Palatal ancient schwannoma: optical, immunohistochemical and ultrastructural study with literature review.

Schwannoma or neurilemmoma is a benign encapsulated slow-growing tumor that originates from a Schwann cell of a nerve, and is rare at intraoral locations. Different histological variants of schwannomas have been described, of these degenerative or ancient schwannoma is probably one of the least common in the oral cavity with only 16 previously reported cases, of which only one has been described in palatal location. Although ancient schwannoma shows particular characteristics, it is difficult to diagnose based on clinical and imaging appearance alone; as a result, morphological examination assisted by ancillary techniques is necessary to establish a definite diagnosis. We present a clinicopathological description of this rare variant of schwannoma, located in an unusual intraoral site, of a 26-year-old female. We illustrate the optical, immunohistochemical and ultrastructural characterization that aid diagnosis, as well as providing a review of the relevant published data of this unusual tumor.

Local intralesional talimogene laherparepvec therapy with complete local response in oral palatine mucosal melanoma: a case report.

BACKGROUND: Mucosal melanoma, an aggressive type of malignancy different from the cutaneous melanomas commonly seen in the head and neck region, represents < 1% of all malignant melanomas. The pathogenesis of mucosal melanoma is unknown. Targetable mutations commonly seen in cutaneous melanoma, such as in the BRAF and NRAS genes, have a lower incidence in mucosal melanoma. Mucosal melanoma carries a distinct mutational pattern from cutaneous melanoma. Surgery with negative margins is the first-line treatment for mucosal melanoma, and systemic therapy is not well defined. Talimogene laherparepvec, an oncolytic viral immunotherapy, is United States Food and Drug Administration approved for the treatment of advanced malignant cutaneous melanoma, with local therapeutic benefits. Mucosal melanoma was initially excluded from talimogene laherparepvec's initial phase III clinical trial. CASE PRESENTATION: We present the case of a white female patient in her 40s with past medical history of systemic lupus erythematous, scleroderma, and estrogen-receptor-positive invasive ductal breast carcinoma. Following a bilateral mastectomy, the patient was found to have BRAF-negative mucosal melanoma of her hard palate with a soft palate skip lesion. Owing to the presence of a skip mucosal lesion as well as the anticipated defect and need for free-flap reconstructive surgery, nonsurgical management was considered. The patient was referred to medical oncology, where-based on the patient's complicated medical history and the risk of immunotherapy possibly worsening her prior autoimmune diseases-local talimogene laherparepvec injections were chosen as the primary therapy for her mucosal lesions. Though talimogene laherparepvec is approved for the treatment of cutaneous melanoma, there are limited data available on the use of talimogene laherparepvec in mucosal melanomas. CONCLUSION: The patient had a complete local tumor response at both the primary lesion as well as the skip lesion with the local injections. She had no side effects and maintained a high quality of life during treatment.

A matched cohort comparison of mTHPC-mediated photodynamic therapy and trans-oral surgery of early stage oral cavity squamous cell cancer.

Photodynamic therapy (PDT) of early stage oral cavity tumors have been thoroughly reported. However, statistical comparison of PDT to the surgical treatment is not available in published literature. We have identified and matched cohorts of patients with early stage oral cavity cancers undergoing surgery (n = 43) and PDT (n = 55) from a single institute experience. The groups are matched demographically and had the same pre-treatment screening and follow-up schedule. Both groups consisted only of tumors thinner than 5 mm to ensure comparability. The endpoints were local disease free survival, disease free survival, overall survival and response to initial treatment. Local disease free survival at 5 years were 67 and 74 % for PDT and surgery groups, respectively [univariate HR = 1.9 (p = 0.26), multivariable HR = 2.7 (p = 0.13)]. Disease free survival at 5 years are 47 and 53 % for PDT and surgery groups, respectively [univariate HR = 0.8 (p = 0.52), multivariable HR = 0.75 (p = 0.45)]. Overall survival was 83 and 75 % for PDT and surgery groups, respectively [(univariate HR = 0.5 (p = 0.19), multivariable HR = 0.5 (p = 0.17)]. In the PDT group, six patients (11 %) and in the surgery group 11 patients (26 %) had to receive additional treatments after the initial. All of the tested parameters did not have statistical significant difference. Although there is probably a selection bias due to the non-randomized design, this study shows that PDT of early stage oral cavity cancer is comparable in terms of disease control and survival to trans-oral resection and can be offered as an alternative to surgical treatment.

Primary oral mucosal melanoma: advocate a wait-and-see policy in the clinically N0 patient.

PURPOSE: Oral mucosal melanoma (OMM) is a rare disease associated with a very poor prognosis. Because well-established treatment protocols for OMM are in short supply, prognostic information regarding recent treatment modalities for this disease were sought. PATIENTS AND METHODS: A retrospective chart review was performed of 61 patients who were treated for OMM from 1998 through 2005. The clinical features and treatment modalities were identified and correlated with the outcomes. RESULTS: There were 41 male and 20 female patients (ratio, 2.1:1) with a mean age of 54.1 years. The mean follow-up was 31.9 months, and the overall 2-year and 5-year survival rates were 51.1% and 30.3%, respectively. According to the seventh edition of the American Joint Committee on Cancer staging system, there were 31 patients (50.8%) with stage III tumors. A more advanced stage and a tumor of at least 2 cm were associated with worse survival (P < .001 and P = .036, respectively). Elective lymph node dissection and biochemotherapy were not associated with a higher total survival rate (P = .53 and P = .76, respectively). CONCLUSIONS: OMM has a male predilection. The seventh edition of the American Joint Committee on Cancer stage and tumor size are effective prognostic parameters for patients with OMM. The American Joint Committee on Cancer staging system provides useful information for predicting the ultimate outcome and should be used as the primary staging system. Elective node dissection and adjuvant biochemotherapy offer no additional advantage in increasing the patient survival rate. A wait-and-see policy is advocated for patients with clinical stage N0 cancer.

Oral pigmentation: case report and review of malignant melanoma with flow charts for diagnosis and treatment.

The color of the human oral mucosa is not uniform. Chromatic variations can occur, depending on the degree of keratinization, melanogenic activity, number of melanocytes, vascularization, and type of submucosal tissue. If a pathological pigmentation is suspected, its benign or malignant nature will guide the treatment plan. This article presents a rare case of malignant melanoma of the anterior maxilla with asymptomatic palatal pigmentation and regional lymph node metastasis. Flow charts for the diagnosis and treatment of pigmented lesions (with special emphasis on treatment of primary and metastatic melanoma) are included.

Primary myoepithelial carcinoma of palate.

OBJECTIVES: The aim of this study was to present a rare neoplasm, Primary myoepithelial carcinoma arising from the palate, and to review its diagnostic criteria, pathologic and clinical characteristics, treatment options and prognosis. CLINICAL PRESENTATION AND INTERVENTION: Myoepitheliomas are tumors arising from myoepithelial cells mainly or exclusively. Myoepitheliomas mostly occur in salivary glands, as well as in breast, skin, and lung. Case of myoepitheliomas in palate has rarely been reported. Myoepithelial carcinoma is malignant counterpart of myoepitheliomas. Adenomyoepithelioma is also a different disease from myoepitheliaomas. Immunohistochemically, tumor cells of myoepithelial carcinoma express not only epithelial markers such as cytokeratin, epithelial membrane antigen (EMA), but also markers of smooth muscle origin such as calponin. The immunohistochemical criteria of myoepithelial differentiation are double positive for both cytokeratins and one or more myoepithelial immunomarkers (i.e., S-100 protein, calponin, p63, GFAP, maspin, and actins). Myoepithelial carcinomas of salivary and breast demonstrate copy number gains and gene deletion. The overall prognosis of myoepithelial carcinoma is poor. There is rarely recurrence or metastasis in benign myoepithelial tumors. Complete excision with tumor-free margin is always the preferred treatment, while local radiation therapy and chemotherapy are suggestive treatment options. Here, a rare case of myoepithelial carcinoma arising from the palate has been described and discussed for the treatment and outcome. Pathological and clinical characters of myoepitheliomas are also compared and discussed. CONCLUSION: The case report serves to increase awareness and improve the index of diagnosis and treatment of myoepitheliomas.

Pediatric rhabdomyosarcoma in an oronasal fistula in a repaired cleft palate: a case report.

Rhabdomyosarcoma is a rare soft tissue tumor that was found incidentally in a repaired cleft palate oronasal fistula of a 3-year-old boy. This situation has yet to be reported in the literature. During this fistula repair, a small piece of tissue was sent for routine pathology, which showed embryonal rhabdomyosarcoma. A literature review as well as diagnostic and treatment algorithms are discussed concerning management of this rare malignancy. Also, routine pathology should be reviewed carefully because unsuspected tissues may have pathologic findings that dramatically affect patient diagnosis and management.

Recurrent pterygo-palatal angiofibroma with intracranial extension: case report.

We report the case of a patient with recurrent pterygo-palatal angiofibroma and its treatment. A 21-year-old male patient had a long history of recurrent epistaxis with progressive nasal obstruction. He was diagnosed with an angiofibroma centered in the right pterygo-palatine fossa. Initially, he underwent surgical excision with removal of the entire tumor. The evolution was clinically good with no signs of recurrence on the cervico-facial scan of control (CT). Nine months after, he presented a reappearance of epistaxis. A cervico-facial MRI was performed and showed a recurrence of the tumor process, which this time was considered inextirpable, hence the decision to opt for radiotherapy with intensity modulated radiation therapy (IMRT). He has improved clinically with a clear reduction in tumor mass on CT scan. This technique represents an interesting alternative to overcome anatomical complexity of the region, cover the tumor and preserve the organs at risk.

Adenoid cystic carcinoma of head and neck. A 5-year retrospective study: Experience in a single third-level reference center.

INTRODUCTION: Adenoid cystic carcinoma (ACC) is a rare tumor, with an annual incidence of 3-4.5 cases/million. MATERIALS AND METHODS: 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. RESULTS: 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%. CONCLUSIONS: Cystic adenoid carcinoma is characterized by a high recurrence rate. Nevertheless, it has a high overall 5-year survival, which justifies an aggressive treatment.

ANTECEDENTES: El carcinoma adenoide quístico es un tumor raro, con una incidencia anual de 3-4.5 casos por millón. MATERIALES Y MÉTODOS: 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. RESULTADOS: 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%. CONCLUSIONES: 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.

Necrotizing sialometaplasia of the palate associated with angiocentric T-cell lymphoma.

In this article we present 2 cases of necrotizing sialometaplasia (NS) associated with angiocentric lymphoma of the midline. Immunohistochemical analysis confirmed a T-cell origin, and in situ hybridization in one case revealed its relationship to Epstein-Barr virus. These findings suggest that vascular occlusion by the neoplastic cells produces ischemia, which leads to local infarction contributing to the salivary gland lesion. To our knowledge, the association between angiocentric lymphoma and NS has been previously reported in only one instance, and we suggest that this particular type of lymphoma should be added to the list of related conditions for NS.

Prognostic factors in patients with soft palate squamous cell carcinoma.

BACKGROUND: To define the prognostic factors associated with outcome in patients with soft palate squamous cell carcinoma (SCC). METHODS: Previously untreated patients with soft palate and uvula SCC treated in our institution between 1997 and 2012 were collected. The prognostic value of clinical, hematological, and treatment characteristics was examined. RESULTS: We identified 156 patients, median age 58 years, with 71% drinkers, 91% smokers; 19% had synchronous cancer. Front-line treatment was chemoradiotherapy in 58 (37%), radiotherapy alone in 60 (39%), surgery in 17 (11%), and induction chemotherapy in 21 patients (14%). The 5-year actuarial overall survival (OS) and progression-free survival (PFS) were 41% and 37%, respectively. In univariate analysis, T3-T4 vs T1-T2 stage, N2-N3 vs N0-N1 stage, and neutrophil count >7 g/L were associated with worse OS and PFS (P < .05). CONCLUSION: In patients with soft palate SCC, inflammation biomarkers were associated with OS.

Primary extranodal non-Hodgkin lymphoma of the oral cavity.

Lymphoma is the second most common neoplasm of the head and neck after squamous cell carcinoma. Nearly 24% to 48% of non-Hodgkin lymphoma can arise in extranodal localizations and 3% to 5% of which were primarily located in the oral cavity. Waldeyer ring is the most frequently involved site. The authors report 2 cases of primary extranodal non-Hodgkin lymphoma of the oral cavity, not of the Waldeyer ring.

Robotic radiosurgery vs. brachytherapy as a boost to intensity modulated radiotherapy for tonsillar fossa and soft palate tumors: the clinical and economic impact of an emerging technology.

As a basis for making decisions regarding optimal treatment for patients with tonsillar fossa and soft palate tumors, we conducted a preliminary investigation of costs and quality of life (QoL) for two modalities [brachytherapy (BT) and robotic radiosurgery] used to boost radiation to the primary tumors following external beam radiotherapy. BT was well established in our center; a boost by robotic radiosurgery was begun more recently in patients for whom BT was not technically feasible. Robotic radiosurgery boost treatment has the advantage of being non-invasive and is able to reach tumors in cases where there is deep parapharyngeal tumor extension. A neck dissection was performed for patients with nodal-positive disease. Quality of life (pain and difficulty swallowing) was established in long-term follow-up for patients undergoing BT and over a one-year follow-up in robotic radiosurgery patients. Total hospital costs for both groups were computed. Our results show that efficacy and quality of life at one year are comparable for BT and robotic radiosurgery. Total cost for robotic radiosurgery was found to be less than BT primarily due to the elimination of hospital admission and operating room expenses. Confirmation of robotic radiosurgery treatment efficacy and reduced morbidity in the long term requires further study. Quality of life and cost analyses are critical to Health Technology Assessments (HTA). The present study shows how a preliminary HTA of a new medical technology such as robotic radiosurgery with its typical hypofractionation characteristics might be based on short-term clinical outcomes and assumptions of equivalence.

Delayed Diagnosis of a Nasal Type Lymphoma Misdiagnosed as Persistent Sinusitis.

Nasal Type T/natural killer (NK)-cell lymphomas are rare clinical entities, highly aggressive with a very poor prognosis. We present a case of a 37-year-old immunocompetent man presenting with deep palatal ulceration and a 3-month history of symptoms, which appear to have been misdiagnosed by physicians. The final diagnosis was achieved by a 15-day diagnostic algorithm, during which time the clinical status of the patient worsened severely. In this article, we also provide a succinct update on the clinical and histopathological findings of Peripheral T/NK-cell lymphomas and propose that symptoms that are consistent with these clinical entities should be considered from the early stages to inform a suitable diagnostic pathway Because of their highly aggressive behavior, we suggest that early therapy of T/NK-cell lymphomas may be started before completing the specific diagnostic investigations.

Cervical metastasis from squamous cell carcinoma of the maxillary alveolus and hard palate.

OBJECTIVES: Squamous cell carcinoma has a predilection for regional lymphatic metastasis. The occurrence of occult cervical metastases from squamous cell carcinoma of the hard palate and maxillary alveolar ridge has not been studied systematically. We have observed many patients who have returned after resection of a primary cancer in these sites with a delayed cervical metastasis. Some of these patients have died of regional or distant metastasis despite control of their primary cancer. METHODS: We have studied 26 patients with squamous cell carcinoma of the maxillary alveolar ridge and hard palate to define incidence of cervical metastasis. RESULTS: Overall incidence of cervical metastasis was: clinical 2 of 26 (7.6%) and occult 7 of 26 (27%) for a total of 9 of 26 (34.6%). The 5-year disease-specific survival was 13 of 22 (59%). Surgery for regional failure was successful in 66% (6 of 9). Radiation was administered after surgery in eight of nine patients. CONCLUSION: Cervical metastasis from cancer of the palate and alveolar ridge is significant. Regional surgery for recurrent disease usually requires radical or modified radical neck dissection. Selective elective neck dissection should be offered to patients with cancer of the hard palate and alveolar ridge. It affords the patient and the treatment team valuable histologic information, which may help to guide therapy and reduce the potential need for future hospitalization, chemoradiation, and more radical surgery.

Cavernous hemangioma of the palate. A review of etiology, pathogenesis and treatment options.

Cavernous hemangiomas of the palate are a rare but not infrequent sighting in the oral and maxillofacial region. The etiology and pathogenesis of these lesions are not definitively understood and are currently being explored. Treatment options usually include, but are not limited to, surgical excision of the lesion. Alternative management paradigms should be considered when dealing with this type of lesion. Precaution should be taken before any surgical intervention.

Management of the clinically N0 neck in squamous cell carcinoma of the maxillary alveolus and hard palate.

BACKGROUND: The purpose of this study was to evaluate active surveillance strategy in the clinically negative neck in maxillary squamous cell carcinoma (SCC). METHODS: One hundred fourteen consecutive patients diagnosed with oral maxillary SCC were analyzed retrospectively from 3 centers in The Netherlands. Analysis parameters included regional disease-free survival of N0 patients stratified for T classification, elective radiotherapy (RT) of the neck; and overall survival of the whole cohort, stratified by N classification; salvage neck surgery rates. RESULTS: Within the N0 cohort, 26.0% of the patients developed neck metastasis in the follow-up visits. Regional recurrence was not related to T classification or postoperative RT of the neck. Regional and locoregional recurrence were associated with diminished overall survival (p < .05). Regional metastasis was operable in 22 of 26 cases (85%). Only 1 patient presented with inoperable neck metastasis without local recurrence. CONCLUSION: Watchful waiting was feasible in this cohort. If meticulous follow-up is not available, elective neck dissection is recommended. © 2016 Wiley Periodicals, Inc. Head Neck 38: 1794-1798, 2016.

Common benign oral soft tissue masses.

This article reviews some of the more common benign oral soft tissue masses with emphasis on their etiology, epidemiology, clinical presentation, histopathology, and treatment. These lesions include traumatic fibroma, mucocele, warts/papilloma, pyogenic granuloma, peripheral giant cell granuloma, generalized gingival hyperplasia, gingival fibromatosis, lateral periodontal cyst, lipoma, and denture-induced hyperplasia.