Imaging of Malignant Minor Salivary Gland Tumors of the Head and Neck.

Minor salivary gland carcinomas (MSGCs) are nonminor tumors in the head and neck region and account for about half of all salivary gland carcinomas. Imaging evaluation based on the anatomy of minor salivary and mucous glands in the head and neck region as well as invasion patterns in each site helps in identifying optimal treatment modalities and planning suitable treatment strategies. MSGCs can be divided radiologically into localized and invasive subtypes on the basis of the clinical utility of such categorization. Characteristic invasion patterns of the invasive type include deep submucosal extension, bone marrow infiltration, and perineural spread, which are difficult to assess clinically. MSGCs easily invade adjacent structures because of their submucosal location and may spread along the muscles, nerves, periosteum, and dura mater. Moreover, the tumor may spread into the bone marrow without obvious bone destruction. In addition to imaging classification, examining the anatomy and distribution of the minor salivary glands, including the palatal, lingual, buccal, labial, and retromolar glands, as well as other mucous glands, including the glands of the sinonasal cavity and nasopharynx, lacrimal glands, ceruminous glands, laryngeal glands, and tracheal glands, facilitates MSGC diagnosis and tumor extension assessment. The authors review the precise anatomy of the minor salivary and mucous glands in the head and neck region, discuss the MSGC imaging classifications, and describe how to evaluate the extent of MSGCs in each site on the basis of the imaging classification and invasion patterns. ©RSNA, 2020.

Ultra-high frequency ultrasonography (UHFUS)-guided minor salivary gland biopsy: A promising procedure to optimize labial salivary gland biopsy in Sjögren's syndrome.

BACKGROUND: Sjögren's syndrome (SS) is an autoimmune disease characterized by an inflammatory infiltrate of exocrine salivary and lachrymal glands. Diagnosis is complex, and minor salivary gland biopsy and subsequent focus score (FS) calculation appear of extreme importance in the diagnostic work-up of the disease. Ultra-high frequency ultrasonography (UHFUS) is a recently introduced diagnostic technique, which is gaining an increasingly important role in intraoral imaging. This study aims at exploring the usefulness of UHFUS for obtaining valuable labial salivary gland samples to assess the histopathological features of SS patients. METHODS: Patients with clinical suspect of SS and eligible for minor salivary gland biopsy were enrolled. UHFUS scan of the lower lip was performed. Glandular echostructure was classified according to Outcome Measures in Rheumatology (OMERACT) scoring system. The glands to be sampled were selected on the basis of UHFUS evaluation and biopsied. The areas of the samples were recorded and compared with those obtained without UHFUS guidance. The correlation between UHFUS grade and labial gland FS was also assessed. RESULTS: The areas of the samples obtained with UHFUS guidance were significantly higher (7.25 ± 3.98 mm2 ) than those obtained by conventional procedures (5.79 ± 3.49 mm2 , P = .02). UHFUS correlated significantly with the salivary gland FS (r = .532, P = .001). CONCLUSION: UHFUS seems a promising tool in SS diagnostic algorithm, being able to provide a valuable support to the biopsy procedure. Further studies are mandatory to confirm the role of UHFUS in SS.

Systematic Review of Clinical and Radiographic Signs of Pediatric Pleomorphic Adenoma of Minor Salivary Glands.

AIM AND OBJECTIVE: To examine the clinical signs, radiographical features, and demographics of pediatric pleomorphic adenoma (PA) in the minor salivary glands. MATERIALS AND METHODS: Several databases were searched for relevant studies. The included studies were assessed for methodological quality. Demographic, clinical, and radiographic data were collected. RESULTS: Sixteen of 3,121 articles met the inclusion criteria (17 lesions). The mean age was 9.7 ± 3.9 years and majority were females n = 10 (59%). It is commonly presented as asymptomatic swelling n = 16 (94.1%), in the hard palate 13 (76.5%). Radiographically, most were well-defined n = 15 (93.7%) and 8 (47%) caused erosion or displacement of surrounding tissues. CONCLUSION: The small size and asymptomatic nature of pediatric PA can render these lesions undiagnosed. On rare occurrences, PA can show calcifications, MRI, or CT enhancement. MRI is the best imaging modality to depict soft tissue content but not subtle erosion of adjacent bony structures. CLINICAL SIGNIFICANCE: The dentist can be the first to detect PA in the mouth of a child. Augmenting clinical examination with radiographic examination is paramount to ensure adequate diagnosis of PA, examine effects on surrounding bone, and maintain close follow-up as watchful waiting is not safe in this population.

Ultra-high frequency ultrasonography of labial glands is a highly sensitive tool for the diagnosis of Sjögren's syndrome: a preliminary study.

OBJECTIVES: Ultra-high frequency ultrasonography (UHFUS) has been recently introduced in oral medicine due to its ability to image small anatomical structures including labial salivary glands (LSG). To date no ultrasonography morphological studies of labial salivary glands (LSG) have been carried out in SS. In this pilot study we aimed at analysing the distribution of UHFUS findings in LSG of patients with suspected SS, focusing in particular on the association with patients' oral dysfunction, antibody profiles and histopathology. METHODS: Consecutive patients undergoing a LSG biopsy for clinically suspected SS were included in this study between January 2018 and January 2020. Intraoral UHFUS scan of the lip mucosa was performed with Vevo MD equipment, using a 70 MHz probe with a standardised protocol. LSG were assessed by using a four-grade semiquantitative scoring system (0-3), similar to the OMERACT scoring system used for major salivary glands. The distribution of UHFUS grades was compared in patients stratified according their final diagnosis, patients antibody profiles and LSG histopathology. RESULTS: We included 128 patients with suspected SS: out of them, 54 (42.2%) received a final diagnosis of SS, made according to the ACR 2016 criteria and 74 (57.8%) were diagnosed as no-SS sicca controls. We found that LSG inhomogeneity was significantly greater in patients with SS than in no-SS subjects (p<0.0001). We also found that higher UHFUS pattern of inhomogeneity (i.e. grade 2 and 3) were significantly more frequent in both SSA+/SSB- and SSA+/SSB+ patients (p=0.001). A normal UHFUS pattern, by contrast, was significantly more common in SSA-/SSB- subjects (i.e. 15/83 (18.1%) vs. 1/33 (3%) vs. 0/12 (0%), p=0.001). Finally, LSG inhomogeneity was significantly associated with both the number of foci (p<0.001) and focus score (p<0.001). Particularly, we found that both the number of foci and the FS were significantly higher in patients presenting a UHFUS grading of 2 and 3 with respect to those presenting a UHFUS grading of 0 and 1 (p=0.01). CONCLUSIONS: This preliminary study demonstrates the optimal feasibility of UHFUS and its high sensitivity in identifying negative patients on subsequent lip biopsy, thus avoiding invasive procedures in selected cases. Further studies are in progress to define the clinical and predictive role of the various patterns observed and their added value with respect to traditional salivary gland ultrasonography.

Image analysis of fatty infiltration in labial salivary gland biopsies: extent and its correlation to age, obesity and diabetes.

BACKGROUND: Fatty infiltration of minor salivary gland parenchyma is relatively frequent, but not extensively examined histopathological phenomenon in biopsy samples. Its extent and relation to several suspected background diseases are not well understood. METHODS: In this study, we examined the presence and extent of fatty infiltration on digitally scanned versions of the periodic acid/Schiff-stained minor salivary gland slides of 275 patients. As a result of the image analysis, fatty infiltration was expressed in per cent of the whole selected area. The presence and extent of this change were compared to age, diabetes mellitus and body mass index in various statistical analyses. RESULTS: Significantly higher age and body mass index values were found in the fatty infiltration positive than in the negative group. We also found that not only the number of fatty infiltration positive cases was increased significantly in the gradually worsened body mass index groups, but the extent of fatty infiltration also increased as the obesity worsened. Age also showed significant correlation with the extent of fatty infiltration. DISCUSSION: All of these findings support that the age (which seemed the only independent variable) shows strong correlation with the presence of the fatty infiltration but obesity may also play important role in the development and the extent of this change. Because of its frequency in elderly, at least partly, the fatty infiltration might be responsible for the xerostomia. We also think that presence of fatty infiltration should be mentioned in the histopathological report of salivary gland biopsies.

Minor Salivary Gland Acinic Cell Carcinoma With Orbital Extension.

An 80-year-old male presented with painless blurred vision from the right eye following endoscopic biopsy of a right maxillary sinus mass with the otolaryngology service. The patient admitted to progressive right-sided proptosis over the past few weeks. Past medical history was significant for acinic cell adenocarcinoma of buccal space origin with resection 3 years prior. Orbital CT revealed a large, poorly circumscribed destructive mass in the right masticator space with extension to the paranasal sinuses, inferior and medial orbit, and middle cranial fossa. Histopathology confirmed the mass to represent a recurrent acinic cell carcinoma. The patient was treated with chemotherapy, and at last follow-up 2 years after presentation had stable painless globe dystopia and visual acuity. The authors describe a very rare case of a recurrent minor salivary gland acinic cell carcinoma with secondary orbital extension.

The Application of Absolute Ethanol in the Treatment of Mucocele of the Glands of Blandin-Nuhn.

Mucocele of the anterior lingual salivary glands is a more common cystic lesion, especially in patients aged less than 20 years. The study is aimed to observe the effect of treatment by injection of absolute ethanol instead of surgery. Fourteen outpatients diagnosed as mucocele of the glands of Blandin-Nuhn were selected. These patients, after blood investigation, were treated by injection of absolute ethanol into a mucous cavity of lesion under superficial anesthesia with 2% lidocaine once a week and followed up from 3 months to 2 years. Mucocele of the glands of Blandin-Nuhn was extirpated in all patients after 1 to 3 injections. There were no other complications except slight distending pain occurred on the same day when the patients were treated. The recurrence was not observed during the follow-up period. In conclusion, the study suggests that injection of absolute ethanol may be an alternative means for treating mucocele of the glands of Blandin-Nuhn, because it is mininvasive, safe, effective, economic, and simply manipulated compared with surgical treatment.

Mucoceles of minor salivary glands in children. Own clinical observations.

INTRODUCTION: Mucoceles are benign lesions associated with the pathology of the oral mucosa of minor salivary glands. Two types of cysts are distinguished depending on their pathogenesis. Most often they occur as a result of mechanical trauma and mucus extravasation into tissues or obstruction of the gland ducts. AIM: The aim of the study was to present our own experiences regarding mucoceles of minor salivary glands in the oral cavity taking into account how frequently the individual types of cysts occur in children. MATERIAL AND METHODS: The research was carried out based on medical files from the years 2005-2015. These were: medical case records, operating books and the medical registry of patients treated at the Clinic of Maxillofacial Surgery, Frederic Chopin Clinical Regional Hospital in Rzeszow. In that period 64 children and teenagers, 28 girls and 36 boys were treated. What was considered was the age and gender of the patients, the reason for their appointment with a doctor, the location, size and histopathological type of the cysts, as well as the course and results of the diagnostic and therapeutic process. RESULTS: In the group analyzed, the reasons for referral to the Clinic were: in 25 patients accidental ascertainment of a non-symptomatic tumor in the oral cavity during examination by a dentist, pediatrician or laryngologist which had not caused any discomfort to the children; in 13 patients concern had been raised by a gradually increasing tumor; in 18 cases there was an increased tissue tension surrounding the tumor, while in 3 children red oedema was observed in the oral cavity (suspicion of abscess). The most frequent mucocele location was the lower lip (34 children). The most frequent size was 2.1-3 cm (28 children). The most frequent histological type was MEP. All the patients were treated at the Clinic in the one-day surgery mode, with good outcome. CONCLUSIONS: Mucocele ascertainment in children's oral cavity could be made accidentally in routine pediatric examination, therefore it is necessary to extend pediatricians' knowledge about small salivary gland mucoceles. The most frequent type of MEP could be related to different types of trauma in the oral mucose.

Ultrasound shear wave elastography for assessing minor salivary gland involvement in anti-centromere antibody-positive primary Sjögren's syndrome: a retrospective study.

The aim of this study is to investigate salivary gland involvement in patients with anti-centromere antibody (ACA)-positive primary Sjögren's syndrome (pSS). We retrospectively evaluated 134 patients with pSS. Patients were divided into four groups based on the results of ACA and SSA antibodies. We compared clinical manifestations, laboratory findings, salivary gland shear wave elastography, minor salivary gland biopsy results, and EULAR Sjögren's syndrome disease activity index (ESSDAI) scores among the four groups. A total of 134 patients were classified as having pSS and divided into three groups based on serum ACA and anti-SSA antibody status: ACA + SSA + , ACA + SSA-, ACA-SSA + , and seronegative. The primary analysis focused on comparing the clinical and SWE findings between the ACA + SSA + and ACA + SSA- groups. In the double-positive group, SWE revealed fewer minor salivary glands along with higher mean (Emean) and maximum (Emax) values of Young's moduli than those in the ACA-negative group. Patients in the positive group had increased occurrence of Raynaud's phenomenon, liver involvement, and a higher incidence of malignancy (P < 0.05). ACA-positive pSS patients are a subgroup with different clinical manifestations and more pronounced involvement of the minor salivary glands. SWE findings revealed that ACA-positive patients exhibit significantly higher mean and maximum stiffness values compared to ACA-negative patients, indicating more extensive glandular fibrosis and involvement. These results underscore the utility of SWE as a valuable method for evaluating salivary gland pathology and supporting the stratification of pSS patients.

Non-invasive imaging for predicting labial salivary gland biopsy outcomes in patients with suspected primary Sjögren syndrome.

To identify the value of salivary gland ultrasound (SGUS) combined with magnetic resonance imaging (MRI) and magnetic resonance sialography (MRS) in predicting the results of labial salivary gland biopsy (LSGB) in patients with suspected primary Sjögren syndrome (pSS), and construct a nomogram model to predict LSGB results. A total of 181 patients who were admitted with suspected pSS from December 2018 to April 2023 were examined and divided into a training set (n = 120) and a validation set (n = 61). Baseline data of the two groups were examined, and the value of SGUS, MRI, and MRS in predicting LSGB was analyzed. Multivariate logistic analysis was used to screen for risk factors, and nomogram prediction models were constructed using these results. In the training set, the SGUS, MRI, and MRS scores of patients in the LSGB + group were higher than those in the LSGB - group (all P < 0.001). The positive prediction value (PPV) was 91% for an SGUS score of 3, and 82% for MRI and MRS scores of 2 or more. We developed a nomogram prediction model based on SGUS, MRI, and MRS data, and it had a concordance index (C-index) of 0.94. The Hosmer-Lemeshow test (χ2 = 3.17, P = 0.92) also indicated the nomogram prediction model had good accuracy and calibration for prediction of LSGB results. A nomogram model based on SGUS, MRI, and MRS results can help rheumatologists decide whether LSGB should be performed in patients with suspected pSS.

Minor salivary gland mucinous adenocarcinoma of buccal mucosa - case report and review of the literature.

Mucinous adenocarcinoma (MAC) is commonly found in the gastrointestinal tract but head and neck localisations are very rare. This article presents the case of a 67-year-old patient suffering from a minor salivary gland MAC of the left buccal mucosa, who was treated in the Department of Cranio-Maxillofacial Surgery in Krakow due to multiple recurrences of the tumour. The results of immunohistochemical staining, the course of surgical treatment and follow-up, as well as a review of literature are also discussed.

[Imaging findings of carcinoma ex pleomorphic adenoma in minor salivary glands].

PURPOSE: To summarize the CT and MR imaging features of carcinoma ex pleomorphic adenoma(Ca-ex-PA) in minor salivary gland, and analyze the correlation between various features and pathological classification. METHODS: Forty-three patients with Ca-ex-PA in minor salivary gland were collected. The CT and MRI findings were retrospectively analyzed and correlated with their pathological types. Fisher's exact test was used to analyze the correlation between various imaging features (tumor morphology, boundary, internal structure, bone invasion, cervical lymph node metastasis) and pathological types with SPSS 25.0 software package. RESULTS: Among the 43 patients with Ca-ex-PA, 83.7%(36/43) of the tumors were lobulated; 81.4%(35/43) showed cystic degeneration or necrosis, with heterogeneous enhancement. Coarse calcification or mixed calcification was found in 37.2%(16/43), 25.6%(11/43) had compressive absorption of adjacent bone. 75%(12/16) of type Ⅰ/Ⅱ tumors had regular morphology (round or oval), and 77.8%(21/27) of type Ⅲ tumors had irregular morphology, 93.8%(15/16) of type Ⅰ/Ⅱ tumors had well-defined margin and 66.7%(18/27) of type Ⅲ tumors had ill-defined margin. Osteolytic bone resorption occurred in 59.3%(16/27) of type Ⅲ tumors. The average maximum diameter of type Ⅰ/Ⅱ tumors was significantly shorter than that of type Ⅲ(P＜0.05). Fisher's exact test showed the characteristics of tumor morphology, boundary and osteolytic bone resorption were related to pathological grouping(P＜0.001). CONCLUSIONS: Most Ca-ex-PA in minor salivary glands is characterized by lobular and heterogeneous enhanced neoplasm on CT and MR imaging. A round or oval tumor with well-defined margin usually correlates with typeⅠ and Ⅱ, contrarily, an irregular mass with ill-defined margin and osteolytic bone destruction usually correlates with type Ⅲ. Combining the three characteristics of morphology, boundary and osteolysis is more helpful to distinguish type Ⅰ/Ⅱ and type Ⅲ tumors.

Correlations between salivary gland scintigraphy and histopathologic data of salivary glands in patients with primary Sjogren's syndrome.

OBJECTIVES: Our aim was to evaluate the association between salivary gland scintigraphy and the clinical parameters, including histological characteristics of salivary glands, in patients with primary Sjogren's syndrome (pSS). METHODS: Forty-one pSS patients were included in the study. The patients who had received salivary gland scintigraphy and minor salivary gland biopsy were retrospectively analyzed. Salivary gland scintigraphy was interpreted via semi-quantitative methods obtained by calculating the peak uptake and washout of each gland using regions of interest. All specimens were examined by pathologists for focus scores and leukocyte common antigen (LCA) to determine the degree of inflammatory infiltration. RESULTS: The mean age of pSS patients was 46.4 years, 82.9% were female, and the mean duration of symptoms was 2.5 years. The focus score was negatively correlated to the mean peak uptake (r = ‒0.396; p = 0.019), mean uptake (r = ‒0.388; p = 0.021), and mean percentage washout (r = ‒0.391; p = 0.02). In addition, the focus score and number of LCA positive cells per mm2 were correlated with the clinical parameters including erythrocyte sedimentation rate, globulin, rheumatoid factor, unstimulated whole saliva, and stimulated whole saliva flow. The number of LCA positive cells per mm2 was negatively correlated to leukocytes and hemoglobin. CONCLUSION: Although the diagnostic role of salivary gland biopsy is widely accepted and features in the classification criteria of Sjogren's syndrome, salivary gland scintigraphy may be an acceptable alternative method especially if a non-invasive test is required.

Mammary analogue secretory carcinoma in minor salivary gland.

Mammary analogue secretory carcinoma is a rare neoplasm usually confused with other neoplasms in the salivary glands region. It has great similarity with the breast carcinoma. We report a case of a patient who presented with gingival submucosal bleeding and lesion, with the initial histopathological examination revealing salivary gland neoplasm of low crane. Computed tomography revealed the lesion near the tooth 27, with extension to the floor of the left maxillary sinus and to the palate mucosa. Resection of the infra-structure was performed, with a diagnosis of breast cancer secretory carcinoma in the minor salivary gland.

Endobronchial sialolipoma. Case report.

BACKGROUND: A 52-year-old woman presented with shortness of breath and cough. An endobronchial sialolipoma was found at the left entrance of the main bronchus. Sialolipoma is an exceedingly rare type of lipoma reported of the minor salivary glands, especially within the bronchus. CASE PRESENTATION: A 52-year-old woman presented with shortness of breath and cough with 6 months´ evolution. Endobronchial endoscopy revealed a tumour at the left entrance of the main bronchus. The entire removal of the tumour was removed using a cryoprobe device. Pathological examination showed a tumour consistent with the diagnosis of sialolipoma due to the presence of mature adipose cells blended with acinar, ductal, basal, and myoepithelial cells. The patient had a favourable outcome. CONCLUSION: The infrequent tracheobronchial presentation of this tumour can be challenging for correct diagnosis.

Prediction of Minor Salivary Gland Carcinoma: A Novel Nomogram and Risk Classification System for Overall Survival and Cancer-Specific Survival.

OBJECTIVE: Minor salivary gland carcinoma (MiSGC) is rare, and the understanding of this disease is insufficient. This study aimed to identify independent risk factors and develop a nomogram for evaluating the overall survival (OS) and cancer-specific survival (CSS) of patients with MiSGC. STUDY DESIGN: Retrospective cohort study. SETTING: SEER database (Surveillance, Epidemiology, and End Results). SUBJECTS AND METHODS: We collected data from patients diagnosed with MiSGC between 2004 and 2015 from the SEER database. According to patient registration, all patients were randomly allocated to training sets and validation sets (2:1). Then, Kaplan-Meier product limit curves and Cox proportional hazard regressions were performed to estimate the prognostic effect of variables. Nomograms based on Cox proportional hazard regressions were established to estimate 3- and 5-year OS and CSS. Finally, the nomogram was developed by the training set, and validation was performed with the concordance index, calibration curves, and decision curve analyses. RESULTS: In total, 1787 MiSGC cases were registered in SEER. The concordance index for internal validation of OS and CSS prediction was 0.842 and 0.816; that of external validation was 0.871 and 0.831. The calibration plots showed good consistency between nomogram prediction and actual survival. The decision curve analysis showed substantial net benefits of the new predictive model. CONCLUSIONS: We constructed nomograms and a corresponding risk classification system predicting the OS and CSS of patients with MiSGC. These tools can generate simple-to-use clinical risk grouping and determine the relationship between adjuvant therapy and active surveillance.

Diagnosis and treatment of secretory carcinoma arising from the oral minor salivary gland: Two case reports.

INTRODUCTION: Secretory carcinoma (SC) is a malignancy of the salivary glands, which is similar to SC of the breast regarding its association with neurotrophic tyrosine receptor kinase fusion-positive gene. SC is a recently described salivary gland tumor, and there are a few reports describing oral minor salivary gland-derived SC. We reported two cases of SC in the oral cavity and reviewed the literature. PATIENT CONCERNS: The patients included a 65-year-old Japanese woman who presented with a mass of the upper lip and an 84-year-old Japanese man who presented with a mass on the buccal mucosa. DIAGNOSIS: Diagnosis was based on histomorphological and immunohistochemical findings and identification of a specific translocation of the ETS variant 6-neurotrophic receptor tyrosine kinase 3 gene fusion. Case 1 was finally diagnosed using reverse transcription-polymerase chain reaction with formalin-fixed paraffin-embedded tissue samples, while case 2 was diagnosed using fluorescence in situ hybridization analysis. INTERVENTIONS AND OUTCOMES: In case 1, excisional biopsy was done and there was no recurrence observed in five-year follow-up. In case 2, tumor resection was done and there was no recurrence observed in two-year follow-up. CONCLUSION: It is highly likely for many cases of SC to be initially diagnosed as acinic cell carcinoma (AciCC) owing to their similar histological findings. The treatment strategy for minor salivary gland-originated SC is similar to that of AciCC; however, SC is often highly malignant and involves a high risk of cervical lymph node metastasis. Thus, establishing an accurate diagnosis together with pathologists and confirming the presence of the ETS variant 6-neurotrophic receptor tyrosine kinase 3 fusion gene using genetic analysis is important.

Rapidly progressive case of type I Madelung disease with bilateral parotid and minor salivary glands involvement.

Context: Madelung disease has been literally classified as a rare disease, more common in Mediterranean population. It is characterized by the presence of multiple symmetrical, non-capsulated storages of adipose tissue.Case description: We present a 56 year old woman with a sudden unexplained progression of swelling in the neck and upper thorax region associated with severe dyspnea. She was admitted with angioedema in the Allergology Department. Radiology images identified a bilateral adipose degeneration of the parotid and minor salivary glands.Conclusions: The diefinitive diagnosis concluded was Type 1 Madelung Disease. The decision was to keep the patient under periodic observation and if any deterioration takes place, surgical intervention will be taken under consideration. Despite non previous findings of Madelung disease in our population this case presented a challenge of emergency diagnosis.

Primary pleomorphic adenoma of minor salivary gland in the parapharyngeal space.

BACKGROUND: World literature suggests parapharyngeal space lesions account for only 0.5% head and neck tumours and the majority of the minor salivary gland tumours are malignant. The parapharyngeal space is very rare site for this tumour. CASE PRESENTATION: Two cases of primary pleomorphic adenomas arising de novo from minor salivary glands in the para pharyngeal space are reported. Review of literature, clinical features, pathology, radiological findings and treatment of these tumours are discussed. CONCLUSION: Pleomorphic adenoma arising de novo in the parapharyngeal space is of rare occurrence. High index of suspicion and an adequate clearance of the tumour with a cuff of surrounding dispensable normal tissues is the key to successful treatment of such tumours.