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Background There have hardly been any reported cases of children presenting with Kaposi sarcoma as a second malignancy following treatment for acute lymphoblastic leukemia outside a transplant setting. Case presentation We report a case of a 5-year-old boy of Bantu origin, which, to our knowledge, could be only the second reported case of oral–visceral Kaposi sarcoma after acute lymphoblastic leukemia treatment. The patient presented with a 1-month history of progressive, non-painful, soft tissue oral mass, 1 month after completing treatment for high-risk acute lymphoblastic leukemia. He was successfully treated for Kaposi sarcoma on a two-drug regimen (bleomycin and vincristine) with good clinical response. Conclusion Visceral Kaposi sarcoma as a second malignancy may occur after pediatric acute lymphoblastic leukemia treatment, but its rarity makes it unlikely to raise suspicion among clinicians, thus precluding early diagnosis and treatment. We recommend routine evaluation for Kaposi sarcoma lesions in children undergoing long-term surveillance following treatment for childhood acute leukemia.

Oral cancer remains an important global health issue and despite recent diagnostic and therapeutic advances, it continues to have an unfavorable prognostic and decreased survival. Although palatal tumors represent one of the rarest locations of oral squamous cell carcinomas (SCCs), they are among the most aggressive local tumors, leaving behind important morpho-functional disabilities. In order to explain such local aggressiveness, the present study aims to investigate the immunohistochemical expression in palate SCCs of some markers known to be involved in the process of tumor invasiveness, such as Wiskott-Aldrich syndrome like (WASL), Claudin-1 (CLDN1), Integrin beta-6 (ITGB6) and c-Mesenchymal to epithelial transition protein (c-Met). We have found here a higher tumor WASL and CLDN1 reactivity in well-differentiated (G1) palate SCCs, and regardless the histological type, degree of differentiation or tumor topography, an overexpression at the invasion front, and in those palate' SCC cases with muscular invasiveness and with lymph node (LN) dissemination. ITGB6 and c-Met had a higher reactivity in moderately differentiated (G2) palate SCCs, especially at the periphery of tumor proliferations, at the invasion front and in those high invasive cases and as well as in those that associated LN dissemination. All four investigated markers were also positive at the level of LN metastatic proliferations. None of the markers could statistically stratify on age group and pain, and on bone and perineural invasion while all of them statistically stratified on survival and grading. We concluded that these markers have a prognostic role allowing the identification of those cases with an unfavorable clinical evolution and decreased survival.

Background Salivary cancer is rare and comprises a variety of histological subtypes and clinical behaviors. There is no agreed method of estimating the risk of occult metastasis or managing the clinically N0 neck.Sentinel node biopsy (SNB) may offer a solution but previous studies have not produced a reliable imaging protocol. This study uses novel technology and trial methodology to develop a reliable SNB technique, with primary aim to identify peri-and intraglandular sentinel nodes. Methods IDEAL framework was used to undertake SNB in clinically node negative salivary gland cancer. Patients with cT1-2 N0 salivary cancer were eligible. Lymphoscintigraphy was undertaken using Tc-99 m labelled nanocoll. Injection technique as well as adjunctive use of freehand SPECT (fhSPECT), near-infrared (NIR) fluorescence imaging, and navigation-guided surgery were used and optimisied during the study protocol. Results 10 patients were recruited. Initial protocol of peritumoural injection of Tc99 m nanocoll showed poor image resolution. Subsequent adjustment to single intratumoural injection allowed identification of intraglandular sentinel nodes. Fh/SPECT and NIR fluorescence imaging found intraglandular lymph nodes otherwise not recognizable to the naked eye. In two cases occult lymph node metastasis were identified. Conclusion This study has shown the IDEAL framework is vital in allowing iterative changes in surgical protocol in the light of experience. This study has produced a reliable method for detection of sentinel nodes, in particular the ability to identify intra- and periglandular nodes with diagnosis of occult metastatic deposits and no false negative results. Our protocol can be readily transferred in to larger scale studies.

Medpor porous polyethylene implants are commonly used for facial skeletal reconstruction due to reported biocompatibility, fibrovascularization, and durability. While uncommon, late implant infections are an important consideration. We report delayed infections in 2 patients after unilateral total oncologic maxillectomy and reconstruction using Medpor implants for an ossifying fibroma and squamous cell carcinoma, respectively. In the first patient, annual interval computed tomography (CT) scans showed no recurrence of tumor or inflammatory changes. The second was lost to follow-up after adjuvant chemoradiation 1 year after resection. Patients both presented with swelling, drainage, and erythema around the implant at a mean of 4.5 years following maxillectomy. Both failed several attempts at conservative treatment. Cultures of implants removed at a mean of 2.5 months after infection grew α-hemolytic Streptococcus in the first and multiple organisms in the second, showing that the potential for delayed infection should be considered years after reconstruction.

Computed tomography (CT) and magnetic resonance imaging (MRI) can be useful for the evaluation of palatal lesions, and strain elastography (performed together with intraoral ultrasonography) is a relatively new sonographic imaging modality. This report describes 2 clinical cases in which strain elastography was used to assess palatal tumors in conjunction with intraoral ultrasonography, CT, and MRI. In the first case, diagnosed as a myoepithelioma, the strain was determined to be 0.000% (strain of normal tissue, 0.556%). In the second case, diagnosed as an adenoid cystic carcinoma, the determined strain was 0.000% (strain of normal tissue, 1.077%). Therefore, we conclude that intraoral strain elastography can be useful for evaluating palatal lesions.

Objective To report long-term oncological and functional outcome of Transoral Robotic Surgery escalated treatment including radiotherapy or chemoradiotherapy for Stage III-IV HPV negative oropharyngeal malignancies. Method From March 2013 to September 2015, 153 patients with oropharyngeal carcinoma were included in the study. Patients were evaluated for disease free survival, overall survival and post-treatment functional outcomes. Results 153 patients (96 males and 57 females) underwent TORS for oropharyngeal carcinoma. 142 patients on final histopathology had stage III and IV disease and received adjuvant treatment based on final histopathology. One hundred and sixteen (81.7%) patients were disease free on average follow-up of 48 months with an overall survival of 91.5% at mean follow-up of 48 months. Conclusion TORS can be used to intensify treatment of Stage III/IV oropharyngeal carcinoma and avoid early and late toxicities due to higher doses of upfront RT/CTRT and achieve better oncological outcome.

Salivary gland tumors are rare, constituting approximately 0.5% of pediatric malignancies, yet account for over 50% of malignant salivary gland neoplasms, of which a majority are mucoepidermoid carcinomas (MECs). We present a case of MEC involving the palatal minor salivary gland of an adolescent patient successfully reconstructed using buccal myomucosal flap. The subject presented as a 17-year-old male with a painless left hard palatal mass found to have imaging suspicious for minor salivary gland tumor and punch biopsy consistent with a low-grade MEC. The subject underwent wide local excision of a 1.0 × 1.2 cm mass with 1.0 cm margins down to hard palate nasal mucosa excluding the abutting first and second molars of the ipsilateral maxilla. Pathology confirmed low-grade MEC confined to hard palate. Two weeks, thereafter, the subject underwent buccal myomucosal flap reconstruction inset into the palatal defect and divided 2 weeks thereafter. Postoperative course was complicated by a pinpoint oronasal fistula at the posterior aspect of the flap-palate junction requiring reelevation and advancement. The subject subsequently recovered without complication. Mucoepidermoid carcinomas represent rare, malignant minor salivary gland tumors with nonspecific presentations that require multidisciplinary workup and management. The authors recommend reconstruction of resultant palatal defects to prevent progression to oronasal fistulae or speech and swallow impairment.

The incidence of oral cavity squamous cell carcinoma (OSCC) is particularly high in South Asia. According to the National Comprehensive Cancer Network, OSCC can arise in several subsites. We investigated survival rates and the clinical and pathological characteristics of OSCC in different anatomical subsites in the Taiwanese population. We retrospectively analyzed data for 3010 patients with OSCC treated at the Changhua Christian Hospital. Subsequently, we compared clinical and pathological features of OSCC in different subsites. Pathological T4 stage OSCCs occurred in the alveolar ridge and retromolar trigone in 56.4% and 43.7% of cases, respectively. More than 25% of patients with tongue OSCC and 23.4% of those with retromolar OSCC had lymph node metastasis. The prognosis was worst for hard palate OSCC (hazard ratio 1.848; p p = 0.017). Retromolar OSCC recurred most often and tongue OSCC second most often. The risk for cancer-related mortality was highest for hard palate OSCC, followed by alveolar ridge and retromolar OSCC. We found distinct differences in survival among the different subsites of OSCC. Our findings may also help prompt future investigations of OSCC in different subsites in Taiwanese patients.

We report a palatal mucoepidermoid carcinoma (MEC) with unusual pathological features showing salivary duct cyst-like architecture, varied epithelial linings (excretory duct, mucous goblet cell, and apocrine epithelium phenotypes), scarce luminal tumor plaques, and a large intraluminal oncocytic polyp. To our knowledge, such a unicystic variant of MEC with pedicled oncocytic proliferation in the lumen is unprecedented in the literature. In this particular case, the microscopic diagnosis of MEC is problematic because of the large number of potential mimics.

Carney complex (CNC) is a rare, autosomal dominant multiple neoplasia syndrome. Although cutaneous myxomas commonly occur in CNC patients, intraoral myxomas are extremely rare. We present a case of a palatal myxoma in a 21-year-old female patient with CNC, along with a review of the pertinent literature. She presented with a sessile nodule on the hard palate that microscopically showed a multilobulated and highly vascularized myxomatous tissue composed of loosely-arranged spindle, polygonal, and stellate cells, suggestive of myxoid neurofibroma. Six years after the oral lesion was removed, she presented with a growth hormone (GH)-producing pituitary adenoma, a cardiac myxoma, two cutaneous myxomas on the lower abdomen area, and one myxoma in the vaginal mucosa. Therefore, the final diagnosis of the palatal lesion was of a soft tissue myxoma related to CNC. The patient remains on close follow-up, with no recurrences of the palatal myxoma after 7 years.

Myxofibrosarcoma (MFS) is a fibroblastic soft tissue sarcoma that is extremely rare in the maxillofacial region. Due to its non-specific clinicoradiographic findings and challenging histopathological features, the diagnosis is difficult. Here, we present a case of MFS which was first diagnosed as nodular fasciitis. The initial examination of the incisional biopsy showed a benign-appearing proliferation of fibroblasts without features of malignancy. The patient returned with recurrence four months after surgical excision of the primary lesion. The second histologic study revealed a high-grade spindle cell sarcoma with myxoid features most compatible with MFS. Definitive diagnosis of MFS was confirmed by these histopathologic features and supportive immunohistochemical stains. Unfortunately, the patient died of disease 3 months later.

Introduction The soft palate is a multilayered intrinsically mobile structure, with a critical role in maintaining the airway, swallowing, and speech. We describe our technique of microsurgical reconstruction and analyze patient reported outcomes. Methods A retrospective analysis was performed using the University of Washington head and neck disease specific Quality of Life (UWQOL) questionnaire and the Hirose Standard speech analysis tool. Swallowing function and diet were recorded. Results Twenty cases were reviewed; nine were available for post hoc analysis. Anterolateral thigh flaps were used in 19 cases, in combination with vastus lateralis muscle in 5 cases. Speech intelligibility was graded "excellent" in 8 of 9 cases and moderate in 1 of 9. All tolerated an oral diet. Mean UWQOL score was 424.4/600 (SD ± 141.9) for physical and 461.7/600 (SD ± 141.9) for emotional-social domains. Conclusion Reconstruction of the soft palate using velopharyngeal narrowing with free anterolateral thigh flap delivers good functional outcomes.

Osteomas are benign slow-growing osteogenic lesions of unknown aetiology which can be central, peripheral or extraskeletal. Peripheral osteomas of the maxilla are very uncommon. We report a 72-year-old female patient who presented to the Department of Oral & Maxillofacial Surgery, Dental School of Athens, Athens, Greece, in 2015 with swelling of the palate following a tooth extraction. Clinical and radiographical features were indicative of a solitary peripheral osteoma of the hard palate. An excisional biopsy and histological examination of the lesion confirmed the diagnosis. No complications occurred during the postoperative period and there was no evidence of recurrence at a one-year follow-up.

Background Myoepitheliomas account for less than 1% of salivary gland tumors. They mostly affect the parotid glands of adults during the third to fifth decades. Case presentation A 10-year-old Indian boy reported a small swelling in the roof of his mouth of 10 days’ duration. History revealed that the lesion was painless and not associated with bleeding or pus discharge. On examination, a purplish well-circumscribed growth was noted on his posterior hard palate. Magnetic resonance imaging was suggestive of a well-encapsulated hemangioma. An excisional biopsy was performed and histopathology along with immunohistochemistry analysis showed that the lesion was a spindle cell variant of benign myoepithelioma. Conclusion Palatal myoepitheliomas are rare and their occurrence in young individuals is rarer.

INTRODUCTION Postablative palatomaxillary defects (PAPMDs) represent a challenging reconstructive problem. Temporalis muscle flap (TMF) has been widely used for reconstruction of these defects with minimal morbidity and satisfactory outcome. AIM OF THE STUDY To presents the authors' experience in the reconstruction of PAPMDs with TMF and to evaluate the validity of TMF in the reconstruction of such defects. METHODS This prospective study was conducted between July 2011 and July 2016 on selected patients for primary reconstruction of PAPMDs with TMF. Temporalis muscle flaps were assessed during surgery and postoperatively. Patients were followed up to evaluate functional and esthetic outcomes and detect complications. RESULTS This study included 32 patients with mean age 48.3 years. The pathology was squamous cell carcinoma in 15 patients (46.9%). Twenty-one patients (65.6%) had type II maxillectomy. Mean time of flap harvesting was 43 minutes. Zygomatic arch osteotomy was done in 3 patients while Coronoid osteotomy in 4 patients. Postoperatively, flaps were viable in 31 patients (96.9%) with good healing of recipient site. Flap epithelization completed within 28 to 59 days. Follow-up period was 13 to 55 months. Satisfactory functional and esthetic outcomes were reported in most of patients with no recurrence. Transient temporal nerve palsy occurred in 2 patients, limited mouth opening in 5 patients. One patient had Transient diplopia with enopthalmos and hypophthalmos. Flap failure occurred in another patient. CONCLUSIONS Temporalis muscle flap is still a valid reliable and versatile reconstructive tool in palatomaxillary reconstruction after ablative surgery. It has a good cosmetic and functional outcomes and minimal morbidity.

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Adenoid cystic carcinoma (ACC) is a rare tumor, with an annual incidence of 3-4.5 cases/million.We conducted a descriptive and retrospective study. We included patients diagnosed with ACC of head and neck of a single third-level referral center, from January 2008 to December 2013.A total of 23 patients were included in the study, 10 males (43.5%) and 13 females (56.5%) (F:M ratio 1.3:1). Median age was 52 years. Localization was predominantly observed in the oral cavity (26%) followed by the palate (17.4%). At presentation, 15 patients (65.2%) had clinical Stage II. The most frequent histology was the cribriform type in 10 patients (43.5%). Following resection, positive margins were observed in 15 (65.2%), neural invasion in 8 (34.8%), and lymphovascular invasion in 2 (8.7%) patients. The median time to recurrence in our population was 23 months. Recurrence at 5 years was observed in 15 patients (65%). At 2 years, recurrence was predominantly local in 7 patients (46%); whereas after 2 years, it was predominantly pulmonary 6 patients (40%). Overall, 5 year-survival was 78%.Cystic adenoid carcinoma is characterized by a high recurrence rate. Nevertheless, it has a high overall 5-year survival, which justifies an aggressive treatment.El carcinoma adenoide quístico es un tumor raro, con una incidencia anual de 3-4.5 casos por millón.Estudio descriptivo, retrospectivo, que incluyó pacientes con carcinoma adenoide quístico de cabeza y cuello de un centro de referencia de tercer nivel, desde enero de 2008 hasta diciembre de 2013.Se incluyeron 23 pacientes, 10 hombres (43.5%) y 13 mujeres (56.5%) (relación 1.3:1), con una media de edad de 52 años. Predominó en la cavidad bucal (26%), seguida del paladar (17.4%). Al diagnóstico, 15 pacientes (65.2%) tenían estadio clínico II. La histología cribiforme fue la más frecuente (10 pacientes; 43.5%). Posterior a la cirugía, se observaron márgenes positivos en 15 (65.2%), invasión neural en 8 (34.8%) e invasión linfovascular en 2 (8.7%) pacientes. La media de recurrencia fue de 23 meses. La recurrencia a 5 años se observó en 15 pacientes (65%). A los 2 años predominó la recurrencia local (7 pacientes; 46%); después de los 2 años predominaron las metástasis pulmonares (6 pacientes; 40%). La supervivencia a 5 años fue del 78%.El carcinoma adenoide quístico se caracteriza por una alta tasa de recurrencia. Sin embargo, tiene una alta supervivencia global a 5 años, lo que justifica un tratamiento agresivo.

Introduction Kindler syndrome is a very rare, autosomal recessive genodermatosis characterized by skin fragility and photosensitivity in infancy with progressive poikiloderma. Case report We report the case of a young woman with a history of Kindler syndrome predominantly characterized by extensive involvement of the oropharyngeal mucosa. The patient presented with an ulcerative lesion of the palate. Computed tomography and biopsy concluded on unresectable invasive squamous cell carcinoma of the hard palate. Neoadjuvant chemotherapy was proposed, but the patient died after the first course of chemotherapy in a context of severe gastrointestinal mucositis and generalized sepsis. Discussion Mucosal manifestations of Kindler syndrome have been described in the literature, but very few cases of malignant transformation to squamous cell carcinoma have been reported, although it is a very well known, long-term complication of this disease. To our knowledge, this is the second reported case of Kindler syndrome complicated by invasive squamous cell carcinoma of the hard palate.

Background To report our institution's experience treating soft palate squamous cell carcinoma with radiotherapy alone or combined with adjuvant chemotherapy and/or neck dissection for residual disease. Methods We analyzed 159 patients treated curatively between 1963 and 2016. Median follow-up was 4 years. Results The 5-year local control rates were T1, 90%; T2, 90%; T3, 70%; and T4, 59%. The 5-year cause-specific survival (CSS) rate was nearly identical for patients with stage I-III disease (88%, 86%, and 88%, respectively) compared to stage IVA/B (58%). Five-year overall survival was similar between patients with stage I-III disease (50%, 57%, and 54%, respectively) and approximately double that of patients with stage IVA/B disease (26%). Thirteen patients (8%) had severe complications related to radiotherapy. Conclusions The likelihood of cure after definitive radiotherapy is relatively high in patients with stage I-III disease with soft palate carcinoma. Patients with stage IVA/B disease have a lower cure rate but with a 5-year CSS exceeding 50%.

Integrity of the palatal mucosa may be disrupted due to various pathological and congenital factors. In maxillofacial defects, the buccal fat pad has been used frequently to repair the palatal region and close oro-nasal communications. In this case report, the use of buccal fat pad for the closure of a surgical palatal defect is presented with a 3-year follow-up. A 75-year-old female patient visited our clinic with a complaint of a non-adaptive maxillary denture. During clinical and radiographic examination, a well-demarcated mass on the palatal mucosa extending to the soft palate of the left side was observed. Subsequent to resection of the tumor, a defect occurred on the palatal region extending toward the soft palate. The pedicled buccal fat pad was chosen for closure of the defect. The patient was followed-up at 3, 7, 14, and 30 days after surgery. Epithelization and healing of the surgical wound were uneventful. At the 3-year follow-up, functional closure of the left palatal region was observed with no recurrence. Buccal fat pad is an easily manipulated flap with predictable results for the reconstruction of medium-sized defects of the oral and maxillofacial region with minimal postoperative discomfort for the patient.

OBJECTIVES To establish a predictive clinical index of malignancy risk in palatal salivary gland tumors (PSGT). MATERIALS AND METHODS One hundred cases of PSGT were evaluated. Clinical data were retrieved from the patient's files. Representative clinical photographs of each tumor were evaluated to identify clinical features suggestive of a malignant tumor. Features significantly associated with malignancy were included in a binary logistic regression model. RESULTS Malignant tumors were more common in the hard palate, in women and in older patients. Features associated with a malignant diagnosis included pain (p = .017), irregular surface (p = .004), bluish/purple coloration (p

Clear cell acanthoma (CCA), also known as pale cell acanthoma, represents a rare benign epidermal tumor with strong predilection for the lower extremities of middle-aged individuals and no frank gender preference. The etiology of CCA is poorly understood, although a localized psoriasiform reaction is favored. Herein, we report on the clinicopathologic and immunohistochemical features, and HPV status of an apparent example of oral CCA. A 58-year-old female presented with a well-circumscribed, asymptomatic, exophytic, sessile and erythematous nodule of the right hard palate, measuring 0.7 cm in greatest dimension. Microscopically, the lesion featured parakeratosis and acanthosis with neutrophilic microabscesses and broad elongated rete pegs. In areas, spinous epithelial cells exhibited pale or clear cytoplasm without nuclear pleomorphism, mitoses or cytologic atypia. The supporting connective tissue revealed mild chronic inflammation with few scattered neutrophils and numerous capillary vessels. PAS histochemical stain with and without diastase disclosed the presence of cytoplasmic glycogen in the pale cells. The majority of glycogen-rich epithelial cells stained strongly for EMA and were negative for D2-40. Ki-67 immunostaining was confined only to the basal cell layer of the epithelium. A diagnosis of CCA was rendered. The lesion was negative for human papillomavirus (HPV) infection, as assessed by HPV-DNA PCR using the MY09/11 primers for the L1 conserved region, thus HPV infection does not appear to contribute to the pathogenesis of oral CCA. In conclusion, we report an intraoral example of CCA in order to raise awareness about this entity.

Background When amenable to radical excision, cancer involving the maxilla is typically treated with maxillectomy followed by adjuvant therapy. Posterior tumor extension beyond the maxillary box leads to the invasion of complex areas, where achieving clear margins may be challenging. Methods Patients undergoing endoscopic-assisted maxillectomy for nasoethmoidal, maxillary, or hard palate cancer between 2007 and 2017 were included in the study. Surgical technique, margin status, and recurrences were analyzed. Extension of posterior resection was classified in 3 types (type 1: resection of the pterygopalatine fossa; type 2: resection of the pterygoid plates and related muscles; type 3: resection of the upper parapharyngeal space). The analysis of putative risk factors for involvement of margins and local recurrence was performed with special focus on the posterior and medial margin. Results The study included 79 patients (75 with available follow-up; mean: 20.6 months, range: 6–101 months), 37 (46.8%) of whom underwent type 1 resection, 34 (43.0%) type 2, and 8 (10.1%) type 3. According to pT category, 57 (72.2%) tumors were classified as T4a/T4b. Posterior and medial clear margins were achieved in 76/79 (96.2%) and 75/79 (94.9%) patients, respectively. T4b category, extension to the ethmoid, sphenoid sinus, pterygoid process, orbital cavity, and premaxillary tissues were significantly associated with a higher rate of margin involvement. None of the factors was significantly associated with medial margin involvement. Conclusion Endoscopic-assisted maxillectomy combines several refinements including the facilitated detachment of the maxilla from the skull base and precise delineation of the posterior and medial margins of resection.

Sialadenoma papilliferum (SP) is a rare benign salivary gland tumor with unclear cell origin. This report presents a new case of SP of the hard palate occurring in a 50-year-old female. The lesion was completely excised, and the microscopic features were consistent with SP. The knowledge of this rare entity contributes to proper diagnosis and prevents unnecessary radical surgery and treatment.