna, es necesario realizar resección completa dada su alta tasa de recurrencia y potencial de transformación maligna. Presentamos el caso de un paciente de 52 años que en estudio de vértigo se detecta incidentalmente una masa tumoral del espacio parafaríngeo de 45 mm × 32 mm de diámetro, de etiología incierta. Se realizó una biopsia incisional endoscópica que evidenció un adenoma pleomórfico, que actualmente se encuentra en etapa de evaluación y planificación de escisión completa. En conclusión, dado que los hallazgos clínicos y radiológicos no siempre son suficientes para determinar la naturaleza de las lesiones del espacio parafaríngeo, es importante contar con un diagnóstico histológico. Los avances en las técnicas de cirugía endoscópica sinusal han permitido acceder a esta zona con una mínima morbilidad y estadía hospitalaria. Debido a la posibilidad de transformación maligna los adenomas pleomórficos requieren un manejo activo.

Abstract Tumors of the parapharyngeal space are rare. Benign neoplasms are the most frequent and, among them, pleomorphic adenomas stand out. These tumors are usually asymptomatic until they reach a size large enough to produce symptoms due to mass effect. Despite its benign nature, it is necessary to perform a complete resection given its high recurrence rate and its potential for malignant transformation. We present the case of a 52-year-old man in whom a study of vertigo incidentally detected a mass in the parapharyngeal space measuring 45 mm × 32 mm in diameter, of unknown etiology. An endoscopic incisional biopsy was performed, showing a pleomorphic adenoma, which is currently under assessment to plan complete removal. In conclusion, as clinical and radiological findings are not always sufficient to determine the nature of parapharyngeal space lesions, it is important to obtain a histological diagnosis. Advances in endoscopic sinus surgery techniques have allowed access to this area with minimal morbidity and hospital stay. Due to the possibility of malignant transformation pleomorphic adenomas require active management.

Malignant peripheral nerve sheath tumor of the parapharyngeal space arising from cervical sympathetic chain: A rare entity.

Malignant peripheral nerve sheath tumors (MPNSTs) of parapharyngeal space are rare and if present are most often in association with neurofibromatosis type 1 (NF-1). Only a few cases of MPNST have been reported in the literature without coexisting NF. We report one such case of an MPNST of parapharyngeal space tumor in a 35-year-old female with no associated features of NF-1. She presented with right-sided neck swelling and ptosis. Magnetic resonance imaging showed a 7 cm × 8 cm × 11 cm irregular swelling in the right parapharyngeal space with invasion of surrounding muscles. The mass was excised using a transcervical approach. Postoperative histopathological examination of the specimen revealed MPNST possibly arising from the cervical sympathetic chain.