Basal cell adenocarcinoma of the soft palate: A closer look for an exceedingly rare salivary gland tumor.

Basal cell adenocarcinoma (BCAd) is an extremely rare primary biphasic carcinoma of the salivary glands with few well-documented cases reported in the literature. Herein, we report a rare case of a 44-year-old male patient who presented an oral medicine service with an erythematous nodular lesion on the soft palate, measuring 1.5 cm in its largest diameter, with a 5-year duration. The clinical diagnosis was pleomorphic adenoma, and an excisional biopsy was performed. Histopathological analysis revealed a biphasic infiltrative tumor composed of a mixture of central ductal cells and abluminal basal cells with slight atypia arranged in solid, trabecular, tubular and cribriform growth patterns in a loose stroma. The peripheral cells show a palisading arrangement with round hyperchromatic nuclei and scanty cytoplasm. Occasional mitotic figures were seen. Few spindle-shaped cells suggestive of myoepithelial cells were present in the stroma surrounding the basaloid tumor nests. The diagnosis was BCAd. The patient was referred to a head and neck service and has been followed up for 8 months with no signs of recurrence. In conclusion, although the diagnosis of BCAd can be challenging due to its rarity and morphological overlap with other salivary gland lesions, a meticulous morphological assessment is key for accurate diagnosis, especially in cases originating from minor salivary glands. Surgical excision with a wide safety margin is the treatment of choice and long-term follow-up is recommended to monitor possible recurrences.

Can a diagnosis of Leiomyosarcoma soft palate be missed in the era of commoner oral carcinomas? - Rare case report and review of literature.

Leiomyosarcomas (LMSs) of the head and neck are an extremely rare entity. Of all smooth muscle tumors, 4%-10% occur in the head and neck and only 0.06% in the oral cavity. Because of its infrequency, it has been associated with both delayed diagnosis and misdiagnosis. Here, we report the clinicopathological findings of a case of primary LMS of the soft palate in a 42-year-old male patient with an emphasis on the judicious use of ancillary diagnostic modalities to arrive at a definitive diagnosis. Intraorally, LMSs present as painless, lobulated, fixed masses of the submucosal tissues in middle-aged or older individuals. The treatment modalities and lymph nodal dissection criteria are dissimilar to more common oral carcinomas. Hence, definitive diagnosis is necessary.

Pseudoxanthoma-elasticum-like changes on the soft palate.

Pseudoxanthoma elasticum (PXE) is an autosomal recessive genetic disorder characterized by aberrant fragmentation and calcification of elastic fibers, leading to characteristic cutaneous, ophthalmic, and cardiovascular manifestations. PXE demonstrates significant phenotypic variability; involvement of the oral mucosa may be the only clue to the diagnosis. Reports on mucous membrane involvement in PXE are scarce. Here, we present a case of PXE-like changes in the oral cavity. A 70-year-old male patient presented with a painless leukoplakic lesion on the soft palate. Biopsy revealed numerous degenerated fibers in the lamina propria. Verhoeff-van Gieson and von Kossa staining confirmed their identity as calcified elastic fibers. A histopathological diagnosis of PXE-like changes was made; the patient was referred to ophthalmology where angioid streaks were visualized fundoscopically. PXE-like changes in the absence of the characteristic genetic mutation have also been reported with or without systemic manifestations. Furthermore, PXE-like changes have been reported in up to 10% of oral biopsy specimens undertaken without clinical suspicion for PXE. Therefore, the significance of such changes in isolation is unclear. Clinicians and pathologists should be aware of the potential oral manifestations of PXE to facilitate prompt diagnosis and subspecialist referral.

Large Palatal Pleomorphic Adenoma Complicated by Dysphagia: A Case Report.

A 60-year-old Japanese woman presented with a palatal pleomorphic adenoma measuring 5 cm in size. In addition to impairments during the oral preparatory and oral transport phases, dysphagia with nasopharyngeal closure disorder was observed in the pharyngeal phase. After resection of the tumor, dysphagia resolved, and the patient was immediately able to eat a standard meal. A videofluoroscopic swallowing study confirmed improvement in the movement of the soft palate compared with the pre-operative condition.

A rare case of solitary intraoral superficial angiomyxoma arising in the soft palate.

Superficial angiomyxomas are myxoid mesenchymal tumors, and intraoral superficial angiomyxoma is extremely rare. This paper reports a novel case of a 41-year-old Japanese male patient with a 32 × 22 mm superficial angiomyxoma in the right soft palate. Tumor resection was performed and a polyglycolic acid sheet was attached. Over a 28-month follow-up, there was no evidence of disease recurrence. This paper also reviewed 11 cases of intraoral superficial angiomyxomas reported in previous literature. The condition was more common among middle-aged men. Surgical resection was the most common treatment, and local recurrence was observed in only one case.

Spindle cell carcinoma masquerading as a benign polyp of the soft palate.

Spindle cell carcinoma of the head and neck is a rare biphasic neoplasm. The presentation mimics other head-and-neck malignancies and hence the diagnosis hinges upon histopathological confirmation along with positive immunohistochemistry (IHC) markers denoting the presence of both epithelial and mesenchymal components. At present, there are no standard management criteria for these tumors with the options varying from surgery alone to surgery combined with adjuvant radiotherapy. We discuss here the case of a patient presenting with an oropharyngeal mass that had benign clinical features and the final diagnosis of spindle cell carcinoma could only be established after histopathology with IHC typing.

Zymogen-poor acinic cell carcinoma of the soft palate with high-grade transformation.

Acinic cell carcinoma is an uncommon malignant salivary gland tumour accounting for approximately 6-7% of all salivary gland neoplasms. The key diagnostic feature of acinic cell carcinoma is the presence of acinar cell differentiation characterised by cytoplasmic zymogen secretory granules. This tumour shows a variety of growth patterns, including solid, microcystic, follicular and papillary cystic patterns. Acinic cell carcinoma is typically a cytologically low-grade malignancy. Acinic cell carcinomas with high-grade transformation (HGT) are exceedingly rare and are reported to have a more aggressive clinical course than conventional acinic cell carcinoma. This is a case report of this uncommon entity with high-grade transformation arising on the soft palate in a 64-year-old woman.

Transoral Robotic Surgery for Cancer of the Soft Palate Posterior Surface.

The surgical treatment of oropharyngeal squamous cell carcinoma (OSCC) has undergone a considerable evolution, with, among others, transoral robotic surgery (TORS). However, TORS is rarely used for the surgery of soft palate. A 73-year-old man presented a human papilloma virus OSCC of the posterior soft palate. The tumor was resected using TORS. This report describes the surgical technique step by step of the tumor resection. The resection of early stage OSCC of the posterior surface of soft palate is possible through TORS. The procedure is noninvasive, rapid, and seems to be associated with excellent functional outcomes.

Epithelioid Soft Tissue Neoplasm of the Soft Palate with a PTCH1-GLI1 Fusion: A Case Report and Review of the Literature.

GLI1 fusions involving ACTB, MALAT1, PTCH1 and FOXO4 genes have been reported in a subset of malignant mesenchymal tumors with a characteristic nested epithelioid morphology and frequent S100 positivity. Typically, these multilobulated tumors consist of uniform epithelioid cells with bland nuclei and are organized into distinct nests and cords with conspicuously rich vasculature. We herein expand earlier findings by reporting a case of a 34-year-old female with an epithelioid mesenchymal tumor of the palate. The neoplastic cells stained positive for S100 protein and D2-40, whereas multiple other markers were negative. Genetic alterations were investigated by targeted RNA sequencing, and a PTCH1-GLI1 fusion was detected. Epithelioid mesenchymal tumors harboring a PTCH1-GLI1 fusion are vanishingly rare with only three cases reported so far. Due to the unique location in the mucosa of the soft palate adjacent to minor salivary glands, multilobulated growth, nested epithelioid morphology, focal clearing of the cytoplasm, and immunopositivity for S100 protein and D2-40, the differential diagnoses include primary salivary gland epithelial tumors, in particular myoepithelioma and myoepithelial carcinoma. Another differential diagnostic possibility is the ectomesenchymal chondromyxoid tumor. Useful diagnostic clues for tumors with a GLI1 rearrangement include a rich vascular network between the nests of neoplastic cells, tumor tissue bulging into vascular spaces, and absence of SOX10, GFAP and cytokeratin immunopositivity. Identifying areas with features of GLI1-rearranged tumors should trigger subsequent molecular confirmation. This is important for appropriate treatment measures as PTCH1-GLI1 positive mesenchymal epithelioid neoplasms have a propensity for locoregional lymph node and distant lung metastases.

Evaluation of the clinical efficacy of nasal surgery in the treatment of obstructive sleep apnoea.

STUDY OBJECTIVES: The aim of the study was to evaluate the clinical efficacy of nasal surgery in the treatment of obstructive sleep apnoea (OSA) by comparing the improvement of subjective symptoms and objective metrics before surgery and after 6 months of surgery. METHODS: Patients with the main complaint of nasal congestion combined with habitual snoring who were hospitalized and treated were selected. Patients underwent subjective symptom tests and objective indicator monitoring both before surgery and 6 months after surgery. Comparisons between groups were performed using the independent samples t-test. RESULTS: Subjective scale evaluations demonstrated that nasal congestion, daytime sleepiness, snoring, nose-related symptoms, and sleep symptoms in patients with simple snoring or with OSA were improved after nasal surgery. Additionally, vitality was improved in all groups except for the patients with simple snoring and emotional consequence was improved in patients with simple snoring and mild OSA. Objective evaluations indicated the apnoea-hypopnoea index (AHI), the thickness of the soft palate, and the maximum cross-sectional area of the sagittal plane of the soft palate decreased after surgery in patients with mild OSA. The lowest blood oxygen concentration (LSaO2) and anteroposterior diameter of the soft palate increased after surgery in patients with mild OSA. The arousal index also significantly decreased in patients with mild and moderate OSA. The nasal cavity volumes (NCVs) and the nasal minimal cross-sectional areas (NMCAs) of all groups showed significant differences after surgery. CONCLUSIONS: Nasal surgery can effectively improve nose and sleep symptoms in patients with simple snoring or with OSA. It can significantly reduce the nasal resistance and increase the ventilation volume. STATEMENT OF SIGNIFICANCE: Obstructive sleep apnoea (OSA) is becoming a global health problem. OSA is associated with several coexisting conditions, reduced health-related quality of life, and impaired work productivity. This study performed nasal surgery on OSA patients with the main complaint of nasal congestion combined with snoring and patients with simple snoring to compare the improvement of subjective symptoms and objective metrics before and after surgery. We found that: (1) symptoms such as nasal congestion, daytime sleepiness or snoring were improved after nasal surgery; (2) the apnoea-hypopnoea index (AHI) and arousal index decreased after surgery in patients with OSA; (3) the nasal and oropharyngeal cavity volumes increased after surgery. These findings suggest that patients with OSA or with simple snoring could benefit from nasal surgery.

Polymorphous Adenocarcinoma, Low Grade Variant, Colliding with a Neurofibroma.

Collision tumors, composed of two distinct benign or malignant neoplasms, are rarely reported in the oral cavity. We present a case of a 61-year-old female with an asymptomatic non-demarcated lump on the soft palate of unknown duration. An incisional biopsy revealed the presence of two neoplastic populations, a neurofibroma that was partially infiltrated by a polymorphous adenocarcinoma, low-grade variant. Total surgical excision was performed, with uneventful follow-up period. The development of collision tumors may be incidental, although molecular events may influence the pathogenetic mechanism of the phenomenon.

Uvulopalatopharyngoplasty for nasal blockage 21 years after total rhinectomy.

BACKGROUND: Total rhinectomy is an invasive procedure that significantly impairs the intranasal turbulence, humidification and heating of inspired air. The use of uvulopalatopharyngoplasty for the treatment of sleep-disordered breathing disorders such as primary snoring and obstructive sleep apnoea has diminished over the past years because of the emergence of less invasive procedures and alternative therapeutic options. This clinical record presents the treatment of a long-term side effect of total rhinectomy using uvulopalatopharyngoplasty. CASE REPORT: In 1997, a 62-year-old male underwent total rhinectomy for a nasal schwannoma, followed by rehabilitation with a nasal prosthesis. Twenty-one years later, he presented with severe complaints of nasal blockage and breathing difficulties during both daytime and night-time. Clinical examination revealed no major anomalies besides significant velopharyngeal narrowing. Thus, in 2019, uvulopalatopharyngoplasty was performed to re-establish velopharyngeal patency. Hereafter, the symptoms of nasal blockage disappeared, resulting in an improved quality of life. CONCLUSION: Uvulopalatopharyngoplasty may prove useful to treat selected patients with daytime breathing difficulties due to velopharyngeal narrowing.

Congenital Hairy Polyp Causing Severe Upper Airway Obstruction in a Newborn: A Case Report.

BACKGROUND Hairy polyps are rare tumors mainly comprising fatty tissues covered by skin and hair follicles, with varied localizations and sizes. Early excision of the polyps by surgery is an effective treatment resulting in a permanent cure. We present a case of successful management of severe obstruction of the oropharynx in a newborn who presented with a large mass of congenital hairy polyp. CASE REPORT A vaginally delivered infant, weighing 3 kg, presented immediately after birth with cyanosis symptoms, failure of the first cry, and respiratory distress signs. The newborn was born to a mother with an uneventful pregnancy. Screening tests during the pregnancy reported no congenital anomalies. The newborn's hematological and biochemical test results were normal. After presenting these symptoms, the newborn was immediately intubated and put on a nasogastric feeding tube, which revealed a small portion of a polyp-like mass. A computed tomography (CT) scan further confirmed a large pedunculated mass, measuring 3×2 cm, arising from the soft palate, and obstructing the oropharynx. Histopathological examination confirmed the presence of a hairy polyp. The polyp was wholly removed transorally using the Covidien LigaSure device without the need for endoscopy. This procedure allowed safe extubation, and the baby was discharged home without symptoms 4 days after birth. CONCLUSIONS This case sheds light on the importance of considering hairy polyp in the differential diagnosis of pharyngeal mass with respiratory distress in pediatric patients. This report also describes our experience using the LigaSure surgical device without needing endoscopic visualization to successfully resect the hairy polyp without complications.

Imaging features of pharyngeal hairy polyps in infants.

BACKGROUND: Hairy polyps are the most common congenital deformity of the oral-nasopharynx/oral cavity in infants, which may lead to severe complications in the newborns. However, few studies have been published with respect to their radiological features, and most are case reports. OBJECTIVE: This study aimed to analyze radiological features of the oral­nasopharyngeal hairy polyps and to identify the radiological features with the highest diagnostic value. MATERIALS AND METHODS: From 2014 to 2019, pediatric cases pathologically diagnosed as hairy polyps and having received radiological examination at the Children's Hospital Affiliated to Zhejiang University were retrospectively analyzed. Radiological evaluations were conducted on tumor size, location, morphology, density or signal features as well as the enhancement pattern. RESULTS: A total of six infants were recruited. Clinical features observed in these cases included choking on milk with cyanosis, intermittent dyspnea, oropharyngeal mass, and snoring. Lesions were derived from the left tubal torus in three out of six cases, from the left lateral aspect of soft palate in one, from the left lateral pharyngeal wall in one, and from the right tubal torus in one. They were shown as pedicled polyp-like well-circumscribed mass with fat density or signal as well as a stalk on CT or MRI. In the contrast-enhanced scan, the fat components were not enhanced, while the stalk was mildly enhanced. CONCLUSION: Hairy polyps had typical radiological features. Hairy polyps should be considered for pedicled polyp-like well-circumscribed mass in the oral-nasopharynx of infants with fat density or signal as well as a stalk.

Clinical Radiographic Predictors of Response to Hypoglossal Nerve Stimulation for Obstructive Sleep Apnea.

OBJECTIVE: To determine if clinically acquired cephalometric measurements, specifically soft palate size, can predict hypoglossal nerve stimulation outcomes. STUDY DESIGN: Combined prospective cohort study and retrospective review. SETTING: US sleep otolaryngology training program. METHODS: Adults with obstructive sleep apnea and apneahypopnea index greater than 15 events/h who underwent hypoglossal nerve stimulation. Eligible subjects had diagnostic preoperative sleep studies and full-night efficacy postoperative studies for analysis. Lateral neck x-rays were obtained as part of routine clinical care and measured for key cephalometric variables by trained head and neck radiologists. Continuous variables were compared using the Student t test, while χ2 testing was used for categorical variables. RESULTS: Fifty-one patients met all study criteria. On average, patients were white, middle aged, and overweight. Following hypoglossal nerve stimulation, the overall cohort achieved a significant apnea-hypopnea index reduction from 36.7 events/h to 20.6 events/h (P < .01) and a response rate of 47% (defined as apnea-hypopnea index reduction >50% and apnea-hypopnea index <20 events/h). On average, therapy responders had significantly thinner soft palates than nonresponders (13.4 ± 3.8 mm vs 16.0 ± 3.4 mm, P = .045). CONCLUSIONS: Patient-specific anatomic factors, specifically soft palate thickness, may help identify optimal candidates for hypoglossal nerve stimulation. A larger, prospective study including both anatomic and physiologic variables is required to validate these findings.

Endoscopic findings in the soft palatal mucosa are associated with the risk of esophageal squamous cell carcinoma.

BACKGROUND AND AIM: We investigated endoscopic findings of the soft palatal mucosa to identify factors associated with esophageal squamous cell carcinoma (ESCC). METHODS: This study was conducted during endoscopic examinations of subjects at Osaka International Cancer Institute from January 2020 through May 2020. We took endoscopic images of the soft palate under non-magnifying and mild-magnifying observations. Subjects with ESCC or a history of ESCC were defined as the ESCC group. Two endoscopists who were blinded to subjects' clinical information interpreted 10 endoscopic findings: melanosis, brownish changes, whitish epithelium, vasodilation, circular alignment of dilated vessels, uneven surface, uneven epithelial color, uneven vessel visibility, palate ridge, and erosion. Subjects were interviewed about their alcohol use, smoking, and flushing reactions. RESULTS: Two hundred eighty-two subjects, including 151 in the ESCC group and 131 in the non-ESCC group, were included in the analysis. Univariate analyses and multivariate logistic regression demonstrated that melanosis, whitish epithelium, and vasodilation were significantly associated with ESCC. The positive likelihood ratios (PLRs) of melanosis, whitish epithelium, and vasodilation were 3.3, 4.2, and 2.8, respectively. Additionally, the PLRs for three of the endoscopic findings in subjects with drinking and smoking habits were higher than in those without these habits-PLRs ranging from 7.23 to 19.1. High PLRs for three endoscopic findings suggested a high possibility of ESCC. Interobserver agreement was substantial for whitish epithelium, moderate for melanosis, and fair for vasodilation. CONCLUSIONS: Three endoscopic findings in soft palate were considered to be useful as alarming signs that indicate ESCC risk.

Extranodal NK/T Cell Lymphoma, Nasal Type with Palatal Involvement: A Rare Case Report and Literature Review.

T-cell lymphomas are infrequently encountered in the head and neck area, with the most common subtype being Extranodal NK/T cell lymphoma, nasal type (ENKTL-NT). ENKTL-NT shows a predilection for midline facial structures presenting with ulcerative destructive lesions, whereas palatal involvement is one of the most prominent signs from the oral cavity. Herein, we describe a case of a 76-year-old Greek man with nasal obstruction and an extensive painful necrotic ulcer with ragged borders on the left distal portion of the soft palate and palatine tonsil of 4-months duration. After an initial non-diagnostic biopsy from the nasopharynx, a second incisional biopsy from the palatal lesions was performed. Histopathology was suggestive of an angiocentric lymphoproliferative neoplasm and immunohistochemical examination and in situ hybridization for EBV RNA led to a final diagnosis of ENKTL-NT. The patient was placed under combined chemotherapy and radiotherapy and no recurrence has been noted. Additionally, a retrospective review of the cases in the English literature with an established diagnosis of ENTKL-NT between 2000 and 2019, based on the latest WHO classification of Head and Neck tumors, is performed.

A Cadaveric Study of the Buccal Fat Pad: Implications for Closure of Palatal Fistulae and Donor-Site Morbidity.

BACKGROUND: For the cleft surgeon, palatal fistulae after cleft palate repair remain a difficult problem, with a paucity of local tissue options to aid closure. Small clinical series have described the use of the buccal fat pad flap to repair palatal fistulae; however, there is no literature detailing the anatomical coverage of the flap. This study delineates the anatomy of the buccal fat pad flap to guide surgeons in patient selection and examines the residual buccal fat after flap harvest to provide new information with regard to possible effects on the donor site. METHODS: Buccal fat pad flaps were raised in 30 hemicadavers. The reach of the flap across the midline, anteriorly and posteriorly, was recorded. In 18 hemicadavers, the entire buccal fat pad was then exposed to determine the effects of flap harvest on movement and volume of the residual fat. RESULTS: All buccal fat pad flaps provided coverage from the soft palate to the posterior third of the hard palate and all across the midline. Approximately three-fourths of flaps would cover the mid hard palate. The flap constitutes 36 percent of the total buccal fat pad on average, and a series of retaining ligaments were identified that may prevent overresection. CONCLUSIONS: The buccal fat pad flap is a useful tool for coverage of fistulae in the soft palate to the posterior third of the hard palate. In most cases, it will also reach the middle third; however, it is not suitable for more anterior defects. On average, two-thirds of the buccal fat pad remains within the cheek after flap harvest, which may protect against unwanted alteration in aesthetics.